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i

NeuroRehabilitation
A Multidisciplinary Approach

Dr. V. C. Jacob (PT)


Dr. Hema Biju (OT)
Dr. Alok Sharma

NeuroGen Brain and Spine Institute


Center for Stem Cell Therapy and Neurorehabilitation
Chembur, Mumbai. India.
[Link]
ii
iii

Authors
Dr. V.C. Jacob (PT) has been the Deputy Director of NeuroGen Brain and Spine
Institute since its inception and is currently the Head of Neurorehabilitation at
NeuroGen. He is a Trustee of Nina Foundation which is an NGO set up for the
rehabilitation of spinal cord injury persons. He has been a physiotherapist at
LTMG Hospital, Sion, Mumbai for 35 years and is now retired. For a period of
38 years, from 1968 to 2006 he was part of the Executive Committee of the
Paraplegic Foundation, a charitable organization for the comprehensive care of
the paraplegics. He has also organized several sports events for these paraplegics
on a national level. In addition, he has also served as President of the Indian
Association of Physiotherapists in the past. Apart from all these activities, Dr.
Jacob has been involved in disaster management during calamities such as the catastrophic earthquake at
Latur and also in Gujarat. He is wholeheartedly committed to the cause of Rehabilitation of patients
suffering from neurological disorders.

Dr. Hema Biju (OT) is currently working as Head of Occupational Therapy


Department at NeuroGen Brain and Spine Institute which is engaged in cutting
edge research in the field of Neuroregenerative rehabilitation involving stem
cell therapy.
With over 17 years of national and international work experience in the varied
fields within Occupational Therapy, she has specialized in the field of
neurological rehabilitation.
Having practiced in the U.S.A as a registered and licensed Occupational
Therapist, she is currently associated as a consultant with numerous hospitals
in Mumbai and was instrumental in setting up the rehabilitation departments at several of them .
Since 2002, along with her professional career, she has also been continuing her pursuit in the field of
academics and research.

Dr. Alok Sharma is a Neurosurgeon who is presently the Professor & Head of
Department of Neurosurgery at the LTMG Hospital & LTM Medical College in
Sion, Mumbaias well as the Director of the NeuroGen Brain & Spine Institute in
Chembur and Consultant Neurosurgeon at the Fortis Hospital in Mulund. He
has been committed to both basic as well as clinical research in attempting to
find an answer to the problems of paralysis and neurological deficits that occur
following injury and diseases of the nervous system. He completed his graduate
and post graduate studies from the Seth G.S. Medical College and KEM Hospital
of Mumbai University. Subsequently he did fellowships in two departments
that were the first to do Neural Transplantation.
In 1995, he worked at the Karolinska Hospital in Stockholm Sweden, where Neural transplantation was
done for the first time in the world and in 1998 worked at the University of Colorado Health Sciences
Center in Denver, USA where the world's first randomized trial for fetal cell transplantation was done for
Parkinson's Disease. Its his life's mission and passion to bring about regeneration within the nervous
system. He setup the stem cell and genetic research laboratory at the LTMG hospital which was the first
of its type in Mumbai. He is a Neurosurgeon, Medical teacher and Scientist attempting to combine the
best of science, medicine and humanity to alleviate the suffering of patients with neurological disorders.
He is a staunch believer that stem cell therapy can relieve a lot of human suffering of neurological patients
and makes every attempt to popularize this new approach amongst the medical community. This book is
one such attempt for the same purpose. He can be reached at alok276@[Link] or Ph: +91 22 25283706,
+91 9820046663
iv
v

NeuroRehabilitation
A Multidisciplinary Approach

Dr. V. C. Jacob, [Link]. D.P.T, MIAP.


Dy. Director , NeuroGen,
Senior Consultant Physiotherapist and Head of NeuroRehabilitation,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.
Retd. Physiotherapist,
LTMG Hospital & LTM Medical College,
Sion, Mumbai.
Past President of Indian Association of Physiotherapist,
Past E. C. Member of Paraplegic Foundation.
Trustee of Nina Foundation.

Dr. Hema Biju, MOTh (Neuro), AIOTA (Mum), OTR (USA)


Consultant Occupational Therapist
NeuroGen Brain & Spine Institute, Chembur, Mumbai

Dr. Alok Sharma, M.S, [Link].


Professor of Neurosurgery and Head of Department
LTMG Hospital and LTM Medical College, Sion, Mumbai
Director,
NeuroGen Brain & Spine Institute, Chembur, Mumbai
Consultant Neurosurgeon
Fortis Hospital, Mulund, Mumbai, India

Scientific and Editorial Co-ordinator


Dr. Nancy Thomas, MPTh (Neuro), MIAP, MOTPT
Neuro Physiotherapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai
vi

NeuroRehabilitation
A Multidisciplinary Approach
© 2012 by NeuroGen Brain and Spine Institute

ISBN 81-86876-08-1

All rights reserved.

This book is protected by copyright. No part of this book may be reproduced in any form
by any means, including photocopying, or utilized by any information storage and retrieval
system without written permission from the copyright owner, except for the brief
quotations embodied in critical articles and reviews.

This book has been published in good faith that the material presented is original. Every
effort is made to ensure accuracy of material, but the publisher, printer and author will
not be held responsible for any inadvertent error(s).

Cover design by

Sunakshi Savla & Sonam Gupta

Published by

NeuroGen Brain and Spine Institute

Printed by

SUREKHA PRESS
A-20, Shalimar Industrial Estate,
Matunga Labour camp,
Matunga, Mumbai 400 019.

Price : ` 2000
vii

Contributors
1. Dr. Prerna Badhe, M.D., Consultant Neuropathologist,
Deputy Director, NeuroGen Brain & Spine Institute,
Chembur, Mumbai.

2. Dr. Hema Biju, MOTh (Neuro), AIOTA (Mum), OTR (USA)


Consultant Occupational Therapist
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

3. Dr. Guneet Chopra, MBBS, PGDM (Medical Genetics)


Clinical Research,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

4. Dr. Myola D'sa, B.O.T.H ,


Occupational Therapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

5. Dr. Nandini Gokulchandran, M.D,


Head-Medical Services & Clinical Research
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

6. Dr. V. C. Jacob, [Link]. D.P.T, MIAP.


Dy. Director , NeuroGen,
Consultant Senior Physiotherapist & Head of NeuroRehabilitation,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.
Retd. Physiotherapist, LTMG Hospital & LTM Medical College,
Sion, Mumbai.
Past. President of Indian Association of Physiotherapist,
Past E. C. Member of Paraplegic Foundation.
Trustee of Nina Foundation.

7. Dr. Manasi Jani,


Consultant Speech Therapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

8. Dr. Joji George Joseph, B.P.T, F.C.R.


Consultant Physiotherapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

9. Dr. Sanjay Kukreja, M.B.B.S.


Clinical Associate,
Department of Neurosurgery,
LTMG Hospital & LTM Medical College, Sion, Mumbai.

10. Dr. Vijay Kulkarni, M.S.


Consultant Andrologist & Microsurgeon,
LTM Medical College & LTM Hospital, Bhatia Hospital,
S L Raheja Hospital & All India Institute for Diabetes.
National Institute for Research in Reproductive Health,
Paraplegic Foundation,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.
viii

11. Ms. Pooja Kulkarni, [Link]. (Biotechnology)


Clinical Research Associate,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

12. Dr. Mamta Lohia, [Link], MIAP, F.N.R, CBE(USA)


Physiotherapist & Clinical Research Associate,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

13. Dr. Dhruv Mehta, M.P.T (Neuro),


Professor , MGM, School of Physiotherapist, Vashi, Mumbai.
Consultant Physiotherapist,
Cheshire Home,
C. P. Centre, H.I.F.I.V.E, Child Development Centre,
Head of Physiotherapy Department, Yusuf Mehralli Centre, Village Tara.
Associate Member, Nina Foundation.

14. Dr. MS. Ketna Mehta, Ph.D.


Founder, Trustee ,Nina Foundation,
NGO for Rehabilitation of People with Spinal Injuries.
Management Educationalist,
Editor & Associate Dean Research,
Welingkar Institute of Management & Research, Mumbai.

15. Dr. Priti Mishra, [Link], F.N.R.


Occupational Therapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.

16. Dr. Naren Naik, M.S, MCh.


Assistant Professor, Department of Neurosurgery,
LTMG Hospital & LTM Medical College, Sion, Mumbai.

17. Dr. Anita Patel, MCh, DNBE,FRCS(Urol),


Consultant Urologist,
Endoskopik Klinik &Hospital, Mumbai.
Visting Consultant Urologist, MPUH, Nadiad.

18. Dr. Ashok Patil, M.P.T (Neuro),


Professor and Principal,
Smt. Kashibai Navale, College of Physiotherapy, Pune.

19. Dr. Alok Sharma, M.S, [Link].


Professor of Neurosurgery and Head of Department
LTMG Hospital and LTM Medical College, Sion, Mumbai
Director, NeuroGen Brain & Spine Institute, Chembur, Mumbai
Consultant Neurosurgeon
Fortis Hospital, Mulund, Mumbai, India

20. MS. Akshata Shetty, M.A.


Clinical Psychologist,
NeuroGen Brain & Spine Institute,
Chembur, Mumbai.

21. Dr. Nancy Thomas, [Link], (Neuro), MIAP, MOTPT,


Neuro Physiotherapist,
NeuroGen Brain & Spine Institute, Chembur, Mumbai.
ix

This book is dedicated to


all rehabilitative therapists,
physicians, care-takers,
institute, rehabilitative centers,
NGOs, societies who have devoted
their lives looking after
the neurological and
musculoskeletal disease
and injuries.
x

Acknowledgement

Publishing a book of this nature could not have materialized


had it not been from the valuable contribution of all the authors
who have spent their valuable time and energy. Most of them
are from the staff of NeuroGen Brain & Spine Institute and the
visiting consultants from various disciplines. So rightly we
can say that it is an effort of a multi disciplinary team. We
acknowledge our sincere appreciation to all of them. We also
thank all our juniors and family members who have also
extended a helping hand in whatever way they could.

Dr. V. C. Jacob
Dr. Hema Biju
Dr. Alok Sharma
xi

Preface
NeuroRehabilitation - A Renaissance
This book (which is the first of its kind published from India) has been written for the following purposes:-
1] To provide in one source a comprehensive overview of all aspects of NeuroRehabilitation
2] To provide both undergraduate and postgraduate students of physiotherapy and occupational
therapy with all the information they will need from this field for their examinations.
3] To make available to our younger therapists the knowledge and wisdom of years of experience of
the senior authors and contributors of this book.
4] To stimulate younger rehabilitation therapists to take up NeuroRehabilitation as a career choice.
5] To emphasize the importance of a multidisciplinary team approach in NeuroRehabilitation.
6] To introduce students, teachers, junior and senior practicing rehabilitation therapists to the fascinating
possibilities of clinical improvements that can occur using Neuro-Regenerative-Rehabilitation-
Therapy (which is a combination of Stem cell therapy with NeuroRehabilitation)

Of all the injuries and diseases that have afflicted mankind, those of the nervous system have proved
to be the most difficult to treat. Whereas modern medical advances have significantly impacted the survival
from infectious diseases and improved the longevity from life threatening cardiological and other critical
illnesses, the management of neurological problems has not been as successful. There are still many
neurological conditions where no drugs were available and where there are no further neurosurgical
options. In such cases the only options for the patients to improve the quality of their lives was
rehabilitation. But even in rehabilitation of these conditions, things were not so simple. Having to deal
with patients who had no movements or limb strength when combined with other aspects such as spasticity,
contractures, loss of sensations, cognitive impairment etc made the rehabilitation process very difficult.
The work required to rehabilitate these patients was very physically intensive, requiring many hours of
laborious work over long periods of time. The results after all this hard work were most often not upto
the patients and their relatives satisfaction since what the patients would want is to start walking normally
again and this was in most cases just not possible. The subsequent reluctance of patients to pay for the
services appropriate to the efforts that were put in became another factor.

All these facts (the complicated nature of the rehabilitation, the poor response, the lack of willingness
to pay) resulted in most younger therapists opting for the less physically demanding and more financially
rewarding other options in rehabilitation that were also available more commonly. NeuroRehabilitation
in its original sense was there becoming a dying science and skill. Another limiting aspect of treating this
was the artificial barriers we had put up between the different branches of rehabilitation mainly
physiotherapy and occupational therapy. We all tended to look at the patients from our specialty point of
view and this often times did not let us see the whole picture. This was in a sense unfortunate since this
was one aspect of rehabilitation where a real difference could be made.

However all this is now undergoing a major transformation. The availability of regenerative medicine
using healthy cells (such as Stem cells) to replace damaged cells has opened up a entire new world of
treatment options giving fresh hope to millions of patients who had given up on any hope for improvement.
What Stem cells do is that they initiate a process of repair, replacement and regeneration of the damaged
cells by a process of release of various growth factors, increased blood supply and cellular replacement.
This sort of reenergizes the nervous and musculoskeletal system. However this is only part of the
improvement process. The real functional changes in the patients lives come from rehabilitation. What
the Stem cell therapy does is that it makes the rehabilitation process more effective and productive.
Improvements not seen earlier just with rehabilitation are now visible. This combination of Regenerative
therapy and NeuroRehabilitation has therefore opened up an entire new world of opportunities for
xii

rehabilitation therapists. Not only is this work exciting and challenging and on the frontline of modern
medicine, it is also very rewarding in all aspects i.e. clinically, academically as well as materially. A entire
group of patients who had given up on rehabilitation since they were not getting encouraging
improvements are now coming back to rehabilitation. The newer fancier corporate hospitals that are just
about introducing stem cell therapy are now seeing rehabilitation in a new way. This is an incredible
opportunity. A field that had been forgotten and given up as not being useful or remunerative enough is
now being looked upon with a whole new interest. It's a rebirth or a renaissance of sorts. Much like how
the older radio had almost died in the 80's and 90's with the advent of television but had a rebirth in the
last decade with the introduction of FM radio.

In this book all the different aspects of rehabilitation (physiotherapy, occupational therapy, speech
therapy, counseling, clinical and surgical aspects ) have all been put together in individual chapters that
are focused on an individual diseases or medical condition. This is intentional and meant to highlight the
multidisciplinary approach to NeuroRehabilitation. Various theoretical aspects have been covered along
with practical advice and suggestions on management. Extensive literature reviews have been done to
incorporate the best of whatever is available from all across the world. Valuable individual information
gathered from years of experience have also been incorporated. This is important. To highlight this let me
state that at the NeuroGen Brain and spine institute we receive patients regularly from all over the world.
These patients have already been to the best rehabilitation centers in the USA and elsewhere. And yet
despite this virtually all the patients without any exceptions have one thing to say. " that they have never
received the kind of rehabilitation in all the centers they had been to, which they received at NeuroGen".
What is the difference between what is offered at NeuroGen and what is offered in the bigger rehabilitation
centers all over the world. At NeuroGen there is the human touch. There are virtually no fancy electronic
machines or equipment. Our rehabilitation makes a difference because its more hands on with the
rehabilitation therapist working intensely and closely with the patients instead of depending on machines
and watching from a distance.
This is work that requires dedication, commitment, hard work and perseverance. The joy of this
treatment lies not as much in the money but in the small small functional improvements seen in the
patients and their gratefulness and gratitude for the same. There were very few therapists who have
dedicated their lives to this work. And these therapists belong to an older generation. It was important
that the wisdom and knowledge of their years of experience was passed on to today's younger generation.
This is therefore one of the important intentions of this book.
It is fortunate for the readers of this book that through this book they will get together in one place
the lifetimes wisdom and experience of the senior author of this book Dr. V. C. Jacob. Dr. Jacob, who as
you all well know, is one of the countries senior most and highly respected physiotherapist who had
devoted his whole life to NeuroRehabilitation through his clinical and teaching work at the LTM Medical
college and the charitable work at the Paraplegic Foundation. Inputs from the occupational therapy aspects
of the work come from Dr. Hema Biju who has worked for several years in the USA before coming back
to India. All the other contributors and authors are experts in their respective fields and we are grateful to
their contributions which have resulted in this unique collection of knowledge and wisdom. On behalf of
the Neurogen Brain and Spine Institute, I take this opportunity to thank all the authors and contributors.
Grateful acknowledgement also goes out to all the members of the NeuroGen team who have despite
great difficulties helped and supported all the work that had to be done to make this book a reality. The
biggest thanks of course goes to Dr. Nancy Thomas, the scientific and editorial coordinator, who had
worked tirelessly for several months to put all this wonderful information together. This book could not
have been put together without her sincere efforts. All of us from Neurogen as well as all the contributors
now place this book in your hands with the hope that it will inform, enrich an contribute to your lives and
the lives of your patients in some important way. Should any of you need any more information please
feel free to reach out to us. We are always more than happy to help any of you in any way that your
desire. Wish you all students as well a practicing therapists happy reading and happy learning.

Dr. Alok Sharma


(alok276@[Link] / 9820046663)
xiii

Contents

Section 1: Spine
1 Spinal Cord Injury. 01
Dr. V. C. Jacob, [Link], D.P.T, Dr. Dhruv Mehta, MPT(Neuro), Dr. Joji George Joseph, B.P.T, F.N.R,
Dr. Anita Patel, MCh, DNBE,FRCS(Urol), [Link] Kulkarni M.S, Dr. Ms. Ketna. L. Mehta, PhD, Dr.
Alok Sharma, M.S, MCh, [Link] Naik M.S, MCh, Dr. Sanjay Kukreja, M.B.B.S, [Link] Biju,
MOTH, [Link] D'Sa, [Link], Ms. Akshata Shetty, M.A.(Clinical Psychologist)

Section 2: Brain and Spine Related Disorders


2 Stroke 93
Dr. Mamta Lohia, [Link], F.N.R, CBE (USA), Dr. Hema Biju, MOTH(Neuro), Ms. Akshata Shetty,
M.A.(Clinical Psychologist), Dr. Manasi Jani( Speech Therapist).

3 Cerebral Palsy 142


Dr. Mamta Lohia, [Link], F.N.R,CBE(USA), Dr. Myola D'Sa, [Link]., Ms. Akshata Shetty, M.A. (Clinical
Psychologist), Dr. Manasi Jani (Speech Therapist).

4 Head Injury 172


Dr. Ashok Patil, MPT(Neuro), Dr. Alok Sharma, M.S, MCh, [Link] Kukreja, M.B.B.S, Dr. Naren
Naik, M.S,MCh., Dr. Hema Biju, MOTH (Neuro), Ms. Akshata Shetty, M.A.(Clinical Psychologist),
Dr. Manasi Jani (Speech Therapist).

5 Autism 218
Ms. Akshata Shetty, M.A.(Clinical Psychologist), [Link] Mishra, [Link], F.N.R., Dr. Manasi Jani
(Speech Therapist).

6 Dementia 255
Ms. Akshata Shetty, M.A.(Clinical Psychologist), Dr. Myola D'Sa, [Link].

7 Cerebellar Ataxia 296


Dr. Nancy Thomas, MPTh (Neuro), [Link] Mishra, [Link], F.N.R., Ms. Akshata Shetty, M.A.(Clinical
Psychologist), Dr. Manasi Jani( Speech Therapist).

8 Multiple Sclerosis 329


[Link] Mishra, [Link], F.N.R., Dr. Mamta Lohia, [Link], F.N.R,CBE(USA), Ms. Akshata Shetty,
M.A.(Clinical Psychologist), Dr. Manasi Jani( Speech Therapist).

Section 3: Muscle and Nerve Related Disorders


9 Muscular Dystrophy 351
Dr. Nancy Thomas, MPTh (Neuro), Dr. Hema Biju, MOTH(Neuro), Ms. Akshata Shetty, M.A.(Clinical
Psychologist).

10 Motor Neuron Disease 393


Dr. Mamta Lohia, [Link], F.N.R,CBE(USA), [Link] Mishra, [Link], F.N.R., Ms. Akshata Shetty,
M.A.(Clinical Psychologist), Dr. Manasi Jani( Speech Therapist).

11 Polyneuropathy 411
[Link] Mishra, [Link], F.N.R., Dr. Nancy Thomas, MPTh (Neuro).

Section 4: Miscellaneous.
12 Spasticity 437
Dr. Alok Sharma, M.S, MCh, Dr. Sanjay Kukreja, M.B.B.S, Dr. Naren Naik, M.S, MCh, Dr. Nancy
Thomas, MPTh (Neuro), Dr. Hema Biju, MOTH(Neuro)
xiv

13 Orthosis 455
Dr. [Link], [Link], D.P.T, Dr. Hema Biju, MOTH(Neuro).

14 Psychological Rehabilitation 472


Ms. Akshata Shetty, M.A.(Clinical Psychologist).

15 Speech Rehabilitation 494


Dr. Manasi Jani( Speech Therapist).

Section 5: Stem Cell Therapy


16 An Overview on Stem Cells and Stem Cell Therapy 499
Dr. Prerna Badhe, M.D., Dr. Nandini Gokulchandran, M.D, Dr. Guneet Chopra, M.B.B.S, (PGDM),
Ms. Pooja Kulkarni, M.S. (Biotechnology), Dr. Alok Sharma, M.S., [Link].

17 Role of Rehabilitation Therapist in Stem Cell Therapy 510


Dr. Mamta Lohia, [Link], F.N.R,CBE (USA), Dr. Guneet Chopra, M.B.B.S, (PGDM),
Dr. Prerna Badhe, M.D., Dr. Nandini Gokulchandran, M.D, Dr. Alok Sharma, M.S., [Link].

18 Clinical Improvements in Neurological Disorders after Stem Cell Therapy 521


Dr. Nandini Gokulchandran, M.D, Dr. Guneet Chopra, M.B.B.S,(PGDM), Dr. Prerna Badhe, M.D.,
Ms. Pooja Kulkarni, M.S.(Biotechnology), Dr. Mamta Lohia, [Link], F.N.R,CBE(USA),
Dr. Alok Sharma, M.S., [Link].
xv

Section 1
Spine
xvi
Ch.1 Spinal Cord Injury
Dr. V. C. Jacob, [Link]., D.P.T., Dr. Dhruv Mehta, MPT; Dr. Joji Joseph, BPTH, F.N.R;[Link] Patel, [Link].,
DNBE, FRCS (Urol), [Link] Kulkarni, M.S, [Link]. Ketna Mehta, Ph.D., Dr. Alok Sharma, M.S, MCh, Dr.
Naren Naik, M.S.,[Link].; Dr. Sanjay Kukreja, M.B.B.S., Dr. Hema Biju, [Link],(Neuro), Dr. Myola D'sa,
[Link]., Ms. Akshata Shetty, M.A. (Clinical Psycologist)

INTRODUCTION function in a normal or as near as normal manner


as possible. Having to give up independence and
“So many of our dreams at first seem impossible, becoming totally dependent on others is a
then they seem improbable and when we summon frustrating and debilitating affair for a person with
the will they soon become inevitable.” SCI. with a unified team approach, a person with a
– Christopher Reeve SCI himself or herself would benefit in gaining not
Rehabilitation of the spinal cord injured individuals only physical independence but also economic and
is by far one of the most challenging fields for the social acceptance. The relatives and the individual
rehabilitation experts. Till today there is very little with SCI are as much a part of the team as
to claim as a panacea for the spinal cord injury (SCI) professionals as eventually they will have to share
or regeneration of the damaged spinal cord. the responsibility for the success in getting back the
However the researchers all over the world find person into the society and not becoming a burden
great hope in stem cell therapy (SCT) and it has to the society.
been proved beyond doubt that the results with SCT Dr. Ketna Mehta. PhD., a SCI person herself says,
coupled with good comprehensive rehabilitation is it starts not just from movements of the limb done
better than just giving SCT. Therefore rehabilitation in an attempt to gain mobility and avoid
is being given a better facelift by SCT. contractures and other related complications but
As everyone knows that the role of all the members from mobilization of the mind by constant
of the rehabilitation team is equally important and motivation so as to prevent from developing the
goes hand in hand with each other. No one can worst ever contracture called “depression”. Having
claim that other’s role is less important. Perhaps in to give up independence to a totally dependent life,
one case one of the team member’s role may be all of a sudden can be largely frustrating and
more significant than other’s, whereas it may be devastating. It definitely is a good growth medium
the other member’s role in yet another case. But for harmful parasitic thoughts of hopelessness,
overall each one has to put in his best foot forward helplessness and worthlessness.
to achieve the desired goal. Therefore it always pays To help of patient come out of this dreadful
if we can achieve the co-operation and co- situation, it requires effort from a team of members,
ordination of all the team members. It is not always which is rightly called as a multidisciplinary team.
possible to get everyone together all the time. But a
good network of communication between the team MULTI DISCIPLINARY TEAM
would bring in good results.
An ideal multi disciplinary team should comprise
In Latin, rehabilitare means make fit again. of all of the following members:
Definition: 1. Spinal surgeon

Rehabilitation is defined as 2. Urologist


“Rehabilitation is a treatment or treatments 3. Physiotherapist
designed to facilitate the process of recovery from 4. Occupational therapist
injury, illness or disease to as normal a condition
as possible.” 5. Medical social worker
– Medical Dictionary 6. Psychological counselor
In other words, it is the process of restoration of 7. Rehabilitation nurse
skills by a person who has had an illness or injury 8. Care givers
so as to regain maximum self sufficiency and
2 Neuro-Rehabilitation : A multi disciplinary approach

However the team would not be complete without but since life expectancy has improved
the SCI person himself whose willingness to co- considerably, the number of living SCI patients
operate with the team members alone would make have increased. Though the prevalence is not
the entire process easier. Ideally the team members much compared to the population, their
should take collective decisions in setting a goal or presence is significant.
outcome measures, which is nothing but a guideline
to achieve the expected level of recovery. For this Causes and risk factors:
each member has to set a realistic achievable goal.
Spinal cord trauma can be caused by any number
The patient’s expectation may be too much, so also
of injuries to the spine. They can result from motor
of the team members. The expectation may be
vehicle accidents, falls, sports injuries (particularly
realistic only if one takes into consideration the
diving into shallow water), industrial accidents,
medical problems and the psychological status of
gunshot wounds, assault, and other causes.
the patient.
A minor injury can cause spinal cord trauma if the
It is advisable and ethical to involve the patient in
spine is weakened (such as from Rheumatoid
planning the treatment pathway from time to time.
Arthritis or Osteoporosis) or if the spinal canal
However very few patients have the technical
protecting the spinal cord has become too narrow
knowledge regarding the management of their
(spinal stenosis) due to the normal aging process.
condition. Hence the therapists have to make efforts
to explain the advantages and disadvantages of Direct injury, such as cuts, can occur to the spinal
various approaches. Many a times it is beneficial to cord, particularly if the bones or the disks have been
show other rehabilitated patients who have damaged. Fragments of bone (for example, from
successfully undergone the rehab process and how broken vertebrae) or fragments of metal (such as
they have solved their own problems, thereby living from a traffic accident or gunshot) can cut or
a productive life within their face of limitations. damage the spinal cord.
Before taking the final decision it is mandatory on
Direct damage can also occur if the spinal cord is
the part of the therapist to consult other team
pulled, pressed sideways, or compressed. This may
members and each one of them has to make a
occur if the head, neck, or back are twisted
thorough evaluation of the motor skills, functional
abnormally during an accident or injury.
skills, home assessment, ADL, etc. Rehab begins
from the day of the injury. All the interventions Bleeding, fluid accumulation, and swelling can
planned for the patient from day one focuses on occur inside the spinal cord or outside the spinal
prevention of further damage to the spinal cord and cord (but within the spinal canal). The accumulation
to preserve the functional capabilities, most vital of blood or fluid can compress the spinal cord and
to save the patient. So a brief review of the SCI is damage it.
discussed below. In the initial phase after the SCI, Most spinal cord trauma happens to young, healthy
surgeon plays a vital role especially to decompress individuals. Men ages 15 - 35 are most commonly
and stabilize the spine so that the actual physical affected. The death rate tends to be higher in young
rehabilitation can commence at the earliest. How children with spinal injuries.
big is the problem? To put light to the intensity of
the SCI issue, the epidemiology is stated below. Risk factors include participating in risky physical
activities, not wearing protective gear during work
Epidemiology: or play, or diving into shallow water.
Older people with weakened spines (from
• Before World War 2, the average life
osteoporosis) may be more likely to have a spinal
expectancy of a SCI person was just 2yrs. With
cord injury. Patients who have other medical
the advent of antibiotics and improved
problems that make them prone to falling from
therapeutic measures, the management of
weakness or clumsiness (from stroke, for example)
complications of SCI has improved
may also be more susceptible.
remarkably. Acute care has also improved
considerably. After the rehabilitation, they
Symptoms:
learn to live rather independently or with
minimal help of the family members. Symptoms vary somewhat depending on the
location of the injury. Spinal cord injury causes
• The annual incidence of SCI has not changed weakness and sensory loss at and below the point
Spinal Cord Injury 3

of the injury. The severity of symptoms depends • Loss of normal bowel and bladder control
on whether the entire cord is severely injured (constipation, leakage, and bladder spasms)
(complete) or only partially injured (incomplete).
• Numbness
The spinal cord doesn't go below the 1st lumbar
• Pain
vertebra, so injuries at and below this level do not
cause spinal cord injury. However, they may cause • Sensory changes
"cauda equina syndrome" -- injury to the nerve roots • Spasticity (increased muscle tone)
in this area.
• Weakness and paralysis
1. CERVICAL LEVEL INJURIES
COMPLETE SPINAL CORD INJURY
When spinal cord injuries occur in the neck area,
symptoms can affect the arms, legs, and middle of In complete lesion, there is no sensory or motor
the body. The symptoms may occur on one or both function below the level of lesion. It is caused by a
sides of the body. Symptoms can include: complete transection or severe compression or
extensive vascular impairment (due to lack of blood
• Breathing difficulties (from paralysis of the supply to the spinal cord).
breathing muscles, if the injury is high up in
the neck) INCOMPLETE SPINAL CORD INJURY
• Loss of normal bowel and bladder control An incomplete spinal cord injury is the term used
(may include constipation, incontinence, to describe damage to the spinal cord that is not
bladder spasms) absolute. The incomplete injury will vary
enormously from person to person and will be
• Numbness
entirely dependant on the way the spinal cord has
• Sensory changes been compromised.
• Spasticity (increased muscle tone)
TYPES OF INCOMPLETE SPINAL CORD
• Pain INJURY
• Weakness, paralysis
• Central Cord Syndrome:
2. THORACIC LEVEL INJURIES This is the commonest of the incomplete syndrome
(CCS). It is characterized by weakness in upper
When spinal injuries occur at thoracic level,
more than lower limbs with sacral sparing. In
symptoms can affect the legs:
addition, sensory loss below the lesion with partial
• Loss of normal bowel and bladder control sensory deficit. Schneidan and others noted that the
(may include constipation, incontinence, etiologic factor was hyperextension with
bladder spasms simultaneous compression of the cord by either
• Numbness anterior osteophytes and posterior impingement
caused by buckling of the ligmentum flavum. It is
• Sensory changes
more common with elderly group of patients with
• Spasticity (increased muscle tone) cervical spondylosis, than with younger group of
• Pain traumatic patients. Pathology of the CCS is
probably due to the injury to the central part of the
• Weakness, paralysis
cord. Depending on the severity of the lesion both
Injuries to the high thoracic spinal cord may also upper and lower limbs getting affected with upper
result in blood pressure problems, abnormal more than lower because in cervical fibers are more
sweating, and trouble maintaining normal body centrally located compared to dorsal, lumbar and
temperature. sacral spine.
Quencer etal by MRI study found that CCS is
3. LUMBO SACRAL INJURIES
predominately a white matter peripheral injury and
When spinal injuries occur at the lumbo sacral level, not intramedullary hemorrhage. CCS usually have
varying degrees of symptoms can affect one or both good prognosis. The pattern of recovery also seen
legs, as well as the muscles that control bladder and starting with lower limbs followed by bladder
bowel: bowel function and then Upper limb starting from
4 Neuro-Rehabilitation : A multi disciplinary approach

proximal to distal and finally intrinsic muscle in etc.) Clinically they present loss of motor and
Upper extremity. However younger patients pinprick sensation with relative preservation of
recover faster than elderly patients. Similarly proprioception, deep pressure sensation. Usually
bladder recovery is also seen in younger more than the prognosis is very poor with poor motor recovery
elderly patients. Patients with ASIA scale ‘D’ are and coordination.
able to recover well and are ambulatory.
• Conus medullaries and
• Brown –Sequard Syndrome (BSS) : Cauda equina lesion:
It occurs in cases of stab injuries with ipsilateral It is the terminal part of the cord which lies at the
loss of all sensory modalities at the level of injury: inferior aspect of L1 vertebrae. The segment above
• With ipsilateral flaccid motor paralysis. conus medullaris is termed as epiconus consisting
of cord segments [Link] roots then travel from
• With ipsilateral loss of position sense and conus medullaries caudal as the cauda equine.
vibration sense below the lesion.
Injuries to the conus will present LMN deficits of
• With ipsilateral loss of motor function anal sphincter and bladder. If L3-S2 nerve roots are
below the lesion. not affected, motor strength in the legs and feet may
• With contralateral loss of pain and not be affected. If the roots are affected, it will give
temperature sensation. LMN damage with diminished reflexes.

This is because of the crossing of spinothalamic Injuries below L1 usually give a LMN weakness
fibres which carry pain and temperature fibres supplying the lumbar and sacral segments causing
whereas corticospinal tracts cross in the brainstem muscle atrophy with bladder bowel involvement
Brown sequard plus syndrome (BSPS) is much with loss of plantar reflexes, anal reflexes and
more common compared to pure BSS i.e. with bulbocaverous reflexes and impotence.
ipsilateral motor weakness (hemiplegia ) and Cauda equine lesions have better prognosis than
contralateral hemiagnosia. other SCI.
Numerous nontraumatic causes of Brown- Fracture L1 may result in conus damage whereas
Séquard syndrome have also been reported, fracture L2 downwards will affect cauda equina.
including the following:
Isolated conus injury is very rare.
• Tumor (primary or metastatic)
Incidence of conus and cauda equina injuries are
• Multiple sclerosis common.
• Disk herniation
DIAGNOSTIC MEASURES:
• Herniation of the spinal cord through a
Spinal cord injury is a medical emergency requiring
dural defect (idiopathic or posttraumatic)
immediate attention.
• Epidural hematoma
The health care provider will perform a physical
• Vertebral artery dissection exam, including a neurological exam. This will help
identify the exact location of the injury, if it is not
• Transverse myelitis
already known. Some of the person's reflexes may
• Radiation be abnormal or absent. Once swelling goes down,
• Type II decompression sickness some reflexes may slowly recover.

• Intravenous drug use The following tests may be ordered:


• A CT scan or MRI of the spine may show the
• Anterior Cord Syndrome: location and extent of the damage and reveal
Damage to the anterior 2/3 of the spinal cord problems such as blood clots (hematomas).
preserving the posterior column due to retropulsed
• Myelogram may be necessary in rare cases.
bone fragments either due to direct injury to the
anterior cord or anterior spinal artery which supply • Somatosensory evoked potential (SSEP)
blood to the anterior part of the spinal cord.( due testing or magnetic stimulation may show if
to aortic or cardiac surgery, embolism, angioplasty nerve signals can pass through the spinal cord.
Spinal Cord Injury 5

• Spine x-rays may show fracture or damage to II. Maintaining Airway Patency: To keep the
the bones of the spine. airway patent, modified jaw thrust and
insertion of an oral airway is required in
As discussed earlier, the management of SCI begins
some patients and, endotracheal
at the location of the trauma. The initial focus
intubation in others. When indicated
should be to reach the patient immediately to
intubation should be done carefully in
hospital with utmost care not to aggravate the
these patients to avoid spine movement
injury (well supported horizontal position of the
and further spinal cord injury.
spine).
b. Circulation Management: In patients with
MEDICAL MANAGEMENT: acute spinal cord injury, shock may be
A spinal cord trauma is a medical emergency hemorrhagic and/or neurogenic. In these
requiring immediate treatment to reduce the long- patients, a diligent search for occult sources
term effects. The time between the injury and of hemorrhage must be made before making
treatment is a critical factor affecting the eventual the diagnosis of neurogenic shock as these
outcome. Initial treatment of patients with cord patients have high incidence of associated
injury focuses on two aspects - preventing further injuries.
damage and resuscitation. I. Excluding Haemorrhagic shock:
Computed tomography (CT) scan or X-
Prehospital Management: Ray will reveal the most common sources
Spinal cord injury isn't always obvious. Mechanism of occult hemorrhage like chest,
of injury, pain in the vertebral column, or neurologic abdominal, retroperitoneal injuries and
symptoms gives a clue to the emergency medical fractures of the pelvis or long-bones.
personnel regarding suspected spinal injury.
Injuries involving the head, pelvis and those II. Neurogenic shock and its management:
resulting from falling from heights should be History of spinal cord injury with
suspected for spinal cord damage. exclusion of haemorrhage suggests spinal
shock.
Emergency medical personnel stabilize and
i. Fluid resuscitation: Fluid resuscitation
immobilize the spine at the scene of injury and
transport the patient to the emergency department with isotonic crystalloid solution is the
(ED) taking care in not moving the spine. Many a initial treatment of choice for neurogenic
times it is the bad lifting and transport that causes shock. These patients are at risk for the
damage to the spinal cord. acute respiratory distress syndrome
(ARDS) and hence volume overload
Emergency Department Management: should be avoided with judicious fluid
resuscitation.
A. ABC Resuscitation: Airway, breathing and
circulation may be compromised because of spinal ii. Maintaining blood pressure: Systolic
cord injury or associated injuries. Resuscitation is blood pressure should be maintained
aimed at airway maintenance, adequate oxygen above 90 mm Hg and hypotension should
saturation of peripheral blood, restoring blood be avoided in these patients.
pressure to acceptable limits, preventing iii. Maintaining oxygenation: Supplemental
bradycardia, done simultaneously to prevent any oxygenation and/or mechanical
ischemic damage to the already compromised cord. ventilation should be given to maintain
a. Airway management: Spinal cord injury adequate oxygenation and perfusion of
makes the assessment and management of the injured spinal cord.
airway complex and difficult. Immobilization iv. Monitoring heart rate: Heart rate should
of cervical spine must be continued in neutral be 60-100 beats per minute (bpm) in
alignment during airway assessment and normal sinus rhythm. Atropine may be
management. given for treatment of hemodynamically
I. Restoring Airway Patency: Oral significant bradycardia.
secretions are cleared to maintain airway v. Maintaining urine output: Foley catheter
patency and to prevent aspiration.
6 Neuro-Rehabilitation : A multi disciplinary approach

should be inserted to decompress the hospital should have a policy regarding


neurogenic bladder. Urine output should administration of steroids to spinal cord injury
be monitoted and maintained more than patients.
30 mL/h.
Monosialotetrahexosyl ganglioside (GM-1): GM-1
vi. Inotropic support: Dopamine or administered following acute spinal cord injury as
norepinephrine is should be reserved for an adjunct after the administration of
patients who have decreased urinary methylprednisolone may be of benefit.
output despite adequate fluid
resuscitation. A low dose of dopamine in Spinal traction: Spinal traction is a more
the 2- to 5-mcg/kg/min range is usually conservative and less invasive approach to allow
sufficient. the bones to heal naturally without surgery.
Traction prevents movement of the spine. The skull
vii. Prevention of hypothermia: Hypo-
may be held in place with tongs (metal braces
thermia should be prevented in these
placed in the skull and attached to traction weights
patients.
or to a harness on the body). The spine braces may
B. Assessment and management of associated need to be worn for a long time.
injuries: Twenty five percent of patients with
spinal cord injury have associated head injury. Surgical Management:
A thorough evaluation for intracranial injury Surgery may be required in the treatment of spinal
should be done in the presence of amnesia, cord injury patients. There are three major goals of
external signs of head injury or basilar skull surgery-
fracture, focal neurologic deficits, associated
alcohol intoxication or drug abuse, and a A. Spinal decompression: This involves removal
history of loss of consciousness. of fluid tissue, bone fragments, disk fragments,
or foreign objects that presses on the spinal
Corticosteroid Therapy: cord.
Methyl prednisolone has been shown by the B. Prevention of tethering: Neurological
NASCIS-II (National Acute Spinal Cord Injury disorders related to pulling of the spinal cord
Studies) trial to be significantly beneficial in causing at the base of the spinal canal causing
favorable neurological outcome if administered stretching and damage. Surgery may prevent
early enough (within 8 hours) of the primary tethetering of the cord. [14]
[Link] appears to work by reducing damage to
C. Stabilization of the spine: The weakened
nerve cells and decreasing inflammation near the
vertebrae from fracture may not be capable of
site of injury. To be effective corticosteroids should
supporting the normal weight from the body
be given within 8 hours of injury. The
and protect the spinal cord. Spinal
recommended dose is 30mg/kg bolus to be
instrumentation and fusion can be used to
administered over 15 minutes and after a 45-minute
provide permanent stability to the spinal
pause 5.4 mg/kg/hr over the next 23 hours.
column. These procedures correct, join, and
Nesathurai and Shanker questioned the validity of solidify the level where a spinal element has
the results of NASCIS trial. These authors cited been damaged. A combination of metal
concerns about the randomization, clinical screws, rods, plates or cage may be necessary
endpoints and statistical analysis used in the study. to help hold the vertebrae together and
In addition, the investigators noted that even if the stabilize them until the bones heal.
benefits of steroid therapy were valid, the clinical
Timing of surgery: There are no established
gains were questionable. The steroid therapy is not
standards regarding when to operate the patients
free of side effects. An increased incidence of
with spinal cord injury because of the lack of well
infection and avascular necrosis has been
designed studies. Emergency decompression of the
documented.
spinal is suggested in the following indications-
The Congress of Neurological Surgeons (CNS) has
1. Acute spinal cord injury with progressive
stated that steroid therapy "should only be
neurologic deterioration, facet dislocation, or
undertaken with the knowledge that the evidence
bilateral locked facets.
suggesting harmful side effects is more consistent
than any suggestion of clinical benefit." Every
Spinal Cord Injury 7

2. Spinal nerve impingement with progressive b. Posterior lumbar interbody fusion (PLIF)
radiculopathy. - the disc is accessed from a posterior
incision. c. Transforaminal lumbar
3. Extradural lesions such as epidural
interbody fusion
hematomas or abscesses.
c. Transforaminal interbody fusion(TLIF) -
4. Cauda equina syndrome.
the disc is accessed from a posterior
Type of surgery: incision on one side of the spine.
Instrumentation with screws, rods, plates
The type of surgery performed (anterior vs.
or cages may be required to provide
posterior), distraction forces during surgery,
permanent stability to the spinal cord.
preoperative grade all influence the outcome. The
surgery may be- Figure 1.1.4
Surgery for prevention of tethering: After complete
Spinal decompression: release and reconstruction of the spinal cord, a
The major types of surgery for spinal Gore-Tex surgical membrane can be placed over
decompression are- the cord and fixed to the lateral dural surface with
a. Microdiscectomy /Corpectomy stay sutures to prevent tethering.

b. Laminectomy Post operative physiotherapy


Post operative physiotherapy starts immediately
Stabilization of the spine:
after the surgery and is continued during the
Can be achieved by spinal fusion with or without convalescent period. Respiratory management
instrumentation. plays a very important role to prevent post
1. In the Craniovertebral junction, stabilization operative complications. Restorative physiotherapy
may be achieved by starts immediately after the patient is stabilized.
a. C1-C2 fixation using pedicle screws/
REHABILITATION OF PATIENTS
transarticular screw/sublaminar wires
WITH LESIONS BELOW CERVICAL
b. Occipitocervical fixation
SPINE (PARAPLEGICS)
Figure 1.1.1
The acute stage of rehabilitation is followed by the
2. In the cervical spine stabilization may be convalescent stage, where the patient is thoroughly
achieved by evaluated by the team for further management. The
a. Anterior approach using vertebral body management is based on classification of SCI as per
plates and screw the ASIA Scale.

b. Posterior approach using lateral mass The American Spinal Injury Association (ASIA) first
screws published an international classification of spinal
cord injury in 1982, called the International
Figure 1.1.2 and 1.1.3 Standards for Neurological and Functional
3. In the dorsal spine stabilization may be Classification of Spinal Cord Injury. Now in its sixth
achieved by edition, the International Standards for
Neurological Classification of Spinal Cord Injury
a. Anterior transthoracic approach for
(ISNCSCI) is still widely used to document sensory
interbody cage placement
and motor impairments following SCI. It is based
b. Posterior approach using transpedicular on neurological responses, touch and pinprick
screw and rod system sensations tested in each dermatome, and strength
of ten key muscles on each side of the body,
3. In the lumbar spine, types of stabilization
including hip flexion (L2), shoulder shrug (C4),
procedures are-
elbow flexion (C5), wrist extension (C6), and elbow
a. Anterior lumbar interbody fusion (ALIF) extension (C7).Traumatic spinal cord injury is
- the disc is accessed from an anterior classified into five categories on the ASIA
abdominal incision. Impairment Scale:
8 Neuro-Rehabilitation : A multi disciplinary approach

Fig 1: X-ray lateral view CV Fig. 2: Intraoperative picture Fig. 3: X-ray lateral view cervical
junction showing occipito cervical showing placement of vertebral spine showing cervical fixation
fixation. body plate. using vertebral plate and screws.

Fig. 4: X-ray lateral view lumbar spine showing Fig. 1.1.4: CT Cervical spine sagittal view: showing
instrumentation using transpedicular screws and post traumatic subluxation of C4 over C5 with canal
rods and interbody cage placement. compromise.

Fig. 1.1.5: Intraoperative image following reduction and stabilization of C4.C5 subluxation.
Spinal Cord Injury 9

ASIA IMPAIRMENT SCALE After evaluation of the client based on the


• A indicates a "complete" spinal cord injury classification, it is vital to set goals or achieving the
where no motor or sensory function is realistic expected outcome.
preserved in the sacral segments S4-S5.
Prediction of outcome:
• B indicates an "incomplete" spinal cord injury
Physical examination is the most important mehtod
where sensory but not motor function is
to predict the outcome.
preserved below the neurological level and
includes the sacral segments S4-S5. This is Initial presence of muscle power would give a clear
typically a transient phase and if the person prediction. The earlier the muscle shows some
recovers any motor function below the power the faster muscle could come to functional
neurological level, that person essentially level i.e above 3.
becomes a motor incomplete, i.e. ASIA C or Within the first 6 months the recovery seen is
D. maximum and the improvement in the first three
• C indicates an "incomplete" spinal cord injury months is usually more than the next three months.
where motor function is preserved below the If one gets back some power initially the chances
neurological level and more than half of key of improvement is better than the muscles
muscles below the neurological level have a remaining “0”.
muscle grade of less than 3, which indicates
active movement with full range of motion However those who have an incomplete injury
against gravity. initially have better prognosis for ambulation. MRI
is the most superior radiological test for
• D indicates an "incomplete" spinal cord injury determining the prognosis. E.g. of a hemorrhage
where motor function is preserved below the seen initially, the prognosis is poor. Contusion is
neurological level and at least half of the key worse than edema. If no hemorrhage is seen then
muscles below the neurological level have a damage to the cord is incomplete and the prognosis
muscle grade of 3 or more. is better. The degree and the extent of edema is also
• E indicates "normal" where motor and sensory determines the chances of recovery. In other words,
scores are normal. Note that it is possible to along with clinical assessment, MRI can give the
have spinal cord injury and neurological prediction better. The other investigations include
deficits with completely normal motor and nerve conduction studies, late responses (H-reflex
sensory scores. and F-wave) and somatosensory evoked potentials.
Whatever the prognosis is, rehab has a definite role
MODIFIED ASHWORTH SCALE: to play and whenever any scientific advances come,
FOR GRADING TONE they could try at that stage. Till then the patient
has to come to terms and try to become as
• Grade 0: no increased muscle tone
independent as possible.
• Grade 1: Slight increase in muscle tone,
manifested by a catch and release, or by Outcome charts
minimal resistance at end of ROM when These are merely meant to act as a guide. Perhaps
affected part is moved in flexion or extension. the therapist’s expectations would be too much or
• Grade 1+ : slight increase in muscle tone too little. So the purpose is to describe the expected
manifested by a catch, followed by minimal levels of safe functions in SCI patients. Considering
resistance throughout the remainder (<50%) associated medical problems and psychological
of ROM. status, therapists have to do systematic treatment
planning.
• Grade 2: more marked increase in muscle tone
through most of ROM and affected part is Prescription of the equipments:
easily moved.
Things to be considered are:
• Grade 3: considerable increase in muscle tone,
1. Easy availability.
passive movement is difficult.
2. Accessibility
• Grade 4: limb rigid in flexion or extension.
3. Durability
10 Neuro-Rehabilitation : A multi disciplinary approach

4. Safety 1. Bony prominences and vulnerable


5. Comfort area of skin.
6. Cost 2. Scar tissue.
It is advisable to involve the patients in planning. 3. Pressure sores.
However very few patients can make their own 4. Secondary bad postures because of
decisions and therefore therapist has to explain pressure sores.
advantages and disadvantages, often trying out b) Sensory Evaluation:
different techniques. Perhaps, it will be more
beneficial to show other patients who have 1. Superficial and deep sensations like
experienced the same problems and have solved pain, temperature, touch joint
their problems. Many a times it would be essential position etc.
to set a trial period, trying out trial calipers/ shoes. 2. Patient’s awareness about sensory
Eventually the therapist can take a final decision in deficits.
prescribing the necessary equipments. Before taking 3. Implications because of sensory
the final decision therapist has to make a thorough deficits namely burns, bruises lack
evaluation of motor skills, functional skills, home of coordination because of lack of
assessment, ADL and opinions of other team kinesthetic sense.
members and taking patients’ consent. 4. Damage to skin due to faulty
equipments like orthosis.
Evaluation:
c) Respiratory Evaluation:
A) Data collection:
1. General observations:
1. From patients and relatives.
2. From medical records including X-rays, a) Shape and symmetry of chest.
MRI, and other reports. b) Muscle tone.
c) Wasting.
d) Presence of artificial airways like
tracheostomy, intercostals tubes.
Respiratory history and associated
medical history may indicate that he
may require preventive measures
for chest complications. Evaluation
of upper and lower chest expansions
may give a clue as to which lobes
require to be attended. Lack of chest
expansion and mobility may lead to
[Link] of Lumbar Spine decreased vital capacity. (Normal
value of chest expansion at xiphoid
It is essential to explain to patients the need process should be at least 2 1/2 to3
for evaluation and taking proper history so inches.) Lack of ROM at shoulder
that he actively participates in the actual level may lead to poor chest
rehabilitation process. expansion. It is essential to have
normal flexibility of trunk and hips
B) Evaluation Procedures:
as it is easier for him to cough
• Assessment of strength. effectively. Normal posture of trunk
• ROM is essential for rib mobility as
• Sensations excessive tightness of ribs would
• Tone lead to poor Vital capacity.
• Girth measurement 2. Respiratory Muscle Strength Evaluation:

C) Physical Evaluation: 1. Diaphragm.


2. Abdominals.
a) Skin Evaluation:
Spinal Cord Injury 11

3. Accessory muscles like intercostals, ROM of Joints:


scalenes, serratus anterior, pectorals, • Rolling may be more difficult with
erector spinae and overstretched back and trunk muscles.
sternocleidomastoid.
• Selective tightness of the long flexors of the
Implications : If abdominal muscles are weak,
hand will help in grasp (Tendodesis action).
it may effect in poor coughing because of
inability to build up intrathoracic pressure to • Maintaining of adequate ROM is essential for
expel air and therefore bronchial hygiene may helping the totally dependent patients to move
be affected very badly. for changing the positions.
1. An abdominal binder may be useful. • If a patient can be moved easily, there is less
chances for pressure sores.
2. Increased respiratory rate indicate
shallow breaths.. MuscleTone:
3. When abdominals are paralysed, they 1. Mild increase: Resistance for passive stretching
breathe best in lying. with full ROM, function not being affected
4. Hypoventilation in patient may also 2. Moderate increase : More resistance and full
cause drowsiness and irritability. This ROM is possible but function being affected.
usually happens among quadriplegics or
high level paraplegics because of 3. Severe increase: Full ROM is not possible and
weakness of accessory muscles. many functional skills impossible.

5. Hypoventilation may cause fainting, Description of quality of Tone:


tingling and numbness. 1. Constant or Fluctuating?
6. Poor co ordination of breathing with 2. Is it related to position change?
functional activities may cause lack of
endurance. 3. Symmetrical or Asymmetrical?

7. Deep breathing exercises would increase 4. Fluctuation related to time.


inspiratory capacity which will improve 5. Is increased tone beneficial in functional
coughing and ventilation of all the lobes activities? e.g minimal increase in tone in
thus decreasing respiratory extensors of hip & knee is beneficial in
complications. standing.

Personal hygiene (C) Recovery process:


1. Evaluation of cough and Vital Capacity 1. Spinal Shock: 3 to 6 weeks.
2. Evaluation of patients’ knowledge of postural In UMN lesions, tone usually increases for 1st
drainage. 2 yrs and then levels off. Flexor tone is seen
initially, extensor tone sets in after 6 months.
IMPLICATION FOR PATIENTS
2. Effect of medications.
1. Vital capacity may be used as an indicator of
Implications for the Patients:
weakness of respiratory muscles.
Muscle tone may benefit or hamper ADL.
2. Decreased vital capacity leads to decreased
cough effectiveness. Chances for getting pressure sore are more
with increased tone because of the difficulty
3. Postural drainage and coughing are very
in turning.
important for a SCI patient as it would prevent
infection.
Strength:
4. Movements would decrease the possibility of Complete evaluation of all muscles.
accumulation of secretions and infections in
the lungs. Difficultes in evaluating the muscles:
1. Immobilisation device.
12 Neuro-Rehabilitation : A multi disciplinary approach

2. Poor stabilization because of weak muscles. Providing a trunk support may also help.
3. Trick movements.
Coordination:
Implications: a) Kinesthetic sense or awareness and accuracy
of movements depend on timing. Find out as
Some of the key muscles:
to how good was his performing skill prior to
1. Scapular stabilizers useful in one man pivot injury in comparison to the present skill.
transfer.
b) It may require a lot of repetitions for
2. Pectoralis Major essential for rolling. improving the skill, also repeated instructions
3. Triceps for bed mobility skills. and explanations are beneficial.

4. Wrist Extensors: c) ADL training is the ultimate goal.

1. Tenodesis. Endurance :
2. Moving wheelchair. 1) Evaluation of cardiopulmonary status.
3. use as a hook in other ADL. 2) Evaluation of breathing with different
activities.
5. Triceps: are essential for Wheelchair push ups.
Lateral transfers. 3) Because of poor endurance, ADL may become
slow.
6. Knee flexors and extensors for ambulation
with AFO. 4) Many a times Exercise sessions have to be
made short for want of endurance.
7. Hip flexors: very useful in ambulation.
8. Ankle Dorsiflexors: for clearing the ground Emotional Status:
and ambulation without AFO 1) Evaluation of patients understanding of the
disability, whether he is realistic or not in his
Tolerance for Vertical Position: expectation of the outcome.
1. Lying to sitting in bed.
2) Evaluation of his accepting the disability.
2. Reclining wheelchair.
3) Does he take active interest in solving his
3. Tilt table. problems by himself? Is he interested in having
Implications: equipments, which would improve the quality
of life? e.g An orthosis may be useful for
1. ADL independence, especially ambulation or perhaps he may have to use a
a) Transfers. W/C for the rest of his life
b) Wheelchair movements. 4) Is there a desire for him to be independent in
c) Dressing. his ADL or does he expect all the help from
spouse or caretaker?
Balance :
• Evaluation of positive supporting Relevant Information required in the Chart:
reaction. A) Age: Younger patients may be able to adapt
• Evaluation of equilibrium reaction. or perform better. Would like to be
• Evaluation of static balance/ Dynamic independent in ADL
balance. B) Body build :
Implications: 1) Has he lost weight or put on with heavy
weight? With heavy weights the
Unless patient had a head injury also, loss of
movements are reduced, the chances of
balance is due to posterior column
getting pressure sores are more. At the
involvement and weakness of certain group
same time, the thinner the person more
of muscles.
the bony prominences and they are also
Therefore he needs to learn to use some other prone to have pressure sores.
group of muscles.
Spinal Cord Injury 13

2) Standard wheelchairs are easily available, main problems of the patient. It is only to see
where as over size wheelchairs and whether we are able to go towards the expected
cushions are to be custom made. Over goal and final evaluation before discharge is to see
size wheelchair may not pass through whether he has achieved the outcomes so that he
standard doors. Wheelchair movements can lead a meaningful life at home.
may also require larger area for mobility.
3) Heavier patients may require more PHYSIOTHERAPY DURING THE
number of people for ADL especially REHABILITATIVE PHASE
transferring. Many people prefer
institutional life. BED MOBILITY
C) Type of Injury: The most essential need of a SCI patient is some
amount of mobility in the bed. The very first thing
Type of injury and extent of injury would help
that he attempts is going on to the side by using
in knowing the prognosis. e.g. Cases with
upper trunk movements and scapular movements.
complete transection of the cord, almost
The therapist can assist him initially by just doing
always will not have any chance of recovery
passive rotation and asking him to assist as much
and hence the patients have to live with
as possible. Next, he is asked to do this by himself
disability. Similarly the level of injury would
and the therapist helping only as much as he wants.
also give us an idea about the outcome as well
When the patient can do by himself, more and more
as the chances of complications.
resistance has to be applied. He may use
D) General Health : momentum, even weight cuffs may be applied to
the hands to increase the momentum and facilitate
Other injuries like head injury, limb fractures,
movements. It also strengthens the muscles.
cardiac, respiratory problems, ectopic bone
formation, bladder and bowel problems are to These activities give him awareness that with swift
be taken into consideration. They all affect the movements of the upper trunk he can initiate lower
therapy and functional outcome. e.g. Ectopic trunk movements.
bone formation affect ROM. Cardiac & Rolling can be made easy if he flexes his one hip
Respiratory problems may affect the and knee or both hips are flexed.
performance and endurance.
BED MOBILITY FOR PARAPLEGICS
E) Personality and Life style:
1. Whether his work was sedentary nature Outcome: Patient should do all bed mobilities
or not. independently and without any specific equipment.
2. Educational level /occupation How to achieve:
3. Willingness for change of lifestyle or job. • Rolling to the left and right.
4. Acceptance of disability: Whether he is
• Rolling from prone to supine and back.
flexible or not?
• Sitting with extended legs from supine as well
F) Family Support:
as with flexed knees
1. Is family supportive?
• Moving to either side of bed.
2. Will he need another care giver?
• Moving to head and foot of the bed.
3. Source of income for maintenance?
• Doing push-ups in bed to relieve pressure.
4. Will they continue the same therapy at
• Coming to quadruped(All Fours)position.
home also?
• Crawling
5. Will they arrange for Vocational
Rehabilitation so that he too can become • Kneel
an earning member and he too can live
with dignity? Considerations:
Evaluation is a continuous process. Initial • Upper extremity strength and ROM.
evaluation is only a baseline evaluation to find out • Spasticity.
14 Neuro-Rehabilitation : A multi disciplinary approach

Fig 2. Rolling Quadruped

Crawling Kneel Standing

• Pressure Sore. pressure sore are much less. Therefore careful


clinical observations can help a patient to develop
• Age.
a treatment protocol which he can follow by
• Obesity. himself.
Process:
Pressure Relieving Maneuvers:
• Evaluate and improve motor skills routinely.
1. Push- ups with hands on armrest of
• Appropriate bed mobility techniques. wheelchair lifting buttocks off the seat. (Strong
• Make a routine exercise program to improve triceps and shoulder extensors essential)
corresponding muscles.

PRESSURE RELIEF
Due to lack of sensation, they don’t perceive the
discomfort of pressure on certain vulnerable points
because of squeezing of the local blood supply and
resulting into ischemia over certain area and that
will lead into pressure sore. They also do not have
the ability to move the part to relieve the pressure.
Therefore they have to develop new methods
consciously. Patients who have very pointed bony
prominences are prone to develop pressure sores
than others (e.g. Some have anatomically pointed
ischial tuberosities.) Therefore the therapists have
to make a protocol for each patient and see as to Push Ups
how often they have to do the pressure relieving
measures. After the patient becomes active in 2. Forward leaning: Trunk muscles, pectoral
wheelchair activities, the chances of getting muscles, triceps are essential. However
Spinal Cord Injury 15

passively leaning forward can also relieve other pressure relief skills like forward leaning, side
pressure to some extent. leaning etc.
3. Side lean by hooking on the rim of the
wheelchair on the opposite side and leaning TRANSFERS
to one side and then the other. For doing this Moving from one surface to other is a major task
he needs strong biceps. for almost all SCI patients and needs special training
4. Electrically operated reclining wheelchair can and effort. They need the support of their strong
also relieve the pressure. hands to do that. They have to take extra care that
Cushions they don’t fall on the ground while transferring and
also see that they do not hurt themselves.
It is proven that no perfect cushion exists which is
suitable for all the patients. We have to look into As an initial step they are taught to maneuver to
the lifestyle needs. Some of the cushions are too move themselves on the bed itself. Firmer beds are
expensive. Some are easily punctured. Foam essential for the initial training. One has to learn
cushions need to be replaced every 6-18 months. step by step and may take several days in achieving
each step. For instance,
Arteriolar pressure in the skin capillaries is
appropriately 32 mm hg but none of the cushions i. Lifting and moving the legs to the left and right
available in the market can maintain pressure below with their own hands;
32 mm. Perhaps, it is the posture control that ii. Managing to lift their body up by pushing on
matters most. Foam cushion has the benefit of being the beds.
more porous with less moisture formation. iii. Shifting the body to the left and right on the
However it is essential that the covering should be bed; maintaining their balance.
of light cotton material and not plastic. Another Some of them require improving their sitting
disadvantage of foam cushion is that if the balance especially the higher level paraplegics.
atmospheric temperature is high, the skin Some may require strengthening their triceps.
temperature also tends to increase. Older patients Some may require improving their ROM like
tend to have lower skin capillary pressures (even rotation of trunk, elbow movements. How the
as low as 20mm). Thin patients have less fat to cover various components are to be continued will
bony prominences so also, flaccid patients. For these depend on his skill. He should be encouraged
patients, cushions with lower contact pressure relief to try different methods and should see that
are recommended. he never gives up trying. It would be ideal to
Those who have sensation intact, find contoured show other patients who have achieved the
foam cushions uncomfortable. Those who have task by trying different methods and have
bladder bowel incontinence, less porous covers managed. Many a times the therapist has to
have to be given. try different methods before the patient finds
With contoured cushions, transferring is more a better method for him i.e. much
difficult. All these factors should be taken into experimentation has to be made.
consideration. No cushion can substitute for iv. After achieving the ability to move from one
frequent pressure releasing maneuvers, like wt end of the cot to the other he has to learn to
shifting and pushups. Other factors like protein transfer from the cot to the wheelchair. It
deficiency, anemia and infection also should be would be easy for him if the WC is of the same
controlled in order to prevent pressure sores. height as the cot. If the WC is at a lower height
he can easily go from the cot to WC but would
Mechanical Methods
find it difficult to transfer back. One needs very
Electrically operated reclining wheelchair is strong Upper extremities. A transfer board
probably the best pressure relieving method would facilitate the movements especially for
especially for higher level paraplegic and higher level SCI cases who cannot maintain
tetraplegics. the balance while transferring. However with
Relieve pressure on ischium, sacrum, and greater repeated attempts they learn to manage well
trochanter. Factors to be considered are ROM even to a higher level. Keeping the Transfer
(especially Hip and Elbow), Tone, obesity, cushions, board under the mid thigh is the most difficult
adjustable arm rests, Ability to do pushups and thing.
16 Neuro-Rehabilitation : A multi disciplinary approach

v. The next transfer the paraplegics have to age, safety, amount of assistance required
master is, from wheelchair to the commode to transfer and time consumption. If the
and back. For this, bars need to be fitted on wheelchair would fit, lateral transfer is
the side walls to make use of while very practical. Raised toilet seats, grab
transferring. bars are necessary. There is no one way
to approach bath transfers. Selection of
vi. It is ideal for paraplegics to learn to transfer
specific techniques will depend on each
to the floor and back, though this is not a very
individual patient.
easy maneuver for obese and higher level
paraplegics. Before attempting this it would Transfer to Car and back to Wheelchair:
be ideal for them to try it step by step. As a
Difficulty is because of limited space for movement
first step they can try corner- shifting in one
and bringing the wheelchair closer to the car seat.
attempt, then they can try holding on the two
Therefore longer sliding boards are required. One
cots they can go up to the floor and back. Next
man pivot transfer is also difficult because of the
they could go on to the floor with one hand
limited space. Finding the right method is a matter
on the floor and then going up with both hands
of experimentation usually while getting in legs
on the cot and twisting their body. This is
should go in last and while getting out legs should
possible for patients with lower level of
come out first.
paraplegia and with satisfactory abdominal
muscles. Transfer from Wheelchair to Floor:
Prior to learning, these transfer activities, it is This is a frightening maneuver for a patient who
necessary for the patient to develop good does not have strong abdominals and triceps.
abdominals. He should attempt to go on all However, repeated attempts would give them
four position and do cat and camel exercises. confidence. First they have to scoot to the edge of
Next he should attempt to do side-sitting on the W/C and then come down straight on one hand
either side. Next he could attempt kneel and then the other with both knees following and
standing with a hyper lordotic lumbar spine coming on All fours position. The other alternative
and balance. If he has a strong Quadratus is going on the sides with straight legs sliding
lumborum and Lattissimus dorsi he can be forward while landing on one hand, taking full
taught kneel walking in parallel bars. All these weight with extended elbow and gradually
exercises are preparatory exercises for walking bringing down the buttocks.
with orthosis.
Floor to Wheelchair:
vii. Next he can learn a stand pivot transfer. This
he can perform only if he has strong 1. One of the easiest method is to have an
abdominals and trunk balance. intermediate step and then lifting the body to
the wheelchair.
Dependent Transfers 2. Another method in which patient has to get
1. One man pivot transfer into kneeling position and then pull himself
into the wheelchair. However getting into
• Anterior- arm around shoulder kneeling position is not that easy. Patients need
• Sideways – arm around the waist. a lot of strength and balance. Removing the
foot pedals is important.
2. Two man lifts
3. Yet another method is using the extensor tone
3. Three man lifts. which would assist in the pushing back to
4. Mechanical lift wheelchair. Here again the upper limb /lower
limb strength and extensor tone are very
• Swivel bar transfer: Useful in cases of important.
limited Hip ROM due to spasticity or
ectopic bone formation around hip joints. The factors that determine the process of
transferring independently are the following:
• Bathroom Transfers: Before beginning
these transfers, it is important to assess 1. Age. 2. Obesity. 3. Pressure sores. 4. Spasticity.
the patients own bathroom i.e. size of the 5. ROM.
bathroom; width of the entrance door,
Spinal Cord Injury 17

The actual process includes: posture of the trunk and strong upper extremity.
1. Positioning of the wheelchair and The muscles to be developed are shoulder flexors
management of its parts viz. brakes, and extensors, elbow flexors and extensors, so also
detachable arm rests, foot rests. good hand grip. Maintaining a good posture is
essential for various reasons mainly for
2. Positioning of the transfer board (if necessary). conservation of energy, proper chest expansion,
3. Ability to maintain sitting balance. Ability to endurance, prevention of pressure sores (as
lift body weight with elbows extended. awkward sitting can cause unequal pressure
leading to pressure sore.) Bad alignment cause
4. Ability to slide from one surface to the rest
postural pain in the back. Wheelchair with higher
(lateral transfer) including the management of
back may help patient in maintaining a good
both lower extremities.
posture. However, wheelchairs with higher back
support are not good for fast movement. With lower
WHEELCHAIR MOBILITY back support, the movements of the arms are
As a first step, the paraplegic has to accept, the very swifter.
concept of using a wheelchair for mobility. This is A good pelvic support is essential. If there is any
not an easy thing for a person who was walking pelvic obliquity it needs to be corrected.
and running or driving a vehicle a few days or
Sling seat causes adduction of lower extremities and
months back. For him to accept this slow moving
causes the base of support becoming smaller and
mode of mobility is very difficult. Not only that,
this would cause instability. Sometimes leg straps
the stigma attached to the wheelchair is too much.
are necessary to keep the lower extremities in
However when he realizes that he is bedridden and
abduction.
that without moving from the bed, life becomes
monotonous and a desire could come to him to get Gel cushions also may cause instability. Some prefer
into a wheelchair. If he observes another wheelchair higher foot rests in order to get pelvic stability,
bound patient moving around comfortably, he too preventing forward movement of the pelvis.
would consider getting into a wheelchair and Slight inclined back support with lumbar roll is a
attempt moving at least within house. When he good option the lumbar roll will maintain in a good
observes people even get out of the house in a position; prevent kyphosis, scoliosis and pelvic
wheelchair and face other people in the society, obliquities. It also helps in maintain the pressure
slowly he gets a desire to accept a wheelchair for anterior to the ischial tuberosities and thighs.
mobility rather than remain in a bedridden Adjustment of the height of the arm rest is also
condition. This acceptance is the first step towards important as they are essential for adjusting and
rehabilitation. maintaining the trunk in good posture.
SCI above 1st lumbar spine should almost always Wheelchair movements are better with strong
consider wheelchair as a mode of mobility whereas upper extremities. However more than the
cauda equina lesion with good abdominal muscles strength, good timing and coordination would help
along with quadratus lumborum, should think of in skillful movement.
appliances in the form of orthosis like KAFO and a For forward movements, the muscle essential are:
pair of crutches either elbow crutches or axillary shoulder flexors, adductors and external rotators
crutches. Between wheelchair and orthosis the whereas for backward movements: shoulder
energy consumption is a much more with orthosis. extensors and adductors and internal rotators.
However everyone has a desire in him for walking
than being on a wheelchair. But when he realizes Propelling of Wheelchair:
that wearing a caliper and crutches take time he 1. Trunk flexors and extensors would facilitate
would think in terms of getting into wheelchair and forward push.
moving fast. However wheelchair needs more
space to maneuver. Some of the bathrooms may 2. Avoid holding the tyre as it can get caught in
not have enough space for movement. the brake and cause injury. Grip should be
always on the rim.
Achieving good wheelchair mobility is essential for
all high level paraplegics. For developing Wheelies:
wheelchair skills, it is essential to have a good Wheelie is one of the most advanced skills, a
18 Neuro-Rehabilitation : A multi disciplinary approach

paraplegic can learn i.e. to lift the front wheels up have no hip flexors. This is because wheelchair is
and maintain the balance on the back wheels only. more practical and energy saving and time saving.
This position would help a patient to negotiate
There is a debate on this issue even among the
curbs up. Without achieving a “Full Wheelie” a
medical personnel. Those who are in favor of
patient can negotiate curbs up to 2 inches. When
ambulation and standing believe there is a
he approaches the curb, he should stop and raise
physiological benefit for the patient.
the front wheel then lean forward push the back
wheel up. i. Most of the body’s calcium is found in the
bone.
For negotiating higher curbs i.e. more than 2 inches,
one has to gain momentum by going fast for 5 to 10 ii. SCI patients have a high calcium washout and
feet and go into wheelie and drop front casters on incidence of osteoporosis is evidently seen in
the curb and then lean forward then push the rear the bones.
wheels. One needs good rhythm and timing which iii. This calcium washout is considered to be
everyone can’t achieve. predisposing factor for formation of bladder
For a patient to become independent in mobility stone and ectopic bone formation which are
both indoors and outdoors, he has to do step by seen in SCI patients.
step training like: iv. Weight bearing can increase the bone density.
• Trunk mobility in sitting. v. It is assumed that with ambulation and weight
• Balance and equilibrium reach outs in bearing, calcium excretion will be decreased
sitting. and therefore less osteoporosis, ectopic bone
formation and fewer bladder stones.
• Use of reach out.
However there is a counterargument that standing
• Use of upper extremity in sitting.
and ambulation alone are not sufficient and more
• Wheelies. pressure is needed to promote bone growth. Muscle
contraction can provide much more compressive
• Improve endurance.
force also shearing and torsion forces increase bone
• Forward and backward and turns. density significantly.
• Management of brakes and removing and One study done by Jacqueline Claus-Walker and
putting back arm rests, foot rests etc. several physicians at TIPR in 70’s found that
• Movements on surfaces like tiles, carpets calcium excretion increases during the first ten days,
etc. and continues to increase in the next six months
and after 1 year it lowers to normal. They also found
• Movements through narrow passages, that position of the body will influence fluid
elevators, ramps. distribution. Position is registered by the
• Movements curbs bumps, cracks in side baroreceptors and transmitted via neural pathways
walk etc. to CNS. In the SCI patient, weight bearing is not
often perceived as the neural pathways are not
• Crossing the street safely.
working. In their study they also found complete
recumbency for 3 days did not increase
AMBULATION:
hypercalciuera in quadriplegics whereas in normal
To Walk or not to Walk?
healthy subjects, calcinera increased b 1.5 times.
If you ask any patient this question, he would
certainly say that he wants to walk. Initial stage he The question still remains whether to encourage
would say that he cannot accept a wheelchair as a wheelchair mobility or weight bearing ambulation?
mode of ambulation. However as time passes and There is no doubt that standing and walking would
he realizes that there is a lot of energy consumption certainly boost up the morale of the patient as he
he slowly accepts the fact that wheelchair may be feels that he is more like the normal people.
better mode of locomotion. However those who are However in course of time, he would realize that
functionally able to manage with orthosis will never walking is much more time consuming and energy
take the idea of a wheelchair. Therapists would like consuming. Therefore, it is essential that he should
to sell the idea of wheelchair to those patients who be given a chance to be on his feet with appliances.
Spinal Cord Injury 19

However considering the cost of the equipment Once he is able to move comfortably in the parallel
before prescribing them it is advisable to try bars he is brought out first in the walker and later
temporary trial orthosis like Push Knee Splints and with crutches. Initially, he could be given axillary
high boots with posterior steel shank, for a few days crutches and later on elbow crutches. Older patients
and gait training could be given. If he is comfortable prefer walker as it gives much more balance than
with these temporary orthotic devices, a any of the crutches.
prescription could be given for KAFO. Many SCI
cases still prefer these temporary devices, as they Once he gains confidence in moving indoor, he can
are easy to wear and light in weight. The therapist be brought outdoor. Initially he is taught to walk
should give the choice to the patients. Finally the long distances so as to gain confidence and
patient forms his opinion as to which mode of endurance.
ambulation he prefers.
The next step could be to climb stairs. Initially he is
GAIT TRAINING taught to hold onto one side on the railings and the
A gait with orthosis like KAFO and crutches other side on crutch and lift his body up and climb
requires very high energy consumption because the one step and bring the crutch to the standing level
patient has to maintain the balance in a and then lift once again holding the crutch and
PARAPLEGIC STANCE both in the dynamic railing climb another step.
movements and while resting, i.e. keeping the spine Another method is facing the railing and holding it
in a hyper extended position and taking the weight with both hands, swing one limb in one pendulum
on the crutches. Therefore the patients get
cycle and then hike the hip and place the foot on
exhausted after walking a few steps. It is essential
the higher step; then bring the other foot up. This
that the energy consumption should be bare
is possible only if he has strong hip flexors or
minimum.
quadratus lumborum. There is yet another way of
Depending on the patients muscle power, "four climbing up i.e. Jack kniving, Here the patient has
point gait" or ”swing to gait" are the easiest to to lift himself up higher than the height of a step
learn. If he can master these gaits, he can easily
with back facing the staircase and swinging both
negotiate narrow spaces. Wheel chair may not pass
legs backwards so as to reach the higher step. The
through very narrow passages. Swing through gait
person has to use one crutch in one hand and
needs more strength, coordination and are difficult
to learn but once mastered the energy consumption holding the railing with the other hand or using
is less. crutches in both hands. This is possible only for
patients who have very good control of the trunk
Gait training should be started in the parallel bars
as well as very strong Triceps and shoulder muscles.
first. Initially the patient is taught to be in the
paraplegic stance i.e. hip in extension and spine in
OCCUPATIONAL THERAPY
hyper lordotic posture. The therapist stands on the
side and pushes the shoulders backwards and IN PARAPLEGIA
pelvis forward. Optimum position of the ankle is Paraplegia
also important as with hyper dorsiflexion and Paraplegia affects the person’s functional
plantarflexion, one tends to loose balance. High independence and has a major impact on quality
boots with posterior steel shank can maintain ankle of life, sense of self worth and consequential social
mobility in neutral position with minimal ankle participation. Whilst it is generally expected that
mobility required for ambulation. After this, patient the degree of functional independence achievable
is able to balance in standing. He is taught four point is dependent on a person’s SCI level, a person’s
gait pattern which is the easiest to learn. It is easier neurological level should not be viewed as strictly
for patients who have some power in hip flexors. If predictive but rather as indicative of potential
he has no hip flexors, he may use adductors by function.
externally rotating at the hips and dragging his feet
alternatively. The third option is by hiking the pelvis Expected Levels of Functional Independence at
by quadratus lumborum. The other option is by different thoracic and lumbar levels
“swing to or swing through” gait.
20 Neuro-Rehabilitation : A multi disciplinary approach

Level Abilities Expected Levels of Functional Independence


T1 Has added strength and precision Daily tasks: Can live independently without assistive
of fingers that result in limited or devices in feeding, bathing, grooming, oral and facial
natural hand function. hygiene, dressing, bladder management and bowel
management.
Mobility: Uses manual wheelchair. Can transfer
independently.
T2-T6 Has normal motor function in head, Daily tasks: Should be totally independent with all
neck, shoulders, arms, hands and activities.
fingers. Has increased use of rib and
chest muscles, or trunk control. Mobility: A few individuals are capable of limited
walking with extensive bracing. This requires
extremely high energy and puts stress on the upper
body, offering no functional advantage. Can lead to
damage of upper joints.
T7- Has added motor function from Daily tasks: Able to perform unsupported seated
T12 increased abdominal control. activities.
Mobility: Same as above.
Health care: Has improved cough effectiveness.
L1-L5 Has additional return of motor Mobility: Walking can be a viable function, with the
movement in the hips and knees. help of specialized leg and ankle braces. Lower levels
walk with greater ease with the help of assistive
devices.
S1-S5 Depending on level of injury, there Mobility: Increased ability to walk with fewer or no
are various degrees of return of supportive devices.
voluntary bladder, bowel and
sexual functions.

OT in Paraplegia overcomes difficulties successfully with the


The purpose of OT in paraplegia is to use of various aids and adaptive equipment
and modifications in the home and place of
1. Evaluate a person’s ability and level of
work environment. A variety of purposeful
functioning in his/her home, at work, and
activities are also used and modified to
while engaging in leisure activities and
promote self-esteem by highlighting functional
hobbies;
skills and emphasizing the tangible
2. To provide individualized therapy to retrain development of a client’s physical, social,
people to perform daily living skills using emotional, sensory and cognitive abilities.
adaptive techniques; OT’s unique contribution to occupational
3. To facilitate coping skills that could help a performance lies in its use of purposeful
person overcome the effects of SCI. activities to promote psychological and
physical health and maximum functional
Role of the Occupational therapist independence. As a part of complex
• The occupational therapist is particularly rehabilitation, it is applied in solving the
concerned in helping the paraplegic reach the problems of occupation (self-care, work and
highest level of independence both physically leisure) of patients. It is emphasized that early
and psychologically that his injury, home, and OT, started immediately after stabilization of
work environment will allow. The paraplegic patient’s functional state, is of great
with the help of the occupational therapist importance.
Spinal Cord Injury 21

Occupational therapy Evaluation In order to determine the functional status of the


Evaluation is an ongoing process that begins from patient the occupational therapists use the
the day of admission and continues long after following assessment tools:-
discharge on an OPD basis. An accurate and
comprehensive formal evaluation is needed to 1. Functional Independence scale (FIM)
determine basic neurological, clinical and -It assesses six areas of function (Self-care,
functional status of the patient. Medical files Sphincter control, Mobility, Locomotion,
provide demographic data, the diagnosis and Communication and Social cognition), which
present and past history and other required fall under two dimensions (Motor and
information like associated injuries and Cognitive).
complications, fractures, cardiac conditions etc. -It consists of 18 items assessing the 6 areas of
Occupational therapists evaluate function
• Passive and active range of motion (ROM) - 2. Klein Bell ADL Scale
of all joints in the upper and lower extremity
using Goniometry -Measure ADL independence to determine
current status, change in status, & sub activities
• Manual muscle testing- of all muscles using to focus on in rehabilitation
accepted muscle testing techniques and
-Areas assessed are dressing, mobility,
• Sensory evaluation- of all dermatomes of the elimination, bathing & hygiene, eating &
body, including evaluation for light touch, pin emergency communications
prick, joint proprioception, stereognosis, and
kinesthesia, to determine areas of loss or 3. Spinal Cord Injury Functional Ambulation
impaired sensation. It helps in identifying Inventory (SCI-FAI)
functional limitations and in establishing level -Assesses functional walking ability in
of injury. ambulatory individuals with SCI (Field-Fote
et al, 2001).
- It is composed of three sub-scales, these
include:
• Gait
• Assistive Device
• Walking Mobility
4. The Spinal Cord Independence Measure
(SCIM)
-The SCIM, now in its third iteration, is a new
disability scale developed to specifically
address the ability of SCI patients to
accomplish ADLs (Catz et al. 1997).
- 19 items assesses 3 domains:
Self-care (6 items, scores range from 0–20)

MRI of Lumbar Spine Respiration and sphincter management (4


items, scores range from 0–40)
• The other areas assessed are:- Mobility (9 items, scores range from 0–40)
ADLs, Reflexes present or lost, Muscle tone (once
spinal shock subsides), Endurance and perceptual Occupational Therapy Intervention
and cognitive skills in case of a combined SCI and
TBI diagnosis. A work history, home accessibility, The Acute Phase:
social, lifestyle, leisure and driving interests are also When the patient arrives in the hospital he will
considered. mostly be confined to bed waiting for or recovering
22 Neuro-Rehabilitation : A multi disciplinary approach

from surgery to stabilize the spine which may be extremity muscles. As muscle strength
immobilized in traction or in a halo brace or body increases, the amount of resistance should be
jacket and prohibited from flexing, extending, and increased to help the patient increase tolerance
rotating the spine. Occupational therapy begins and endurance. Shoulder exercises should
within the first 48 hours of admission. emphasize the shoulder depressors (latissimus
dorsi), the flexors, abductors, and extensors
After the evaluation a daily range of motion
(deltoids), and the scapular musculature. The
program should be started with active and active-
triceps, pectoralis, and latissimus dorsi are
assisted ROM of all joints within strength, ability,
required for weight shifts in the wheelchair
and tolerance level. Positioning should be evaluated
and for transfers.
and instruction to the staff, patient, and family
members should be given if necessary. Participation
in self care activities (eating, combing, and writing)
should be encouraged. Discussions regarding
further therapy and rehabilitation are initiated to
prepare the patient and the family members for
discharge.

The Rehabilitation Phase:


The Rehabilitation Phase is also known as the Active
or Mobilization phase. In this phase the patient can
sit in a wheelchair therefore upright sitting
tolerance should be developed. The occupational
therapist also works on:-

1. Bed Mobility
Bed mobility skills like rolling, coming to sit
from supine, scooting and sitting at edge of
bed techniques are taught to the patient. The
patient is instructed and assistance is provided
until patient is independent. Weight bearing
while performing therapeutic activities in
different positions like prone on arms,
quadriped and kneeling are initiated to
improve upper extremity and trunk stability,
balance and develop skills for transfers. Fig 3 fig 4

3. Endurance Training
The intervention progamme should be graded
to increase the amount of resistance that can
be tolerated during the activity. As muscle
power and endurance increases, increase the
amount of time in wheelchair activities which
helps patient participate in activities and
occupation throughout the day.

4. Self care retraining-


Self-care retraining may commence whilst
Fig 2
patient is still in bed, focusing on feeding and
grooming. There are many assistive devices/
2. Upper Extremity Strengthening
specialized items of equipment that O.T.
The Occupational therapist uses weights/ prescribes to help you achieve greater
resistance for progressive resistive exercises independence in feeding, grooming,
and resistive activities to strengthen upper
Spinal Cord Injury 23

showering, dressing, and bladder and bowel Fig. 5,6,7,8,9,10


management. Some of the most commonly used pieces of adaptive
• Grooming equipment (AE) used during dressing include:
Grooming tasks include brushing teeth, • Dressing sticks
washing face, combing hair, shaving and • Reachers
applying make-up. As with, upper body
dressing a person with a paraplegia usually • Long-handled shoe horns
has full use of their arms and grooming is • Button hooks
completed without difficulty from a
• Velcro
wheelchair as long as items are in reach.
• Elastic shoe laces
• Feeding
• Sock aids
Feeding, like upper body dressing and
grooming, is usually not difficult for a person • Legs straps
with a paraplegic level of injury.
• Toileting
• Upper body dressing (UBD) Toileting includes the ability to pull down
Upper body dressing (UBD) includes donning clothing in preparation for elimination,
and doffing of clothing from waist upwards. cleaning of the perineal area and pulling
As in paraplegics the upper extremities are clothing up after completion. A person is often
spared so they can independently perform able to independently complete the process
upper body dressing if the clothing’s are in with the correct technique and needed
reach. In absence of independent sitting, equipment.
supported sitting enables the patient to
complete UBD. Examples include:
• Leaning on one elbow to raise a hip and pull
• Lower body dressing (LBD) down clothing from side to side
Lower body dressing (LBD) includes putting Leg straps to assist lifting legs
on and taking off any clothing item from the
• Raised chair with a cut hole
waist down. When dressing the lower body,
persons with a paraplegic level of injury might • A standard-height toilet with a raised toilet
find it helpful to use a combination of seat and a cutout in the front and back to allow
alternative techniques and adaptive an individual or attendant access for digital
equipment. The most common position for stimulation and hygiene purposes
performing LBD is circle sitting or long sitting
• Specially designed seats with attached arms,
in bed. This allows the person to reach his/
if needed.
her feet from a large base of support, which
increases balance. Technique to dress lower • Sufficient area around the toilet to permit
body is as follows: wheelchair transfer
Person sits up in the bed. One leg is bent at the knee • Grab bars at a height to maximize the person's
and lifted over the other, making sure that the leg ability
is not dragging on the bed. Socks can be put in this
position while checking placement of seams to • Bathing
avoid pressure on toes. The process is repeated with The initial days or weeks following injury, will
the other leg. Trousers are put on after the socks to most likely be sponge bath from bed. Once
avoid toes being caught in the seams. The process patient is stable showering is allowed. The
is the same as the wearing of socks. Trousers are following aids assist with safety and
then pulled up as far as possible. Then the person completion of bathing:
lies on the bed and pulls up the trousers by rolling
Shower chair
from one side to the other to pull it over the
buttocks. By sitting up again, shoes can be put in Tub chair/tub bench with a back
the same way. Transfer board
24 Neuro-Rehabilitation : A multi disciplinary approach

Fig. 5 Fig. 6

Fig. 7 Fig. 8

Fig. 9 Fig. 10

Hand held shower device, with or without facilitatory equipment,


Long handled sponge by suggesting the best possible technique in
Grab bars on three sides of the bathroom the best possible position.
Thermometer
Transfers:
Transfer board
For independent transfers in paraplegics, muscle
Hand held shower
strength of upper limbs is very important. Upper
Thermometer Towel racks, soap dishes, and
limbs have to be strong enough to lift and support
shelves within reach
the body while transferring. Transfer board to
Full length mirrors should be installed bridge the gap between your wheelchair and the
wherever possible bed, commode, or bathtub.
Non skid floors
The Two methods commonly used are taught to
Insulated hot water pipes to prevent burns
the patient for independent transfers:
Bowel and bladder training
Forward-on:
Therapists may assist in independent
stimulation and applying a urinary collection The wheelchair is placed at right angles to the bed
and patient lifts the legs onto the surface (bed or
Spinal Cord Injury 25

chair) one at a time. Footrests are removed or • Lift-off


swayed away and wheelchair is moved closer. The
Make sure that patient clears the surface
person moves forward as necessary until buttocks
during lifting to avoid shearing and pressure
are on the surface. Process is reversed to get back
sores.
on the chair.
Mobility
Legs down (side-transfer method):
The International Classification of Function defines
The wheelchair is positioned beside the bed.
mobility as “moving by changing body positions
Footplates are swung away or removed. The person
or locations by transferring from one place to
moves forward on the seat until the feet are flat on
another, by carrying, moving or manipulating
the floor. The nearest armrest is removed, and the
objects, by walking, running or climbing, and by
hand is placed on the farer armrest. And the person
using various forms of transportation.” Individuals
lifts the buttocks off the chair and on the bed or
with paraplegia due to the loss or impairment of
chair. Then legs are repositioned.
motor function in lower extremities are able to move
Some transfer rules and technique:- through use of technology such as wheelchairs,
walkers, orthosis such as leg braces and crutches.
• Transfers best done at same height levels
• Get the patient scoot as close as possible to the Wheelchairs
surface he has to be moved to It is the most primary means of mobility. The
• Lock wheelchair if transfer is from or to a occupational therapist helps the patient in selecting
wheelchair. the most appropriate type of wheelchair (manual
or powered), adjusting it well (wheelchair, armrests
• Get armrest out of the way on the side next to
or casters height/width), training the patient in safe
the surface patient is transferring to.
propulsion techniques and effective use in different
• Transfer using a closed fist with wrist in settings. The Wheelchair Skill Test
neutral for an increase in vertical excursion. ([Link].) can be used to
determine which wheelchair skills are required to
• Depress shoulders for additional height while
be addressed and to document any progress after
transferring.
intervention.
• When transferring, Lean trunk forward, head
Parts of wheelchair and its uses, techniques to
should move in the opposite direction of your
ascend and descend ramps, negotiating curbs and
hips. This is known as a head-hips relationship
steps, taking a turn, transfers from wheelchair to
and can help with movement and clearing
different heights and surfaces and vice- versa,
obstacles.
protecting oneself in case of falls are some of the
• To protect shoulders, position arms as close wheelchair skills the patient needs training in. The
to the body as possible (about 30-45 degrees family members are also educated regarding the
away from your body) while lifting weight. care and use of the wheelchair.

Walkers
Walkers are metal frames designed to provide
support and stability while walking. Walkers may
be folding or fixed; height-adjustable or non-height-
adjustable; equipped with wheels on the front, all
four, or none of the legs; suited for stairs; and/or
equipped with seats

Crutches
There are two basic styles of crutches: traditional
under-arm axillary crutches or forearm crutches,
whichever suits best can be prescribed.
Environmental Barriers, Home and Work (Job/
Fig 11
26 Neuro-Rehabilitation : A multi disciplinary approach

School/Play) Barriers assessment and social and psychological well being. A variety of
recommendations instruments have developed that address the
impact of environmental factors on function for e.g.:
Environmental Barriers, Home and Work Barriers
are physical impediments that keep patients from Craig Handicap assessment & reporting Techniques
functioning optimally in their surroundings. (CHART)- This instrument was developed to
Occupational therapists use the results of tests and document an individual’s functioning with in his
measures to identify variety of possible or her societal context. It examines levels of
impediments including: involvement within six domains of functioning:
physical independence, cognitive independence,
– Safety Hazards (e.g. throw rug, slippery surfaces
mobility, occupation, social integration & economic
for patient with help of lower extremity orthosis &
self-sufficiency. The CHART-sf2 is a 20-item
walker etc)
shortened version of the CHART.
– Access problems (e.g. narrow doors, high
thresholds & steps, absence of elevators) Domestic retraining
– Home & Office design barriers (e.g. excessive As part of occupational therapy program, patients
distances to negotiate, multi storey environments, have the opportunity to practice homemaking
sinks, bathrooms, counters and placement of activities in a simulated or in the environment the
controls or switches) patient will be returning. E.g. cooking in a
wheelchair accessible kitchen, where appropriate
Occupational therapists after identifying the
skills will be taught and opportunities to practice
impediments use the results of tests to suggest
different pieces of equipment that can enhance
modification to the environment to improve
patient level of independence in this area. Other
functioning in the home, workplace and other
domestic skills may also be addressed according to
settings:
your individual need.
• Construction of ramps or lifts to home. A number of products are commercially available
• Railings and grabs around the house to facilitate independence in performing home
management tasks. Examples of these products
• Electrical points fixed at a height accessible to
include:-
patient from wheelchair
Easy reachers, long-handled dustpans, brooms or
• Enlarged doorways and passages for easy vacuum cleaners
access of wheelchairs
Trays or trolleys can be utilised to transport items
• Removal of cabinets from under sinks and or carry hot items to reduce the risk of burns on
platforms in kitchen lower limbs. A front loading washing machine, and
• Removal or rearranging furniture that a lowered clothes line or front loading dryer, can
hampers wheelchair access facilitate independence in laundry tasks.

• Alter thresholds to no more than 3/4” in height Assistance with return to driving
• Position the heights of bed and chair in level For a paraplegic who wants to resume driving, – a
with cushion of wheelchair to ensure easy driving assessment with an Occupational Therapist
transfers. who specializes in driving assessment and
Tests and measures may include those that intervention is usually required. The driving
characterize or quantify:- assessment will determine readiness to resume
driving and recommend modifications that are
• Current & Potential barriers:- e.g. checklists, required to enable safe and functional driving, e.g.
interviews, observations or questionnaires hand controls. O.T. aims to provide comprehensive
• Physical space & environment:- observations, education and retraining to help patient return to
photographic assessments, questionnaires, driving. Transportation options are also addressed
structural specifications, technology assisted (e. g. modified vehicle and public transport). Motor
or videographic assessments vehicle adaptations are selected based on physical
capacity of an individual.
The environment directly impairs patients’s ability
to perform tasks and abilities that support physical, Common car adaptations include:-
Spinal Cord Injury 27

– Hand controls to operate accelerator and brake patients post injury than before injury as it helps to
– Handles to assist in transfers from a maintain the competitive instinct and to help
wheelchair to a car or van, integration back into the community.

– Ramps, Depending on the severity of their injury, they may


have to learn new leisure skills. The therapists
– Wheelchair and transfer lifts
provide counseling about returning to pre-injury
– Raised ceilings, leisure interests, developing new leisure interests
– Lowered floors, and and possible adaptations needed to pursue those
interests. People are also encouraged to identify
– Wheelchair carriers attached to the outside of
benefits of their leisure time and to consider the
the car or van
important part leisure played in their lives before
their injury.

Vocational rehabilitation
Vocational options are discussed with the patient
by the occupational therapist. An occupation is of
varying importance to patients, but most will see it
as giving a sense of purpose to their life and will
want to return to their former work if at all possible.
Early contact with the patient's employer to discuss
the feasibility of an eventual return to his previous
job is important. If the degree of the patient's
disability precludes this some employers are
sympathetic and flexible, and will offer a job that
will be possible from a wheelchair. If appropriate,
a work site assessment may be arranged. An on-
site work task analysis performed at the client’s
Fig 12 workplace as well as examination of the worker and
the work environment will provide the therapist
Leisure skills valuable insight about the present skills of the
Leisure time means free time and excludes time patient. However, many patients-find life outside
spent on essential activities such as paid hospital-difficult enough initially, even without the
employment, chores, eating, sleeping, and going to added responsibility of a job, and in these
school. Leisure activities not only put high emphasis circumstances a period of adjustment at home is
on restful pursuits such as reading, watching TV, advisable before they return to work.
stamp collecting or watching performances, but also If a patient is planning to return to his previous
on more physical activities such as sports like employer, school, or college the occupational
basketball, swimming, table tennis and weight therapist should assess the suitability of the
lifting. Many patients with spinal cord injuries are premises for wheelchair accessibility. Ideally, if a
unemployed and therefore have more leisure time patient is considering returning to work the
available. To maintain their self esteem suitable therapist assists him by assessing his work abilities
hobbies and sporting activities should be in a simulated work environment. In addition the
encouraged. patient will build up his strength and stamina and
Together with the patient the O.T., investigates both he and the staff will have a clearer idea of his
options for returning to previous leisure interests, employment capabilities. The patient should be
and also developing new pursuits. Recreation and taught proper body mechanisms and energy
leisure activities assist people to not only pursue conservation to perform work in a safe manner. The
their individual talents, abilities and interests but occupational therapists provides referrals for a
also develop important and valuable social number of services to provide vocational
networks and relationships in the broader counselling, rehabilitation and assistance with
community. finding employment in the community.
Leisure activities are more important for the
28 Neuro-Rehabilitation : A multi disciplinary approach

REHABILITATION OF PATIENTS 1) PT management at the site of injury.


WITH LESIONS OF CERVICAL 2) PT during the acute stage ( intensive care)
SPINE (TETRAPLEGICS) 3) PT in the sub-acute stage( outside intensive
Tetraplegia refers to partial or complete paralysis care, in the wards)
of all four extremities and trunk including the 4) Active rehab/sports
respiratory muscles; it results from lesion of the
5) PT at home and in the community
cervical cord.
In India approximately 15 lakh (1.5 million) people 1) PT management at the site of injury
are living with SCI. Approximately, 10,000 new Move the patient with great care to avoid
cases each year; about 8,000 are persons with bending of neck/back. Have another person
paraplegia, and 2,000 persons with tetraplegia. put the stretcher in place. If the neck is injured
82.5% are males, age group 16-30. or broken, put sand bags or tightly folded
clothing or boxes that are taped on sides of
Causes head to keep head/neck from moving. If there
1. Traumatic causes. is more help the 10 steps should be followed
• Road traffic accidents, • Application of steady but gentle manual
• Fall from height (tree, train, buildings, traction to the head and neck and airway
govinda) opened by modified jaw thrust.
• Fall of objects on spine (buildings, • Placing a rigid collar around the patient’s neck.
earthquake, mining, load taken on head
upper back, working under vehicles) • Board is placed parallel to the patient
• Diving • Kneel at patients side, opposite the board,
• Violence leaving room to roll patient towards you, one
at the shoulder, one at the waist, one at the
• Sports
knee, one maintains traction.
2. Non Traumatic causes • Log roll to side, as one unit.
• Transverse myelitis • Waist level person grips spine board and pulls
• Syringomyelia into position against the patient.
• Multiple sclerosis • Roll the patient onto the board
• Amyotropic lateral sclerosis
• The patients head is secured to board with
• Pott’s spine cravat or 3” tape. Place folded clothing besides
• Neoplasms head and neck for additional protection, these
• Disc prolapses. must be secured by a cravat over forehead and
then tied to the sides of the board.
C 5 is the most common injury level, followed by
C4 then C 6. • Additional 3 straps, one across chest, one hips,
and one across the knees are put, traction is
Percentage of injuries by ASIA classification maintained on the neck.
• Incomplete Tetraplegia 34.5 • Move patient and board on wheeled
• Complete Tetraplegia 18.4 % ambulance stretcher.
• Incomplete Paraplegia 17.5 % This safe lifting at site, on the ambulance, or at times
• Complete Paraplegia 18.4 % onto a plane and then to a hospital which has
expertise in treating people with spinal cord injuries
PHYSIOTHERAPY MANAGEMENT will ensure that no further neurological damage
OF TETRAPLEGIA occurs. With good handling, an incomplete injury
will not progress to become a complete injury.
Physiotherapy (PT) which plays a very vital role in When it is a high cervical spine injury, even the
the rehabilitation and well-being of persons with diaphragm, besides the intercostal muscles would
spinal cord injuries can be broadly discussed as be affected, and breathing is severely affected. Thus
Spinal Cord Injury 29

an ambulance with well equipped artificial FIGURE 1.1-2


respiratory kits would be needed. Assessment tools for quadriplegia are the same as
The physiotherapist can be part of this emergency we use in evaluating paraplegia. i.e. ASIA Scale,
evacuation team, or get involved in training Modified Ashworth Scale, etc. besides these, there
personnel that safe lifting techniques of the person are various other measures of assessment which are
injured, and those lifting are adopted. This will described below.
prevent neurological damage to the person injured, The neurological level is the most caudal
and will prevent musculo-skeletal damage to those neurological segment of the spinal cord that retains
who are lifting. normal sensory and motor function on both sides
of the body. Sensory and motor levels may vary
2) Physiotherapy in the acute stage from side to side and even on a given side.
From the time the patient is admitted in
4 levels, sensory and motor, both right side and left
intensive care, physiotherapy is begun for
side are examined. 10 myotomes and 28
a) Chest care dermatomes are checked.
b) Maintaining joint mobility The 5 key muscles in upper limb and 5 key muscles
c) Posture in lower limb are

d) Pressure Relief Upper limb:

Other assessment includes – C5 elbow flexors

• Level of arousal – C6 Wrist extensors

• Cranial nerves assessment – C7 Elbow extensors

• Associated injuries like head injury, chest – C8 Finger flexors (distal phalanx of
wall injury, fractures of bones, neuro- middle finger )
vascular injury. – T1 Finger abductor, little finger.
• The type of respiration and chest mobility Lower limb:
• Ability to cough – L2 Hip flexors
• The nature of the injury – L3 Knee extensors
• Occupation of the person – L4 Ankle dorsiflexors
The acute phase is very critical as complications can – L5 Long toe extensors
occur which we should be very much aware of
– S1 Ankle plantar flexors.
1. Pneumonia, atelectasis
Grading is done from 0 to 5 strength, total score 25
2. Deep vein thrombosis (DVT for each limb,50 for upper limbs, 50 for lower limbs,
3. Pressure sores total 100 for key muscles of upper and lower limbs.

4. Urinary tract infections(UTI) 28 dermatomes from C2 to S4/S5 are examined for


pin prick and light touch
5. Autonomic dysreflexia
– 0 absent sensation
6. Burns due to sensory loss
– 1 impaired sensation
7. Heterotropic ossification
– 2 normal sensation.
8. Osteoporosis
Thus a total score of 56 each for pin prick and 56
9. Orthostatic hypotension for light touch, total 112 for each limb.

Assessment of Respiratory system


• Diaphragmatic movement
• Chest wall movement
30 Neuro-Rehabilitation : A multi disciplinary approach

Deep Vein Thrombosis Heterotopic Ossification

Osteoporosis Pressure Sore Diagram

Pressure Sore – Stage 1 Pressure Sore – Stage 2

Pressure Sore – Stage 3 Pressure Sore – Stage 4 Pressure Sore – Stage 5


Spinal Cord Injury 31

• Paradoxical movement lateral and posterior aspects of the chest wall.


• Use of accessory muscles (sternocleido- Abdominal support given whilst coughing and
mastoid, scalenes, trapezius) huffing can also help.
• Rate and depth of respiration Exercises for Sternocleidomastoid, trapezius,levator
• Chest expansion measurements, at axilla, scapulae,platysma are given as they help to increase
at nipple level and at xiphisternum AP diameter and tidal volume.
• Breath sounds (air entry and added Glossopharyngeal breathing
sounds) a. The mouth and throat are filled with air, the
• Assessment of ability to cough tongue, jaw and larynx are depressed.
b. The lips are closed and the soft palate is raised
Assessment of intugementary system to trap the air.
Occiput, scapulae, elbows, spinous process, sacrum, c. The larynx is opened, the jaw and floor of the
coccyx, ischial tuberosities, greater trochanters, mouth and larynx are then raised.
lateral and medial femoral condyles, lateral and
d. With repeated motion of the tongue air is
medial malleoli andheels are vulnerable to develop
forced through the opened larynx in the
pressure sores.
trachea.
These areas have a greater potential for breakdown, e. The larynx is closed and air is trapped in the
due to sustained loading in one posture, trachea and lungs.
compounded by sensory loss makes these areas
f. 14-48 strokes/min, can supplement air for
vulnerable.
effective coughing and secretion clearance.
Poor nutrition, smoking, swelling, incontinence and
lack of pressure relief mechanisms are potential Summed breathing
causes for skin breakdown. This may increase tidal volume. It is carried out by
In acute stage, chest physiotherapy is very vital. In encouraging patient to take several quick shallow
first 3 months post SCI, death occurs mainly due to but cumulative breaths before expiration.
pulmonary complications.
THE SUB-ACUTE STAGE
Paralysis of trunk and extremity muscles causes
immobility and due to abdominal muscle weakness, (IN THE WARD)
coughing is severely affected. BED MOBILITY
Loss of innervations of intercostal muscles and 1) Rolling
decreased diaphragm movement may result in
Steps:
decreased lung volumes.
• Gaining momentum by swinging upper limbs
Sympathetic cardiac innervation is lost making side to side.
postural changes more precarious. There is
• Turning the head in direction of the
decreased vital capacity, total lung capacity,
movement, interlocking arms if weak triceps
expiratory reserve volume, and FEV1.
in one arm.
Use of accessory muscles for respiration is • Inhaling with extension, exhaling with flexion
increased. wraps for the extremities, a G suit and helps.
Mas trousers may be used to minimize orthostatic
• Bending hip, knee and internal rotation helps,
hypotension until vasomotor control is established.
crossing legs over also helps.
Due to reduced vital capacity, there is decreased • Weights cuffs (2-3lbs), bed rails and loops at
deep breathing and inability to cough, inability to the side of the bed would help.
change position which are the causes for the
• Air splints may hold the patients elbow
retention of secretions, atelactasis and pneumonia.
straight as he/ she swings arms.
Assisted breathing exercises and coughing can help
in the prevention of these complications. • For log rolling, wedges and pillows can be
used.
Stryker frame is used for positioning and turning.
• Also draw sheet rolling starting from side-
Percussions and vibrations are given to the anterior, lying can also be done.
32 Neuro-Rehabilitation : A multi disciplinary approach

4) Prone on Elbows

Steps:
• Hands near shoulders, elbows close to the
trunk, push of elbows on the mat and lifting
upper trunk.
• Final position
a. either patient shifts weight from side to
side moving elbows up under the
shoulders.
Rolling done in Group Therapy b. pushes entire body backwards until
elbows are under the shoulders.
2) Supine on elbows
SITTING STATIC
Steps:
Long leg sitting on the bed is graded as follows:
• Roll to one side and come up on bottom elbow.
As rolling is done back to supine position, 1 Poor minus, requires maximum assistance to
quickly extend the top arm and place the elbow maintain static position
under the shoulder. Shift weight onto the 2 Poor requires moderate assistance to maintain
extremity and position the other elbow under static position
the shoulder, good speed and timing are 3 Poor+ requires minimal assistance to maintain
requisite for this. static position
• Place hands under their hips or hook thumbs 4 Fair minus, requires contact guard to maintain
into pockets. By using wrist extensors or their static position
biceps to pull half way up. Then as they shift
5 Fair maintains static position with closed
their weight from side to side they are able to
supervision(< 2 minutes)
reposition their elbows under their shoulders.
6 Fair+ maintains static position with closed
• Momentum of forcefully flexing arms and
supervision,> 2 minutes.
head to curl forward and then quickly throw
elbow back behind the shoulders. 7 Good minus, maintains static position against
minimal resistance.
3) Supine/prone to long sitting 8 Good maintains static position against
moderate resistance.
Steps:
9 Good+ maintains static position against
• From supine on elbows, rocking from side to
maximal resistance.
side attempting to build momentum, throw
one arm behind and immediately shift weight
DYNAMIC BALANCE
onto this extended arm.
1) Poor minus : requires maximal assistance to
• Next throw the other arm behind and walk up move on either sides, unable to move
on extended arms to a sitting position ( can be voluntarily from the midline.
maintained by pectoralis muscles with
shoulder placed in extension and external 2) Poor: able to move through 25-50 % range,
rotation). requires maximal assistance to return.
3) Poor plus : able to move through 50 % range,
• If prone on elbows used, curled into C-curve,
requires maximal assistance to return.
one arm is hooked under knee, pull with this
arm, other arm flinged behind, locking the 4) Fair minus : able to move through 50-75 %
elbow into extension. range, requires contact guard to return.

• The first arm is then thrown behind and 5) Fair: able to move through 75 % range with
patient walks with the extended arms the rest contact guard or 50-75% with closed
of the way up. supervision
Spinal Cord Injury 33

6) Fair plus : able to move through full range FIGURE4


with contact guard in all directions. 4) Tilt back in a manual wheel-chair with
7) Good minus: independent in functional assistance, or in a powered chair, hold for a
dynamic balance activities. minute.
8) Good: independent in functional dynamic 5) If there is strength in triceps, do push- ups in
balance activities. wheel-chair, holding the tyres, or arm rests and
sustain the push-up for one minute. To wear
9) Good plus: independent in high level dynamic
mittens to prevent injury to hand and
balance activities.
callosities.
TRANSFERS Along with pressure relieving maneuvers a good
Transfers/ transitions is the manner in which you cushion, mattress will aid in pressure relief, and
move from one place to another, in bed, from bed prevent skin breakdown.
to wheel-chair, from bed to furniture, wheel-chair Cushions come in different forms-
to commode, wheelchair to car/vehicle.
a) contoured foam
For person with tetraplegia, sliding board transfers
b) contoured foam with gel filled pad.
should be done first, antero-posterior transfers
should be tried, so also lateral transfers, a wheel- c) Viscoelastic foam with casing.
chair with removable arm rest is useful for the
d) Polyurethane foam.
sideways transfer.
e) Air filled villous roho cushion
Where the arm strength is very weak to do a push-
up lift, assistance through sling, by one person or f) Water filled seat cushion
two persons assist is done. Mechanical and g) Low cost cardboard cushion.
mechanized hoist also are safe lifting and transfer
FIGURE5
devices.
DEEP BREATHING EXERCISES
Goal is to strengthen diaphragm, intercostals if
innervated, or accessory muscles for a deeper
inhalation or to focus on intercostals if inervated
for a more forceful exhalation needed for a
productive cough.
Diaphragm position for maximum contraction-
Patient is supine with a pillow, and posterior pelvic
tilt, with arms resting at the sides in shoulder
adduction and internal rotation will slacken the
accessory muscles and enhance diaphragmatic
function. Patient is told to take deep breath from
the belly, and to relax the shoulders, also therapist
can provide firm manual pressure to the shoulders
Lifters in the inferior direction. Stretch just before
inspiration as in costophrenic cough technique.
Weight relieving maneuvers Weights can be used on the abdomen, but we
1) Lifting leg through a thigh loop, holding this should get correct motor performance, and
position for half to one minute. discourage accessory muscle breathing. Later
diaphragmatic activities in other functional
2) Slump over feet, hooking handle or holding positions used.
onto loops, sustain for half to one minute.
2) Leaning onto sides, holding arm rest of wheel- Strengthening the accessory muscles
chair, or removing arm rest and leaning on cot, The patient is instructed to take a deep breath, while
sustain position for half to one minute. raising his shoulders and contracting the innervated
34 Neuro-Rehabilitation : A multi disciplinary approach

Weight relieving Maneuver -1 Weight relieving Maneuver-2 Weight relieving Maneuver-3

Weight relieving Maneuver-4 Weight relieving Maneuver-5

Weight relieving Maneuver-6

Cushion-1 Cushion-2
Spinal Cord Injury 35

muscles in the neck and pectoral regions. Position i. The therapist places the palmar surface of
further facilitated by not using a pillow, shoulders hands along the costophrenic angles of the rib
in abduction and external rotation, and anterior cage with fingers laterally spread in the
pelvic tilt, mirror can be used to give feedback. direction of the ribs.
Therapist can apply manual pressure at exhalation
ii. Quick stretch is given downwards and
to inhibit diaphragmatic contraction. Prone on
inwards on exhalation.
elbows is a diaphragmatic inhibiting position that
allows the patient to better use accessory muscles. iii. This quick stretch can facilitate an improved
contraction of the diaphragm and the
Glossopharyngeal breathing intercostals muscles.
If only accessory muscles used, it is tiring. Patient iv. After inspiration hold air and instruct to
should be taught glossopharyngeal breathing, also cough.
called frog breathing or air stacking. Muscles of the
v. Quickly apply a lateral and inferior manual
tongue, soft palate, pharynx and larynx work
force to enhance the force of the patients
together to create a pumping action that forces
exhaled air flow.
gulps of air into the trachea and lungs. Patient traps
air in a pocket of negative pressure within their vi. Best position is side-lying.
mouths, which allows them to maximize that space,
pulling in more air, then closing their lips, and Anterior chest compression
forcing the air back and down the throat with It is modification of the costophrenic assist, which
stroking maneuvers of the tongue, pharynx and facilitates the upper chest rather than the lower.
larynx. More than 60 % of patients with high SCI
i. The therapist places one hand or entire
who are unable to breathe without the ventilator,
forearm across the chest wall and the other
can achieve autonomous breathing for hours or
across the lower chest wall.
even all day with this technique.
ii. As the patient coughs, the lower hand or
Assisted Cough Techniques forearm still compresses to assist exhalation
• Heimlich assisted cough while the upper hand stabilizes and
• Costophrenic cough compresses the upper chest as well.
• Anterior chest compression Counter-rotation Assisted Cough
• Tetraplegia long-sit assist It is also called Massery’s Counter rotation assisted
• Prone on elbows, self assist cough technique.
i. The counter-rotation assisted cough
Heimlich assisted cough compresses the thorax in 3 planes for a
It is also called abdominal thrust assist. maximal exhalation.
i. Therapist places the heel of one hand just ii. It is performed in side-lying, for patients where
proximal to the patient’s navel and the xiphoid there is no contraindication for spinal rotation.
process. iii. When patient is left side-lying with 45 degrees
ii. Instruct the patient to take deep breath or air of hip flexion, therapist kneels behind the
stack with hold for several seconds. patient diagonally facing patient’s shoulder.

iii. Instruct the patient to cough, therapist iv. The therapist’s left hand should be on patient’s
simultaneously applies an anterior and right scapula and right hand on patient’s ASIS.
superior force to increase the expiratory effort v. Patient is instructed to take a deep breath,
of the patient. therapist pushes the upper thorax superiorly
and anteriorly with the left hand and pulls the
iv. Also can be done in sitting in a wheel-chair or
pelvis inferiorly and posteriorly, with the right
when in a postural drainage position,
hand the therapist pulls the upper chest
including supine or side lying ( with one hand
inferiorly and posteriorly with the left hand
on the posterior thorax for stability)
and simultaneously pushes the gluteal region
Costophrenic Assisted Cough (for those who cannot superiorly and anteriorly with the right,
tolerate Heimlich assisted cough) patient is told to cough.
36 Neuro-Rehabilitation : A multi disciplinary approach

vi. This maneuver functions in the same manner 2) Do not do hot water fomentation (burns)
as innervated intercostal muscles. 3) Do not lie on hard bed, sit on hard chair
without cushion (sores)
Tetraplegia long sitting self-assist
4) Do not stay immobile in one position for too
i. This is performed in long-sitting position or
long (sores, swelling, stiffness)
with legs externally rotated and flexed.
5) Do not sit for too long without support, (back
ii. The patient is instructed to take deep breath
pain)
with cues to extend neck, scapular retraction,
then instructed to cough while bringing head 6) Do not lie too curled up, in a flexed position
forwards to assist with upper chest (contractures and deformities)
compression. 7) Do not sit with legs dangling (swelling and
iii. The short-sitting self assist is performed with contractures)
the patient sitting in a wheel-chair or on side 8) Do not put on weight ( mobility severly
of bed or mat. hampered)
iv. Same process as above, hands interlocked, 9) Do not change position abruptly, (postural
forearms in the Heimlich position press hypotension)
inward and upward on abdomen to assist with
10) If using axillary crutches, do not hang on them
forced exhalation.
(neuropraxia)
v. Patient can try it in prone position if it is not
11) Do not use improper aids (neck, back pain)
contraindicated.
vi. Also in quadruped position, but requires more 12) Do not wear ill fitting orthosis /worn out
balance and motor control. rubber tips, (abrasions, risk of falls)

vii. Instructed to rock forwards extending his head 13) Do not stay in sun too long (autonomic
and trunk as he inhales. dysrefexia)

viii. Then rocks back, bringing hips posterior and 14) Do not smoke, consume alcohol (decreased
inferior and flexing trunk as he` coughs. cardio-respiratory endurance)
15) Do not travel without proper footwear, socks,
Role of Surgery ( Tendon transfers) wounds, burns.
1) Biceps to triceps transfer for elbow extension. 16) Do not cut down on your water intake.
2) Brachioradialis to radial wrist extensor with 17) Do not seek advise from those who are not
FPL tenodesis. ( Mobergs tenodesis) professionals.
3) Brachioradialis to flexor pollicis longus for
DO’S FOR PERSONS WITH SCI
active pinch.
1) Exercise daily, put the joints, muscles under
4) Radial wrist extensor to flexor digitorum active control through full range of motion, 20
profundus.\ repetitions twice a day. ( upper limbs can help
5) Transfer of posterior deltoid to triceps the lower limb, stronger. Upper limb may
assist the weaker limb, or assistive devices are
Above transfers help in increasing pinch force,
used. Do warm up slowly and after exercise
improve hand function, and activities of daily
cool down, do gentle stretches.
living, eliminates need of multiple adaptive
equipments, restores reachable work space with the 2) If unwell do gentle ROM.
biceps transfer, eliminates reliance on shoulder 3) Do deep breathing exercises, twice daily, also
external rotation and locking of the elbow joint, huffing and coughing.
improves proximal control. In incomplete
tetraplegia may help in holding walker/crutches. 4) Do lie with a good posture, sit with an erect
posture.
DON’TS FOR PERSON WITH SCI 5) Do strengthen the whole body, head, neck,
1) Do not exercise vigorously or jerkily ( increase trunk, upper and lower limbs (whatever
of spasticity, myositis ossificans, sprains, possible)
fractures.
Spinal Cord Injury 37

6) Inspect body parts twice daily, particularly 5) Maintain lordosis while lifting.
bony prominences using a long handled
6) Get close to the person and object they are
mirror.
lifting.
7) Do lie on soft, firm (not sagging) bed, do use
7) Keep a broad base while lifting.
soft cushion when you sit
8) Do dynamic bracing (lower abdomen
8) Do sit up/ stand slowly and lower yourself.
contracted with a pull upwards and towards
9) Do push-ups in prone, sitting, and if possible the sides of the waist).
in standing.
9) Avoid jerky movements, leg movements
10) Do roll on your sides every two hourly. should be fluid.
11) Do weight shifting and change of position 10) Lift in the direction of the movement.
frequently, every 15 minutes for 15 seconds to
When we lift a person (with help), we lift ½ of
1minute.
persons weight and 70% of our own body weight (
12) Do activities that improve trunk balance and HAT head, arms and trunk),multiplied by the
co-ordination, especially of the trunk. distance from the body, multiplied by the degrees
of bending( at 45 degrees multiplied by 5), also if
13) Do improve protective extension of arms in
rotation in the lift, load on the spine doubles.
all positions.
If weight 20 cms from the body,
14) Do your shifting in bed, chair and transfers
moment at the lumbar spine 60 Nm.
smoothly, with adequate clearance, lift.
If weight 40 cms from the body,
15) Do inspect aids, daily and see that they are in
Moment at Lumbar spine is 80 Nm.
proper condition.
Bending moment with spine straight,
16) Do go out of the house daily/ weekly by self hips and knees bend 151Nm.
or with help. If trunk balance is poor see that
straps are fastened when on W/C. Bending with spine bend,
knees straight 192.5 Nm.
17) Do be in the morning sun, in the house or
Bending with spine bend,
outside (15mins to 30 mins), avoid afternoon
hips and knees bend, load away 212.5 Nm.
sun.
With trunk flexed, knees straight, force on LS
18) Take part in sports, ball games. Exercise
junction goes up to 750 kg. Thus bending at hips
individually, also in group sessions.
and knees with a straight spine creates an air
19) Wear mittens when propelling a W/C. cushion in the abdomino-thoracic cavity unloading
20) Do seek advise from professionals only. the spine.

Lifting Principles and Musculo-Skeletal Care Nachemsons chart:


The objective of any lift done with and for a patient • supine lying - 25,
is to change his or her position or place with ease • standing - 100,
and comfort to the person being lifted and the lifter, • sitting with back rest - 150,
alone or with the help of other persons, with or • sitting leaning forward - 175,
without adaptations. The therapist should:
• knees straight - 200.
1) Let patients participate as much as they can,
Bracing, lateral, antero-posterior rocking facilitate
during the lift and shift.
shifting and lifting, Gaining momentum aids the
2) Arch backwards few times before lifting. lift. Safe position of lifter/s and the person being
3) Before lifting, bend hip, knee and ankle joints lifted is very important. The choice of the lift, how
few times. many persons would be involved, who is holding
where, the direction of movement, clear instructions
4) Work over bend hip, knee and ankles, and commands should be given., the start of the
vertebral column should be bending as little movement, the transit and the lowering should be
as possible. smooth.
38 Neuro-Rehabilitation : A multi disciplinary approach

Aids to lifting/ shifting. Ramps


Grab rails, overhead bars, bed-ladder, draw sheet,
push-up blocks, transfer boards, slings, belts, loops,
manual or mechanized hoist.

TYPES OF LIFTS

Through arm lift.

Ramp

For wheel chair users, the incline/ slope gradient


is a very important consideration. For every inch
of vertical rise there should be 12-14 inches of
length.
This gradient is possible for average riders and
strong persons with quadriplegia. This is the best
slope for public buildings and rehab centres.
Lifter
There should be width of 36 inches to 48 inches (
For patients who slump in sitting, a lifter alone and 91.4 -121.9 cms), continuous bilateral hand rails
for moving up in bed, into and out of bath. with height of 34 -38 inches situated 1.5 cms (3.8
Orthodox lift. cms) from the wall, with circular cross section of
1.25 -2 inches (3.2 -5.1 cms), with hand railings
Only should be used when a patient may not have extending 12 inches (30.5 cms) beyond the top and
pressure on the axilla or chest wall, as following bottom of the ramp.
mastectomy or when totally helpless.
There should be a non-skid surface and for a long
Half-shoulder/ half orthodox lift. and curved ramp there must be a level platform at
10 feet intervals.
When it is impossible because of patients condition
to use shoulder lift on one side, then it may be If for each inch of vertical rise, there is length of 10
necessary to use the orthodox lift on that side, while inches, it is a fairly steep slope and possible for
the shoulder lift is used on the opposite side (could riders with strong arms and person with paraplegia
be avoided, an oblique lift) who are strong.
If only 6 inches length for each inch of vertical rise,
Grasps
it is a very steep slope, only possible for those using
Finger grasp, double wrist grasp, double forearm a powered chair or with help, rarely possible for
grasp, single wrist grasp, relaxed hand grasp, rider alone, chair may tip backwards.
through arm grasp, axillary grasp,, elbow grasp,
waistband grasp, buttock grasp, forearm grasp, Orthosis
palm-to-palm thumb grasp. • Hard Cervical Collar (Thomas’s Collar)
Stances for lifting and supporting • Philadelphia Collar
Walk standing • Sterno-Occipital Mandib
Lunge standing. • Ular Immobilzer(SOMI),
Stride standing. • Poster Type Cervicothoracic Orthosis
Ten to two standing. • Minerva Cervicothoracic Orthosis
Spinal Cord Injury 39

• Cuirass Type Cervico-Thoracic Orthosis Weight >23 kg for person weighing 114 kg, seat
• Yale Orthosis width 20”

• Halo-Vest Orthosis Standard 18-23 kg, Lightweight 9-18 kg, Ultra light
weight (sports wheel-chair) 7-9 kg.
• Opponens Orthosis
• Opponens Orthosis With Lumbrical Bar Measurements of wheelchair
• Wrist Control Orthosis,To Assisting Tenodesis 1) Back height distance from buttocks to level of
• Universal Cuff scapulae.

• Tailors Brace 2) Arm rest distance from buttocks to olecranon,


elbows flexed 90 degrees.
3) Seat depth distance from back of buttocks to
popliteal fold, add 1-2 inches.
4) Seat height distance from heel to popliteal fold,
add 2 inches to provide clearance of foot rests
when approaching slope, ramps.
5) Seat width widest distance across hips, add1-
2 inches for clothing, splints

Types of Wheelchairs:
• Conventional wheelchair, non folding, fixed
arm rests, small castors.
Philadelphia Collar
• Folding, removable arm rests, flipawayor
Wheel-chairs elevating foot rests,
Wheelchair should be appropriate for the patients • Semireclining wheel-chair.
needs. Trial should be done indoors and outdoors • Reclining wheel-chair
in its safe use by patient and care-givers. Patient
should learn skills of maneuvering the wheel-chair, • Stand-up wheel-chair
manual for persons with paraplegia, with • Manual wheel-chair with add on power unit.
projections on outer rim, wheel or a powered chair • Electric wheel-chairs
for person with tetraplegia. Patient should learn
some mode of transfer to and from the wheel-chair, • Tricycles
and if possible learn assembly, disassembly, and • Electric carts.
maintenance. (Lubrication, and charging batteries
• Adapted vehicles.
of powered chairs)

Wheelchairs
40 Neuro-Rehabilitation : A multi disciplinary approach

Ambulation 6) Between catheterizations do not drink more


The physiotherapist should be involved with than what your bladder will hold(<400cc).
training skills to gain independence through wheel- 7) A daily intake of 2 to 2.5 litres of fluid is
chair and later introduce ambulation if a person sufficient.
with incomplete tetraplegia wishes to do so.
8) Avoid drinking at bedtime, unless, patient or
Standing and weight-bearing have to be given to care-giver is going to catheterize.
all whether injury is partial or complete, to prevent
9) Bladder stimulation by suprapubic jabbing
osteoporosis, prevent bladder and bowel
may help, by pressing deep over the bladder
complications, elevate their spirits and mood.
to mechanically stretch its wall, rather than
Let the person with tetraplegia(incomplete) decide suprapubic tapping,or stroking or pinching the
whether ambulation is practical or not, tilt-table perineal skin.
standing is done gradually.
10) Transurethral electrical bladder stimulation
Gait training should begin between parallel bars activates mechanoreceptor afferents which
and with a mirror in front for postural reeducation. restore the sensation of bladder filling and in
Walking is begun in parallel bars with, KAFO’s and turn activate efferent nerves, resulting in
abdominal binder/spinal brace, later one bar and detrusor contraction.
one crutch, later bilateral crutches, if possible. 11) Valsalva manouvere- increase intravescical
A modified walker, forearm gutter walker, a walker pressure by increasing intra abdominal
without wheels, or a wheeled-walker can be used. pressure. It involves sitting and resting
abdomen forward on the thighs for both men
Walking with HKAFO and spinal braces increases and women. During straining, hugging of the
the energy cost of walking and is only possible for knees may prevent bulge of abdomen,.All of
young athletic persons, for functional needs the increase in intra abdominal pressure is
wheelchair becomes more practical. transferred to the bladder and pelvic floor. (in
If possible we should teach the skills of wearing patients with vescicoureteral reflux, it is contra
and removing the appliances, standing from bed, indicated. adverse effects may also include
lowering to bed from standing, walking forwards, hemorrhoids, rectal prolapse, or hernia)._
sideways, backwards, going up and down small 12) Crede’s maneuvers increase intravescical
gradients, if possible stair climbing, and falling and pressure by manually pushing down on the
rising. bladder.
Bladder management 13) In the open hand credes maneuver, the thumb
of each hand is placed over the left and right
1) During the acute phase over distension of the
bladder is prevented by an indwelling ASIS and the digits over the suprapubic area
catheter. with slight overlapping of the tips. The slightly
overlapped digits are then pressed into the
2) After 2-4 weeks, intermittent catheterization abdomen. When well behind the symphysis,
may be tried. Proper aseptic precautions the pressure is directed downward to
should be followed, it needs to be done 4-6 compress the fundus of the bladder. Both
times a day. For those who have finger control hands are then pressed as deeply as possible
and power ( C7)are able to do self down ward into the pelvic cavity.
catherisation, also C6 level quadriplegics may
be able to do with tenodesis grip. 14) In the closed-hand Crede’s method,
compression of the bladder is done by using
3) When the injury is above T12 level, there is closed fist or a rolled-up towel.
spastic or reflex bladder, and below T12/L1,
there is flaccid/non-reflex bladder. 15) Kegels exercises can be done in women.
4) To avoid problems, we should not allow more a. Females can use diapers,
than 400 cc of urine to fill in the bladder at any b. men can use condom catheters.
one point.
16) In those where there are complications of
5) A voiding diary should be kept, a diary of
bladder stones, hematuria, bactermia, penile
water intake versus urine output.
Spinal Cord Injury 41

and scrotal fistulas, uretheral stricture, ambulation with modified walkers/crutches, and
uretheral diverticulum, bladder carcinoma, lower limb orthosis, with abdominal binder, and if
suprapubic catherization is preferrable. possible outdoor walking skills.
17) Whenever there is urinary infection, Besides physically helping, supporting the person
appropriate antibiotics are given along with with tetraplegia to gain functional independence
plenty of citrus fruits. in attaining motor skills, therapist cheers, boosts the
morale, raises the spirit, infuses positivity in the
Bowel Care person with tetraplegia, their families and the care-
1) Have a well-balanced fibre rich diet, with givers. The Physiotherapists should strive hard for
roughage. whole grain cereals, fruits with skin, giving them independence in ADLs like bed
green leafy vegetables. mobility skills, transfers, wheel chair mobility,
bladder and bowel management rather than
2) Drink plenty of fluids.
stressing on ambulation, which is more time and
3) Stay active. energy consuming.
4) Stick to a scheduled bowel program
OCCUPATIONAL THERAPY IN
5) Automatic bowel responds to a suppository
QUADRIPLEGIA
or stimulation by a finger..
6) Preferably sit on a toilet seat/commode for 15 OT in Quadriplegia
minutes after taking suppository, if you Quadriplegia is defined as the partial or complete
cannot, insert it while lying on your left side, paralysis of all four limbs and trunk including
use a gloved lubricated finger, push respiratory muscles, as a result of damage to
suppository 2 cm in anus, drink a cup of hot cervical spine.
water, tea/coffee/lime juice or do after food,
as gastro-colic reflex helps bowel movement/ Occupational therapy assessment
peristalsis. Regardless of where the patient begins in the
rehabilitation process, an assessment is always
7) Sitting time should not exceed 30 minutes,
completed on admission. The assessment will help
massing the abdomen from right to left and
in establishing a diagnosis as well as determining
down several times helps.
the most appropriate therapeutic intervention.
8) If suppositories do not help then only do
digital stimulation. History Taking
In summary, a physiotherapist’s role is very vital The first step involves review of medical record to
in the total well-being of person with tetraplegia, gather background information and identify
right from the site of injury, in the intensive care medical precautions. The history should include
unit, in the step down unit, in the home setting, demographic data, social history, occupational &
and the community by optimizing functional skills. leisure history, past and present medical & surgical
The therapist is cognizant and prevents negative history, associated conditions, social habits and
effects of cardio-respiratory de-conditioning, allergies. Even though most of the information is
prevents pneumonia, atelectasis, deep vein already noted in the medical file, asking the patient
thrombosis, pressure sores, contractures and about his hobbies, work or family often opens
deformities, renal calculi and osteoporosis, and communication and helps establish a rapport
postural hypotension through graduated between the therapist and the patient. Therapists
verticalisation and weight-bearing. Therapist later conduct a Qual-OT assessment to identify which
on strengthens all the innervated muscles, keeps areas of potential quality of life are most important
up the mobility in the joints where active movement to the patient.
is not possible, enhances bed mobility skills, and
transfers with/without assistive devices, improves Physical Status
dynamic sitting balance, wheel-chair skills for Before evaluating the physical status of the patient,
indoors and outdoors and also for getting in and the therapist should first obtain specific medical
out of vehicle,, weight relieving maneuvers, in precautions from the physicians. The physical
persons with incomplete tetraplegia household status includes:
42 Neuro-Rehabilitation : A multi disciplinary approach

• Muscle tone & Deep tendon Reflexes common hand grips. The test consists of 20
Muscle tone should be evaluated with activities of daily living.
reference to the quality, muscles groups
involved and any factors that appear to • Cognitive and perceptual evaluation
increase or decrease tone. It is evaluated using If a head injury is also suspected, then the
the modified Ashworth Scale. Deep tendon assessment also includes the patient's ability
reflexes evaluation is also done. The most to initiate tasks, follow directions, carry over
common examined are the biceps, triceps, learning day to day, and do problem solving.
extensor carpi radialis longus, quadriceps and • Clinical observation is used to evaluate
gastrocnemius. posture & trunk control, endurance, lower
limb functional strength and total body
• Range of Motion function and more specific evaluation may be
Range of motion evaluation includes required depending on the individual.
measurements of all extremity joints and all
digits. It should be measured before muscle Functional Status
testing to determine available pain–free range. A detailed functional evaluation is usually not
It helps to identify if there are any contractures carried out until the patient is in the active or
or potential to develop the same. In cases of rehabilitative phase where the patient will be
spinal instability caution should be used while medically stable. In the acute phase a functional
performing any movement. evaluation includes performing light activities of
daily living (ADLs) such as feeding, light hygiene
• Joint Integrity & mobility and object manipulation, to determine present and
The therapist should check for any soft tissue potential levels of functional ability and should
swelling, inflammation or restriction. Also begin as soon as the patient is cleared of bedrest
joint hyper or hypo mobility should be precautions, depending on the level of injury.
checked for and noted. During the rehabilitation phase performances in the
following areas are assessed:-
• Muscle strength
Manual muscle testing includes testing of the ADLs
muscles of the scapula, shoulder, elbow, wrist, ADLs are the basic tasks performed on a daily basis
and digits, as well as grip and pinch strength in order to engage in daily routine. It includes
measurements. bathing, toileting, dressing, getting in and out of
bed or a chair, hygiene, and eating skills.
• Sensation Occupational therapy scales like FIM, Spinal Cord
Sensory evaluation of all dermatomes of the Independence Measure (SCIM) & Quadriplegic
upper body includes evaluation for light Index of Function (QIF) evaluate different areas of
touch, pin prick, joint proprioception, function. The QIF assesses 10 ADL's:
stereognosis, and kinesthesia. Sensation is
1. Transfers
indicated as intact, impaired or absent per
dermatome. This helps in establishing the level 2. Grooming
of injury and to determine functional 3. Bathing
limitations. Using a dermatomal map aids in 4. Dressing
easy documentation. 5. Feeding
• Wrist and Hand function 6. Mobility
A wrist and hand function evaluation 7. Bed activities
determines the degree to which the patient can 8. Bladder program
manipulate objects. This information helps the 9. Bowel program
therapist to suggest the required splints (e.g. 10. Personal care
tenodesis splint) which will aid hand function.
Sollerman Hand Test is a standardised hand The QIF was specifically designed for SCI patients
function test based on seven of the eight most and is focused on persons with tetraplegia
Spinal Cord Injury 43

Environmental Barriers – Instructing others in care for pressure


Barriers present in the patients home and work reliefs, skin precautions, upper extremity
environment can be identified using questionnaires ROM exercises, equipment setup and
or interviews. Also photographs or video recording maintenance, positioning, etc.
of the particular environment aid in identifying – Independently propelling a power
barriers. wheelchair with portable respirator and
Other evaluations include assessment of chin or breath control on hard, level
performance in areas such as: surfaces
1. Vocation – Operating communication devices,
2. Access to home and community computers and environmental control
systems electronic page turners, with
3. Leisure time activities
head pointer, mouthstick or pneumatic
4. Driving control
5. Appropriateness of treatment and
– Leisure activities, such as computer and
equipment.
electronic games, reading and painting
Goals – Vocational skills involving use of a
Once the patient is evaluated, goal planning is computer
started. Goal planning is based on patient
• Total paralysis of the trunk, UEs and LEs
involvement as an active participant and not
dictate:
therapist-led practice, with the recognition and the
utilization of the patient’s strengths to meet – Total ADL and respiratory dependence
identified needs significant to the patient in regard – Full-time attendant care
to his perception of his spinal cord injury. Goals
are then set within the rehabilitation team with C4
specific, measurable and realistic targets to be • Innervated diaphragm(C3-5), trapezius,
achieved in an agreed time. Regular review and cervical and paraspinal muscles permit
monitoring success is necessary. respiration, scapular elevation, and neck
movements, which permit:
Some general goals of occupational therapy
intervention are: – Limited self-feeding with a long straw
• Maintain/achieve full ROM in all joints and straw holder and externally powered
flexor hinge splints and mobile arm
• Prevent deformities
supports
• Increase strength of innervated or partially
– Propelling power wheelchair on uneven
innervated musculature and increase physical
terrain using head, mouth, chin, breath
endurance
or sip-and-puff controls
• Achieve maximal level of functional
independence – Note taking in school or business with an
adapted tape recorder
• To explore leisure interests and vocational
potential – Writing/art using pencil attached to
mouthstick
• To train the patient in use and care of necessary
equipment. – Using a mobile with speaker phone and
mouthstick
Functional Expectations
• Paralysis of trunk, UEs and LEs dictate:
The following are the anticipated functional
abilities and limitations that a patient with – Full-time wheelchair use
quadriplegia may achieve at each level:- – Part- or full-time respiratory assistance
– Full-time attendant care
C1 - 3
• Innervated facial and neck muscles permit C5
chewing, swallowing, talking, blowing, and • Innervated shoulder muscles, biceps,
neck control, which permit: brachialis, brachioradialis, supinator,
44 Neuro-Rehabilitation : A multi disciplinary approach

infraspinatus, rhomboids and deltoid permit C6


shoulder external rotation, abduction to 90° • Innervation of the pectoralis major(C5-8,T1),
and limited flexion; elbow flexion and serratus anterior(C5-7), lattisimus dorsi(C6-8),
supination; and gravity assisted shoulder pronator teres(C6,7), and Extensor carpi
adduction, pronation, and internal rotation, radialis longus and brevis(C6-7) permit
which permit: strength for all shoulder movements, elbow
– Self-feeding with a mobile arm support flexion, forearm pronation and supination, and
or suspension sling, dorsal wrist splint radial wrist extension (tenodesis grasp), which
with u-cuff, and other devices such as a permit:
plate guard or scoop dish, stabilized cup – Use of a tenodesis splint or universal cuff
or cup holder, straw with holder and an for increased ease and independence
angled spoon or fork
– Self-feeding with a cup with large
– Limited Upper Extremity dressing and handles, a rocker or sharp paring knife
fully dependent in lower extremity for cutting, and does not need straws and
dressing plate guard
– Self-grooming, with a wash mitt and a – Grooming, including teeth brushing,
quad grip long handle sponge/ makeup application, and hair care, with
hairbrush/toothbrush, to aid in face tenodesis grasp and/or modified
washing, upper extremity bathing, hair equipment
brushing, teeth brushing, with moderate – Bathing, with a shower/tub bench, faucet
assistance within easy reach, and hand-held shower
– Maximum assistance with lower hose
extremity bathing – Typing, telephoning, turning appliances
– Upper extremity ROM with maximum and equipment on and off
assistance – Independent skin inspection and
– Propelling hand-controlled power pressure reliefs when sitting using loops
wheelchair over obstacles with assistance, and forward weight shift
and manual wheelchair with projection – Assist in bowel and bladder care,
knobs on level surfaces for short distances
– Dressing of upper extremity and pants in
– Pressure relief with power recline or tilt bed (using momentum and substitute
wheelchair with elbow or head switches movements to turn over, sit up and pull
– Typing stick in dorsal wrist splint with up clothing) or button hook and zipper
u-cuff for operating computers and long pull, with maximum assistance required
writing orthosis for writing, turning book for shoes and socks
pages using a book holder stick in dorsal – Upper extremity ROM
wrist splint with u-cuff.
– Propelling manual wheelchair, with
– Driving a van with Hand controls friction material or rims on uneven
• Absence of elbow extension and pronation, all surfaces for short or moderate length
wrist and hand movements, total paralysis of distances; moderate assistance needed for
trunk and LEs and Low endurance and low architectural obstacles
respiratory reserve result in: – Independent transfers with a transfer
– Inability to roll over or come to sitting board and partial depression or swivel
without hospital bed with rails transfer
– Assistance needed for transfers, skin – Rolling and moving from supine to long-
inspection, bowel and bladder sitting with loops suspended over the bed
management or by grasping the bedrail
– Atleast part-time attendant care – Driving using hand controls with U-
shaped Cuff attached to steering wheel
Spinal Cord Injury 45

– Light kitchen activities in a wheelchair- Acute Phase


accessible kitchen Intervention Immediately after the initial
– Use of specially Adapted electronic evaluation:
machines in offices 1. A daily range of motion program should be
– Participating in leisure activities like started, with gentle, assisted, active
watching TV using the remote, listening movements given to all muscles. Progression
to radio and playing adapted table top is made to unassisted active exercises by
games or sports, such as quad rugby, encouraging the patient to move his arms
swimming independently and functionally within
strength, ability, and tolerance levels. Basic
• Absence of wrist flexion and total paralysis of
reasons for range of motion exercises include
the trunk and LEs result in the need for a part-
a) prevention of contractures, b) prevention
time attendant for:
of joint pain caused by contractures, c)
– Some assistance with dressing prevention of joint deformities, and d) mobility
of the joints.
– Moderate assistance for negotiating
obstacles in a wheelchair and positioning 2. Total body positioning should be evaluated
and instruction to the staff, patient, and family
– Maximum assistance with heavy work
members should be given if necessary.
C7
• In supine,
• Innervation of the triceps, extensor carpi
The patient's shoulders should be externally
ulnaris(C6-8), and flexor carpi radialis, flexor
rotated and abducted to 90°, elbows flexed,
digitorum superficialis and profundus(C7-8,
forearms supinated, and a pillow placed under
T1) permit elbow extension, wrist flexion and
the forearms. The upper extremities should be
extension and PIP & DIP flexion which permit:
intermittently positioned in 80° of shoulder
– Self-care same as C6 except easier abduction, external rotation with scapular
dressing, bathing/grooming, and depression, and full elbow extension to
bladder/bowel management alleviate pain in the shoulders and ROM
– Propelling manual wheelchair over limitations. In patients with injuries at the C5
inclines and in/out of elevators, with level, the forearm should be positioned in
some supervision for rough terrain and pronation to prevent supination contractures
some assistance for doors
• In side lying:
– Independent pressure reliefs with push A pillow should be placed in a vertical position
ups under the thoracic region of the trunk. The
– Independent transfers to and from bed upper extremity under the patient should be
and wheelchair placed in 100° of shoulder flexion. The elbow
should be flexed or extended with the forearm
– Independent in Bed mobility, with
in supination. The upper extremity on top of
minimal assistance with padding and
the patient should be placed in pillows in front
positioning
of the patient
– Participating in preparation for sexual
3. The occupational therapist may also provide
activity
the patient with hand splints to maintain
– Driving a modified car proper hand positioning and prevent
– All communication, recreational, and contractures and joint deformities. Dorsal,
vocational activities for C6, but with rather than ventral splints should be used to
greater ease allow maximum sensation when the hand is
resting on a surface.
• Lack of trunk muscles result in lack of full
shoulder stability, weak trunk control, reduced • If there is inadequate musculature to support
endurance due to low respiratory reserve, and wrist and hands properly for function and/or
paralysis of the lower extremities. cosmesis, the wrist should be supported in
46 Neuro-Rehabilitation : A multi disciplinary approach

extension, with the thumb in opposition. This patient’s strength. Shoulder and scapular muscles
will maintain the thumb web space and allow are exercised for increasing proximal support. Wrist
the fingers to flex naturally exercises should emphasize the extensors to
maximize natural tenodesis function for functional
• If there is at least F+(3+) strength of wrist
grasp and release. Assistive devices prescribed to
extension, short opponens splints should be
enable efficient performance should be cost
used to maintain the web space and support
effective and not bulky also. Use of assistive devices
the thumb in opposition
should be kept to a minimum with an emphasis on
4. Facilitation of a tenodesis grasp which modified techniques.
involves fingers flexion while wrist is
maintained in an extended position and
extension of fingers while wrist is maintained
in a flexed position should be begun during
range of motion of the hand.
5. The patient should be provided with a basic
environmental control, such as a television,
telephone, and nurse call system, based on the
patient's capabilities and needs, to promote the
patient's sense of self-control and
independence
6. Education of the patient is begun, including
the importance of skin management, pressure
relief, and daily ROM
Fig 1 : Patient using suspension for active assisted
7. Education of the family is begun, including exercises
discussion of anticipated medical equipment,
home modifications, and caregiver training
should be initiated
8. As the patient becomes medically stable, gentle
resisted movements can be gradually
introduced as indicated. Strong unilateral
exercise for the whole arm involves head
movement and is therefore completely
avoided till spine is completely stabilized. All
movements must be given with carefully
graded resistance, avoiding any neck
movements. Gentle static neck exercises are
Fig 2 Facilitation of tenodesis action. Courtesy
given 6 weeks post-injury if there are no
Electronic Textbook of surgery
contraindications. ADL training should be
begun, particularly for patients who are on ADLs
prolonged bed rest, followed by transfer to a
Activities of daily living (ADL) program can be
wheelchair and training to tolerate an upright
expanded to include independent feeding, oral
sitting position.
hygiene, and upper body bathing and dressing,
Active Phase with or without devices. Bowel and bladder care,
such as independent stimulation and applying a
In the active phase emphasis is on maximizing urinary collection device, can be carried out with
functional independence. Passive and active Range or without facilitory equipment, using the best
of motion and strengthening the innervated
possible technique in the best possible position.
musculature using resistive activities and building
Transfers using a sliding board are taught to the
endurance should be continued. The use of weights, patient. Communicating skills can be worked upon
pulley systems, skateboards, suspension slings, and via writing & use of the telephone, tape recorder,
mobile arm supports are used depending on the stereo, and personal computer.
Spinal Cord Injury 47

Fig 3 Patient using U-cuff for eating Fig 4 Use of U-cuff for combing hair &
brushing teeth

Fig 5 Fig 7

Fig 7 Fig 8
Fig 5, 6, 7 & 8 Patient performing dressing of upper extremity independently

Mobility Orthosis
Wheelchair mobility provides high-level Splints are usually required to maintain position,
quadriplegic persons with one of their most correct a contracture or to encourage function, and
achievable functional activities and also allows can be made from several materials, e.g. plaster of
them to regain some control over the environment. Paris, synthetic plaster materials, thermo-plastics
When prescribing any wheelchair, it is important or neoprene. Some patients require two splints: one
to consider the patient's living situation, educational may need to be worn at night to maintain hand
and vocational potential, transportation, and position and another during the day to aid
maintenance. The occupational therapist has the independence. Some examples of the ways in which
primary role of informing the patient and the family splints may be used:
of options, costs, maintenance record, and
• Long paddle splint (from forearm to finger tips
transportability of the wheelchair.
with the fingers slightly flexed) for a patient
48 Neuro-Rehabilitation : A multi disciplinary approach

with a lesion at C4 or above to maintain the • Select and learn to use any assistive equipment
hand in a good position and give some control they may need to enter or re-enter the work
over the arm. force or engage in another method of
productivity.
• Wrist extension splint for a patient with a
lesion at C5 to inhibit lengthening of the • Develop personal supports, such as peer-to-
extensor tendons and allow the hand to be peer mentor programs and appropriate
used with simple gadgets. interpersonal advocacy
• Identify potential work, educational, or other
• A patient with a lesion at C6 with wrist
community environments where the person
extensors less than grade 3, as for C5 above
can be productive;
but may also need tapes over the fingers to
hold them in finger flexion to encourage the • Identify and implement the necessary assistive
tenodesis position. equipment, environmental modifications, task
restructuring, task modification, use of
• A knuckle duster splint to inhibit
coworkers, students, or other members of the
metacarpophalangeal hyperextension for a
self-directed, productive group as personal
patient with a lesion at C7.
assistants, etc and
• A splint to encourage opposition of the thumb
• Develop modified hand movements and
may be needed by a patient with an incomplete
assistive devices to compensate for lost hand
lesion or one at C7.
function
Leisure
Psychological support:
Participation in Leisure activities is necessary as a
The therapist provides psychological support by
means of self-expression, release, and socialization
allowing and encouraging the patient to express
for the people with tetraplegia.
frustration, anger, fears, and concerns. The therapist
• Time should be taken out for leisure activities also identifies and addresses each patient's
like viewing TV, listening to music, playing psychological problems, such as denial, apathy,
cards, table games, computer-assisted depression, etc. Then she identifies and emphasizes
programs, reading materials, outings to the on the patient's strengths and skills. Group therapy
movies, theatre, sports events, sightseeing with similar level of patients or forming of support
tours, museums and concerts, shopping, groups is also encouraged.
restaurants, and clubs etc.
Adaptive Equipment:
Vocational rehabilitation Following are some of the adaptive equipment
The likelihood of a person with tetraplegia being suitable to help people with tetraplegia:-
able to work is strongly dependent upon hand
Dressing and Grooming: Dressing sticks, zipper
function. Tetraplegics without hand function have
pulls and d-rings, Sock aid, Button hooker, Dressing
less than 2% likelihood of being employed. The
ladder, Velcro fasteners, Loops in clothes, Long
recovery of hand function will increase the
handle sponges and bath mitts, enlarged handles
likelihood of a person working by more than
for toothbrushes, combs, razors and hairbrushes
fourfold. Prior education also enhances the
and Long handled mirrors.
likelihood of a person being able to work. (Spinal
Cord Injury: Functional Outcomes in 2009 and Eating: Spoons, forks & knives with special handles,
Beyond Harry M. Koslowski, MD). Vocational Plate guards, long straws, Universal cuffs to hold
therapy begins with a comprehensive vocational spoons, forks, or knives
evaluation to determine a person's basic skills, Writing: Special holders for pens & pencils, Special
including their dexterity and other physical pointing tools/mouthsticks to type on a keyboard
capabilities, as well as their cognitive capabilities.
Bathing: rolling shower chair, grab bars, soap on a
The evaluation process also includes a component
string, lever type taps
that determines changes in physical and cognitive
capabilities and interests over time. Once the Cooking: Mirror over stove to view into vessel from
evaluation is complete, the occupational specialist the wheelchair. Special tools to open jars like
helps the person to: reachers, universal cuff, Tenodesis splint
Spinal Cord Injury 49

Environmental Control:-This technology allows of elevated blood pressure. SCI patients, caregivers,
patients to do things like turn on lights, open doors, and medical professionals must be knowledgeable
answer the phone, and control the temperature. about this syndrome and its management.
Many of these technologies are “hands-free,”
allowing the patient to control his or her Epidemiology
environment by using puffs of air, voice, head Reported prevalence rates vary for Autonomic
movement, or even blinking. In addition, the home Dysreflexia (AD) in the United States, but the
can generally be outfitted so that it operates off of generally accepted rate is 48-90% of all individuals
one remote control unit who are injured at T6 and above. Some incidence
Driving: hand controls that allow braking and has been reported in SCI as low as T10.
acceleration, easy-touch pads for ignition and The occurrence of AD increases as the patient
shifting, and joysticks and spinner knobs. evolves out of spinal shock. With the return of sacral
Other Adaptive Equipment: Prism glasses, reflexes, the possibility of AD increases. AD occurs
Mouthsticks, Telephone holders or a speaker phone. during labor in approximately two thirds of
Special electronic devices for hands-free control of pregnant women with SCI above the level of T6.
phones and to turn on radios, TVs, lamps, etc The male-to-female ratio for sustaining SCI is 4:1;
Fig 9 - 17 therefore, autonomic dysreflexia is primarily a male
phenomenon.
Care giver Training
An important aspect in rehabilitation is educating Etiology
the patient and the caregivers in proper handling, Autonomic dysreflexia (AD) occurs after the phase
transferring and pressure relieving techniques. of spinal shock in which reflexes return. Individuals
Energy conservation and joint protection methods with injury above the major splanchnic outflow may
are also taught to prevent pain related to overuse. develop AD. Below the injury, intact peripheral
sensory nerves transmit impulses that ascend in the
Complications Following Spinal Cord Injury: spinothalamic and posterior columns to stimulate
Some of the common complications following SCI sympathetic neurons located in the
are : intermediolateral gray matter of the spinal cord. The
• Autonomic dysreflexia inhibitory outflow above the SCI from cerebral
vasomotor centers is increased, but it is unable to
• Deep vein thrombosis
pass below the block of the SCI.
• Heterotopic ossification
This large sympathetic outflow causes release of
• Neuropathic pain various neurotransmitters (norepinephrine,
• Osteoporosis dopamine-b-hydroxylase, dopamine), causing
• Spasticity/contractures piloerection, skin pallor, and severe
• Pressure ulcers vasoconstriction in arterial vasculature. The result
is sudden elevation in blood pressure and
1. AUTONOMIC DYSREFLEXIA vasodilation above the level of injury. Patients
commonly have a headache caused by vasodilation
Autonomic dysreflexia (AD) is a syndrome of
of pain-sensitive intracranial vessels.
massive imbalanced reflex sympathetic discharge
occurring in patients with spinal cord injury (SCI) Vasomotor brainstem reflexes attempt to lower
above the splanchnic sympathetic outflow (T5-T6). blood pressure by increasing parasympathetic
Anthony Bowlby first recognized this syndrome in stimulation to the heart through the vagus nerve to
1890 when he described profuse sweating and cause compensatory bradycardia. The fact that this
erythematous rash of the head and neck initiated reflex action cannot compensate for severe
by bladder catheterization in an 18-year-old patient vasoconstriction is explained by the Poiseuille
with SCI. Guttmann and Whitteridge completed a formula, which demonstrates that pressure in a tube
full description of the syndrome in 1947. is affected to the fourth power by a change in radius
(vasoconstriction); the pressure is affected only
This condition represents a medical emergency, so
linearly by a change in flow rate (bradycardia).
recognizing and treating the earliest signs and
Parasympathetic nerves prevail above the level of
symptoms efficiently can avoid dangerous sequelae
50 Neuro-Rehabilitation : A multi disciplinary approach

Fig 9: Use of Velcro instead of buttons for clothes Fig 10: Enlarged handles of eating tools
for ease in grasping

Fig 11 Use of mouthstick in operating a computer Fig 12 Different types of Reachers

Fig 13 Use of a page turner to promote Fig 14 Example of Adaptive Seating in


independence in reading bathroom & toilet

Fig 15 A example of an adaptive Fig 16 A example of a Wheelchair Fig 17 A example of a modified


bathroom accessible kitchen car with hand controls
Spinal Cord Injury 51

injury, which may be characterized by profuse • Other


sweating and vasodilation with skin flushing. – Heterotopic ossification ("Myositis
There can be many stimuli that cause autonomic ossificans", "Heterotopic bone")
dysreflexia. Anything that would have been – Acute abdominal conditions (gastric
painful, uncomfortable or physically irritating ulcer, colitis, peritonitis)
before the injury may cause autonomic dysreflexia – Skeletal fractures
after the injury.
In general, noxious stimuli (irritants, things which History and Physical Examination
would ordinarily cause pain) to areas of body below History
the level of spinal injury. Things to consider include:
The patient with autonomic dysreflexia (AD)
• Bladder (most common) - from overstretch or generally gives a history of blurry vision,
irritation of bladder wall headaches, and a sense of anxiety. Feelings of
– Urinary tract infection apprehension or anxiety over an impending
– Urinary retention physical problem commonly are exhibited.
– Blocked catheter
Physical Examination
– Overfilled collection bag
A patient with AD may have 1 or more of the
– Non-compliance with intermittent following findings on physical examination:
catheterization program
• Sudden, significant rise in systolic and diastolic
• Bowel - over distention or irritation blood pressure
– Constipation / impaction
• Profuse sweating above the level of lesion -
– Distention during bowel program (digital Especially in the face, neck, and shoulders;
stimulation) rarely occurs below the level of the lesion
– Hemorrhoids or anal fissures because of sympathetic cholinergic activity
– Infection or irritation (eg. appendicitis) • Goose bumps above, or possibly below, the
• Skin-related Disorders level of the lesion
– Any direct irritant below the level of • Flushing of the skin above the level of the
injury (eg. - prolonged pressure by object lesion - Especially in the face, neck, and
in shoe or chair, cut, bruise, abrasion) shoulders; this is a frequent symptom
– Pressure sores (decubitus ulcer) • Blurred vision
– Ingrown toenails
• Spots in the patient's visual field
– Burns (eg. - sunburn, burns from using
hot water) • Nasal congestion – A common symptom
– Tight or restrictive clothing or pressure With regard to the first item above, the sudden rise
to skin from sitting on wrinkled clothing in blood pressure in AD is usually associated with
bradycardia. Normal systolic blood pressure for SCI
• Sexual Activity
above T6 is 90-110 mm Hg; blood pressure 20-40
– Over stimulation during sexual activity mm Hg above the reference range for such patients
[stimuli to the pelvic region which would may be a sign of AD. However, patients with AD
ordinarily be painful if sensation were may display no symptoms, despite elevated blood
present] pressure. Differentials for AD include essential
– Menstrual cramps hypertension and pheochromocytoma.
– Labor and delivery
52 Neuro-Rehabilitation : A multi disciplinary approach

PATHOPHYSIOLOGY
Patient exposure to noxious stimuli (pain or pressure below the level of injury)

Signal sent to autonomic nervous system via the sympathetic pathway

Exaggerated Reflex ( vasoconstriction and hypertension)

In normal cases In spinal cord injury patients

Baroreceptors send Baroreceptor activity is interrupted


signal to brainstem
No compensatory mechanism
Vasodilatation
Elevated Blood pressure and heart rate
Slowing of heart rate

Prevention Treatment of Autonomic Dysreflexia:


The following are precautions you can take which 1. Recognise the symptom and remove the
may prevent episodes: precipitating stimuli. Check the BP.
• Frequent pressure relief in bed/chair 2. Check urinary blockage. Look for the kinks.
• Avoidance of sun burn/scalds (avoid 3. Flush the catheter.
overexposure, use of #15 or greater sunscreen,
watch water temperatures) 4. If on intermittent catheter, do catheterization.
• Maintain a regular bowel program. 5. Consider medical management. If systolic BP
• Well balanced diet and adequate fluid intake remains above 150mm.

• Compliance with medications 6. Check if there is a fecal impaction in rectum.


• Persons at risk and those close to them should N.B.: Teaching the patient and the caretaker
be educated in the causes, signs and about this complication is essential.
symptoms, first aid, and prevention of
autonomic dysreflexia. 2. DEEP VEIN THROMBOSIS
• If you have an indwelling catheter: Deep Vein Thrombosis (DVT) is a common
complication of acute spinal cord injury (SCI) but
Keep the tubing free of kinks.
also major cause of morbidity and mortality.
• Keep the drainage bags empty Morbidities from DVT include thromboembolism,
• Check daily for grits (deposits) inside of the prolonged edema, and pressure ulcers.
catheter.
Definition
• If you are on an intermittent catheterization
program, catheterize yourself as often as Deep vein thrombosis (DVT) is a condition in which
necessary to prevent overfilling. a blood clot (thrombus) forms in one or more of the
deep veins in the body, usually in the legs. Deep
• If you have spontaneous voiding, make sure
vein thrombosis can cause leg pain, but often
you have an adequate output.
without any symptoms.
• Carry an intermittent catheter kit when you
are away from home. There are changes in the normal neurologic control
of the blood vessels that can result in occlusion of
• Perform routine skin assessments
Spinal Cord Injury 53

the normal flow. DVT in the lower leg is almost anticoagulants, whenever possible. While they
universal during the early phases of recovery and don't break up existing blood clots, they can
rehabilitation. Thromboses in the thigh, however, prevent clots from getting bigger or reduce the
are a great concern, as they are at risk for becoming risk of developing additional clots.
dislodged and passing through the vessels to the
As soon as the DVT is suspected in SCI
lungs. A major obstruction of the arteries leading
infusion of the blood thinner like heparin is
to the lung can potentially be fatal.
given for a few days. After the course of
heparin injections, oral medicines like warfarin
INCIDENCE
are usually given. Oral blood thinners are
The incidence of DVT following acute spinal cord required for at least for a minimum period of
injury has been reported more than 20%. How ever 3 months. However, these blood thinners have
the clinical symptoms are seen in only 15% of serious side effects like risk of bleeding
patients with acute spinal cord injury, and especially when the dose is too high, how ever
pulmonary edema develops in approximately 5% if the dose is too low there can be chances of
of these patients. The risk of DVT is highest within getting additional blood clots. Therefore it is
the first 2 weeks following injury, with peak necessary to get blood clotting time bleeding
occurrence between 7 and 10 days. DVT has been time done periodically and the physician has
detected as early as 72 hours post injury; however, to adjust the optimum dosage. In the event of
risk before this time appears to be low. a Paraplegic getting a life threatening
complication like Pulmonary embolism, the
CAUSES physician may give other medications like
1. Inactivity after a spinal cord injury tissue plasminogen( TPA )
2. Absence of pumping action in the leg • In cases where one cannot be given blood
musculature due to paralysis. thinners, Filters are inserted in the venacava
3. Trauma which prevents clots that break loose from
entering the lung.
SYMPTOMS • Compression stockings. These help preventing
– Swelling in the affected calf muscle in the swelling that is associated with DVT. These
affected leg, including swelling in ankle and stockings are worn on the leg from foot to knee.
foot. They are to be worn for a period of at least
– Pain in the leg mainly in ankle and foot. The one year.
pain often starts in the calf and can feel like
cramping. However majority of paraplegics do 3. HETEROTOPIC OSSIFICATION
not have normal sensation and therefore one
has to look for other symptoms to diagnose INTRODUCTION:
and treat the case immediately. Heterotopic ossification following spinal cord
injury (SCI) was first described by Dejerine and
– Warmth over the affected area.
Ceillier in 1918 as para osteoarthropathy. The
– Changes in the skin colour, such as turning ossification process involves the formation of
pale, red or blue. mature lamellar bone, which is indistinguishable
from normal bone, in soft tissues surrounding
TREATMENT paralyzed joints. The bone is not connected to
The goal of deep vein thrombosis treatment periosteum and becomes encapsulated as it
matures.
• Preventing blood clot from getting any bigger.
• Preventing the clot from dislodging and The pathology is similar to that of fracture callus,
causing a pulmonary embolism except that bone forms in the connective tissue
between the muscle planes (histologic findings in
• Reducing the chances of recurrence of DVT. neurogenic heterotopic ossification are similar to
Deep vein thrombosis treatment options include: those in healing fracture callus). Heterotopic
ossification is also seen after other neurologic
• Blood thinners. Medications used to treat deep
insults, such as traumatic brain injury (TBI) and
vein thrombosis include the use of
54 Neuro-Rehabilitation : A multi disciplinary approach

stroke, as well as after thermal injuries and masses with a cartilaginous consistency;
orthopedic procedures (eg, total hip replacement). within 4-7 weeks, a solid mass of bone can be
felt. Common sites include the pectoralis
In experimental models of heterotopic ossification
major, the biceps, and thigh muscles. a
formation, ischemia and tissue expression of bone
nontraumatic type of myositis ossificans also
morphogenic proteins have been shown to play
may exist.
important roles. Bone morphogenic proteins likely
act on mesenchymal stem cells present in tissue, 3. NEUROGENIC HETEROTOPIC
activating the cells to differentiate into osteoblasts. OSSIFICATION - this condition is the one
that comes to mind when the generic phrase
INCIDENCE: heterotopic ossification is used. This type of
The incidence of heterotopic ossification in spinal heterotopic ossification is the subject of this..
cord injury is between 16% and 53%, depending the various terms mentioned at the outset all
on the incidence reports from various institutions. refer to this type of heterotopic ossification.
Once present, neurogenic heterotopic ossification
is clinically significant in 18-27% of cases. PATHOPHYSIOLOGY OF HETEROTOPIC
Fortunately, only 3-5% of cases involve joint OSSIFICATION
ankylosis. The mechanism underlying heterotopic ossification
There is no known race or sex predilection for following spinal cord injury is not fully understood
neurogenic heterotopic ossification; however, the but it appears to be initiated by mesenchymal cells
incidence of neurogenic heterotopic ossification into bone precursor cells. It has been noted that
after spinal cord injury is lower in pediatric patients mesenchymal stem cells can differentiate into
than in adults, ranging from 3% to 10%. In addition, osteogenic cells given the right stimuli within the
spontaneous resorption of the neurogenic right environment, even soft tissues. These
heterotopic ossification is frequently seen in mesenchymal stem cells can generate cartilage,
pediatric patients. bone, muscles, tendons, ligaments or fat and are
thought to play a pivotal role in the development
CLASSIFICATION OF HETEROTOPIC of Heterotopic Ossification. Heterotopic
OSSIFICATION Ossification then forms through a typical process
beginning with the formation of a protein mixture
HETEROTOPIC OSSIFICATION can be classified
created by bone cells (osteoid) that eventually
into the following 3 types:
calcifies within a matter of weeks. Over the next
1. MYOSITIS OSSIFICANS PROGRESSIVA few months, the calcified osteoid remodels and
(FIBRODYSPLASIA OSSIFICANS matures into well-organized trabecular bone.
PROGRESSIVA) - this disorder is among the Months following the initial trauma patients
rarest genetic conditions, with an incidence of develop bone formation in muscle and soft tissues
1 case per 2 million persons. transmission is adjacent to a joint (paraarticular) with resultant
autosomal dominant with variable expression. restriction in range of motion, pain and ankylosis.
the condition is characterized by (a) recurrent, The bony lesion has a high metabolic rate, adding
painful soft-tissue swelling that leads to new bone at more than three times the rate of
heterotopic ossification and (b) congenital normal bone. Osteoclastic (bone removal cell)
malformation of the great toe. there is no density is more than twice that found in healthy
treatment for this form of heterotopic bone. It is suspected there may be a neurogenic
ossification. limited benefits have been factor contributing to Heterotopic Ossification but
reported using corticosteroids and etidronate. the mechanism is poorly understood.
most patients die early from restricted lung
disease and pneumonia; however some CAUSES:
patients live productive lives. No one is quite sure what causes heterotopic bone
formation. Many reports propose it is related to a
2. TRAUMATIC MYOSITIS OSSIFICANS - in
type of mesenchymal metaplasia, meaning that
this condition, a painful area develops in
connective tissue cells change their characteristics
muscle or soft tissue following a single blow
into bone forming cells. It is not known why the
to the area, a muscle tear, or repeated minor
cells change function but it is thought to be some
trauma. The painful area gradually develops
type of inflammatory reaction.
Spinal Cord Injury 55

In individuals with spinal cord injury, heterotopic The swelling usually is localized more than it is in
ossification will usually begin forming in the thrombophlebitis, and within several days, a more
intramuscular connective tissue within four months circumscribed, firmer mass is palpable within the
of the injury. When the reaction begins, there will edematous area.
be a deposition of calcium phosphate in the
If the mass is adjacent to a joint, gradual loss of
intramuscular tissue. However, ossification occurs
passive range of motion may follow.
only when the calcified material goes on to form
hydroxyapatite crystals. Once bone formation has With the development of early heterotopic
occurred, it rarely disappears spontaneously ossification at the hip or knee, effusion may be noted
at the knee.
HISTORY
The onset of heterotopic ossification usually is 1-4 MANAGEMENT
months after injury in SCI patients, although it may
occur in earlier weeks or as late as 1 year following MEDICAL MANAGEMENT
injury. The condition may occur later with other In the later stages of the development of mature
precipitating circumstances (eg, fracture, surgery, bone, medical treatment is ineffective. Etidronate
severe systemic illness). (Didronel) is the only available medication for the
treatment of HO after SCI. Treatment with NSAIDs
Commonly, incidental heterotopic ossification that
may be required initially, until the resolution of
was not noted clinically may be detected much later
inflammation and the normalization of CRP levels.
on radiographs.
HO always occurs below the level of injury in SCI PHYSICAL REHABILITATION
patients. The use of physical therapy (PT) in HO has long
HO tends to occur more frequently with complete been controversial. Rossier and co-investigators
injuries. noted occasional transverse microfractures on
sections of HO that they thought might be caused
In SCI patients with HO, the hips are most by spasticity or by overly aggressive Passive range
commonly involved. of motion (PROM). Since then, the debate between
• At the hip, the flexors and abductors tend to resting the joint and aggressive PROM has
be involved more frequently than are the continued. In the literature, however, the
extensors or adductors. developing consensus appears to be that aggressive
• At the knee, the medial aspect is most PROM and continued mobilization, once acute
commonly affected by heterotopic ossification. inflammatory signs have subsided, are indicated,
• Shoulders and elbows are the most commonly because they help to maintain ROM and (in more
affected upper extremity joints. extensive HO) they may lead to the formation of a
pseudarthrosis. Resting the joint appears more
• Involvement of the metacarpophalangeal
likely to lead to decreased ROM or to ankylosis.
joints of the hand is rarely seen.
• The lumbar paravertebral region also has been During the acute inflammatory stage, the patient
noted though very rarely. should rest the involved joint in a functional
position, and the physical therapist should initiate
PHYSICAL EXAMINATION: gentle PROM as soon as possible. The role of
continuous PROM machines has not been studied
A diagnosis of heterotopic ossification can be made
in this situation. For patients with incomplete SCI
clinically if localized inflammatory reaction,
or head injuries, maintaining ROM may be difficult
palpable mass, or limited Range of motion is
because of pain from ROM exercises. The use of
observed.
joint manipulation has been reported in patients
Clinically, the onset of larger masses of heterotopic with HO. but because of limited joint ROM, they
ossification is often characteristic of any have functional limitations. However, such
inflammatory reaction. manipulation is controversial owing to the risk of
Fairly, a warm and swollen extremity becomes the formation of new hematoma and because of the
obvious, and fever is present. chance that long-bone fracture will occur in patients
with secondary osteoporosis.
If sensation is intact, the area of swelling is painful.
56 Neuro-Rehabilitation : A multi disciplinary approach

NEUROGENIC PAIN Nerve root entrapement pain often begins days to


weeks after injury and may worsen over time. It
INTRODUCTION occurs at or just below the level of injury and has a
Longstanding pain is experienced by many distinct pattern. You may feel brief waves of
Paraplegics below the site of lesion. This is one of stabbing or sharp pain or a band of burning pain at
the most challenging medical problems after spinal the point where the normal feeling stops. Some
cord injury (SCI).This neuropathic pain is very times a light touch could trigger a severe pain.. The
difficult to be treated and patients spend many pain stems from compression of a nerve root by a
sleepless nights. The incidence is very high. A bone or disk. Pain from damage to the cauda equina
review by Bonica an average of two-thirds of the is a type of nerve root pain that is described as a
Paraplegics experience this disturbing pain out of burning feeling in the legs, feet, pelvis, genitals, and
which half describe the pain as very severe, rectum.
interfering with ADL. Syringomyelia is a hollow, fluid filled cavity
(syrinx) in the spinal cord. It is not common, but
CLASSIFICATION OF PAIN IN SPINAL sometimes develops months or years after injury.
CORD INJURY The cavity can slowly increase in size and extend
Individuals with SCI can experience several types up or down the spinal cord. As the syrinx expands,
of pain. The most common ones can be classified it can result in pain along with an increased loss of
into three groups. sensory and motor function.
• Neuropathic pain Musculoskeletal
• Musculo skeletal pain This type of pain is also a concern for individuals
• visceral with spinal cord injury. It occurs in parts of the body
like the bones, joints, and muscles. Musculoskeletal
Neuropathic Pain pain is usually worsened by movement and eased
with rest. It can generally be described as a dull or
There are three varieties viz. SCI central pain,
aching pain, but the pain can also be described in
Segmental, nerve root entrapment pain, pain due
other terms.
to syrinx
Secondary overuse (pressure syndromes) is a very
SCI (Central pain) is described as sharp, shooting,
common cause of musculoskeletal pain. The pain
burning pain, tingling numbness or throbbing.
can occur months or many years after injury. It is
Though the patients do not experience sensations
caused by the overuse of muscles in any part of the
like touch, pin prick or any other sensation, he
body. For example, many people develop tendonitis
experiences a disturbing type of pain. This can begin
of the rotator cuff (shoulder) as a result of pushing
within weeks or months after the injury and is felt
a manual wheelchair for a long period of time.
at the level of injury or below the level where there
is no touch sensation. It is thought that the pain Muscle spasm pain is experienced by some
signals are coming from elsewhere and not related individuals after SCI. The spasms are involuntary
to any action or position of the limbs. movements of the body in areas that have lost some
or all motor function. The pain is caused when
Segmental pain often occurs around the border
muscles and joints are strained.
where there is normal sensation and where there is
loss of sensation as a result of injury. It can be Mechanical instability of the spine is caused by
slightly above the level of injury or slightly below. damaged ligaments or fracture of bones. It occurs
It usually develops during the first few months after most often shortly after injury, but it can also
injury. Segmental pain is often associated with develop later. The pain is usually around the area
allodynia and hyperalgesia in the painful region. of instability.
Allodynia is pain caused by something that does
not normally cause pain. For example, something Visceral
cold, warm or a very light touch to the skin can Visceral pain usually begins a short time following
result in pain. Hyperalgesia means an extremely SCI. It occurs in the abdomen either above or below
painful response to what is normally only mildly the level of injury. The pain is described as burning,
painful. cramping and constant.
Spinal Cord Injury 57

PATHOPHYSIOLOGY CLINICAL PRESENTATION AND PATIENT


EVALUATION
The blockade of nerve conduction in neuropathic
conditions causes nerve dysfunction, which can
result in numbness, weakness and loss of deep
tendon reflexes in the affected nerve area.
Neuropathic conditions also cause aberrant
symptoms of spontaneous and stimulus-evoked
pain. Spontaneous pain (continuous or intermittent)
is commonly described as burning, shooting or
shock-like. Stimulus-evoked pain includes
allodynia (pain evoked by a nonpainful touch) and
hyperalgesia (increased pain evoked by a painful
stimulus). Allodynia can be caused by the lightest
stimulation, such as skin contact with clothing or a
light breeze. These sensory abnormalities may
extend beyond nerve distributions, which may lead
Fig: Neuropathic pain arises following nerve injury to the inappropriate diagnosis of a functional or
or dysfunction. A: After nerve damage, psychosomatic disorder. The diagnosis of
transcription and axonal trafficking of sodium neuropathic pain is based primarily on history and
channels to the site of injury is increased, with findings on physical examination.
concomitant attenuation of potassium channels.
The altered expression of ion channels results in TREATMENT
neurons becoming hyperexcitable and generating Neuropathic pain can be very difficult to treat with
ectopic activity, which is thought to lead to the only some 40-60% of patients achieving partial
genesis of spontaneous and paroxysmal pain. B: At relief. Determining the best treatment for individual
the cell body of primary afferent neurons within patients remains challenging. Establishing an
the dorsal root ganglia (DRG), sympathetic accurate diagnosis is an important first step.
neuronal sprouting occurs and may account for
sympathetically maintained pain. C: Peripheral NONPHARMACOLOGICAL TREATMENT
nerve injury causes a multitude of changes in gene Although many patients with neuropathic pain
transcription and activation of various kinases and pursue complementary and alternative treatments,
proteins, including enhanced N-methyl-D- rigorous evidence supporting efficacy of nondrug
aspartate (NMDA) receptor activity. However, therapy is limited. Some reports suggest benefits
nerve injury also elicits hypertrophy and activation of conservative interventions such as exercise,
of glial cells, including microglia within the grey transcutaneous electrical nerve stimulation,
matter of the spinal cord. Microglia expresses P2X4 percutaneous electrical nerve stimulation, graded
purinergic receptors, allowing them to be activated motor imagery and cognitive behavioural therapy
by adenosine triphosphate (ATP). Following or supportive psychotherapy. Relieving stress
activation, microglia release various pronociceptive related activities is important because these
cytokines, such as interleukin-1 (IL-1), tumour intensify most pain disorders and may be helped
necrosis factor alpha (TNF-a) and neurotrophins, by psychological counselling or psychiatric
including brain-derived neurotrophic factor, which intervention.
in turn exacerbates nociceptive transmission and
contributes PHARMACOLOGICAL TREATMENT
to the sensitization and maintenance of neuropathic It is common practice in medicine to designate
pain. classes of medication according to their most
common or familiar use e.g. as "antidepressants"
Note: Aß = A beta neuron, Ad = A delta neuron, and "anti-epileptic drugs" (AED's). These drugs
C = C nociceptor, 5HT = serotonin, KCC2 = chloride have alternate uses to treat pain because the human
transporter, NA = noradrenaline, Nav = sodium nervous system employs common mechanisms for
channel, NO = nitric oxide, Kv = potassium channel,
different functions, for example ion channels for
PGs = prostaglandins, PKs = protein kinases, P2X4
impulse generation and neurotransmitters for cell-
= purinergic receptor.
to-cell signaling.
58 Neuro-Rehabilitation : A multi disciplinary approach

No one pharmacological protocol can be used in Neuropathic pain is best managed with a
the treatment of all patients. Usually the approach multidisciplinary approach. Nevertheless, several
is to try one agent until pain relief is achieved, a different treatments can be initiated in the primary
maximum dose is obtained, or side effects prohibit care setting. Treatments with the lowest risk of
increasing the dose, before trying another agent. adverse effects should be tried first. Evidence
Favored treatments are certain antidepressants e.g. supporting conservative nonpharmacologic
tricyclics and selective serotonin-norepinephrine treatments (e.g., physiotherapy, exercise,
reuptake inhibitors (SNRI's), anticonvulsants, transcutaneous electrical nerve stimulation) is
especially pregabalin (Lyrica) and gabapentin limited; however, given their presumed safety,
(Neurontin), and topical lidocaine. Opioid nonpharmacologic treatments should be considered
analgesics and tramadol are recognized as useful
whenever appropriate.61 Simple analgesics (e.g.,
agents but are not recommended as first line
acetaminophen, NSAIDs) are usually ineffective in
treatments. Many of the pharmacologic treatments
pure neuropathic pain but may help with a
for chronic neuropathic pain decrease the sensitivity
coexisting nociceptive condition (e.g., sciatica with
of nociceptive receptors, or desensitize C fibers such
that they transmit fewer signals. musculoskeletal low-back pain). Early referrals to
a pain clinic for nerve blocks may be warranted in
Some drugs may exert their influence through some cases to facilitate physiotherapy and pain
descending pain modulating pathways. These rehabilitation.
descending pain modulating pathways originate in
the brainstem. CONCLUSION
The use of intrathecal baclofen, morphine, and Neuropathic pain is a devastating chronic condition
clonidine are newer approaches to decrease this that generally can be diagnosed by history and
type of pain. findings on physical examination. For some
neuropathic pain syndromes, available treatments
SURGICAL TREATMENT are tolerable and afford meaningful relief to a
Surgical intervention is only considered if all else considerable proportion of patients. Nevertheless,
has failed and the neuropathic pain interferes with many patients report intractable and severe pain,
daily activities. The most common surgical and better treatment strategies are desperately
techniques used include dorsal root rhizotomy, needed. The field of neuropathic pain research and
cordotomy, and lumbar sympathectomy. treatment is in the early stages of development, with
many goals yet to be achieved. In particular, future
ALGORITHM FOR THE MANAGEMENT OF laboratory, clinical and epidemiologic research into
NEUROPATHIC PAIN IN PRIMARY CARE. pathogenesis, treatment and prevention of
neuropathic pain is expected as well as improved
dissemination of new information to health
professionals and the public. Over the years to
come, many upcoming advances are expected in
the basic and clinical science of neuropathic pain
as well as in the implementation of improved
therapies for patients who continue to experience
these devastating conditions.

5. OSTEOPOROSIS
Introduction
One of the inevitable complications of spinal cord
injury (SCI) is the associated osteoporosis that
occurs mainly in the pelvis and the lower
extremities. The acute treatment of patients with
spinal cord injury has always been concentrated on
the injury itself and on the subsequent
complications. Osteoporosis or bone loss as a
consequence of spinal cord injury has been of
secondary concern.
Spinal Cord Injury 59

Osteoporosis in persons with spinal cord injury was Fractures and SCI
first studied in relation to calcium metabolism and There is a risk of fractures as the bone mineral
the associated hypercalcemia and renal calculi that density decreases. The incidence of fractures of the
followed. The differences between osteoporosis lower limbs in SCI is quite high.. Most fractures
induced by spinal cord injury and other causes of while doing normal activities such as transferring
bone loss due to disuse because of prolonged bed and not from violent falls. Sometimes people cannot
rest, space travel, and lower motor neuron remember of any trauma at all, but when they notice
disorders, have since become clearer. swelling or redness get investigated and find they
– Bone loss occurs below the level of the have afracture.
spinal cord injury, with no loss of any
bone mass above the lesion. CAUSES OF OSTEOPOROSIS IN SCI
– Trabecular bone is more affected than Various possibilities for osteoporosis are
cortical bone, and in particular femur and – Disuse: or lack of mechanical loading on the
tibia. Studies have shown that there is bone would inhibit stimulation of bone-
about 30% to 40% decease in bone density building cells.
in the lower extremities after SCI.
– Disordered vasoregulation: sluggish blood
– Osteoporosis can be detected on x-rays flow to limbs may contribute to a decrease in
as early as six weeks after injury. Most bone mass.
researchers feel that bone loss is not that
rapid after 6weeks and it levels out by – Poor nutrition: not taking healthy and
around 2 years. But some studies show balanced diet
that there is still possibility of bone loss – Hormonal alterations : proteins in the body are
even after2 years.. responsible for the maintenance bony
– The lumbar spine does not show any formation and resorption.
osteoporosis, the reason being that SCI – Metabolic disturbances (tissue acidosis,
persons sit on the W/C for longer period alkaline phosphatase, hypercalcemia/
and the loading is maximum on sitting hyercalciuria, hydroxyproline excretion):
than any other position. Loading of bone disturbance in metabolites and acidity of the
actually stimulates bone mineral density. blood can influence the balance of bony
The non-weight bearing lower formation and resorption.
extremities don't have this stimulation – Autonomic disregulation: impaired control by
and therefore lose bone mineral density. the self-regulating nervous system can lead to
– Injury level increased imbalance between bone formation
• Individuals with quadriplegia have more and resorption.
osteoporosis than paraplegics because of more
PATHOPHYSIOLOGY
area below the lesion. Paraplegics usually have
bone mineral density preserved in their upper The mechanism behind spinal cord injury (SCI)–
extremities. induced osteoporosis is accepted as being
multifactorial in the acute and chronic stages. These
• In the bone that is affected, the severity of bone mechanisms differ from those observed in subjects
loss is the same both in paraplegia and without spinal cord injury after prolonged bed rest
tetraplegia. and in subjects with other neurologic deficits.
• Severity of injury: Individuals with complete
injuries have more bone loss than those with Disuse structural change and hypercalciuria
incomplete injuries. Spinal cord injury causes immediate and, in some
regions, permanent gravitational unloading. The
– Spasticity may have a beneficial effect in
result is a disuse structural change with associated
maintaining bone mass after SCI, due to
metabolic consequences. Hypercalciuria is seen by
muscle pulling on the bone, similar to the
10 days following the spinal cord injury and reaches
effect of weight-bearing.
a peak 1-6 months postinjury. This level of
– Duration of injury: The longer time the hypercalciuria is 2-4 times that of persons without
since injury, the greater the bone loss is. spinal cord injury who undergo prolonged bed rest.
60 Neuro-Rehabilitation : A multi disciplinary approach

This marked increase in urine calcium is the direct lower extremities). In addition, the prevalence of
result of an imbalance between bone formation and vitamin D deficiency in SCI is increased, and this
bone resorption. may exacerbate bone loss

Osteoblast and osteoclast activity SIGNS AND SYMPTOMS


The activity of osteoblasts and osteoclasts is There are no particular signs and symptoms for
triggered by the spinal cord injury; however, osteoporosis Only when an x ray is taken following
markers of osteoblastic activity rise only slightly, a fracture it is revealed. There is no other physical
whereas osteoclasts have a significant increase in examination to diagnose the condition. At times
their activity, peaking at 10 weeks following the there may be some effusion in the knees or
injury with values 10 times the upper limits of heterotropic ossification
normal (ULN). In addition, the increased bone
resorption precedes the increase in osteoblastic Biomechanical Markers
activity. This model at the skeletal level following The biomechanical markers that have been
spinal cord injury resembles the high bone turnover measured in studies of spinal cord injury (SCI)–
rate seen in postmenopausal osteoporosis. induced osteoporosis include serum calcium,
phosphorous, alkaline phosphatase, 1,25–
Bone muscle traction loss or neuronal factors
dihydroxyvitamin D and calcitonin, and urinary
The loss of bone also may be enhanced by lack of calcium and hydroxyproline.
muscle traction on bone or by other neural factors
associated with spinal cord injury. These other These markers may not be followed routinely in
factors further separate spinal cord injury–induced the ongoing care of the person with spinal cord
osteoporosis from other causes of disuse injury. However, the sensitivity and early response
demineralization. Absorption of calcium from the of these markers indicate that they would be useful
gastrointestinal (GI) tract has been found to in the early identification of patients with spinal
decrease in the acute period following injury. Even cord injury who are at risk of developing severe
so, in the past, dietary calcium reduction was osteoporosis.
commonly recommended as a way to decrease
MANAGEMENT APPROACHES
calcium excretion and prevent the complications of
hypercalciuria. Changes do occur very rapidly in the bones of a
patient with spinal cord injury (SCI), and
Parathyroid hormone interventions must be undertaken quickly. Infact
The body that has sustained spinal cord injury has that there are no effective treatments to restore bone
been considered the model of premature aging, and mineral once it has been lost and therefore early
the role of parathyroid hormone (PTH) in treatment is imperative. Thus, prevention is the
osteoporosis following spinal cord injury illustrates main focus in treating spinal cord injury–induced
this point. Acutely, the parathyroid gland is osteoporosis.
relatively inactive, with low PTH levels observed In the evet of a fracture conservative treatment is
up to the 1-year point following injury. to be tried first. Invariably healing would be seen
Hypercalcemia seen immediately postinjury leads in 3 to 4 weeks. Soft splints may be required. Hard
to this low level. A reversal in activity during years splints and materials should be avoided. However
1-9 is noted. if deformities are seen surgical intervention may
The parathyroid gland is stimulated to the point be [Link] surgeon’s opinion has to
that PTH levels are above the reference range. The be taken.
result is an increase in bone reabsorption or
osteoporosis related to parathyroid dy nosfunction PREVENTION & TREATMENT
in the chronic stages of spinal cord injury. This It is difficult to totally prevent bone
chronic-stage mechanism of osteoporosis is demineralization after spinal cord injury.
balanced by an increase in bone mineral in regions Individuals are at higher risk for osteoporosis
of the body in which weight bearing is resumed following SCI. There is no “standard of care” to treat
(eg, in the upper extremities, spine) and adds to this problem. Each individual needs to be evaluated
the demineralization observed in regions that are to determine if treatment is essential.
chronically non–weight-bearing (eg, the pelvis,
Spinal Cord Injury 61

• Exercise help prevent bone loss and increase bone


Physical activity is supposed to preserve or increase density by 1 to 4%. These drugs are approved
bone mass in able-bodied individuals. Studies has for preventing and managing osteoporosis in
shown that very hard exercises are beneficial the general population.
preventing bone loss in the upper extremities.. But
this type of exercise did not stop demineralization • Protect the Bones.
of the lower body. Individuals with SCI need to be Individuals with SCI need to protect their bones.
cautious when they begin an exercise program as When doing range of motion exercises, limit
overuse syndrome may set in. Therefore a movement to stretches that are easily done. Do not
physiotherapist has to prescribe the level o f force a joint or muscle to move past what is
exercises. Various activities are under study as comfortable. Check with a physical therapist for
forms of exercise to build bone strength in exercises specific to an individual’s needs and
individuals with SCI. These include weight bearing abilities.
using a standing frame or harness; treadmill
training; Parastep; and functional electrical Spasticity can put some force on the bones that helps
stimulation. [Link] ever there is controversy to strengthen them. However strong spasms could
about the effectiveness of thisT of these different cause a weak bone to fracture. Be cautious when
programs. Some of drawbacks to these activities are transferring. Remove feet from heel loops or toe
costs, the need to be done for long periods of time, straps on the foot rests before transferring. If a
and difficulty fitting it in to an individual’s daily person’s balance or strength is weak, he/she needs
schedule. There is also an increased incidence of to ask for help to avoid falling.
fractures if bones are already badly osteoporotic.

• Extra calcium and vitamin D


SPASTICITY
These need to be included in one’s daily diet to help INTRODUCTION:
in preventing osteoporosis. Calcium helps build Spasticity is the involuntary movement (jerking) of
strong bones and vitamin D improves the muscles, which occurs because messages can travel
absorption of calcium. But for individuals with SCI, from parts of your body to the spinal cord and cause
high levels of calcium and Vitamin D may increase reflex activity (muscle movement). This is possible
the risk of urinary calculii. There are no guidelines because the spinal cord has certain normal
on the risks or benefits of calcium and Vitamin D automatic functions, which are under the influence
supplements for individuals with SCI. A of the brain. These functions include muscle tone
supplement of 1000 mg/day will be safe and
and reflexes. Most spinal cord injured persons have
beneficial if the excretion remains less than 250 mg/
a healthy, intact spinal cord below the immediate
day and the individuals’ parathyroid hormone
area of their injury, and thus these automatic
levels are in mid-normal range. It may be necessary
activities can continue to exist. However, they are
to monitor calcium levels in the urine if
no longer under the regulating influence of the
supplements are used.
brain and are thus exaggerated. This is called
• Stop smoking. spasticity.
Cigarette smoking reduces the body’s ability Spasticity is common in varying degrees after spinal
to absorb calcium. This in turn can speed up cord injury. Spasticity is a non-specific symptom,
bone loss in all populations. which may occur in many problems associated with
spinal cord injury.
• Limit caffeine.
Caffeinated drinks act as a diuretic. They speed ETIOLOGY:
up the removal of calcium from the body in After spinal cord injury the nerve cells below the
the urine by about 10 mg per day. level of injury become disconnected from the brain.
Following the period of spinal shock changes occur
• Avoid drinking too much alcohol. in the nerve cells that control muscle activity.
This is linked to bone loss as well as poor Spasticity is an exaggeration of the normal reflexes
nutritional habits. that occur when the body is stimulated in certain
ways. After spinal cord injury, when nerves below
• Medications the injury become disconnected from those above,
A new class of drugs, bisphosphonates, can these responses become exaggerated.
62 Neuro-Rehabilitation : A multi disciplinary approach

Muscle spasms, or spasticity, can occur any time Avoiding situations such as bladder infections, skin
the body is stimulated below the injury. This is breakdowns, or injuries to the feet and legs will also
particularly noticeable when muscles are stretched reduce spasticity. There are three primary
or when there is something irritating the body medications used to treat spasticity, baclofen,
below the injury. Pain, stretch, or other sensations Valium, and Dantrium. All have some side effects
from the body are transmitted to the spinal cord. and do not completely eliminate spasticity. Other
Because of the disconnection, these sensations will interventions to help reduce or control spasms
cause the muscles to contract or spasm. include injections of Botulinum Toxin A (Botox) or
Some stimuli can cause a change in your spasticity. phenol directly into the muscles of concern.
Anything that would ordinarily be uncomfortable Injection therapy usually lasts 3-4 months. Surgical
or painful can cause an increase in your spasticity. interventions include the insertion of an Intrathecal
If you experience a major increase in spasticity, Baclofen Pump. A Baclofen pump trial is conducted
possible causes are: first to make sure there is an adequate response to
• Skin problems - a skin sore or ingrown toenail the intrathecal baclofen. Once it is determined that
a person responds to the intrathecal baclofen
• Bladder problems - high residuals, infection
therapy, surgery is scheduled. The pump must be
or bladder stones
refilled every three months but is dependent upon
• Bowel problems - constipation, impactions or the amount of drug needed by each [Link] the
hemorrhoids case of SCI, the distribution of spasticity tends to
• Medical problems - viral syndrome (infection, be more diffuse, making regional or systemic
influenza, intestinal flu), heterotopic treatment preferable.34 The decision whether or not
ossification or a spinal cyst. to treat spasticity and, if so, in what manner, is
summarized nicely in a flow chart
TRIGGERING FACTORS:
Almost anything can trigger spasticity. Some things,
however, can make spasticity more of a problem.
A bladder infection or kidney infection will often
cause spasticity to increase a great deal. A skin
breakdown will also increase spasms. In a person
who does not perform regular range of motion
exercises, muscles and joints become less flexible
and almost any minor stimulation can cause severe
spasticity.

BENEFITS OF SPASTICITY:
There are some benefits to spasticity. It can serve
as a warning mechanism to identify pain or CONSERVATIVE/PHYSICAL
problems in areas where there is no sensation. Many REHABILITATION MANAGEMENT
people know when a urinary tract infection is
It is generally agreed that physical therapy/
coming on by the increase in muscle spasms.
rehabilitation is an essential component in the
Spasticity also helps to maintain muscle size and
management of spasticity as a first line of defence,
bone strength. It does not replace walking, but it
as well as in a long-term regimen during and after
does help to some degree in preventing
osteoporosis. Spasticity helps maintain circulation the implementation of pharmacological or surgical
in the legs and can be used to improve certain strategies. The goal of physical therapy is to
functional activities such as performing transfers diminish spasticity in order to allow expression of
or walking with braces. For these reasons, treatment voluntary mobility and movements and/or to
is usually started only when spasticity interferes improve the comfort and independence in tasks
with sleep or limits an individual's functional related to Quality of living, such as transfers,
capacity dressing, and using the washroom. The literature
on the conservative/physical treatment of spasticity
MANAGEMENT: is sparse, and some have questioned the
Some spasticity may always be present. The best effectiveness of these management strategies. Table
way to manage or reduce excessive spasms is to below summarizes the most common physical
perform a daily range of motion exercise program. therapy approaches to spasticity management.
Spinal Cord Injury 63

Table - Physical techniques in the management of spasticity.


Physical therapy technique description and Purpose/suggested mechanisms
comment on effectiveness

Positioning

• In bed and during sitting • Reports of clinical • Important to the maintenance of


effectiveness; impact remains to be proven muscle length
scientifically

Range of motion/stretching

• Includes passive stretch and passive length- • Prevents contractures • Causes temporary
ening • Benefits may carry over for several reduction in intensity of muscle contraction in
hours • Effects remain to be quantified and the reaction to muscle stretch • May cause plastic
efficacy remains to be determined despite the changes within the central nervous system and/
clinical evidence for the benefits or mechanical changes at the muscle, tendon,
and soft-tissue level

Weight-bearing

• Using a tilt table or standing frame •Benefits • Prolonged stretch of ankle plantar flexor
are greater than stretching alone and may persist muscles • Mechanism remains uncertain;
into next day • Effectiveness has been questioned suggested to include a modulating influence
from cutaneous and joint receptor input to the
spinal motor neurons, resulting in decreased
excitability

Muscle strengthening

• Progressive addition of resistance to muscles • Emphasis of balance of agonist and antagonist


with voluntary control groups of muscles with voluntary control

Electrical stimulation

• Various methods: stimulation to the antagonist • Stimulation of the antagonist muscle:


muscle, application of tetanic contraction to the augmentation of reciprocal inhibition of the
spastic muscle, functional electrical stimulation spastic muscle • Repetitive tetanic stimulation
(FES), and transcutaneous electrical nerve of spastic muscle: fatigue of the muscle due to
stimulation(TENS) Reports of beneficial effects repetitive tetanic stimulation • FES: change the
between only 10 min and 3 h mechanical properties of a spastic joint by
strengthening the antagonists of the spastic
muscle or might decrease the hyperactivity of
spastic muscles through reciprocal inhibition •
TENS: may involve the stimulation of large
diameter afferent fibers that travel from
mechanoreceptors to the spinal cord14

Epidural spinal cord stimulation

•For mild spasticity and incomplete lesions: • May involve the activation of inhibitory
stimulation below the level of the lesion found networks within the spinal cord • More strongly
effective (spasms) For severe spasticity: affected patients require stronger stimuli and/or
stimulation of dorsal roots of the upper lumbar higher frequencies
cord segment found effective (hypertonus and
spasms) Shown to lack long-term effectiveness
64 Neuro-Rehabilitation : A multi disciplinary approach

Cold/heat application
• Application of a cold pack or a vapocoolant • Cold: may cause slowing of nerve conduction,
spray, or superficial heat • Following cold decrease in sensitivity of cutaneous receptors,
application: tendon reflex excitability and and alteration of CNS excitability • Heat:
clonus may be reduced for a short period of facilitation of uptake of released
time (eg, <1 hr), allowing for intermittent neurotransmitters and return of calcium to the
improved motor function • Following heat
application: subsequent passive stretch is
facilitated
Splinting/orthoses
• Helpful in the continuous application of muscle •Enables long-term stretch Joint can be
stretch • Use of splints is questioned maintained in a position that does not elicit a
spasm

PRESSURE SORES With proper treatment, most pressure sores will


heal.
INTRODUCTION A pressure sore is any redness or break in the skin
Pressure ulcers are an extremely common caused by too much pressure on the skin for too
complication after spinal cord injury. In the acute long a period of time. The pressure prevents blood
stage after spinal cord injury, more than one third to the skin leading to necrosis of skin and even
of patients have pressure ulcers. Pressure ulcers deeper tissues. Normally the nerves send messages
may delay the onset of rehabilitation therapies, of pain or feelings of discomfort to the brain and
lengthen the total hospitalization, and make the the person should change position to relieve the
ultimate adjustment to disability more difficult. In pressure, but due to damage to the spinal cord these
the long term, these ulcers impair an individual’s messages cannot reach the brain.
quality of life because of activity restriction and
Most common sites of pressure sores are as shown
increased need for medical and nursing care, often
in the diagram below
by hospitalization or surgery.
A pressure sore is known by many names, like
pressure ulcer, decubitus ulcer, ischemic ulcer, bed
sore or skin sore. No matter what it is called, it is a
serious problem that can take days, weeks, months
or even longer to heal.
A Pressure Sore is an area of the skin or underlying
tissue that is dead or dying as a result of the loss of
blood flow to the area. It can begin in a number of
ways. The most common is when you rest on a bony
area for a prolonged period of time. The extended
pressure leads to a pressure sore.
Damage from a pressure sore will range from slight
The patient may need to learn new ways to change
discoloration of the skin (stage 1) to open sores that
his/her position to prevent too much pressure.
go all the way to the bone (severe). The affected
Pressure sores can occur, for example, when the
area may feel warmer than the surrounding tissue.
patient sits or lies in one position too long. Shearing
In light-skinned people, the discoloration may
is also a kind of pressure injury. It happens when
appear as dark purple or red. In darker-skinned
the skin moves one way and the bone underneath
people, the discoloration will appear darker than
it moves another way. An example of this is if you
the surrounding tissue.
slouch when you sit.
A pressure sore is serious matter and cannot be
Another type of injury, an abrasion, can occur when
ignored as the sore may deepen in a very short time.
pulling oneself across a surface instead of lifting.
Spinal Cord Injury 65

This is an example of a friction injury. In addition,


short exposure to high pressure, such as a bump or
fall, may cause damage to the skin which may not
show up right away.

STAGES OF PRESSURE SORES AND HOW


TO CARE FOR THEM:
Category/Stage I: Non-blanchable erythema
Intact skin with non- blanchable redness of a
localized area usually over a bony prominence.
Darkly pigmented skin may not have visible
blanching; its colour may differ from the
surrounding area. The area may be painful, firm,
soft, warmer or cooler as compared to adjacent
tissue. Category I may be difficult to detect in Category/Stage III: Full thickness skin loss
individuals with dark skin tones. May indicate “at Full thickness tissue loss. Subcutaneous fat may be
risk” persons. visible but bone, tendon or muscle are not exposed.
Slough may be present but does not obscure the
depth of tissue loss. May include undermining and
tunnelling. The depth of a Category/Stage III
pressure ulcer varies by anatomical location. The
bridge of the nose, ear, occiput and malleolus do
not have (adipose) subcutaneous tissue and
Category/Stage III ulcers can be shallow. In
contrast, areas of significant adiposity can develop
extremely deep Category/Stage III pressure ulcers.
Bone/tendon is not visible or directly palpable.

How to recognize:
Skin is not broken but is red or discoloured. The
redness or change in colour does not fade within
30 minutes after pressure is removed.
Category/Stage II: Partial thickness
Partial thickness loss of dermis presenting as a
shallow open ulcer with a red pink wound bed,
without slough. May also present as an intact or
open/ruptured serum-filled or sero-sanginous
filled blister, presents as a shiny or dry shallow ulcer
without slough or bruising*. This category should
How to recognize:
not be used to describe skin tears, tape burns,
incontinence associated dermatitis, maceration or The break in the skin extends through the dermis
excoriation. (second skin layer) into the subcutaneous and fat
tissue. The wound is deeper than in Stage Two.
*Bruising indicates deep tissue injury. Category/Stage IV: Full thickness tissue loss
How to recognize:
Full thickness tissue loss with exposed bone, tendon
The epidermis or topmost layer of the skin is or muscle. Slough or eschar may be present. Often
broken, creating a shallow open sore. Drainage may includes undermining and [Link] depth of
or may not be present. a Category/Stage IV pressure ulcer varies by
66 Neuro-Rehabilitation : A multi disciplinary approach

anatomical location. The bridge of the nose, ear, can be increased every few days, if hyperaemia
occiput and malleolus do not have (adipose) resolves within 30 minutes. The patients'
subcutaneous tissue and these ulcers can be buttocks should be placed far back in the chair,
shallow. Category/Stage IV ulcers can extend into and the footrests should be adjusted to permit
muscle and/or supporting structures (e.g., fascia, the knees to be at or slightly below the level of
tendon or joint capsule) making osteomyelitis or the hips. Pressure reliefs should be performed
osteitis likely to occur. Exposed bone/muscle is frequently, every 15 to 30 minutes, for one
visible or directly palpable. minute. Recommended methods include
leaning the patient forward, with chest toward
How to recognize:
the thighs, and tipping the wheelchair back 65
The breakdown extends into the muscle and can degree.
extend as far down as the bone. Usually lots of dead
3. Transfers: Transfers should be performed
tissue and drainage are present.
carefully to avoid shearing.
4. Equipment:
• During the acute care phase, the patient
is frequently in a supine position and the
sacrum, occiput, and heels are at high risk
for pressure sores. To prevent pressure
sores, orthotic devices can be used to
completely elevate the head off the bed
and minimal air loss beds are beneficial.
Donut-type devices should not be used.
Thoracic and lumbar fractures are
managed by immobilization in special
beds that allow movement.
• Equipment used in the hospital should
COMPLICATIONS CAUSED BY PRESSURE be as similar as possible to equipment that
SORES: will be used after discharge in the home.
Skin tolerance to standard mattresses and
• Can be life threatening. wheelchair cushions should be
• Infection can spread to the blood, heart, bone. determined before discharge. Air-
flotation and air-fluidized beds, for
• Amputations.
example, are suitable for hospital use in
• Prolonged bed rest. the beginning or to treat pressure ulcers,
• Autonomic dysreflexia. but they are not recommended for home
use. An egg-crate foam mattress pad on
PREVENTION: top of a standard mattress is best suited
to the home. Although wheelchair
1. Turning: Patients should be positioned
cushions reduce pressure over bony
properly in bed at all times. Patients should
prominences, proper skin care and
be turned frequently, every two hours at the
pressure reliefs are essential.
beginning, and skin should be checked
between turns. Turning time can be increased 5. Cushions: Cushions should be used in
when hyperaemia over bony prominences wheelchairs, for many bed positions, etc. Most
resolves within 30 minutes. All positions, cushions are made of foam or gel or filled with
including the prone position and side-lying, air or water.
should be used. In time, patients will be • Foam cushions produce higher skin
independent or able to give instructions to a temperatures and less humidity. Custom
caregiver in bed positioning, turning, and skin contoured foam seat cushions are more
checks. effective than flat foam cushions.
2. Sitting: Patients should sit no more than 30 to • Gel cushions maintain skin temperatures
60 minutes at the beginning. Their sitting time (although they may require cooling after
Spinal Cord Injury 67

3 hours to continue to be able to do this) TREATMENT OF PRESSURE ULCERS


and produce more humidity than foam; ACCORDING TO DIFFERENT STAGES
some gel-type cushions also improve
posture. STAGE I
1. Keep pressure off the sore!
• Air-filled cushions need to be checked
daily for proper air pressure. 2. Maintain good hygiene. Wash with mild soap
and water, rinse well, pat dry carefully (but
• Water-filled cushions decrease skin
gently). Do not rub vigorously directly over
temperature; however, humidity
the wound.
increases more on water-filled cushions.
3. Evaluate patient’s diet by asking - are you
Good body positions getting enough protein, calories, vitamins A
Patient’s position is important to relieve pressure and C, zinc and iron? All of these are necessary
on the sore and prevent new ones. He/she may for healthy skin.
need to switch positions whether in a bed or a chair. 4. Review the mattress, wheelchair cushion,
transfers, pressure releases, and turning
While In Bed
techniques for possible cause of the problem.
1. Do not lie on the pressure sore. The use of pads
or pillows to position the body will help relieve 5. If the sore seems to be caused by friction,
the pressure on specific areas. sometimes a protective transparent dressing
such as Op-Site or Tegaderm may help protect
2. Change positions at least every 2 hours. the area by allowing the skin to slide easily.
3. Avoid lying directly on the hip bone. A 30- 6. If the sore does not heal in a few days or recurs,
degree position is best. consult the health care provider.
4. When lying on the back, keep the lower legs
up by placing a thin foam pad or pillow under STAGE II
the lower part of the legs (midcalf to ankle). Follow steps 1-4 under Stage One. Consult the
Do not place the pad or pillow directly under health care provider for further treatment, which
the knee as this will reduce the flow of blood may include the following:
to the lower leg areas. Do not use donut shaped • Cleanse the wound with saline solution only
cushions as they will reduce the flow of blood. and dry carefully. Apply either a transparent
5. Use pillows or small pads to keep the knees dressing (such as Op-Site or Tegaderm), a
and ankles from touching. hydrocolloid dressing (such as DuoDERM), or
6. Raise the head of the bed as little as possible saline dampened gauze. The first two types of
(30 degrees or less). dressing can be left on until they wrinkle or
loosen (up to 5 days). If using gauze, it should
7. Feed meals in an upright position to prevent be changed twice a day and should remain
choking. damp between dressing changes.
Pressure Relief • Check for signs of wound healing with each
Pressure sores form when there is constant pressure dressing change.
on certain parts of the body. Long periods of • If there are signs of infection consult the health
unrelieved pressure cause or worsen pressure sores care provider for alternative wound care ideas
and slow healing once a sore has formed. Taking and review of possible causes (see step 4 under
pressure off the sore is the first step toward healing. Stage One).
Pressure sores usually form on parts of the body
STAGE III
over bony prominences (such as hips and heels) that
bear weight when one sits or lies down for a long Follow steps 1-4 under Stage One and the additional
time. Pressure can be relieved or reduced by: steps under Stage Two. Always consult the health
care provider. Wounds in this stage frequently need
• Using special surfaces to support the body.
additional wound care with special cleaning or
• Putting the body in certain positions. debriding agents. Different packing agents, and
• Changing positions often. occasionally, antibiotics (creams or oral pills) may
68 Neuro-Rehabilitation : A multi disciplinary approach

be required. One may also qualify for a special bed located between cord level T10 to L2. The actual
or pressure-relieving mattress that can be ordered micturition center coordinating these is located at
by the health care provider. Pons & finally the higher social & permissive
control happens at the level of frontal lobe. The
STAGE IV sensory input is via the pelvic nerves [autonomic]
Consult the health care provider right away. & the motor output is via pelvic [autonomic] &
Surgery is frequently required for this type of pudendal [somatic] nerves. In general sympathetic
wound. system helps us store [ S for storage] &
How to know if the sore is healing: parasympathetic system helps us empty [P for pee!]
The first event during micturition is relaxation of
• The sore will get smaller.
external sphincter, followed by detrusor contraction
• Pinkish tissue usually starts forming along the followed by relaxation of bladder neck, leading to
edges of the sore and moves toward the centre; bladder emptying. The test to study the behavior
one may notice either smooth or bumpy of the lower urinary tract is called “Urodynamic
surfaces of new tissue. evaluation”.
• Some bleeding may be present. This shows
that there is good blood circulation to the area, Types of neurogenic bladder
which helps healing. All patients with spinal injury go through a state
These are commonly seen complications in patients of spinal shock or total unresponsiveness below the
with SCI irrespective of levels of lesion, which level of injury; for a period varying from a few days
become a challenge for the neuro rehabilitation to several months. The urinary bladder during this
team to tackle with. phase is areflexic & asensate. Thus the patient has
painless large volume retention with intermittent
UROLOGICAL REHABILITATION over flow dribble. Once the patient recovers from
OF A SPINALLY INJURED PATIENT the spinal shock, depending on the completeness
of injury, following patterns emerge. While
Injury to the spinal cord leads to neurological describing the patterns, the detrusor & sphincter
damage below the level of injury & in turn produces behavior has to be taken into consideration.
temporary and or permanent changes in the body
As the spinal reflex center for urinary bladder is
organs supplied by the respective nerves. In
situated in the sacral spinal cord, the injuries are
addition to commonly known changes such as
divided into supra sacral, sacral or infra sacral. In
muscle weakness or paralysis as well as impairment
supra sacral, the injury can be above the level of
or loss of sensation, there are changes involving the
thoracic sympathetic outflow, that is above spinal
urinary tract, sexual function as well as bowel.
level T6.
These changes add to significant physical &
emotional stress and urological complications are 1 Supra sacral injury above T6 – Here the
often the major cause of morbidity & mortality in bladder develops high pressure involuntary
these patients. Last 3 decades have witnessed a sea detrusor contractions at low volume, often
change in the way we view the neurologically leading to leakage of urine. The resting bladder
damaged urinary tract [neurogenic bladder]. With pressure may be high leading to upper tract
the concept of clean intermittent catheterization [kidney] damage. The outlet remains closed,
[CIC], the quality of life of patients with various sometimes with active sphincteric contractions
forms of neurogenic urinary dysfunction has during episodes of leak, leading to high
changed dramatically. pressure leak. Such patients may also develop
rise in blood pressure with bladder distension,
Normal urination a condition called autonomic dysreflexia due
All of us have the ability to store & empty our to mass sympathetic discharge. This can
bladder completely at will, at low pressure; without produce dangerour rise in blood pressure &
straining at bladder volumes within a certain limit. can rarely lead to intra cranial bleed. These
The reflex center for micturition [urination] is patients clinically present with storage
located in the sacral cord at S 2-3-4 segments. This symptoms such as frequency with urinary
in turn is modulated by parasympathetic autonomic leak, often associated with headaches & leg
system at S2-3-4 & sympathetic autonomic system spasms. These patients usually have
Spinal Cord Injury 69

constipation, with passage of hard stools every importance as compared to the other issues such
3rd or 4th day. as protecting the kidneys & prevention of
complications.
2 Supra sacral injury below T6 - These patients
have a relatively safe bladder in that there is
Management of neurogenic bladder
no autonomic dysreflexia. The remaining
pattern is the same as that described above. During the acute management of injury, all patients
The patients may also be able to predict when usually have an indwelling foley catheter. However
they will leak urine, thus enabling them to once the patient is stable, clean intermittent
avoid incontinence. Bowel dysfunction catheterization [CIC] should be started either by
remains the same as in group one. teaching it to the patient [in paraplegics] or to the
care giver [in quadriplegics.] The catheter needs to
3 Sacral injury – Those with injury at or below be passed every 4 to 5 hourly to mimic natural
the sacral reflex center have poor active urination.
detrusor contractions with a fixed non
contracting & non relaxing outlet. Thus they Once the state of spinal shock is over, all patients
present with voiding symptoms, such as poor must have a baseline urodynamic evaluation, which
flow, straining to empty or frank retention shows the type of voiding dysfunction. The test can
associated with overflow leak & large residue. then be repeated every 2 years or earlier if a change
Bowels are usually constipated. The large takes place in the urination pattern. All patients
residue predisposes them to infection & upper must have an annual assessment of serum
tract [kidney] damage. creatinine to check renal function & a sonography
of the urinary tract to look for any upper tract
Unfortunately majority of patients may have a dilatation of development of stones. As a rule
partial and-or multiple level injury, thus the urinary adequate water intake [roughly about 2.5 to 3 liters
bladder behavior also may show mixed picture. ] should be ensured to prevent super saturation of
Special mention must be made of spinal urine with crystals
dysrhaphism or spina bifida, presenting in its Technique of CIC - The best catheter or tube for
various forms either at birth [meningo myelocoele] CIC is a 10 or 12 fr soft plastic catheter such as an
or later.[tethered cord]. Nowhere is urological infant feeding tube. This has the lowest chance of
rehabilitation as important as in these kids as it damaging an asensate urethra & is cheap & easily
needs to be done right from birth with tremendous available. Though it is ideal to use a new catheter
input for the parents. Typically low level lesions each time, the same tube can be reused if proper
tend to spare the legs but involve only the bladder clean precautions are taken. The catheter & hands
& bowel, manifesting as failure to empty. Higher need to be washed thoroughly before & after CIC
lesions may present with various forms of & the catheter should be dried from the outside &
paraplegia, often with upper motor neuron type kept in a dry container without folding it. Though
bladder dysfunction +/- DESD. Such patients CIC is easy to learn for a male patient, in female
present with storage symptoms +/- vesico ureteric patients a mirror needs to be used to help them
reflux. All these kids need rehabilitation in the form identify the urethral opening initially. Patients
of starting ISC & anti muscarinic agents right from should be given prophylactic antibiotics [such as
birth to avoid dangerous complication, especially Nitrofurantoin or Cotrimoxazol] in the initial
renal failure in later life. learning phase to avoid infection. In female patients
with severe leg spasms, special appliances can be
AIMS OF UROLOGICAL REHABILITATION made to keep the legs apart during catheterization.
• To protect the upper tracts [kidneys] function During travel or at work, such patients should keep
• Prevent urinary tract infections extra catheters with them to avoid reusing the same
catheter. However, in special situations, CIC is just
• To prevent development of complications such not the most practical option, In that case any of
as kidney stones the options mentioned below can be considered as
• To achieve continence. per the clinical situation.
Though to the patient the most important issue is Timed urination - Some patients with detrusor over
to have continence; that is to be able to store & activity can actually time their urination. They visit
empty urine at will; medically that gets less the toilet at fixed times & sometimes produce reflex
70 Neuro-Rehabilitation : A multi disciplinary approach

voiding by supra pubic tapping with a finger or by Commercially available as Botox [Allergan] or
manually pressing on the bladder. [Crede’s Neuronox [Ranbaxy], it is injected in the detrusor
maneuver]. Patients can also limit their liquid intake muscle to control over activity or in the external
after 6 pm to avoid or reduce the night urination. sphincter to reduce its spasm. Patients with resistant
form of detrusor over activity or those with DESD
Condom catheter – In a male patient with a supra
can benefit by this injection within the detrusor or
sacral injury, a condom catheter may be the most
sphincter respectively. Patients should be counseled
convenient management provided the bladder gets
about its temporary effect as well as about the need
emptied with each void & there is no autonomic
for ISC post such therapy. Patients with recurrence
dysreflexia. It is also the most convenient form of
of symptoms after Botulinum toxin injection can
management in those with leak due to a weak
be offered repeat injection with identiacl benefit as
sphincter.
the first injection.
Diapers - In female patients with severe neurogenic
Surgical management of neurogenic voiding
incontinence and in some incontinent males;
dysfunction - About 10% patients need some
diapers are a practical option. Diapers are available
surgical procedures to maintain their urinary tract
in the market with a holding capacity of up to 1.5
health. A detailed discussion on this is beyond the
litres. However in Indian weather, skin rashes &
scope of this article. However, augmentation
fungal infections are often the undesirable effect of
cystoplasty using a bowel patch [small or large
having a wet diaper constantly in contact with the
bowel] to increase the bladder capacity may be
skin.
required in severe cases of neurogenic detrusor over
Indwelling catheter – In quadriplegics without activity or a small capacity poorly compliant
round the clock care givers and in some paraplegics, bladder. All these patients have to be conversant
an indwelling urethral or supra pubic catheter [16 with the technique of self catheterization. This
Ch in size] may be the best & sometimes the only operation can be combined with creation of an
practical option. These can be changed monthly or abdominal catheterizable stoma for intermittent
3 monthly depending on the type of catheter. In catheterization, especially in spastic overweight
general incidence of infective complications is paraplegic female patients. [These patients
higher with an indwelling urethral catheter than otherwise may find it very difficult to self
with a supra pubic catheter. The urine drainage bag catheterize due to the location of their urethra.] An
can be a hindrance especially during physiotherapy artificial sphincter may be required for cases with
& mobilization. A belly bag or a leg bag is more sphincteric leak. Also neurosurgical procedures
convenient during these times. In special such as placement of nerve root stimulator or a
circumstances, a spigot can be used [to block the neuromodulator may be necessary in selected cases.
catheter] & released every 4 hours to empty the
bladder. This, though not the most ideal option; can Complications in Neurogenic Bladder
be considered if clean handling is ensured. With better understanding of urinary bladder
Medication for incontinence - As the commonest dysfunction, most complications can be avoided by
problem in neurogenic bladder is detrusor over individualizing the management strategies.
activity, anti muscarinic [commonly known as anti Infection - The commonest complication is urinary
cholinergic] drugs such as Oxybutinine or tract infection. Although these patients may have
Tolterodine can be given to prevent episodes of only vague symptoms such as increase in leg
frequency with leak. These drugs are also useful to spasms, frequent episodes of leak etc to indicate
control leak in between 2 catheterizations in those infections, presence of fever in a neurogenic bladder
on CIC & to prevent leak by the side of the catheter patient indicates urinary infection unless proved
in those with indwelling catheters. The commonest otherwise. Each attack has to be treated aggressively
side effect of these drugs is constipation & dry with appropriate antibiotics, often followed by
mouth. In patients with failure to empty, sometimes prolonged course of preventive suppressive agents.
alpha blocker group of drugs such as Tamsulosin Auxillary measures include ensuring adequate
or Alfuzosin are useful. water intake with daily urine output of at least 3
Botulinum toxin - This toxin when injected in a liters, following correct clean technique of CIC,
muscle, paralyses the muscle temporarily for a changing the indwelling catheters or diapers on
variable period from 6 months to over 1 year. time & ensuring proper perineal hygiene.
Spinal Cord Injury 71

Calculus disease- Though the most dreaded SEXUAL REHABILITATION OF


complication, its incidence is decreasing at least in SPINAL CORD INJURED MALES
countries with appropriate bladder rehabilitation
protocol. Prolonged immobilization, inadequate Can a Spinal Cord injured person lead a happy
water intake, repeated infection, presence of foreign Family life ? Undoubtedly yes !
bodies such as catheters, inadequate bladder The physical problems faced by the patients with
drainage, presence of dilated kidneys; encourage SCI are as:
calculus formation in these patients. Smaller
1. Loss of Erection is called Erectile Dysfunction
concretions in the bladder can be washed out but (ED).
larger calculi need appropriate endoscopic
management. [Cystoscopic or ureteroscopic or 2. Loss of Ejaculation is called Ejaculatory
nephroscopic removal. ] These patients may not Dysfunction (EjD)
need major anesthesia but their body deformities, Erectile Dysfunction : Occurs when the
spasms etc make them very challenging even for sympathetic pathways T11,12, l1,2 and
the most skilled urologist. parasympathetic pathways S2,3,4 are involved.
Renal insufficiency - Before the advent of CIC, Erectile Dysfunction can be treated with various
most spinal injury patients eventually succumbed modalities like
to renal failure! Thankfully that incidence has – Oral Erectogenic Drugs –
dropped substantially in recent times. In addition
to the above measures, annual sonography & – Intra-Penile Self Injections
creatinine assessment should be carried out in all – Vacuum Erection Devices (VED)
patients. Any rise in creatinine levels or – Penile Prosthesis
development of kidney damage such as
hydronephrosis must be referred to nephrologists 1. Oral Erectogenic Drugs : ( mainly
or urologist on time. Whenever needed, dialysis PhosphoDiEsterase type 5 Inhibitors) are:
should be started & in selected cases, even a kidney – Sildenafil
transplantation may be required. These transplants
– Tadalafil
have to be performed in centres specialized in
caring for such patients with compromised bladder – Vardenafil
function to ensure a good outcome. PDE5I have revolutionized treatment of Erectile
In summary – From the above text, it is clear that Dysfunction. Sildenafil citrate, presently is the most
patients with spinal injury need major urological commonly used oral erectogenic drug.
rehabilitation. With proper management protocols, PDE5Inhibitors (PDE5I) like Viagra and similar
most of the management goals can be achieved & drugs are available which is need to be taken only
especially the complication can be prevented. before sex but has mild to moderate side effects
Caring for these patient needs a team approach & which is to be taken only with doctor’s instructions
an urologist becomes an important member of that and is not the permanent cure. These oral drugs
team. The patient & his family members should be show side effects like headache, back pain,
counseled about the need for urological dyspepsia, myalgia, nasal congestion, pain in limbs,
rehabilitation right from the start. Needless to say, visual disturbances, flushing of face with nitrates
a sympathetic attitude of relatives, friends & contraindicated in any form.
colleagues at work goes a long way in boosting the One should know that PDE5I is not an aphrodisiac
morale of these patients who have to make a lot of i.e it does not increase libido nor make normal
adjustment with their body & life in general. erection harder or last longer.
[No specific reference is sited in this chapter as
2. Intra-Penile Self Injections:
all these treatment strategies are internationally
accepted with plenty of references available in the It is the clinician who decides what type of injection
literature. However as a comprehensive guide, the and the dose to be required. He gives the injections
reader is requested to refer to the latest edition of in prefilled syringes and trains the patient how to
Campbell’s Urology textbook.] take it at home or giving to his wife before sex. But
72 Neuro-Rehabilitation : A multi disciplinary approach

Fig1 : Intra penile Self Injection. Fig2. Injection Testing.

Fig 3. Self Injection. Fig 4: Wife assisted injection.

Fig 5: Basic design of Vaccum Erection devices. Fig 6: Constriction ring over the base of the cylinder.
Spinal Cord Injury 73

Fig 7: Constriction ring slipped over the base of the penis.

Fig 8 -10 After using the Vaccum Erection Devices.

Fig 11: Malleable & Flexible Penile Prosthesis.

Fig 12a: Placement in body.


74 Neuro-Rehabilitation : A multi disciplinary approach

Fig 12 b: Placement in body. Fig 12 c: Erect State.

Fig 12 d: Erect State. Fig 13: Lifting.

Fig 14: Inflatable Penile Prosthesis. Fig 15 a: Placement in Body.


Spinal Cord Injury 75

Fig15 b: Placement in Body. Fig 15 c: Erect State. Fig 15 d: Erect State.

Fig 17 a : Vibration Therapy.

Fig 16: Inflation.

Fig 17 b: Vibration Therapy. Fig 17 c: Vibration Therapy.

Fig 18: Seager's Electro Ejaculator.


76 Neuro-Rehabilitation : A multi disciplinary approach

Fig 18a: Seager's Electro Ejaculator Procedure. Fig 18 b: Seager's Electro Ejaculator Procedure.

Fig 19: Ejaculation in antegrade direction. Fig 20: Position with women on top.

Fig 21: Testicular sperm retrieval techniques done


percutaneous.
Fig 22: Intra Cytoplasmic Sperm Injection.
Spinal Cord Injury 77

it is not a permanent cure. The commonly used • Non-Inflatable


drugs for Self Injections are Papaverine and
• Inflatable
Prostaglandin E1 in combination with other drugs
like Phentolamine, Chlorpromazine, Atropine. Non Inflatable Type- These are solid rods of medical
grade silicon. There are two types again-
The Stepwise procedures done in Home Self
Injection Program include the following: • Malleable
1. Therapeutic dose determination by the • Flexible
clinician. Fig 11: Malleable & Flexible Penile Prosthesis.
2. Training & supervised self injection by Fig 12a: Placement in body. Fig 12 b: Placement in body.
the patient Fig 12 c: Erect State. Fig 12 d: Erect State. Fig 13:
3. Home self-injection program. [Link] 14: Inflatable Penile Prosthesis.

Patient’s Guide to Self Injection – A simplified The components of inflatable penile prosthesis are
pictorial guide to help the patient. as:

Fig1 : Intra penile Self Injection. Fig2. Injection Testing. 1. A pair of cylinders
Fig 3. Self Injection. Fig 4: Wife assisted injection. 2. A Reservoir
3. A Pump
Side effects:
Fig 15 a: Placement in Body. Fig15 b: Placement in Body.
The local side effects like noduleor small hematoma
Fig 15 c: Erect State. Fig 15 d: Erect State Fig 16:
can happen but most important and dreaded side
Inflation
effect is PRIAPISM which is a rigid erection lasting
for more than 4 hours. If not treated immediately, Thus with such wide ranging treatment modalities
it can cause permanent inability to achieve erection. available, erection dysfunction in a Spinal cord
injury male can be treated effectively.
3. Vacuum Erection Devices (VED): Factors such as age, motivation, financial status and
1. Time-tested. a cooperative partner plays an important role for
2. Non-invasive9. the individual patient.

3. For Select Population Ejaculatory Dysfunction :


BASIC DESIGN Spinal cord injury does not disrupt sperm
production but it affects the sperm transport to
1. Cylinder
outside. The spinal centers of S2,3,4 (Onuf’s
2. Vacuum pump nucleus), when they get involved in the spinal
3. Constriction rings injury, the ejaculatory dysfunction occurs. In most
cases, the sperm instead of coming antegrade
Fig 5: Basic design of Vaccum Erection devices. (outside) tend to go Retrograde (backwards) into
Fig 6: Constriction ring over the base of the cylinder. the urinary bladder. Hence inspite of being able to
Fig 7: Constriction ring slipped over the base of the penis. have sexual intercourse, the man cannot deposit his
Fig 8 -10 After using the Vaccum Erection Devices. sperms into the vagina leading to infertility.
Patients who have limited frequency coitus can be The main causes of infertility are as :
advised to have a combination of intra penile
injection and vaccum erection devices thereby 1. Inability to perform coitus.
avoiding surgery for prosthesis. 2. Inability to ejaculate intra-vaginally.
3. Altered seminal parameters due to infection.
Surgery of Penile Prosthesis :
To overcome these problems, the couple requires
When none of the abovementioned modalities are
doctor’s assistance. Hence we have Assisted
effective enough to make penetrative intercourse
Reproduction Techniques (ART) which are as
possible, then surgery for prosthesis is resorted to.
follows:
There are essentially two types of prosthesis
1. IUI (Intra Uterine Insemination) with sperm-
available.
preparations
78 Neuro-Rehabilitation : A multi disciplinary approach

2. IVF (In Vitro Fertilization ) 1. TESA – Testicular Sperm Aspiration


3. ICSI ( Intra Cytoplasmic Sperm Injection ) / 2. NAB – Needle Aspiration Biopsy
IVF
3. Trucut Needle biopsy
For every procedure mentioned above we need the
Fig 20: Testicular sperm retrieval techniques done
patient’s sperms.
[Link] 21: Intra Cytoplasmic Sperm Injection.
Modalities to recover sperms from SCI patients
IVF-ICSI procedure is very expensive and
include:
exhausting where only 30-35% chance of success is
1. Vibration therapy seen.
2. Electro-ejaculation ICSI has revolutionized the management of male
infertility including azoospermia.
3. TESA (Testicular Sperm Aspiration)
Thus Sexual Rehabilitation plays an integral part
Vibration therapy: is used to stimulate the
of total rehabilitation where the patient is guided
undersurface of glans thereby enabling the patient
correctly by giving the proper awareness of
to fantasy erotic pictures. The procedure takes upto
modalities of treatment available by the clinician.
one hour where patient sits comfortably. It requires
Socio-economic conditions also play a major role
privacy and needs upto four sessions.
in availing of the treatment to the patient.
Fig 17 a- 17 c.
If vibration therapy is not successful in producing PSYCHOLOGICAL REHABILITATION
semen from the SCI patient, then next modality OF SCI
used is: Spinal Cord Injury (SCI) leaves a major impression
on the person’s body and mind. A new spinal cord
Electro-Ejaculation under anesthesia: is a special
injury patient usually has many queries regarding
instrument which uses direct current for
his future and at the same time has a sense that
stimulation. SCI patients do not need anesthesia but
things are not going to be the same. A person who
the equipment is quite expensive.
had been leading an independent satisfying life
Fig 18: Seager’s Electro Ejaculator. Fig 18 a-18b. becomes immobilized, bowel and bladder
incontinence, loss of sexual functioning and
In this metal plate on the rectal probe is directed
becomes dependent on others for every small
towards prostatic plexus and the current in
necessity. The patient not only faces loss of body
increasing voltage (5-50 V) is delivered. At the
control but also experience changes in self worth,
optimum voltage for that particular patient,
ejaculation mostly occurs in antegrade direction. sense of independence, confidence, attractiveness,
sexuality, and relationship with family and friends.
Fig 19: Ejaculation in antegrade direction.
There are various stages that one goes through post
Fig 20 : Position with women on top.
spinal cord injury: 1) shock and denial 2) grieving
The sperms thus obtained are injected into his wife followed by depression or vice versa 3) anxiety /
on the day of her ovulation, resulting in pregnancy. frustration 4) anger /aggression 5) trying to adapt
If this procedure also is unable to result in to the situation. The patients may go through all
pregnancy, then further IVF techniques can be used the above stages or they might not go through the
where the sperms are directly collected from Testis stages given in the above order or they might skip
for test tube baby. Thus, these sperms are injected some stages.
in his wife’s mature eggs where they show
fertilization and when growth happens they are Shock and Denial:
transferred back into her uterus resulting in When the patients come in terms with what has
pregnancy. happened with them they are often not yet ready
to acknowledge, the extent or permanence of their
Obtaining Sperms directly from the testis for the
disabilities. At the other extreme, patients
purpose of IVF – ICSI (In Vitro Fertilization – Intra
sometimes build denial systems based on
Cytoplasmic Sperm Injection) includes testicular
unrealistically high hopes. Clear communication,
sperm retrieval techniques which are done
emphasizing realistic expectations before
Percutaneously by the following methods:
introducing treatment, may prevent some of these
Spinal Cord Injury 79

responses. They should be provided with the spinal cord injury/disease. A person may
information on obtaining recommended future. need to learn new skills because he cannot
perform his old job.
Greif and Mourning:
3. Withdrawing Emotional Investment:
Working through grief and loss is the way people
adjust to losses they have sustained. This The final stage of mourning involves the actual
adjustment requires emotionally "letting go" of "letting go" of life as it used to be before the
something that was valued but can't be replaced. It spinal cord injury/disease and investing
is only after letting go that the person is free to re- energies elsewhere. Perhaps the person learns
invest their emotions into new things. However, to participate in an activity that he did prior
letting go can be difficult, complex, and require a to hospitalization but now does it in a different
great deal of time. manner; or the person who was very "body"
oriented learns to use the "mind" more for
Tasks of Mourning: stimulation, satisfaction, and productivity.
Four tasks of mourning that a person needs to
4. Anxiety:
successfully work through while grieving.
A panic like reaction of initial recognition of
1. Accepting the Reality of the Loss: the enormity of the traumatic event takes place
Patients and families struggle long and hard as the patient is relatively unaware. As, the
with this reality. For some, the struggle and patient comes into terms with the injury and
disbelief continues for years and some never the consequences the level of anxiety
do accept the reality of their loss and will eventually subsides.
forever be in mourning. Their emotional
energy goes to fighting against the reality of Depression:
the spinal cord injury/disease instead of Depression is a common illness and it can affect
concentrating on strengthening and using the anyone. However, it is more common among SCI
remaining intact muscles and nerves. Those patients as about 1 in 5 people. Estimated rate of
who do accept this reality, even a part of it, depression among people with SCI ranges from
can then move forward to the next step. 11% to 37%. Krause, et al suggests that 48% of
patients with SCI in 1997 had clinical symptoms of
2. Experiencing the Emotional Pain Associated
depression at a year or more after injury. Another
with the Loss:
study showed that 60% of Portuguese patients with
Emotional pain is a reflection that something spinal cord injury have depressive symptoms.
which has been valued or treasured has been
lost. The emotions are often very strong and Suicide:
difficult to manage. Anger, sadness, In Denmark, a suicide rate is 5 times higher than a
hopelessness, fear and a sense of injustice are general population and it is not related to the
very common. Those individuals who do not severity of the injury. Suicidal tendencies are higher
successfully handle this task in the grief during the initial days after spinal cord injury.
process may, over the long term, lose
motivation and interest in most or all activities, Independence:
withdraw from friends and family, become Causes of depression after spinal cord injury and
bitter and resentful, stay depressed, develop found that social support and recent stressful events
poor personal hygiene or eating habits, can be used to identify patients at a high risk of
seriously abuse drugs and alcohol or depression but that they are less likely to become
deteriorate physically. Those persons
depressed if they are independent. Adjustment to
successful at this step can move forward to the spinal cord injury and quality of life can be
next. adversely affected by inadequate home facilities
2. Adjustment to the Environment despite that make a person more dependent (Seki, et al.,
Spinal Cord Injury: 2002). Expectations of independence decline
steadily with increasing age. In some patients, there
Adjustment means effectively learning to deal
are secondary gains in their dependent state,
with the world despite the changes caused by
though they may not be consciously aware of this.
80 Neuro-Rehabilitation : A multi disciplinary approach

Body Image: person with spinal cord injury when they talk about
Many spinal cord injury patients value the fact that the accident and the events that follow. Feelings of
they look "normal" except for the wheelchair. The helplessness and hopelessness, guilt, and
magnitude of disability may be "invisible." Patients depression often pervade their lives for a long time.
sometimes report that people stare at them more. There may also be differences between the
Their sense of "being different" and social responses of mother and fathers.
discomfort increases. Spinal cord injury patients
may not integrate disability into their self-concept Children:
for some time. The disruptive and impoverishing effects of spinal
cord injury on families, most people assume that
Adjustment: spinal cord injury of a parent has deleterious effects
Affective internalization, of the functional on children. Killen (1990) assessed roles of children
implications, of the disability along with in families after spinal cord injury and found that
behavioural adaptation to newly perceived life spinal cord injury did not change the roles, i.e.
situation. True adjustment and adaptation begins mothers, fathers, husbands, and wives continued
after discharge from rehabilitation. to play their traditional roles.

Family Issues: Marriage:


Spinal Cord Injury is a life altering event not only The marriage rate of people with spinal cord injury
for the person but also for family member. Family is lower than the general population but the factors
member also suffer from the various stages that a that influence marriage rate may differ from what
person goes through post spinal cord injury. is commonly assumed. Disability appears to exert
Findings suggest that the spouse of a person with a greater effect on the marital status of females than
spinal cord injury usually suffers from emotional males. Although medical complications have an
stress that is comparable to or greater than those of adverse impact on quality of life of adults with
injured partner. The spouse has to now take on pediatric-onset spinal cord injury, the presence of
overall charge of the patient, himself or herself and pressure ulcers, severe urinary tract infections, and
other family members. They normally have to spasticity have little effect on marriage rate of adults
juggle out time for everything. This leaves the with pediatric onset spinal cord injury. In contrast,
caregiver at a higher risk of physical and mental the presence of head injury has much more adverse
stress, burnout, anger, fatigue and resentment. effects on marital status.

Spouses: Sexual satisfaction:


The burden of caregiving most frequently falls on Spinal cord injury obviously impairs sexual
the spouse. Caregiving spouses are often severely function. However, sexual satisfaction does not
stressed, particularly due to health issues that arise appear to be related to physical factors such as
after spinal cord injury. Caregivers have a higher erectile function, genital sensation, or orgasmic
incidence of physical stress, emotional stress, capacity as much as perceived partner satisfaction
burnout, fatigue, anger, and resentment and relationship quality (Phelps, et al., 2001). Fisher,
(Weitzenkamp, et al. 1997) than their partners or et al. (2002) assessed sexual function in 40 people
spouses who are not the caregivers. Chan, et al. (32 men, 8 women) with spinal cord injury. By 6
(2000) report that the impact of spinal cord injury months after discharge from hospital, most of the
is more severe on marriages that began before than participants in the survey had made significant
after the injury. changes in sexual behavior and activity. Most of
the respondents had realistic concerns coupled with
Parents: more requests for sexual health intervention.
However, many were engaging in sexual activity.
When a person is young and not married, the
In Iceland, 55.5% of spinal-injured people are
burden of caregiving frequently is taken up by the
married and 71% had an active sexual life after
parents. There is often nobody else to help ease out
injury (Knutsdottir, 1993). Thus, sex is important
the burden of older parents. Parents often do not
but is not necessary for sexual satisfaction or sexual
forget the incident for years and decades. It is not
activity, and is frequently not the most important
unusual to find tears in the eyes of a parent of a
factor in life satisfaction.
Spinal Cord Injury 81

Psychological Assessments: for the patient to tell his life-story, which enable
Psychological assessments are conducted on the him to gain a sense of order and perspective. It also
patient to gauge the level of impact that the injury helps the patient to vent out his emotions and
feelings regarding the event when spinal cord
of the spinal cord has caused. As, this is a life
injury took place. Also, the patient is able to discuss
changing event which causes the patient to make
with the psychologist the emotional issues
constant adjustments and impacts all the sphere of
regarding family, work, and physical state.
life, hence evaluation of all the aspects is required
to see the coping mechanisms of the patient. Individualised Psychotherapy:
• Becks Depression Inventory (BBDI – II): This Hope is a potentially important coping strategy for
is a 21-question multiple-choice self-report both the person and family with spinal cord injury.
inventory, one of the most widely used Goal-directed hope based on realistic perceptions
instruments for measuring the severity of of life, focusing on progress, positive interpretation
depression. Each answer is scored on a scale of events, and goal setting are important in helping
value of 0 to 3. The cut-offs used differ from: people and families cope with spinal cord injury.
0–13: minimal depression; 14–19: mild The psychologist can help the team of therapists to
depression; 20–28: moderate depression; understand the patient's stage of adjustment, and
29–63: severe depression. provide consultation on behavioural management
approaches [3]. Emotional responses dealt with by
Higher total scores indicate more severe depressive psychotherapy include a range of ego defences,
symptoms. most commonly repression and denial. Typically,
• Stress: The Depression Anxiety Stress Scales as denial decreases over time, depression, anxiety,
(DASS): [1] is made up of 42 self report items and anger increase. How these emotions are
to be completed over five to ten minutes, each expressed depends largely on the patient's
reflecting a negative emotional symptom [ 2 ]. premorbid personality style. Psychotherapy can
Each of these is rated on a four-point Likert help via reinforcing adaptive coping skills and
scale of frequency. These scores ranged from teaching new coping strategies. The psychologist
0, meaning that the client believed the item may also work with the interdisciplinary team to
"did not apply to them at all", to 3 meaning develop behavioural modification programs, based
that the client considered the item to "apply to on learning theory, to decrease these behaviours.
them very much, or most of the time". It is also Contingency management and behavioural
stressed in the instructions that there are no "contracting" are, most frequently used in
right or wrong answers. rehabilitation settings [4]. Approaches emphasizing
positive reinforcement to "shape" desired
• Quality Of Life Questionnaire: The SF-36: behaviours are particularly effective. Cognitive
This is a multi-purpose, short-form health therapy is used to help the client overcome the
survey with only 36 questions. It yields an 8- negative and distorted view of himself or herself
scale profile of functional health and well- or people around him or her.
being scores as well as psychometrically-based
physical and mental health summary Group Therapy:
measures and a preference-based health utility Psychological treatment of spinal cord injury often
index. includes group psychotherapy, which is an
• Suicide Risk Questionnaire to assess the excellent method to both maximize patient learning
suicidal ideation in a person with spinal cord and efficiently use therapist time. Patient groups
injury. can provide emotional support, peer role models;
• Hamilton’s Psychiatric Rating Scale for teach new coping skills, and decrease social
discomfort. Likewise, multiple-family group
Depression to assess the severity of depression.
psychotherapy is a powerful and effective tool for
• Hamilton Anxiety Rating Scale is conducted facilitating family adjustment to spinal cord injury.
on the patients to gauge the level of anxiety in Family members experience similar emotional
the patients. responses to the patient and similarly benefit from
psychological intervention. If not included in the
PSYCHOLOGICAL INTERVENTION:
team effort, a well-meaning family member could
Psychological intervention is important as it acts inadvertently sabotage the independence-oriented
like emotional catharsis, and gives the opportunity rehabilitation approach, or be too psychologically
82 Neuro-Rehabilitation : A multi disciplinary approach

distressed to provide the emotional or physical care She is a Management Educationist, Editor &
the patient needs. Associate Dean, Research, Welingkar Institute of
Management Development & Research, Mumbai.
Sexual Counseling:
Email: ninafoundation@[Link]
Establishing a healthy sexual relationship may Website: [Link]
require professional help. Couples or individuals
who get sexual counseling can learn effective ways I am Ketna Mehta, and I met with a paragliding
to communicate feelings. Patients who are accident on 12th February 1995 leading to a T12
wheelchair bound usually are embarrassed about burst fracture. That’s when I came to know that God
their body and their physical state and hence they wills differently for me, because my life lay totally
need individual psychological session to help open changed from a completely independent, working
up about their feeling and be comfortable or find professional to being dependent on my near and
solutions in their condition. Studies show that males dear ones even for my basic chores, initially after
with spinal cord injury want information about the accident.
sexual issues. Those who receive the proper Little did I know that I would be able to walk again
information have more positive sexual with the help of a walker and come to lead a life
relationships. that I can call independent. It only became possible
due to the help and support of my family, medical
Caring for the Caregivers: team and consistent efforts of the Spinal Cord Injury
Caregivers, play a major role in the life of patients (SCI) – REHABILITATION TEAM
with spinal cord injury. As, caregivers are mostly
It was like being reborn, re-learning how to perform
looking after the patient, all the time they should
every single activity of daily living in a new way,
avoid having burnouts. Hence they should find
to adjust to my new self. But yes, I can surely say,
time for themselves and their recreational activities,
as toddlers our parents rejoiced every new step we
because if the caregivers are emotionally and
took towards growth when we had no clue as to
physically well balanced they will be able to attend
why we were being hugged and kissed for standing
to the patients need in a better way. Also, they
up or walking those baby steps. Looking at the
would be able to give their maximum support in
brighter side of my disability I got to rejoice and
improving the patients condition.
celebrate every new step I took towards
Other Therapies: independence.
Other issues which need to be routinely addressed Spinal Injury (SI) is an aspersion to the spinal cord
by the psychologist, in conjunction with the resulting in a change, either temporary or
rehabilitation team, are vocational rehabilitation permanent, in its normal motor, sensory, or
and pain management training. Prevention of autonomic function. People affiliated with Spinal
medical complications particularly, those which Injury usually have permanent and often
have significant behavioural/emotional devastating neurologic deficits and disability.
components, need to be emphasized. For example Spinal Injury is a high cost disability leading to
pressure sores, which often occur when depression drastic changes in an individual’s life. Due to the
and/or substance abuse lead to poor self care. many changes in the life of a person with spinal
injury emotional and psychological support
REHABILITATION – PERSPECTIVE becomes an essential factor. Also, the financial
OF A SPINAL CORD INJURED impact of Spinal Injury is extremely high as the
ACHIEVER disability leads to lengthy hospitalization, medical
complications, extensive follow up care and
Dr. Ms. Ketna L. Mehta, PhD. is a founder Trustee recurrent hospitalizations.
of a 10 year old NGO, Nina Foundation –
Rehabilitation of people with Spinal Injury. They Rehabilitation is a reiterative, active, educational,
have received prestigious awards such as NCPEDP problem solving process focused on a person’s
Shell Helen Keller Award, NASEOH Award, behaviour (disability), with the following
featured in the Limca Book of World Records and components:
empowered over 600 persons with Spinal Cord • Assessment – Persons Problem
Injury. • Goal setting
Spinal Cord Injury 83

• Intervention – Treatment and Support me through the journey I had I felt reassured
psychologically.
• Evaluation – to check the effects of
intervention. At Hinduja hospital, to my greatest luck Dr. S. Y.
Bhojraj, an ace spine surgeon was available and he
The rehabilitation process aims to:
examined me and arranged for MRI, X-rays and
• Maximize the participation of the person other investigations and fixed me for surgery in the
in his or her social setting same night itself. To have the best doctors both at
• Minimize the pain and distress the site and hospital according to me was a critical
experienced by the person aspect of my rehab journey.

• Minimize the distress of and stress on the After the surgery, Dr. S. Sagade, my Urologist
person’s families and corers. gently informed me about my bladder situation and
advised me to drink over 2 liters of water each day
A rehabilitation service comprises a and keep a tab on the urine output. I had an
multidisciplinary team of people who indwelling catheter for 20 days in the hospital.
• Work together towards common goals for I was informed by Dr. Bhojraj that the surgery was
each person for decompression and stabilization by a Steffi plate
• Involve and educate the person and and 4 screws. I was also told that I was on a
family waterbed in the hospital and that I should turn
every two hours on my sides to avoid bedsores.
• Have relevant knowledge and skills
Later I was put on regular mattress and was asked
• Can resolve most of the common to change positions frequently.
problems of their persons.
I was put on regular physiotherapy at the hospital
This definition emphasises the importance of the by Sharon Vakharia my first Physiotherapist. She
team skilled in achieving the professionally made me exercise regaling me with funny
perceived clinical outcome for the individual with anecdotes and jokes. It was fun time everyday. She
spinal injury. also told me about two other people with spinal
injury in the ward and urged me to visit them once
On 12th Feb 1995 at 12 noon, on the outskirts of
I was mobilized on a wheelchair. My urge to
Mumbai, I was in a paragliding camp and this was
motivate and help others started right from the
my last flight. I trekked up the 40 feet hill and took
hospital. My sister Nina started me on homeopathic
off. Suddenly the wind turned and I swerved to
medicine immediately to aid my neurological
my right without control crash-landing on the rocky
recovery and keep me in a happy and jovial frame
ground with huge impact. T12 burst fracture with
of mind. My movements, sensations, bladder and
para paresis, was the verdict. I assimilate important
bowel were not functioning.
pointers which were favorable in my journey of
Rehabilitation (practical aspects of SI rehabilitation): I was discharged from the hospital after 20 days
stay.
I was carried in the big tent cloth supine with four
people holding the cloth on either side. My house was on the first floor and had to be
carried up physically.
I was laid out straight on my back on rear seat of
the ambassador car from the site of injury up to the Dr. Milka Vivek was my 2nd Physiotherapist who
town. (How we are handled at the time of accident attended me at home. She gave me a lot of courage.
is very important. I was fortunate!) Later, Dr. V. C. Jacob from Sion Hospital came home
to help me out. He was attending to me for 2 long
I was taken to Dr. Riten Pradhan an astute and
years. His experience with SCI was indeed
knowledgeable orthopedic surgeon whose hospital
beneficial for me.
was in Virar. He examined and diagnosed my
condition as Spinal cord injury. He arranged to Dr. Jacob taught me several tricks – climbing stairs
transfer me to Hinduja Hospital. He took me by by personally getting railings installed, he ordered
train to avoid jerks and bumps of the Mumbai good thigh high calipers with shoes which locked
roads. He had administered methyl prednisolone at the knees, then graduating to ankle high shoes
which I was told would help in the recovery if taken and also gave me a goal to help others with SI. To
within 6 hrs after the trauma. Since he accompanied have an ace senior physiotherapist to guide, advice
84 Neuro-Rehabilitation : A multi disciplinary approach

and correctly assess my condition was a very My mind was active professionally too. I had my
important aspect of my rehab. He also took me to management consultancy and continued with my
Paraplegic Foundation a charitable organization for market research projects – I had a PC & workstation
comprehensive care for SCI persons and told me to and would work using the phone, hold meetings
dedicate one day of the week for my other friends at home. It’s an important aspect of rehab to be
with spinal injury. I shared, counseled and learnt active and do what we enjoy.
about their issues and worked out solutions.
With the interaction with the Rehab team I could
learn a lot about the SCI, its complications like
Education and Counseling by a Physiotherapist:
Osteoporosis, Hypercalciurea, Urinary calculii and
A person with spinal injury requires extensive their management. I also learnt the importance of
physiotherapy to gain strength, lost muscle tone dynamic weight bearing, risk of developing
and to become largely functional again. During the contracture and so on. I feel that it is the duty of
treatment a physiotherapist plays a vital role in Physical Therapists to explain to the SCI persons
bringing about a positive approach towards the the benefits of Exercises and the complications that
treatment of the person. A caring touch, constant could happen in the absence of it.
reassurance and reaffirmation from the
physiotherapist help the person to develop a It would be ideal to do a total period of 6 hours of
positive approach as well as to look at the brighter exercises for 6 days a week for 6 months regularly
side of life. The person needs to be explained the and then continue for 2 years with other activities
nature of the treatment, need for compliance and also which includes walking long distances (even
the importance of the regularity of the exercise. in the crowd) and various sports activities.
My sister and brother -in- law being doctors taught
me safe Clean Intermittent Catheterization (CIC)
method. I slowly learnt to manage myself.
Eventually, I could manage both the bladder and
bowel program myself.
Dr. Jacob urged me to teach other female spinal
injury persons the art of doing self catheterizations,
which I did very proudly.
I took part in various sports activities. Sports are
meant not only for recreation but also for improving
strength and balance. No time should be devoted
for idleness and self pity please! There should be
social outing to parks, beaches, sea faces, movies,
plays, wedding, temples etc. Each outing should
She/he is informed about the condition and the be done with a sense of adventure. Never should
likely outcomes. A team approach including feel embarrassed about the disability or the
physiotherapist, occupational therapist, social appliances.
worker and person with Spinal Injury herself/
himself will help the person gain not only physical Travelling was part of my profession. I even
independence but economic independence and ventured travelling by public transport. People of
social acceptance as well. The relatives and person Mumbai in general would always give a helping
with Spinal Injury are as much a part of the team hand if you ask for it. I have no hesitation to hold
as the professionals and must be considered at all on to two persons’ shoulders and climb up or down.
times, because eventually it is they and the person Therefore found that nothing is inaccessible. “God
herself/himself who will share the responsibility bless you” became my lexicon.
for the success or otherwise of attempts to restore I took several trips abroad for holidays with my
her/him for an independent life in the community. family. I even took a solo trip to Thailand for 7 days
In the case of the most severely disabled person the to attend a conference. This was a major adventure.
relatives may be able to undertake nursing care at It certainly boosted up my morale further, feeling
home after suitable instructions and with the a sense of achievement!
necessary equipments.
Spinal Cord Injury 85

Sports and Recreation have helped me in my mental the entire team is to make the treatment sessions
health. Playing Wheelchair games like Throw ball effective and enjoyable. All one needs is to explain
and W/C tennis also gave me a lot of pleasure. to the person with spinal injury that they need to
give it a try, work up ways and means, ask for help
Levels of Rehabilitation whenever required and not lose hope and calm; all
(Source: “Market Potential Study for a World Class they need to have in their mind is that they need to
Spinal Cord Injury Rehabilitation Centre in reach their goal. There is just a need to stimulate
Mumbai” PhD thesis by Dr. Ketna Mehta – India, that urge for living and reignite the fire for setting
2008.) goals and achieving them in a manner possible to
them.
A physiotherapist becomes a friend, confidante,
story teller, advisor and life coach. The trust reposed
on the physiotherapist is immense and with
tremendous patience can resurrect the life of the
person with Spinal Injury bit by bit by bit – always
being positive & keeping hopes alive.
My second life started after an accident at the time
of an adventurous sport, but my adventure
continues with a sense of achievement after going
through a successful Rehabilitation.

Summary:
Nina Foundation ([Link]) is an
This chapter has dealt with almost all aspects of
NGO founded by us in 2001 to spread awareness
rehabilitation of SCI with special emphasis on team
about prevention & rehabilitation of people with
approach . Management of the SCI is a complex
Spinal Injury. We also conduct educational
and challenging task, if all members take it up with
seminars at various physiotherapy colleges,
commitment, great things can be achieved in
sensitizing the budding Physiotherapists.
different stages. Frequent communication among
Rehabilitation plays a major role in the life of a the team members , patient and family is the key
person with spinal cord injury. factor and this alone can bring the person back to
Having a spinal injury is a life changing event for the society and lead a dignified and meaningful life.
both the person and their loved ones. The aim of

PUSH UP PULL UP
86 Neuro-Rehabilitation : A multi disciplinary approach

TRANSFERS FROM BED TO FLOOR READY TO CRAWL

REACHOUTS IN ALL FOURS

WALKING WITH CRUTCHES WALKING WITH A WALKER


Spinal Cord Injury 87

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92 Neuro-Rehabilitation : A multi disciplinary approach

Section 2
Brain and Spine Related Disorders
Ch.2 Stroke
Dr. Mamta Lohia, [Link]., F.N.R., C.B.E.(USA), Dr. Hema Biju, [Link],(Neuro),
Ms. Akshata Shetty, M.A. (Clinical Psycologist)

Definition: 2. Emboli: These are free flowing bodies in the


cerebral blood stream in the form of dislodged
Stroke is defined as sudden neurological deficit thrombus, fats, air, tissue particle, etc which
caused by focal vascular lesion in the brain. The gets trapped at any one point along their
term 'stroke' is synonymous with cerebrovascular course, frequently at the bifurcation of the
accident (CVA) and is purely clinical definition arteries, & cause occlusion to the cerebral
which, according to the World Health Organisation, Vessel.
can be defined as a 'rapidly developed clinical sign
of a focal disturbance of cerebral function of 3. Hemorrhage: It occurs due to the rupture of
presumed vascular origin and of more than 24 the blood vessels in the brain, commonest type
hours duration' Included within this definition are being subarachnoid haemmorhage. Following
most cases of cerebral infarction, cerebral hemorrhage tissue death occurs due to both
haemorrhage and subarachnoid haemmorhage, but ischemia & mechanical injury to the brain
deliberately excluded are those cases in which substance as a result of compression by the
recovery occurs within 24 hours. These are clot. Hemorrhage usually occurs either due to
designated as 'transient ischaemic attacks' (TIA). hypertension, arteriovenous malformation or
The vascular lesion could be either hemmorhagic due to trauma.
or thromboembolic phenomena (leading to
ischaemia) involving the blood vessels supplying
various parts of the brain. The extent of neurological
involvement may range from mild motor deficit to
gross involvement of various function, namely
Sensorimotor, perceptual, emotional, behavioural,
memory intelligence, speech & language function.

Etiology:
The brain is a unique organ in which functioning
of the neurons depend on a continuous blood
supply because metabolism is almost exclusively
aerobic. Different mechanisms have been found to CT Scan Brain showing Hemorrhage
cause vascular insufficiency to the brain resulting
in stroke. However, the most common causes are: There are some risk factors that can predispose to
stroke. The common ones are diabetes, high blood
1. Thrombus: This is mainly due to the presence pressure & cardiac disorders. Modifiable risk
of atherosclerotic plaque in the cerebral factors are cigarette smoking, obesity, sedentary
arteries as a result of severe platelet adhesion, lifestyle & excessive alcohol intake.
fibrinous coagulation & decreased fibrinolysis
activity. Pathophysiology of Cerebral Ischemia &
consequent infarction:
The two pathophysiological changes leading to
cerebral infarction are loss in the supply of oxygen
& glucose due to vascular occlusion, & various
changes in cellular metabolism resulting in
consequent collapse of energy producing processes
with disintegration of cell membrane.
Complete occlusion to brain substance causes
severe damage to it with a zone of infarction, which
is however found to be smaller than the actual area
MRI Brain showing gliotic infarct supplied by the involved artery. The margin of this
94 Neuro-Rehabilitation : A multi disciplinary approach

infarcted zone consists of cells that are alive but Neurovascular Syndromes:
metabolically less active. These surrounding areas Cerebral Blood flow (CBF) is controlled by a
are termed as ischemic penumbra. These areas are number of autoregulatory mechanisms (cerebral)
nourished by meningeal collaterals.
that modulate a constant rate of blood flow through
The necrotic tissue swells rapidly mainly due to the brain. These mechanisms provide homeostatic
excessive intercellular water content. Also, lack of balance, counteracting fluctuations in systolic blood
oxygen is another factor that could contribute to pressure while maintaining a normal flow of 50 to
swelling. This vascular lesion to the brain causes 60 ml / 100 gm of brain tissue per minute. The brain
release of neurotransmitters like glutamate & has high energy requirements & very little
aspartate by the ischemic cells, which excites metabolic reserves. Thus, it requires a continuous
neurons & produces an intracellular influx of Na & rich perfusion of blood to deliver oxygen & glucose
Ca leading to irreversible cell damage. Thus recent to the tissues. Cerebral blood flow represents
research attempts at blocking this action of approximately 17 % of available cardiac output.
gluatamate & aspartate on nearby cells, which will
Depending on the areas of the brain & the arteries
reduce the secondary involvement of surrounding
affected clinical symptoms vary.
viable cells.
Cerebral edema begins within few minutes & A) Occlusion of proximal Middle Cerebral
reaches a maximum by about 4 days, however it Artery (MCA)
mostly disappears by 3 weeks. This edema can It produces extensive neurological damage with
increase the intracranial pressure & can even cause significant cerebral edema & list of clinical
contralateral & caudal shift of brain structure. manifestations called MCA Syndrome:

Structures involved Neurological deficit


Motor area of face, arm, and fibres descending Paralysis of contralateral face, arm and leg
from leg area to enter the coronal radiata
Somatosensory area of face, arm and face leg Sensory impairment over contralateral and leg
Motor (Broca's) area on dominant hemisphere Motor speech disorder
Central language area and parieto-occipital Central aphasia, word deafness, anomia, jargon
cortex of the dominant hemisphere speech, alexia, agraphia, acalculia, and finger
agnosia
Non-dominant parietal lobe Perceptual disorder like unilateral neglect,
anosognosial unawareness of hemiplegic side,
apraxia, and spatial disorganization
Occipital lobe Homonymous hemianopia and loss of conjugate
gaze to the opposite side
Parietal lobe Ataxia of contralateral limbs
Bilateral frontal lobe Brun's ataxia or apraxia of gait
Supramarginal gyrus or inferior parietal lobe Loss or impairment of optokinetic nystagmus
Posterior limb of internal capsule and adjacent Pure motor hemiplegia without sensory and
corona radiata visual involvement

B) Clinical Manifestations of Anterior Cerebral Artery Occlusion are as follows:


Structure involved Neurological deficit
Motor leg area involvement of arm area Paralysis of opposite foot and leg involvement
of opposite arm although this is rare
Sensory area of foot and arm Cortical sensory loss over foot and leg
Stroke 95

Bilateral involvement of posteromedial part of Urinary incontinence


superior frontal gyrus
Medial surface of posterior frontal lobe Contralateral grasp reflex, sucking reflex and
gegenhalten (paratonic rigidity), frontal tremor
Severe frontal lobe infarction Memory loss and behavioral impairments
Supplementary motor area of dominant Aphasia
hemisphere
Corpus callosum Apraxia and agraphia
Bilateral motor area of leg Cerebral hemiplegia

C) Clinical Manifestations of Posterior Cerebral Artery Occlusion are as follows:


Structure involved Neurological deficit
Thalamus Hemianesthesia (contralateral sensory loss) or
thalamic sensory syndromes (unpleasant
hemibody sensation with spontaneous pain)
Occipital cortex Homonymous hemianopia, visual agnosia,
prosopagnosia
Bilateral occipital cortex involvement Cortical blindness
Temporal lobe ischaemia Amnesic syndrome with memory defect
Midbrain Skew deviation, athetoid posturing, postural
tremor, hemiballismus)
Cerebral peduncle Contralateral hemiplegia
Motor tract between red and vestibular nuclei Decerebrate attacks

D) Lacunar Syndromes:
Lacunar syndromes are caused by small vessel disease deep in the cerebral white matter (penetrating
artery disease). Lacunar syndromes are consistent with specific anatomic sites.
Structure Involved Neurological deficit
Posterior limb of internal capsule, pons & Pure motor lacunar stroke
pyramid
Venterolateral thalamus or thalamocortical Pure sensory lacunar stroke
projections
Base of pons, genu of anterior limb or the Dysarthria / Clumsy hand syndromes
internal capsule.
Pons, genu of internal capsule, coronal radiate Ataxic Hemiparesis
or cerebellum.
Junction of internal capsule & thalamus Sensory / Motor Stroke
Putamen, global pallidus, subthalamic nucleus. Dystonia Movements

Synergy Patterns:
Stroke being a central Nervous system disorder, mass movement pattern, called Synergy patterns.
following the acute stage of flaccidity, there is Synergy can be either flexor or extensor. Synergy
severe spasticity in the affected parts of the body. normally involves abnormal movement patterns
This leads to abnormal, stereotyped, primitive, that are not useful for functional activities.
96 Neuro-Rehabilitation : A multi disciplinary approach

Synergy type Upper Limbs Lower Limbs


Flexion Synergy Shoulder girdle retractor and Hip flexion abduction and lateral
elevation, shoulder abduction, rotation, knee flexion, dorsiflexion
external rotation, supination and inversion
flexion of elbow, wrist and finger
flexion.
Extension Synergy Shoulder Girdle protaction and Hip extension, adduction, internal
depression, Shoulder adduction, rotation, knee extension, ankle
internal rotation, elbow extension, plantarflexion, inversion and toe
pronation, wrist and finger flexion. plantarflexion.

The abnormal hemiplegic attitude is due to


combination of strongest component of the flexor
and extensor synergy in both upper and lower
limbs. The strongest components for the upper limb
is the flexor synergy and for lower limb is extensor
synergy. Hence, a hemiplegic patient has an
attitude of shoulder retraction and depression,
shoulder adduction and internal rotation, elbow
flexion, pronation and wrist and finger flexion. The
lower limb adopts an attitude of pelvic rotation, hip
extension, adduction and external rotation, knee
extension, and ankle plantarflexion and inversion.
Abnormal Synergy patterns of lower limbs leads
to abnormal Gait:

Comparison of Normal and


Hemiplegic Gait :
Hemiplegic stance.
Walking is an independent, automatic, symmetrical
and economical event. During a normal walking Under normal circumstances walking is an
there is both vertical and horizontal displacement automatic process which requires contribution and
of CG in the form of a sinusoidal wave that causes integration of various mechanisms, however after
horizontal displacement of 1.7 inch and vertical the brain damage like in cases of stroke all the
displacement of 1.8 inch. The gait determinants are automatic adaptation is lacking and the patient tries
as follows: to compensate for it by putting more voluntary
efforts. The phasic action of muscle group seen
• Knee flexion at heel strike on loading which normally is replaced by abnormal muscular
lowers the CG. combination characteristic of primitive reflexes. The
• Pelvic tilting flexion and extension synergy interferes with the
walking and greatly influence the hemiplegic gait.
• Pelvic Rotation which increases with increase
in the heel strike. The abnormality of gait in hemiplegic patient can
be compared with normal gait taking into
• Foot ankle mechanism
consideration the contribution of the joints for
• Knee flexion on heel rise or heel off. various phases of gait cycle.
• Lateral displacement of pelvis
Ankle Joint:
Early stance (heel strike to foot flat ): In normals
when the heel strikes the ground, the angle at the
ankle is 90 degrees once the weight is transmitted
to the fore foot, the sole is gradually lowered which
Stroke 97

is controlled by eccentric contraction of the controlled by the eccentric contraction of the


Dorsiflexors to prevent slapping of foot. quadriceps to prevent buckling. Immediately
In hemiplegics, the anticipation of the weight afterwards due to concentric work of quadriceps
bearing excites the extensor synergy which induces there is extension at the knee.
plantar flexion instead of dorsiflexion because In hemiplegic patient in flaccid stage there can be
dorsiflexion is not a part of the extensor synergy. buckling due to weight transmission. In stage of
The hemiplegic limb comes down on the entire sole spasticity, the initial flexion does not take place and
and if the plantarflexion is marked then on the toes. the knee continues to remain in hyperextension
Thus the heel strike is completely missing in throughout. Some patients do have a tendency to
hemiplegics. If there is pronounced inversion there buckle initially but immediately rectify by going
is more weight borne by the lateral aspect of the into hyperextension.
foot and when it is moderate it gets corrected on
weight bearing. Late stance :
In normals knee flexes with plantarflexion to give
Mid stance: momentum to the swing phase.
In normal when the sole is firm on the ground the
In Hemiplegics the strong linkage between
leg starts pivoting forwards above the ankle causing
quadriceps and plantarflexors prevents the swing
a relative dorsiflexion. This happens in response to
and the affected limb is moved forward by other
the weight shift from hindfoot to the [Link] compensatory mechanism.
movement of relative dorsiflexion is controlled by
eccentric work of the plantarflexors which Hip Joint :
gradually gets elongated.
Early to midstance:
In hemiplegics when the calf muscle is stretched
by body weight, it stimulates the stretch reflex In normals during weight bearing the abductors of
the hip prevent the pelvis from sagging on the
causing increase in spasticity of plantarflexors and
opposite side along with trunk flexors. The hip
it prevents any further stretch. Thus the forward
extensors work from heel strike to midtsnace. The
shift is prevented in hemiplegics.
short burst of activity reduces just before midstance.
Late Stance (Unloading to push off ) : In hemiplegics due to activation of extensor
In normals towards the end of the stance phase the synergy, the adductors contract in place of
heel raises from ground and the knee flexes. This is abductors and hence there is tredelenberg's sign.
achieved by forceful concentric contraction of Moreover when the adductor spasticity is very
plantarflexors for initiating a forward swing. severe the affected leg is placed very close to normal
limb or may even cross over interfering grossly with
In hemiplegics, the knee does not flex due to lack the forward propulsion.
of dorsiflexion at the ankle, in the earlier phase and
also due to influence of quadriceps spasticity. Swing Phase:
Sometimes even if it does flex it flexs gradually.
In normals this begins in the late stance and is due
Moreover the combination of plantarflexion with
to combined effort of muscles, gravitational force
knee flexion neither belongs to flexion or extensor
and [Link] studies have shown that
synergy, hence is very difficult for a hemiplegic to
muscle activity level is minimal. The hip and knee
propel himself. Due to lack of push off in
flexors along with Dorsiflexors act to help in
hemiplegics the force required to initiate a swing is
adequate ground clearance.
very less that causes slowness in walking.
In hemiplegics the extensor synergy does not let
Knee Joint: go its grip and if it does then it is very gradually so
that the body moves forward in slow manner. Hip
Early and midstance: and knee flexion is absent because of plantarflexion
In normals as heel strikes the ground the knee is at the ankle and retracted pelvis. Thus, there is
extended but as the body weight shifts on to the inadequate ground clearance. In the absence of
loading foot, there is 15 degree flexion at the knee forward rotation of the pelvis and hip flexion, the
under the influence of body weight and it is limb is brought forward by circumduction.
98 Neuro-Rehabilitation : A multi disciplinary approach

Assessment of Stroke Patient : Earlier the adult central nervous system was
considered to be rigid and unalterable. Stroke
Detailed medical history, emphasizing on the onset, rehabilitation was focused on compensatory
type of stroke, cause, territory of the brain affected. approaches for motor impairments and most
Following which examination of patient to confirm clinicians believed that therapeutic interventions
the extent of involvement and examine the had little impact on the process of neurological
voluntary control in limbs. Thorough assessment recovery. But research indicates that the adult
of Sensorimotor control is also mandatory. Few central nervous system has great neuroplasticity
assessments scales are used as a standard tool : and tremendous potential for reorganization, which
STREAM Scoring. has offered new hope to those treating patients with
long-term disability and underlies the increasing
The Brathel Index.
interest in finding new and more effective ways to
Functional Independence Measure (FIM) maximize this potential. In the recent years, it has
become clear that a variety of interventions have
NIH Stroke Scale Work sheet
the potential to favorably influence motor recovery.
([Link])
The ICF model provides a conceptual basis and a
Rehabilitation after Stroke: universal common language for understanding and
describing patients' health status, reaching beyond
Stoke rehabilitation has experienced tremendous
mortality, diseases, and rehabilitation. In the field
growth in recent years. Much of the impact of stroke
of neuro-rehabilitation, it facilitates
on functional independence is due to its effects on
multidisciplinary team communication, to structure
motor function. One of the main reasons of the
the rehabilitation process, for goal setting and
impaired quality of life of these patients is not only
assessment, for documentation, and for reporting.
related to the actual lesion but also to the result of
immobilization and segregation from the ICF model defines various problems at four levels:
community. a) pathophysiology of the HEALTH
The type of rehabilitation and outcomes are CONDITION,
determined by a variety of clinical and social factors, b) impairments at the BODY FUNCTIONS/
including length of time since injury, level of STRUCTURE level,
dependency, characteristics of the residual c) disability at the ACTIVITY level, and
impairment, age of the patient, and resources d) handicaps at the PARTICIPATION level.
available. Based on the first three levels, Levin et al
defined recovery and compensation to explain
Is there any scope of remedial training in the differences and allow better understanding
stroke? about motor recovery after stroke.

ICF Level Recovery Compensation


Health Condition: Restoring neuronal function in the Neural tissue acquires a function
Neuronal Level penumbra (i.e., around the primary that it did not have prior to injury.
lesion) and in the disachisis, which -Abnormal activity in alternative
was initially lost due to injury. brain regions
-restructuring of brain circuits
Body Functions/ Restoring the ability to perform Performing old movement in a
Structure: movement as normally as performed new manner
Performance Level before the injury. -Reduction in truck -Use of excessive shoulder
displacement during reaching elevation & retraction to lift arm
Activity: Successful accomplishment of task Successful completion of task but
-Functional Level normally as seen in nondisabled using alternate strategy.
individuals -Opening package of chips with
-Opening package of chips using both one hand and mouth
hands as nondisabled individuals do
Stroke 99

The mechanism of recovery Principles


Spontaneous recovery is the norm after stroke. This 1. Use It or Lose It : Failure to drive specific brain
recovery is typically most rapid & early after a functions with volitional movements can lead
stroke and generally proceeds at a decelerating to functional degradation.
pace. In case of mild deficits due to small infarcts
2. Use It and Improve It : Training that drives a
and transient ischemic attacks, recovery of near
specific brain function can lead to an
normal motor function may result. The mechanism
enhancement of that function.
of neurological recovery after stroke involves more
than one process. 3. Specificity; The nature of the training
experience dictates the nature of the plasticity.
Recovery of the ischemic penumbra (brain tissue
that has been affected by ischemia but not infarcted) 4. Repetition Matters: Induction of plasticity
and resolution of cerebral oedema early after stroke requires sufficient repetition.
may account for much of the recovery that occurs 5. Intensity Matters: Induction of plasticity
within the first few days. The role of disachisis, a requires sufficient training intensity.
loss of brain function due to reduced neural input
from remote infarcted tissue, may contribute to 6. Time Matters: Different forms of plasticity
deficits that resolve during the early recovery occur at different times during [Link]
period. the rehabilitation intervention better the
prognosis.
Stroke recovery may continue for months after a
stroke. The latter portions of neurological 7. Salience Matters: The training experience must
improvement appear to be substantially due to be sufficiently salient to induce plasticity.
cortical plasticity. The plasticity consists of various 8. Age Matters: Training - induced plasticity
mechanisms like denervation supersensitivity, occurs more readily in younger brains.
unmasking of silent synapses, regenerative and
9. Transference: Plasticity in response to one
reactive synaptogenesis.
training experience can enhance the
Basically it is the rewiring of synaptic connections acquisition of similar behaviors.
within the surviving brain tissue to restore some
10. Interference: Plasticity in response to one
degree of motor functioning. Cerebral plasticity has
experience can interfere with the acquisition
been clearly documented in animal model of
of other behaviors.
cortical stroke and appears active in humans as
well. It is becoming clear that the amount and type of
physical and mental activity as well as attitude and
Animal studies of cerebral function have found
motivation of the patient has an impact on
changes in cortical function that result from exercise
reorganization of the brain. Hence, application of
training for motor tasks in both the intact and the
these new approaches with better understanding
damaged brain and in the absence of exercise of
of recovery and compensation and mechanism for
the paretic limb, the cortical plasticity provides
plasticity will help us in gaining better outcomes
important evidence of the impact of exercise
after stroke rehabilitation.
training on the brain. Thus cortical maps are
dynamic However one should not forget to consider
The role played by physiotherapists in stroke
the various factors such as the patient's age, severity
rehabilitation
of the lesion, effects of drugs post injury that will
have an impact on the outcome. Physical therapists specialize in treating disabilities
related to motor and sensory impairments.
Mechanisms to induce recovery (plasticity i.e., Rehabilitation helps stroke survivors relearn skills
at neuronal level) after brain damage: that are lost when part of the brain is damaged.
For example, these skills can include coordinating
Till date researchers have found following 10
leg movements in order to walk or carrying out the
principles, relevant to rehabilitation outcomes
steps involved in any complex activity.
which might augment brain plasticity leading to
neuronal recovery. Patients need to be explained, Physical therapists help stroke survivors regain the
in details all of them to gain maximum benefit : use of stroke-impaired limbs, and establish exercise
programs to help people retain their newly learned
100 Neuro-Rehabilitation : A multi disciplinary approach

skills. Disabled people tend to avoid using impaired • To restore muscle function
limbs, a behavior called learned non-use. However,
• To control compensation strategies
the repetitive use of impaired limbs encourages
brain plasticity and helps reduce disabilities. • To maintain muscle length
Rehabilitation teaches survivors new ways of • To re-educate balance
performing tasks to circumvent for any residual
disabilities. • To retrain walking and restore mobility

There is a strong consensus among rehabilitation • To maximise functional ability while allowing
experts that the most important element in any on-going neuromuscular recovery
rehabilitation program is carefully directed, well- To deal with these issues, physical therapists adopt
focused, repetitive practice - the same kind of various approaches during the rehabilitation
practice used by all people when they learn a new process.
skill, eg: playing the guitar or learning new game.
Approaches used in Clinical Setting - Early
For some stroke survivors, rehabilitation will be an Rehabilitation
ongoing process to maintain and refine skills and
Rehabilitation therapy begins in the acute-care
could involve working with specialists for months
hospital after the patient's medical condition has
or years after the stroke. Over time rehabilitation
been stabilized, often within 24-48 hours after the
can be quite remarkable. It does take a team of
stroke. Goals of treatment in acute stage are :
professionals and the family to accomplish this.
a) Prevent ignorance or unawareness of
Physiotherapists take a leading role in developing
hemiplegic side.
appropriate policies and strategies with other
exercise professionals and services to address the b) Decrease the tendency to develop synergy in
transition from rehabilitation to an active lifestyle the chronic stage.
following stroke.
c) Prevention of any joint restriction or stiffness.
During this recovery phase, there are many caveats
d) Prevention of complications due to
which physical therapists have to address to
immobilization like chest complications,
enhance the progression of recovery. Few of these
deconditioning of bone muscles, etc.
issues are as follows:
e) Early weight bearing.
• Paralysis or muscle weakness
f) Pscychological councelling.
• Impaired coordination
g) Education to the family.
• Apraxia ( patients loose their ability to plan
the steps involved in a complex task and to The first steps involve promoting independent
carry the steps out in the proper sequence, movement because many patients are paralyzed or
having problems following a set of seriously weakened. Patients are promoted to
instructions.) change positions frequently while lying in bed and
to engage in passive or active range-of-motion
• Impaired muscle tone
exercises to strengthen their affected limbs.
• Loss of feeling Exercises are generally begun with mat activities
like rolling, sitting up from lying, in sitting position
• Speech and language impairments
activities like side shifting are [Link]
• Memory and reasoning problems progress from sitting up and transferring between
• Swallowing difficulties the bed and a chair to standing, bearing their own
weight, and walking, with or without assistance.
• Psychological trauma (fear, anxiety,
frustration, anger, sadness, and a sense of grief, Weight bearing exercises are necessary to promote
clinical depression development of tone in muscles and also to
maintain the absorbtion of calcium in the [Link]
• Problems with vision and visual perception activities like bridging, supine to elbows, sitting
Goals of Treatment in Stroke patient: with weight bearing on affected arms and standing
should be given as soon as possible with in the
• To normalise muscle tone
limitation of patients general limitation. Oromotor
Stroke 101

exercises are also started so as to inhibit drooling Current Bobath concept has accepted that neural
and initiate adequate swallowing with out control is not a simple hierarchial function but it is
chocking. more complex where multiple body systems
participate in executing movement that is organized
Rehabilitation nurses and therapists help patients
by the specific task and constrained by physical
perform progressively more complex and
laws and the environment.
demanding tasks, such as bathing, dressing, and
using a toilet, and they encourage patients to begin
Limitation:
using their stroke-impaired limbs while engaging
in those tasks. Beginning to reacquire the ability to There is currently no evidence that Bobath
carry out these basic activities of daily living interventions are more or less effective than other
represents the first stage in a stroke survivor's therapy approaches (kollen, et al., 2009, Luke, et
return to functional independence. al., 2004; Paci, 2003).

Exercises on vestibular ball are also started to Hence, NDT approach requires evidence for its
improve balance and also normalize tone of the effectiveness over other treatment approaches.
trunk. Vestibular ball may be used to exercise the Proprioceptive (or peripheral) Neuromuscular
upper extremity mainly to achieve control at the Facilitation (PNF)
proximal joint and facilitate extension of fingers
PNF approach uses spiral and diagonal
through stimulation of the proprioreceptors at all
components of movements rather than the
the joints of upper limbs. Once the patient is
traditional movements in cardinal planes of motion
comfortable in doing these exercises, then standing
with the goal of facilitating movement patterns that
and gait training are initiated emphasizing on
will have more functional relevance than the
symmetrical weight bearing on both lower limbs
traditional technique of strengthening individual
Fig : 2, 6, 7, 8, 9, 10, 12, 13, 14 group muscles.
Traditional therapeutic exercise program also It uses resistance with the goal of facilitating
consists of strengthening, mobilization, "irradiation" of muscles to other parts of the body
compensatory techniques, endurance training (e.g., associated with the primary movement.
aerobics). Traditional approaches for improving
motor control and coordination: emphasize need Constraint Induced Movement Therapy (CIMT)
of repetition of specific movements for learning, the CIMT is a behavioral approach consisting of
importance of sensation to the control of movement, different components derived from neuro
and the need to develop basic movements and rehabilitation from basic neuroscience. The
postures. treatment for humans after neurological injury has
three components:
Approaches used in Clinical Setting- Late
Rehabilitation a) Repetitive, task oriented training of the
impaired extremity or function following
Bobath Approach (Neurodevelopmental shaping principles for several hours a day for
Therapy) 10 or 15 consecutive weekdays (depending on
The bobath approach is widely utilized in the severity of the initial deficit);
rehabilitation following stroke and other b) Constraining patients to use the impaired
neurological conditions. It is a problem solving extremity or function during walking hours
concept that allows for a variety of strategies flexible over the course of treatment, sometimes by
enough to be adapted to the strengths and restraining the unimpaired extremity; and
impairments of the individual client rather than a
prescribed treatment of exercise. c) Applying a package of behavioral methods
designed to transfer gains made in the clinical
Traditional Bobath approach was based on the setting to the real-world.
belief that voluntary movements are built on
reflexive movement and that treatment must follow Brunnstorm Approach (Movement Therapy)
the normal developmental sequence. Over the
Brunnstrom approach is based on the concept that
years, the Bobath approach has been modified with
damaged CNS regressed to phylogenetically older
many changes and till today alterations continue.
patterns of movements (limb synergies and
Fig : 2, 6, 7, 8, 9, 10, 12, 13, 14

102 Neuro-Rehabilitation : A multi disciplinary approach

Right Spastic hand with clawing of fingers and Stroke patient attempting to transfer
synergy pattern. from chair to bed.

Bedside shifting for Hemiplegic Patient, teaching Bridging exs in Hemiplegic patient, to aid trunk
independent transfers. strengthening and weight bearing of affected limbs.

Gait Training in Hemiplegic patient with Push knee splint and elbow splint for
affected side of the body

Swiss ball activities to strengthen the trunk muscles. All fours and hip extension of affected limbs to
develop voluntary control & break synergy pattern.
Stroke 103

primitive reflexes); thus, synergies, primitive walking and active repetitive movement training.
reflexes, and other abnormal movements are It has been suggested that through training,
considered normal processes of recovery before functional movements of locomotors patterns,
normal patterns of movements are attained. Hence, sensory inputs, and therefore central neuronal
based on this principle, Brunnstorm approach uses circuits, become activated. Hence, even with several
primitive synergistic patterns in training to improve studies having shown the feasibility of supported
motor control through central facilitation. treadmill ambulation training in patients with
Treatment includes facilitation of specific synergies stroke, whether it is superior to other therapies is
through cutaneous /Proprioceptive stimuli. still under dispute.

Motor Relearning Program/Carr and Shepard Functional electrical stimulation (FES)


Approach (Carr et al., 1985) People with hemiparesis often display abnormal
This approach is based on cognitive motor gait patterns, such as equinovarus (excessive
relearning theory and influenced by Bobath's plantar flexion and inversion) or foot drop
approach. Goal is for the patient to relearn how to (excessive plantar flexion), in which selective
move functionally and how to do problem solving control impairments are particularly prominent in
while attempting new tasks. the feet. During walking, a person's big toe and
outer foot margin rub against the ground, thus
In addition, this approach teaches general strategies
putting the person at risk of sustaining sprains and
for solving motor problems, instead of emphasizing
other ankle injuries. To minimize these patterns,
repetitive performance of a specific movement for
electrical stimulation to correct spastic foot drop in
improving skills.
hemiplegia was first applied by Liberson and co-
Sensorimotor Approach/Rood Approach (Noll, workers in 1961.
Bender, and nelson, 1996) Functional electrical stimulation (FES) based on the
This approach is based on modification of muscle concepts described by Liberson et al, uses electrical
tone and voluntary motor activity using cutaneous signals to activate peripheral nerves and control
sensory motor stimulation. functional movements. This technique makes use
of afferent feedback during contraction, a process
To facilitate muscle tone following cutaneous that, with a patient's help, may minimize motor
stimulation techniques are used: quick stretch, brisk relearning during active repetitive movement
icing, fast brushing, tendon tapping, vibration, and training.
joint compression to promote contraction of
proximal muscles. Similarly, to inhibit muscle tones, In routine clinical practice Functional electrical
following cutaneous stimulation approaches are stimulation time (in minutes) can be adjusted
used: slow sustained stretch, prolonged icing, etc. according to verbal feedback from the patients
during the 20 to 45 minute stimulation period. The
Newer approaches in late rehabilitation patient should be interested to say when they felt
fatigue related to dorsiflexion and eversion
Treadmill mediated gait training movements of the stimulated leg. In that situation,
Gait restoration is a major goal in post-stroke FES should be discontinued for 5 minutes and then
neurological rehabilitation. activated again. As volitional control improves, the
FES amplitude can be reduced.
For this reason, the recovery of independent
walking is important in rehabilitation studies. Two main advantages of using FES combined with
Recently, gait training on a treadmill with body treadmill training.
weight support (BWS) has received special • The first advantage was that the patients often
attention. It consist of a suspension system to which report a preference for walking on the
a patient is connected so that weight shifting, treadmill with BWS combined with FES. They
balance, and stepping can be controlled; walking report that gait training was more comfortable
is facilitated by a treadmill. Increasing evidence has because it was easier to place their foot during
suggested that treadmill training in older subjects early stance.
with hemiparesis improves locomotor capabilities
during over-ground walking and motor relearning, • The other advantage was that training with
because it provides task-oriented practice of FES decreased the participation of the physical
104 Neuro-Rehabilitation : A multi disciplinary approach

therapists. Manual assistance can be provided 3) Bi-Manu-Track- facilitates bilateral passive


to help the subjects optimize gait quality and active movement of the forearm (Prange
during training. et al., 2006)
4) NeReBot- enable movement of the shoulder,
Task-oriented training:
elbow, and wrist (Masiero, et al., 2007)
No conclusive definition of a task-oriented
approach exists in the literature. In the task-oriented 5) MIT-Manus/inMotion2- allows the person to
approach, movement emerges as an interaction person movement without interference from
between many systems in the brain and is organized the robot (although the robot can help perform
around a goal and constrained by the environment. the motion when the person is unable to
(Shumway Cook & Woollacott 2001). Task-oriented initiate or complete the motion)
training includes a wide range of interventions such Robotic-assisted therapy has the potential to
as treadmill training, walking, training on the facilitate improvements in motor control post
ground, bicycling programmes, endurance training stroke, but it is not clear whether this therapy
and circuit training, sit-to-stand exercises, and improves outcomes to a greater extent than
reaching tasks for improving balance. conventional therapy.
Task oriented training needs to be repetitive and
meaningful for the individual. Many interventions Bilateral Movement Training (Stewart et al.,
in task-oriented training that have proved to be 2006)
effective are usable in daily nursing practice, such A primary reason to perform bilateral arm training
as walking on the ground, moving from sitting to is that much of what we do everyday involves the
standing from different chairs, and sitting and use of both arms and therefore, bilateral re-training
reaching. is necessary. For example, daily living activities
such as bathing, dressing, feeding, toileting, as well
Hence, such training is task and patient focused and
as, many other mobility functions such as carrying
not therapist focused. Active use of task-oriented
objects, getting up from bed or chairs, and in
training in the daily nursing care of stroke
driving requires use of both arms and hand.
survivors, will lead to improvements in functional
Instrumental activities such as keyboarding,
outcomes and overall health-related quality of life.
shopping and cooking also rely heavily on bilateral
arm use. Hence, bilateral training has potential to
Robotic-Assisted Therapy (Krebs et al., 1998)
improve arm function post-stroke.
Robotic-Assisted Therapy for stroke rehabilitation
started only since 1990s. in general, robotics Mechanism for Bilateral Movement Training:
interfaces with computer software and hardware 1) Bilateral coordinated movement, the upper
for improving upper extremity function. Most extremities couple and act nearly as a single
robots that have been developed for rehabilitation unit, reflecting inter-hemispheric coupling at
allow several modes of operation including acting the supplementary motor area (SMA), lateral
as a low friction passive support to the upper premotor cortex, the premotor and
extremity, providing active range of motion and/ sensorimotor areas that may facilitate
or soft guidance in a movement pattern when the movement in the affected extremity.
individual is unable to initiate or complete a
movement, and offering some resistance to the 2) There is a reduction in intra-cortical inhibition
movement. with bilateral movement that may facilitate
reorganization in the damaged hemisphere.
Five types of robots have been tested:
Limitation:
1) Assisted Rehabilitation and Measurement
(ARM) Guide- made for straight line trajectory Studies of bilateral training have shown that people
reaching (Reinkensmeyer,et al.2000) improve their motor skills with bilateral training
after stroke, but the results are completely mixed
2) Mirror Image Movement Enabler (MIME) - on whether such training produces greater motor
provides unilateral or bilateral shoulder/ gains than unilateral training.
elbow movement (lure, et al., 2002; Kahn, et
al., 2006) To design rehabilitation protocols for functional
gains with bilateral movements is a challenge.
Stroke 105

Mental Imagery Therapy (Page et al., 2006: 2007) speech and language areas like Broca's area (frontal
Mental practice, using first-person visual and lobe) which causes difficulty in expression,
kinesthetic imagery to practice performance of Wernicke's area (temporo - parietal lobe) which
tasks without activating the muscles involved in causes difficulty in comprehension and jargon
physically performing those activities, activates speech, alexia and agraphia.
many of the same neural areas as does physical Stroke in the verbral and basilar artery affects
practice. In stroke, mental practice has been paired more of the brain stem areas like the cerebellum,
with either practice of daily common tasks or medulla and pons. This leads to dysarthria and
nonfunctional motor tasks and has been performed dysphagia.
from 3-5times per week. Studies have reported a
positive effect of adding mental practice to therapy. COMPLICATIONS IN THE
Thus, mental practice after stroke appears a HEMIPLEGIC PATIENTS :
promising adjuvant to motor rehabilitation.
1. Reflex sympathetic Dystrophy - many
Although unexplored, the ability of individuals
hemiplegic patients develop pain in the upper
with stroke to produce quality detailed images may
extremity. Pitting edema, cyanosis, and limitation
influence response to this therapy. How well motor
of extension in the wrist and finger joints, associated
imagery can be accomplished may depend on
with pain, are common and can be attributed to
stroke location in addition to pre-stroke imagery
malpositioning and lack of therapy
ability.
Treatment :
Virtual reality therapy (Burdea, et al., 1994)
Paraffin bath may be utilized prior to the passive
Virtual reality technology provides the capacity to
assistive exercises, provided the paraffin glove is
create an environment in which the intensity of
molded with the fingers in maximum flexion. The
feedback and training can be systematically
use of a platform or cockup splint, with the fingers
manipulated and enhanced in order to create the
in extension is advised..
most appropriate, individualized motor learning
paradigm. The potential benefits of training in 2. Subluxation of Shoulder: is very common,and
virtual reality environment would be the ability to sling suspension along with weight bearing
increase the duration, frequency, and intensity of exercises are recommended.
therapy that could be provided to patients by using Heterotopic calcification - extra articular
semi-automated programs. Virtual reality calcification has been described in other neurologic
rehabilitation exercises can be made to be engaging, disorders and it may occur in hemiplegic patients.
such that the patient feels immersed in the It is seldom encountered in the patient who has
stimulated world, which is extremely important in been quite active and conscious during the early
terms of the patient motivation. petiod following the stroke. Repeated insignificant
Limitation: trauma may play a role in the development of this,
progressive refractory [Link] may be
Subjects trained on a motor task in a virtual
associated with parietal lobe dysfunction causing
environment demonstrated the ability to improve
the patient to neglect the limb, predisposing it to
performance on the task in that environment, but
repeated injury.
the learning did not always transfer to the real-
world task. The extra-articular calcification occurs about the
lesser trochanter of the femur producing a severe
Hence, these protocols should be explored more in
adduction flexion contracture of the hip similarly,
order to ascertain the use of virtual reality training
it can produce a complete extra-articular bony
as an enhancement to traditional stroke therapy.
ankylosis of the shoulder [Link] extensor surface
Speech Affection in Stroke : of the elbow just above the olecranon process may
also be the site of involvement. The elbow is held
Most of the individuals with stroke present with in an atypical attitude of relative extension rather
speech, language and swallowing problems than flexion as commonly seen in the hemiplegic
depending on the area in the brain, subcortical areas [Link] the elbow is quite painful,
and the brain stem affected. Usually, middle warm,and slighthly swollen, without any joint
cerebral artery infarction causes problems in the effusion. A small amorphous calcific deposit at the
106 Neuro-Rehabilitation : A multi disciplinary approach

insertion of the triceps tendon, seen in the lateral contracture in a painful spastic shoulder.
X-ray film,may be the initianl finding in other Mobilisation of the head of humerus and amnual
patients this complication may not recognized until therapy gives great results in breaking adhesions
the acute phase has subsided the calcification may between the capsule.
progress to complete ossification
Local infiltration of the tender long head of the
Treatment of this complication is very biceps tendon with procaine and corticosterois may
unsatisfactory, particularly when it is advisable to afford considerable relief when the diagnosis is
immobilize the elbow in flexion and avoid all [Link] some instances pendular exercises
motion until the swelling and pain have subsided may produce sufficient relaxation to permit
has been recommended in traumatic conditions but effective mobilization. Oral corticosteroids have
has not been described in hemiplegic patients. been used extensively Physical therapy procedures
are prescribed to relieve the pain and increase the
3. Frozen Shoulder / Adhesive Capsulitis: The
range of [Link] application of hot packs or cold
painful shoulder is probably the most frequent and
packs over the deltoid region has become more or
most disturbing complication encountered in the
less a routine procedure. Frequently it is more
stroke patient. It can be quite disabling since it
desirable to apply the packs over the spastic or
intereferes with active function, including the
contracted pectoral muscles while the shoulder is
performance of simple dressing activities, and may
abducted and in some external rotation rather than
even secondarily immobilize the hand.
adducted and internally rotated. The physical
The cause of the painful shoulder in the hemiplegic therapist can then intiate the shoulder exercises
patient has been the subject of consider able from a more advantageous position diathermy
discussion basically, several factors may be should be used with considerable caution,
involved: particularly in patients having a sensory deficit.
1) preexisting degenerative changes in the rotator Ultrasound therapy has been employed with
cuff; varying results but may be indicated in treating a
persistent localized tendinitis.
2) repeated small trauma to the periarticular
structures, incurred as a result of traction on The application of a modality to the painful
the shoulder when the patient lies unconscious hemiplegic shoulder should always be followed by
on the hemiplegic arm or when he tries to range-of-motion exercises. The patient must be in
move in bed with the arm flaccid at his side; a supine position so that the shoulder can be
adequately stabilized and the effects of gravity
3) excessive traction on the shoulder capsule, eliminared vigorous stretching should not be
with marked inferior displacement of the devoted to this phase of the treatment to permit
humeral herd in a flaccid limb; the the pectoral and other adductor muscles to
4) vigorous stretching of the "tight" spastic relax. Stretching will only elicit a stretch reflex and
shoulder during physical therapy, producing increase the spasticity in these muscles.
further trauma to the cuff and long head of Pulley exercises should not be prescribed unless
the biceps tendon; there is at least 60 to 70 degrees of passive abduction
5) unrecognized trauma such as an impacted present; otherwise they will only increase the
fracture of the surgical neck of the humerus, scapulothoracic substitution. The patient must be
incurred as a result of falling during the onset properly positioned so that the overhead pully is
of the stroke;and slightly behind him to effectively abduct and
externally rotate the shoulder during this exercise.
6) contractures that occur in the untreated patient
or develop as a result of peripheral nerve 4. Vascular complications. Thrombophlebitis in the
lesions and heterotopic calcification. hemiplegic lower extremity occurs not infrequently
during the early convalescent period and may be
The treatment of the painful shoulder should be
overlooked. this could explain the persistent edema
predicated on an accurate diagnosis rather than
in the lower leg of some patients after they are
consist of routine procedures and exercises.
started on ambulation activities.a simple elastic
Although the use of a sling may be of considerabie
compression bandage or stocking applied before
value in supporting the flaccid shoulder, it may
the patient gets out of bed is quite adequate. Ample
only aggravate the adduction internal rotation
provision must be made for the swelling about the
Stroke 107

ankle when fitting the patient for shoes or a short become the accepted procedure in fractures of the
leg brace. The T strap on the brace must be broader femoral neck in the elderly hemiplegic patient.
than usual and well padded to avoid any More recently, consideration is being given to
constriction about the ankle. The lower leg should simply pinning the fracture after closed reduction
be elevated when the patient is sitting in a followed by immediate progressive weight bearing.
wheelchair.
An incipient occlusion of the femoral artery may
Role of Occupational Therapist in
simulate a thrombophlebitis. Evaluation may be Stroke:
difficult, particularly when the patient is aphasic.
Vascular changes may have been present prior to Introduction
the onset of the hemiplegia. Pain in the lower limb
Following stroke, patients may be faced with
is persistent, and there may be evidence of an
occupational dysfunction. The physical, cognitive
ischemic neuropathy particularly involving the
and psychosocial capacity to perform routine tasks
deep branch of the peroneal nerve, which is most
such as self care, work and leisure activities that
sensitive to arterial insufficiency. The foot drops, if
are meaningful to them, may be affected and in turn
present initially, will become more pronounced, the
have an impact on the patients occupational
hyperactive tendon reflexs may be lost, and a
identity, health and well being.
segmental sensry deficit involving especially the
first web space may signal the onset of a major The role of the occupational therapist is to enable
arterial [Link] surgical intervention is patients to regain competence, reengage in
indicated at this stage rather than waiting for the occupations and redevelop a positive occupational
appearance of the cyanosis of the foot and identity1. Occupational therapists are an integral
demarcation of the ischemic skin. part of the team working in the rehabilitation of
patients suffering from stroke. Though stroke is a
5. Seizure.: epileptic seizures may occur as a late
complex condition, there has been constant
complication of a cerebral vascular [Link]
advances in the understanding of the condition,
incidence may be as high as 10%to 15% among
assessment and intervention techniques. It is hence
those patients who survive beyond the first year.
vital that the OTs understand the condition itself,
Since the focal signs are localized in the hemiplegic
know the theoretical basis for intervention and keep
extremities and in the speech mechanism, they often
themselves abreast of the latest advances in the care
lead to the diagnosis of another stroke. However,
and rehabilitation techniques so as to provide the
these patients recover quite rapidly within the first
best possible care for the patients.
24 hours and resume their previous level of activity
6. Decubiti / Pressure Sore : The older hemiplegic Theoretical Basis
patient with peripheral vascular disease must be There are several theoretical constructs, that help
carefully observed during the early phase of the to describe and explain occupational function,
illness to prevent pressure sores, particularly over guide assessments and interventions and predict
the posterior aspect of both heels. Early outcomes. The prominent constructs are:
mobilization helps in preventing it, by improving
blood supply. Conceptual models of practice
7. Trauma.; the hemiplegic patient is accident prone, The Model of Human Occupation (MOHO)2
especially when ataxia is the predominant considers the complexity of human occupation that
peripheral defect. Fracture of the femoral neck is a behaviour is dynamic and context dependent and
frequent mishap, particularly in those ataxic that occupations shape a person's self-perception
patients who walk with a spastic adducted gait and and identity.
invariably fall on the hemiplegic side. This injury This model and its associated tools help
may be associated with an unrecognized impacted occupational therapists to understand the person
fracture of the surgical neck of the humerus. and focus on an integrative view of human
Careful consideration should be given to the occupation. However, therapists are required to
management of the hip fracture. Replacement of draw on other frameworks to understand and
the femoral head with prosthesis, followed by address patient's performance capacity.
immediate ambulation with full weight bearing, has The Canadian Model of Occupational Performance
108 Neuro-Rehabilitation : A multi disciplinary approach

and Engagement (CMOP-E)3 is a social model that non-western perspectives enables therapists to be
considers the spiritual, physical, affective and truly client-centred. Thus, in stroke rehabilitation,
cognitive components of the person whose self-care, reduction of impairments may not be as significant
productivity and leisure occupations occur in the as it is in western cultures. Maximising patients'
context of the physical, institutional, cultural and personal attributes and resources, adapting
social environment. environments and considering interdependence on
family and social participation (social inclusion)
It is a client-centred outcome measure enabling
may be more meaningful than addressing
clients to rate importance, performance and
impairments and activity limitations.
satisfaction with self-care, productivity and leisure
activities that they 'need to', 'want to' or 'are
expected to' do.
Frames of reference
Similar but distinctive models emphasising person, Client-Centred Frame of Reference is a humanistic
environment and occupation are the Person- approach which originated with psychotherapist
Environment-Occupation (PEO) Model4 and Carl Rogers and was further developed by
Person-Environment-Occupational Performance occupational therapists in Canada10. Key concepts
(PEOP) Model5. of the approach include client autonomy and right
to informed choice; partnership between client and
The Australian Occupational Performance Model therapist to work together to negotiate therapy
(OPM(A))6 describes eight interactive constructs, goals and processes; responsibility of the client for
including occupational performance, occupational his/her own health and ethical responsibility of the
roles, occupational performance areas (self- therapist to ensure no harm; empowering and
maintenance, rest, leisure and productivity), enabling clients to achieve their occupational goals;
components of occupational performance understanding clients individual contexts through
(biomechanical, sensorimotor, cognitive, intra- and respect and listening; accessibility of services to
interpersonal skills), core elements of performance meet clients needs; and respect for diversity.
(mind, body and spirit), the performance
environment (sensory, social, physical and social Biomechanical Frame of Reference is a bottom-up
contexts), time and space. frame of reference, useful for understanding
occupational performance capacity in more detail.
The Perceive Plan Recall and Perform (PRPP) It considers the anatomy and physiology and
System of Task Analysis7 was developed as one of mechanics of human movement (kinesiology)
the assessment tools within this model. focusing on musculoskeletal, neuromuscular and
Activities Therapy8 combines psychodynamic, cardiorespiratory systems. Occupational therapy
human developmental and behavioural frames of approaches that fit within this frame of reference
reference. This model suggests that adaptive include graded activities to improve movement
(compensatory/ functional) skills are re/learnt in strength, endurance, range of motion and sensation,
a developmental sequence to achieve mature work hardening, energy conservation, ergonomics,
functioning and influenced by the environment and assistive devices, splinting and joint protection.
their biological composition. In relation to stroke, Thus approaches to prevent deterioration, restore
this model suggests that dysfunction arises when function or compensate for limitations are
patients regress in their sensory integration, significant here. Nevertheless, the primary
cognitive interaction, group interaction, self- assessment and outcome for occupational therapy
identity and/or sexual identity skills. Rehabilitation should always be in the context of meaningful
is based on sequential relearning of adaptive occupation.
(compensatory/functional) sub-skills through Rehabilitative Frame of Reference draws on
graded occupation and patient's innate need for medical, physical and social sciences. It considers
mastery. rehabilitation as the process of helping patients
The Kawa (River) Model9 is an emerging model competently fulfill daily activities and social roles
from an Asian perspective which may address and focuses on therapists teaching, patients
cultural biases of existing models (which value learning adaptive (compensatory/functional)
individualism, autonomy and independence) to methods, assistive equipment and environmental
consider cultural values of collectivism, social modifications to restore function when underlying
hierarchy and interdependence. Appreciation of impairments cannot be remediated and successful
Stroke 109

rehabilitation is dependent on motivation and Sigmund Freud's controversial theories but has
cognitive skills. been developed to focus on understanding the
relationship between past experience and present
Motor Control Frame of Reference considers the
difficulties. It highlights links between unconscious
relationship between the central nervous system in
motivations and emotions which are
relation to motor function and reacquisition of operationalised through interpersonal interaction,
coordinated skilled movement but recognises the behaviour and occupation. For example,
influence of other systems (sensory input and mechanisms such as repression, denial, projection,
cognitive processing),environmental context and reaction formation, intellectualisation,
learning principles (such as attention, feedback, rationalisation, regression, sublimation and
active participation and goal-directed movement). compensation protect the psyche against anxiety
In comparison to a biomechanical frame of arising from unconscious internal conflict. These
reference, emphasis is on muscle tone, reflexes and internal conflicts and underlying emotions and
movement patterns. Many restorative (remedial) motivations can be therapeutically explored and
intervention approaches fall under this heading, symbolically resolved through creative (projective)
including Bobath's neurodevelopmental (normal activities, meaningful occupations, reflection, group
movement) approach, Carr and Shepherd's work processes and therapeutic relationships to
movement science/motor relearning, Rood, achieve a sense of wellness12.
Brunnstrom's Movement Therapy, Proprioceptive Cognitive Perceptual Frame of Reference draws on
Neuromuscular Facilitation, Mental imagery and neuroscience and neuropsychology and focuses on
Constraint-Induced Movement Therapy. the components and interaction of cognitive and
Behavioural Frame of Reference considers learning perceptual skills that impact on occupational
principles arising from stimulus response models performance. Treatment approaches can be
such as Pavlov's classical condition and Skinner's categorised into remedial/bottom-up/skills
operant conditioning where behavioural responses training or adaptive/top-down/strategy training
to stimuli or triggers can be modified through approaches recognising the brain's capacity but
exposure and manipulation of the consequences. limited potential to repair following brain injury13.
This frame of reference is useful for behavior A wide range of cognitive and perceptual tools and
modification such as desensitisation or reduction treatment strategies fall under this umbrella.
of anxiety-related symptoms as well as for new In addition to the above theoretical constructs
learning principles such as repetition and positive which assist in guiding occupational therapy
feedback. practice, the emerging theories of neuroplasticity
Cognitive Frame of Reference originated in are utilised in current neurological practice. A
psychiatry and psychoanalytical theory with the knowledge of neuroplasticity can assist the
work of Aaron Beck. This frame of reference occupational therapist in selecting an intervention/
examines the links between the patients' automatic approach for the individual patient and will assist
thinking, their behaviour and emotional response. in clinical reasoning and justification of the
Cognitive-behavioural therapy (CBT) links the intervention administered.
cognitive and behavioural frames of reference
together. It utilises a problem-focused approach to Neuroplasticity
explore patients' underlying thoughts, beliefs and
Recent advances in neuroscience and functional
physiological responses associated with specific
imaging have demonstrated evidence of
triggers and the consequences of dysfunctional
neuroplasticity - the brain's considerable capacity
behavioural responses that might maintain these.
for neural reorganization14.
Duncan (2006)11 cautions that a cognitive-
Neuronal plasticity after injury occurs as a result
behavioural frame of reference should be used in
of one of two main processes: either the rerouting
conjunction with an occupation-focussed
and subsequent formation of new connections, or
conceptual model of practice to maintain
neurones substituting function of damaged
professional role and identity and to enhance the
neurones to enhance the effectiveness of existing
therapeutic potential of the patient-therapist
connections15. This includes:
partnership.
– The concepts of synaptic strengthening or
Psychodynamic Frame of Reference originated with
potentiation - altering the effectiveness of
110 Neuro-Rehabilitation : A multi disciplinary approach

synapses (short-term potentiation/long-term (ADL). It is based on the belief that man is a


potentiation). functional animal and his ability to do so is essential
– Unmasking of existing silent synapses whose for his well-being19.
function was previously blocked by inhibitory Adaptive (compensatory/functional) approaches
influences. are traditionally used when restoration is unlikely
– Sprouting of new axon terminals. and assumes that certain functions will not
– Changes in dendritic organisation. recover20. Compensation for loss of function is
achieved by changing the activity, environment or
Neuroplasticity redefined recovery as 'the ability
to accomplish the goal in exactly the same way as patient behaviour by using external assistance,
before the injury'16. The loss of normal function modifying the task or changing the goal or by
deprives the CNS of the experiential feedback practice until the task becomes easier in a variety
required to drive adaptive reorganisation and may of environments. The advantages of this approach
subsequently permit maladaptive reorganisation17. are that it is patient-centred, easy to explain, uses
This increased emphasis on remedial approaches problem solving, meets short-term needs and gives
for 'true neuroplastic recovery' early in stroke quick results.
rehabilitation.
Cognitive rehabilitation approach
Intervention approaches Cognitive rehabilitation therapy is a systematic and
Despite evidence of neuroplasticity, predicting functionally oriented approach to improve
recovery potentials remains challenging. Some cognitive functioning either by restoring cognitive
combinations of symptoms will be more amenable processing skills that are impaired and/or helping
to true recovery while other combinations will have the patient learn new ways to compensate for the
limited capacity, requiring an adaptive impairment(s)
(compensatory/functional) approach to learn to
adapt to activity limitations. Thus, occupational Normal movement (Bobath-based approach)
therapists will always need both restorative and
adaptive treatment approaches as components of The normal movement approach is the most
neurorehabilitation. commonly used restorative approach to physical
Neurorehabilitation. It is also known as Bobath or
Restorative approach (remedial approach)
neurodevelopmental treatment (NDT)
The restorative (remedial) approach relies upon
theories of neuroplasticity and the ability of the and is based on neurodevelopmental reflex-
brain to reorganise itself18. Neurophysiological hierarchical theory that hypothesised spasticity as
approaches such as normal movement and motor a product of overactive reflexes. Originally,
relearning are included within the restorative treatment utilized reflex inhibiting patterns and
(remedial) approach. Here, the therapist provides progressed patients through a neurodevelopmental
controlled visual, auditory, vestibular, tactile, sequence21. However, Bobath treatment techniques
proprioceptive and kinaesthetic stimulation to have changed since the last Bobath publication in
promote normal CNS processing of sensory the 1990s. The current 'Bobath Concept' of normal
information. Therefore, normal sensory processing movement has evolved to incorporate present-day
should help the patient make normal perceptual knowledge and a systems theory of motor control,
motor responses required for performance of motor learning, neural and muscle plasticity and
functional tasks. This approach therefore aims to biomechanics.
reduce the impairment to subsequently improve
activity and participation. The Normal movement approach is a problem-
solving or clinical reasoning process based on the
It is implicit within this approach that these tasks assumption that 'too much effort by the patient and
are appropriately graded to challenge the patient
overuse of the unaffected side reinforce abnormal
and encourage the brain to adaptively reorganise
tone and movement of the affected side'. Thus, the
itself for successful behaviours.
approach aims to improve disturbances in function,
Adaptive (compensatory/functional) approach movement and postural control. Key points are
used to:
The adaptive (compensatory/functional) approach
focuses on repetition of particular skills which are (a) Facilitate and control movements; and
normally associated with activities of daily living (b) Alter postural tone.
Stroke 111

Therapists use experience of movement, repetition, the unaffected arm with intensive training of the
speed, voice, environmental manipulation and paretic arm conducted by a clinician using shaping
feedback. and repetition24. Shaping involves small steps of
progressing difficulty and activities are designed
Proprioceptive neuromuscular facilitation to enable patients to carry out parts of a movement
Proprioceptive neuromuscular facilitation (PNF) as sequence; verbal feedback is always positive for any
a neuophysiological treatment approach is based small gains made25.
on the reflexive relationships between agonist and
antagonist muscles which can be manipulated to Bilateral arm training/isokinematic training
control the contraction and relaxation of specific approach
muscles groups and thus facilitate normal
Bilateral arm training is where the unaffected limb
movement. It also emphasises that 'the brain
facilitates the affected limb in synergistic
registers total movement and not individual muscle
coordinated voluntary movements and is
action'. Assessment considers the relationship
recommended for subacute and chronic phases of
between proximal and distal functions, agonists and
antagonists, in total patterns of movement observed recovery26. It is based on theories that
during functional activities. contralesional activation may activate the lesioned
hemisphere or adaptively strengthen ipsilateral
Rood approach pathways to facilitate recovery of the affected limb.
This intervention is based on reflexive and
Mental imagery approach
hierarchical models of the nervous system. Use of
developmental postures and sensory stimulation Mental imagery or mental practice has been
applied to muscles and joints are used to stimulate described as the internal rehearsal of movements
a motor response that can either facilitate or inhibit without any physical movements27. An essential
muscle tone in preparation for normal movement. part of mental imagery is the ability to create clear
Rood's concept is therefore based upon the concept and powerful images of the task required on
of correct sensory stimulation being applied to the demand. The practice must have functional
sensory receptors and eliciting the correct motor relevance and meaning to the individual to enable
reflex which can be utilised in normal movement more successful visualisation.
patterns22.
With advances in neuroimaging techniques, these
Movement science mechanisms could be better understood and assist
in the selection of specific intervention strategies
This remedial approach to physical
either in combination with mental practice or in
neurorehabilitation is also known as motor
isolation28. Using mental imagery may maintain
relearning programming (MRP), functional and
neuronal activity that would deteriorate without
task-oriented approaches, founded by Carr and
stimulation and prime pathways in readiness to
Shepherd in the 1980s 23. It emphasises the practice
promote motor function.
of the functional task or action itself as the remedial
component promoted by principles of motor Electromyographic (bio) feedback
learning, including use of instruction, explanation,
Involves the use of external electrodes applied to
manual assistance, visual and verbal feedback on
muscles and instrumentation to convert electrical
performance, reinforcement and contextual
potentials from muscles into audio or visual
practice. Thus, it aims to facilitate motor relearning
information. This augmented feedback is based on
through use of meaningful activity, feedback and
behavioural and motor learning theory where
practice. This approach emphasises neuroplasticity
extrinsic feedback is used to improve reacquisition
and addresses concerns regarding negative effects
of motor skills. There is some evidence to support
of compensatory use of the affected side, learned
its use to augment standard treatment29 but routine
non-use and use of adaptive aids on motor learning
use outside of clinical trials is not recommended.
by altering task requirements.
Functional electrical stimulation
Constraint-induced movement therapy
approach Electrostimulation is thought to be beneficial to
train and strengthen muscle contractions.
Constraint-induced movement therapy (CIMT) is
a behavioural approach that involves restraint of However, results remain inconclusive.
112 Neuro-Rehabilitation : A multi disciplinary approach

The occupational therapy process I. PERFORMANCE SKILLS


Effective early management is vital in the care and • Motor
rehabilitation process of a patient with a stroke. Key • Sensory
aspects of early management are accumulating • Cognitive
relevant information about patient and completing
• Perceptual
effective assessments to identify areas of
rehabilitation. • Psychological
• Social
Putting all this information together helps to
formulate a clear image of the patient, their II. IMPACT OF ANY IMPAIRMENTS ON A
impairments, skills, goals, motivations, so this PERSON'S ABILITY TO ENGAGE IN
information can be used to prepare a clear OCCUPATION WITHIN A DEFINITE
intervention plan. ENVIRONMENT. (PHYSICAL, SOCIAL AND
CULTURAL)
Information gathering checklist: a. Performance skills
Before assessing the patient, it is important to gather
initial information from varied sources such as : A. Motor
1. Medical history: It is important to document i. Position, Movement, tone of affected
any co morbidities, associated medical extremity
problems, current medical or pharmacological ii. Ability to change or maintain position of
management that the patient has, as these may trunk and extremities
affect their assessment and functional abilities. iii. Presence of pain, shoulder subluxation,
2. Social history: The occupational therapist associated reactions etc.
should document the patient's social network, iv. Ability to perform mobility in bed.
occupation, main roles, financial information,
v. Ability to sit unsupported and maintain
driving status, leisure interests etc.
balance
3. Current physical mobility: to ascertain how the vi. Ability to transfer from supine to sit, bed
patient can be moved and whether any to wheelchair and chair, wheelchair or
equipment or assistance from others is chair to toilet or commode
required.
vii. Ability to stand on affected leg and reach
4. Premorbid level of functioning out in standing while maintaining
balance and safety.
5. Consistent level of functioning post stroke.
viii. Ability to walk and tolerance in different
6. Information from CT Scan and MRI which environments. For eg: Indoors on even
indicate the area and extent of damage caused surfaces, open spaces, uneven surfaces
to the brain and the potential impairments that etc.
the patient may present with.
ix. Ability to perform his or her ADLs for
eg: in early stages of recovery, simple
Assessment
ADLs like feeding, grooming, washing
Based upon their theoretical knowledge and dressing. In later stages, shopping,
(conceptual model and frame of reference) and travelling etc.
clinical experience, the OTs select specific
assessment and evaluation measures that will Standardised Assessments for Motor Impairments
identify and measure the factors affecting the There are two types of tests that are generally
functional abilities of the patient. Assessment selected to assess motor impairment in stroke.
provide information as a base line for intervention
and are useful for planning, intervention and setting 1. Generic activites of daily living for eg Barthel
short term and long goals. index30 is a generic ADL scale

OT assessment at any stage of recovery will involve 2. Specific motor performance tests For eg:
a detailed analysis of Motorocity index31 Rivermead motor
assessment32 Nine hole peg test33:
Stroke 113

B. Sensory name being called.


Sensory impairments like sensory loss are b. Phantom auditory perception e.g.
common following stroke. Also there may be tinnitus or hallucinations
problems in interpreting sensory information
c. Increased sensitivity to sound
i.e. perceptual impairments of
iv. Vestibular
i. Visual
Vestibular Systems along with visual and
ii. Somatosensory
somatosensory input greatly contribute
iii. Auditory towards balance38. Dysfunction leads to
avoidance of activity and social isolation.
iv. Vestibular
OT can observe for:
v. Olfactory and Gustatory
a. balance reactions and reports of dizziness
i. Visual especially with eyes closed or reduced
OT can observe for base of support.

a. visual acuity impairments: difficulty in Standardised Assessment: Sensory


reading, recognizing details or faces, organization Balance test39
squints while trying to focus. v. Olfactory and Gustatory processing:
b. Occulomotor control impairment: Dysfunction in sense of smell and taste
Blurred vision, double vision, difficult in following stroke is rare. OT can observe
focusing, difficulting in reading, for:
watching TV etc.
a. Patients response to pungent odours (like
c. Visual field impairment: Hemianopia and cloves, cinnamon, coffee, vanilla and
quandrantonopia lemon) and different tastes (like sweet,
ii. Somatosensory: sour, salty and bitter).

OT can observe for Assessment of cognitive functions


Impaired or absent tactile sensation or Cognition refers to those mental functions which
proprioception e.g. patients who appear help us to acquire, organise, manipulate and use
to be clumsy and or burn or cut information and knowledge. It includes all of our
themselves. asstereognosis, e.g. patients 'thinking' processes.
who drop objects body image
In the acute phase of recovery from a stroke a
impairments e.g. patients who negect
person may be fatigued and may be dealing with a
their limbs body scheme impairments like
complex set of recovery issues which can impact
unilateral neglect, right/ left discrimation
on their emotional and psychological status. Testing
impairment.
specific cognitive skills at this stage may not give a
true reflection of their abilities and it may be
Standardised Assessments:
advisable to wait until the rehabilitation phase to
1. Rivermead assessment of somatosensory do this.
performance (RASP)34
Cognitive functions which may be impaired
2. Revised Nottingham sensory assessment following a stroke include the following:
(rNSA)35
– Attention - the ability to focus on specific
3. Erasmus MC Modifications to the r NSA36
sensory stimuli and suppress distractions.
4. Stereognosis sub test of Chessington OT Attention is required for many other cognitive
Neurologic assessment battery (COTNAB)37 functions to occur.
iii. Auditory – Memory - the ability to retain and recall
OT can observe for information.
– Perception - 'making sense of the senses' -
a. Hearing loss: e.g. Patients response to
sound during conversation or patients – Language - understanding and expression.
114 Neuro-Rehabilitation : A multi disciplinary approach

– Praxis - motor planning. specific impairments only and some are more
– Executive functions - skills which are needed general. These can be split into three main
to plan organise and execute a task. categories and examples of each are shown below:

OT can observe for cognitive dysfunction during 1. Neglect.


assessment of occupational performance during 2. Spatial.
selected daily living activites like grooming or
preparing tea/ coffee. 3. Multi assessments.
1. Neglect assessments
Cognitive Standardised assessment
i. Baking Tray Test43.
Occupational therapists have a vast array of
ii. Balloons Test44.
standardised assessments and screening tools
available to them which can be used to contribute iii. Behavioural Inattention Test45.
to the assessment of cognitive functions. 2. Spatial assessments
Standardised, impairment-based assessments aim i. Location Learning Test46.
to provide valid and reliable assessments of
performance skills, that is, specific cognitive ii. Rey Figure Copying Test47.
components. These assessments should be used in iii. Visual Object and Space Perception
combination with clinical assessments and Battery48.
reasoning to ascertain extent of impairment impact 3. Multi assessments
on occupational performance.
i. Cortical Visual Screening Test49.
Standardised assessments: Standardised ii. Motor Free Visual Perceptual
observational assessments of performance eg.: Battery50.
MEAMS - Middlesex Elderly Assessment of Mental
iii. Occupational Therapy - Adult
State40
Perceptual Screening Test (OT-
– COTNAB - Chessington Occupational Therapy APST)51.
Neurological Assessment Battery41
iv. Rivermead Perceptual Assessment
– LOTCA - Lowenstein Occupational Therapy Battery52.
Cognitive Assessment42. v. Repeatable Battery for the
Assessment of Neurological
Perceptual assessment
Status53.
Perceptual problems have been shown to be
Many assessments are available from Pearson
common following both right and left hemiplegic
Assessment (combining The Psychological
Stroke. These perceptual problems affect the
Corporation, Thames Valley Test Company and
patients' response to rehabilitation and their ability
to perform activities of daily living. Harcourt Assessment). In addition to the above
assessments, assessments for provision of adaptive
Perceptual assessment will clarify whether patients equipment may also be required.
have any perceptual impairments, including the
type(s) of perceptual impairment(s) present and Assessment for adaptive equipment and
their severity. Perceptual ability can be assessed wheelchairs:
either functionally or by use of standardized
Wheelchair prescription for stroke patients is
assessments.
controversial as some OT's believe that self
Functional assessment propelling the wheelchair increases patients muscle
tone while using unaffected arm and leg. Indoor
Functional assessment of activities of daily living,
powered wheelchair could be considered with
such as personal or domestic activities, will
demonstrate the effect of a mixture of impairments, patients with severe physical disability and those
both physical and cognitive impairments, upon the who have chronic lung and heart conditions.
patient. Outdoor Powered wheelchair could be considered
for patients with severe long term mobility
Standardised perceptual assessments problems.
There are many standardised perceptual While assessing for wheelchair, therapist should
assessments available, some of which are for take into consideration
Stroke 115

- patient's vision, cognition and perception – Loss of food or liquid from the mouth or
drooling
- patients home environment and local area
– Coughing\choking while eating or
- Access to patient's home, type of
drinking.
accommodation, width of doorways, layout of
furniture and fittings, door thresholds and – Pocketing of food inside the mouth.
floor coverings.
– Change in voice quality after eating or
- Raised toilet seats and frames drinking
- Non Slip mats for bathrooms – Frequent pneumonias

II. OTHER IMPAIRMENTS Depression:


IMPACTING ON FUNCTIONAL A depressed patient has decreased motivation to
ABILITY participate in assessments and intervention due to
being preoccupied with their worries and
1) Communication thoughts54 and it was found that in patients, the
2) Dysphagia severity of impairments in functional activity (ADL)
and intellectual function was significantly
3) Depression and Anxiety correlated with the severity of post stroke
4) Fatigue depression early after stroke. Symptoms of
depression include negative thoughts, irrational
5) Emotional lability belief, distortion of reality, self blame low mood,
poor appetite and weight loss or increased appetite
1. Communication:
and weight gain, disturbed sleep, poor
There are three main problems affecting concentration and indecisiveness.
communication:
– Aphasia/dysphasia-disorder of language Assessments for depression:
which can result in difficulty in The assessments that may be used include the
understanding and expressing things following:
verbally, reading and writing. (a) Hospital Anxiety and Depression Scale 55.
– Dysartria-a speech disorder caused by (b) Wakefield Depression Inventory56.
damage to the nerve supplying the
muscle used when speaking. It may (c) Geriatric Depression Scale57.
involve problems with breath control for (d) General Health Questionnaire58.
speech, voice production controlling
whether air is directed orally or nasally Anxiety
during speech and articulation of speech Patients may have anxieties relating to their stroke,
sounds. It can range in severity from for example, fear of having another stroke, fear of
mildly slurred speech to inability to epilepsy, fear regarding their future in terms of
produce any intelligible speech. home, social, sex, employment, etc.
– Verbal apraxia: a disorder affecting Hospital Anxiety and Depression Scale59 may be
purposeful coordination of muscle used for assessment.
movements for speech production. for eg:
struggling to achieve correct sound for Fatigue
words or sequence the sounds in the right
Fatigue can affect the individual physically,
order.
mentally, emotionally or as a mixture of all three60.
Dysphagia: Fatigue can lead to increased tone, poor
Difficulty in moving a\bolus of food, liquid from performance, reduced motivation, etc. OTs should
the mouth to the stomach without aspirating take care not to overtire the patient. Working with
withour aspirating and involves chewing and the therapist involves both physical and mental
tongue movements, preparing food for swallow as effort on patients part and it can be more effective
well as actual swallow. The OT's can observe for: to change the task than stop and rest when the
116 Neuro-Rehabilitation : A multi disciplinary approach

patient appears tired. Suitable relaxation techniques – The opportunity to practise activities in
can be taught as an alternative to activity or rest. the most natural (home-like) setting
Therapist should try to understand the possible possible.
cause of tiredness. (for eg: not sleeping well at night,
– Assessment for, provision of and training
not able to wake up in the morning because of
in the use of equipment and adaptations
medication, have an infection or pathological
that increase safe independence.
condition, poorly nourished, or bored.) and discuss
strategies of coping with it like keeping active, – Training of family and carers in helping
developing interest, using energy conservation the patient.
techniques etc. The therapist can assist the patient The intervention plans should incorporate
in handling fatigue by providing a varied and practicing tasks, particularly personal care tasks.
challenging programme that is not impossible and Further, task-specific training should be used to
not causing stress. improve activities of daily living and mobility: For
eg. Standing up and sitting down.
Goal Setting
is a collaborative process between the therapist, Management of motor impairments:
patient and their family (where appropriate) Following a thorough assessment, th therapist
involving education and negotiation. Initially, needs to incorporate the identified problems into
therapists should ascertain patients' and their an intervention plan. The main aims of OT
family's long-term goals or where they see intervention regarding motor problems are:
themselves at the end of therapy. Long-term goals
are aspirational, giving patients hope and 1. To promote motor recovery in the most
motivation to engage in the therapeutic process. In normal or efficient way to increase functional
contrast short-term goals need to be client-centred independence by practising graded activities
and collaborative, specific, measurable, achievable, of daily living using a restorative (remedial)
realistic and timely (SMART). Short-term goals approach.
form the steps needed to work towards the long- 2. To prevent secondary complications such as
term goal. These can be used to measure outcomes. pain in the shoulder or swelling of the hand.
In addition they allow patients, therapists and team 3. To maximise the patient's independence in
members to maintain direction, motivation, activities of daily living by using an adaptive
monitor progress and gain insight into how (compensatory/functional) approach, when
achievable the longterm goal is or whether the long- the restorative (remedial) approach is felt not
term goal needs to be adjusted, thus allowing to be practical or achievable.
patients to transform their occupational identity to
a more realistic sense of self. 4. To train caretakers in safe techniques for
handling and carry out risk assessment based
Interventions on patient's functional level and equipment
needs, either in preparation for discharge or
Rehabilitation enables the patients to meet their
as ongoing rehabilitation in the community.
goals and ultimately aim to reduce the activity and
participation limitation. Rehabilitation has been In the early stages of recovery, when movements
defined as a problem solving and educational are restricted by the effects of their stroke,
process aimed at maximum recovery by using individuals are unlikely to be able to make the
restorative (remedial) approaches to reduce postural adjustments required, to maintain a
impairments and adaptive (compensatory / symmetrical posture, without assistance. Proper
functional) approach to prevent impairments from positioning in bed help recovery by maintaining
translating into functional disability. (Activity and passive range of movement, allowing the individual
participation limitation) to use the control they have and providing normal
sensory and proprioceptive input.
Following a thorough initial assessment, the
therapist needs to incorporate the identified
Section II.Fig1.1 to Section [Link] 1.6
problems into an intervention plan. Specific
interventions that should be offered (according to 1. While side lying on the affected side keeping
need) include: a pillow under the knee helps to reduce any
developing hip adductor tone.
Stroke 117

Positioning in bed lying on affected side Positioning in bed sidelying on affected side (Rt
(Lt hemiplegia pt) hemiplegia pt)

Positioning in bed lying on unaffected side Positioning in bed lying on unaffected side
(Lt hemiplegia pt) (Rt hemiplegia pt)

Positioning in bed lying back Positioning in bed lying back


(Lt hemiplegia pt) (Rt hemiplegia pt)
118 Neuro-Rehabilitation : A multi disciplinary approach

2. Side lying on the unaffected side restricts use on a restorative (remedial) approach, although it is
of sound upper extremity. However, this may a common practice to teach some adaptive
be the position of choice, (atleast for some time (compensatory/functional) techniques early, that
for patients with over active sound side). This is, dressing techniques to maximize early
position provides proprioceptive feedback independence.
about midline, facilitates elongation of trunk
on sound side and promotes weight bearing When the patient's sitting balance has improved
through over active side. and the required assistance with transfers is
minimal, the occupational therapist can consider
3. Positioning the patient on his /her back can sessions involving showering. Showering can be
restrict their visual fields and use of upper carried out on a shower chair or while seated on a
limbs. However this is a good position to allow
bath board.
pectoral muscles to be stretched. Pillows
should be used to prevent affected shoulder If the patient's overall mobility improves, standing
and hip falling into retraction. should be incorporated into intervention, for
example, standing in the shower or at the sink. The
It is important to consider mattresses when
positioning the patient. A firm, supportive surface therapist should still provide facilitation and
will provide proprioceptive feedback, enable rolling prompts if required, to achieve active incorporation
and promote independence when sitting up. of the affected arm and leg.
However, pressure areas also need to be monitored. Dressing can be graded in a similar way to
Where hospital pressure care mattresses are used, washing/ showering and should be part of the
the patient is likely to require more assistance to same intervention session.
turn and sit up.
_ Patients early post stroke can be taught one-
Encouraging the patient to sit up through side lying handed dressing techniques while seated in a
promotes head righting, weight transference and a wheelchair or armchair; the session would also
sense of midline. More independence is offered to focus on the patient's sitting balance, trunk
the early stroke patient in supported sitting and
control and position and incorporation of the
they gain a more normal visual perspective of their
upper limb as appropriate.
environment. There is scope for the unaffected arm
to be used in a range of functional activities. The _ Patients with improving trunk control could
trunk muscles begin to be used actively and the be taught dressing techniques while seated on
lower limbs begin to form a stable base of support. a plinth. This would also involve practising
It is important to note that sitting is not a passive standing with the necessary prompts or
task; the early patient may develop inappropriate facilitation.
muscle activity and 'holding' postures if they do
not receive sufficient support from the chair or _ The ultimate goal for dressing would be for
pillows. Those with sensory loss will require the patient to be as independent as possible in
pressure areas to be monitored. Where head control the most normal environment, for example,
is still lacking, support must be provided. sitting on the bed or standing in the bedroom
or bathroom.
When the patient begins to gain some active sitting
balance and transfers are progressing, positioning Improve flexibility and joint integrity:
on a perching stool allows for more active sitting,
Soft tissue or joint mobilization and range of motion
improving dynamic control of balance, active
extension of trunk and weight-bearing through exercises are intiated early to maintain joint
lower limbs. The upper limbs are freed to perform integrity and mobility and prevent contractures.
a greater range of activities. The extra seat height Positioining strategies are also important in
and position of the pelvis in anterior tilt facilitates maintaining soft tissue length. Effective positioning
easier transfers into the standing position. of the hemiparetic extremities encourages proper
However, perching stools should only be joint alignment while positioning the limb out of
considered for relatively high functioning patients. typically assumed abnormal postures.
Active and passive ROM with terminal stretch
Self Care Activities: should be performed daily in all motions.
All self-care activities should be graded, depending
Full extension of elbow wrist and fingers is
on the patient's level of functioning. The focus is
important as most stroke patients develop tightness
Stroke 119

of flexor muscle group. This can be done


functionally through sitting, weight bearing on
paretic arm with wrist and fingers extended.
Splints: a resting hand splint can also be provided
to prevent and manage length associated changes
in muscle and connective tissues. Within the
literature, there is conflicting evidence and opinions
on whether to use splinting as a form of intervention
with patients with stroke. The decision whether to
splint must be made on sound clinical reasoning.
Splints should not be considered when there is
active movement that would be restricted if a splint Facilitating weight bearing and trunk rotation
is provided. It is important to teach the family and during peg transfers
caregiver the correct manner to don-doff splint and Avoiding secondary complications:
the wearing schedule for effective long term Pain: Post stroke, shoulder pain is very common
management. and leads to difficulty in participation in functional
activities and can cause low mood, altered sleep
Graded Therapeutic / Remedial Activities:
patterns and therefore have an impact on patients
Activities that offer the patient movement quality of life. Shoulder pain can be caused due to
experience in a controlled environment can be shoulder subluxation abnormal tone either hyper
carried out. The OT sets up the environment and or hypotonicity, proprioceptive impairment and
positions the task to gain specific movements and poor handling.
to maximize the effect of the intervention, for e.g.:
adjusting the height of table the patient is seated or Intervention is aimed at normalizing tone and
standing at, and to encourage the patient to reach reducing pain using proper positioning and
forward and to pick up a card, carry out peg board handling, gentle ROM and gentle stretching,
activities, writing exercises, computer work etc. biofeedback and relaxation techniques. The affected
arm needs to be positioned properly in all positions
Upper limb reeducation: lying sitting and standing. Support can be given
by using pillows in supine side lying sitting, trays
Activities to retrain upper extremity postural
or lapboards for patients using wheelchair or a
support, reaching and manipulation are essential
variety of slings in sitting standing or walking. Use
elements of training. Two techniques that is
of slings is controversial as it may lead to increased
bilateral arm training/ iso kinematic training and
flexor tone and contractures and learned non use.
constrain induced movement training have been
An alternate to the traditional sling is a humeral
shown to be effective in some patients and can be
cuff sling.
incorporated into occupational therapy plans.
Slings should be used for certain upright activities
Joint approximation and extended arm weight
like transfers and gait training and used only for
bearing during early rehab phase promotes
short duration62. As patient recovers and voluntary
proximal stability and counter acts the effects of
movements emerge, use of slings should be
excessive flexor hypertonus and a dominant flexion
discontinued.
synergy.
Family caregivers and hospital staff are instructed
Weight bearing activites can be performed in
and given practices in proper handling and
sitting, quadruped, modified plantigrade and
mobilization of upper extremity, avoiding trauma
standing postions, reaching activities with
and traction injuries during ROM exercises,
unaffected extremities can be given in these
transfers and wheelchair activities as well as proper
postions using the affected upper extremity as a
use and care of slings and splints.
postural support. The occupational therapist
should encourage the patient to use his affected Oedema: Post stroke oedema results in restrictive
extremity for postural assistance during functional mobility and functional use. Oedema is caused due
training activities. E.g. Pushing from side lying into to ineffective muscle pumping activity combined
sitting. Graded reaching and manipulation with poor positioning and effects of gravity.
activities can also be incorporated in the sessions61. Oedema of the hemiplegic arm is commonly seen
120 Neuro-Rehabilitation : A multi disciplinary approach

as an isolated hand and wrist swelling but in some searching environment for hidden objects,
cases it can be a part of a more complex "shoulder Involving patient in games that require them
hand syndrome"63. to switch or track objects such as table tennis.
Intervention includes a positioning program which Therapy within the area of social participation is
includes supported elevation of the arm, passive essential to ensure a patient continues to engage in
ranging and light retrograde massage along with their life roles and the wider environment while
functional use of limb whenever possible. ensuring their safety. For e.g. Developing safe
search strategies, for example, crossing the road -
Management of visual and identifying the left curb, following it along and
sensory impairments scanning from this curb till the patient sees the other
curb. This ensures they have seen the whole road
Intervention for visual processing dysfunction before crossing.
Occupational therapists have a key role in
Management of somatosensory impairments:
identifying visual impairment and referring them
to appropriate specialist. (Opthalmologist, Approximately 50% of stroke patients experience
optometrist or orthoptist) for an in depth somatosensory impairment64. Functionally,
assessment and to incorporate the identified somatosensory processing impairments have
impairments into any further interventions as significant safety implications, particularly for the
vision has a key role to play in functions. detection of protective thermal and pain sensations
and for patients have difficulty regulating grasp for
Following the International Classification of effective object manipulation; particularly for
Functioning, Disability and Health model, fasteners and writing. They are at an increased risk
intervention for visual impairments can be of developing learned non-use as spontaneous use
classified under impairment-based intervention, of the affected hand is diminished contributing to
activity engagement and social participation. further deterioration of motor function and their
ability to relearn skilled movements is affected65.
Visual acuity and Occulomotor control These difficulties may in turn impact on all personal
Impairment needs referral to the optician and domestic and community activities of daily living,
orthoptist for recommended corrective lenses or sexual and leisure activities and thus participation
glasses and occlusion for double vision, prisms etc. in life roles66. Somatosensory impairments are
significantly related to stroke severity and activity
Visual field impairments limitations, which negatively impact on motor
This cannot be rectified by intervention and recovery.
adaptive (compensatory/functional) measures are
used to encourage the patient to scan and search Intervention
within their affected visual field. Occupational Significant tactile, stereognostic and proprioceptive
therapists can provide simple tasks such as upper limb recovery can occur in the first six
scanning sheets, telephone number copying, months post stroke; however, prognosis for
environmental searching, etc, to encourage recovery is poorer than in the lower limb67. Stroke
scanning into the affected vision field. An orthoptist severity is the strongest indicator of impairment
may consider use of prisms to compensate, so and recovery and motor performance significantly
referral is worth considering. influences recovery of stereognosis. However, there
is no recognisable pattern to recovery68.
Activity engagement
O Carey 69 summarises the principles of
Often patients with visual processing impairments intervention from successful training programs
compensate very well and are able to adapt to their which include the following:
impairments; however, it is the role of therapy to
not only increase their confidence in activity – Attention to the sensory stimulus:
engagement but also their efficiency and reduce the – Repetitive stimulation with and without
effort required to complete familiar tasks. vision:
Intervention could include:
– Use of targeted sensory tasks that are
– Engaging the patient in an obstacle course, challenging and motivating, with
Stroke 121

opportunities for success. Adaptive (compensatory/functional)


– Anticipation where patient draws on previous intervention
experiences of what the stimulus should feel – Use of vision to compensate.
like. – Enlarged handles to assist grips, universal
– Focus on the hand. splints when attention is divided.
– Graded progression of tasks for the targeted – Safety - education, safety awareness,
modality from easy to more difficult environmental adaptations.
discrimination. Variation in stimuli is required – Pain - distraction, relaxation, pain clinic,
for generalisation to novel tasks. Transcutaneous Electrical Nerve Stimulation
– Intensity of training programme. (TENS), splinting (alignment), taping/
Functional Electrical Stimulation (FES)/
– Feedback on accuracy and execution in line
support for subluxation.
with learning theory. Calibration of
perceptions with the other hand and visual Intervention for auditory dysfunction
feedback may also be important.
Speech and language therapist generally address
Restorative (remedial) intervention at impairment/ restorative (remedial) intervention.
impairment level Occupational therapists should liaise with these
disciplines regarding recommended strategies and
Shabrun and Hillier70 reviewed the evidence for
enable patients to incorporate these into everyday
passive (excludes muscle contraction) and active
activities. Occupational therapy intervention
sensory retraining following stroke and found that
should primarily focus on adaptive (compensatory
active stimulation involving exercises that practice
/functional), activity / participation-based
discriminating and localising sensations,
intervention.
stereognosis and proprioception may be beneficial.
Restorative (remedial) intervention during Intervention for vestibular dysfunction
functional tasks at activity and participation level Occupational therapists should liaise with the
Encouraging some use of the affected limb during physiotherapist regarding recommended strategies
functional tasks to improve sensory feedback may and consider application to everyday activities. The
help. The therapists should consider the dynamic occupational therapy intervention should primarily
nature of sensory input as the central nervous focus on adaptive (compensatory/ functional),
system responds to change and switches off when activity/participation-based strategies including:
sensory stimulation is constant. Other techniques – Visual compensation.
which may enhance normal sensory input include
– Stabilisation during functional tasks, for
the following:
example, use of perching stool, chair,
– Bobath principles of facilitation and use of stabilizing pelvis against benches/sinks,
handling to prevent abnormal movements and propping through upper limbs.
feed correct sensory input into the sensory – Grading activity demands related to weight
system may have indirect effects on sensory transfer and dynamic sitting and standing
improvement during functional tasks. balance and functional mobility.
– Cognitive processing (inattention to tactile – Graded activities requiring independent
stimuli) to utilise attention/information head/eye movements.
processing strategies.
– Functional activities demanding balance that
– Sensory re-education desensitisation - here the manipulate somatosensory input and visual
central nervous system is overwhelmed and feedback, such as
is over-responsive to change, so needs to learn
– Completing functional tasks with/without
to cope with reduced amounts of input with
shoes on, on different surfaces inside and
slower changes.
outside.
– Constraint-induced movement therapy – Graded visual feedback starting from
downgraded activities where visual feedback
122 Neuro-Rehabilitation : A multi disciplinary approach

is utilised and somatosensory feedback from there should be a regular review of goals with
the lower limb is stable to high-level retraining the patient, family/carer and team72.
where somatosensory feedback is variable (e.g.
– Individualised - a selection of strategies and
outdoor surfaces) and vision is occluded.
intervention techniques may be required as
– Activities which may include reading, tracking people will have individual interests and
objects with/without head movements, responses to interventions.
coping in dynamic environments in the
– Educate and include relevant family/carers/
community, for example, crossing roads,
friends and significant others - so that they
shopping, travelling keeping gaze on fixed
understand the difficulties a person may be
target and moving head horizontally and
having and can assist with the application of
vertically versus moving eyes with head fixed,
strategies and provide support.
travelling on buses, in cars, mobility under
more challenging conditions, for example, – Focus on functional improvement - including
walking, running, moving, standing on a way of measuring this improvement, such
moving surface. as goal attainment and performance measures.
– Falls prevention. – Include psychological and emotional support
- people with cognitive problems can develop
Intervention for Olfactory and Gustatory anxiety, depression and a sense of loss of
process dysfunction control and self-esteem. These should be
Recovery of smell and taste have been reported in acknowledged and interventions provided to
minor strokes71 and a restorative (remedial) support management of these problems, such
approach could be considered on a theoretical basis. as anxiety management training, relaxation
Adaptive (compensatory/ functional) approaches training and medication.
should consider functional implications such as
Intervention strategies
safety (e.g. alerting to spoiled food, gas, fire and
smoke), nutrition and psychosocial implications. – Task-specific training - or functional
retraining, stresses the value of the use of
Management of Cognitive Impairments specific and relevant functional tasks.
Emphasis is placed on task characteristics, in
Cognitive rehabilitation order to support behavioural change73.
The main rehabilitative approaches used by
– Practise - repetition over time and use of
occupational therapists, within cognitive
retained capacity assists learning.
rehabilitation are:
– Errorless learning - people with brain injury,
– Remediation (restoration) and.
including stroke, may not learn from their
– Adaptive (compensatory/functional). mistakes so an approach which supports the
achievement of a successful outcome by cueing
Occupational therapists tend to favour a functional
the correct response is more likely to enhance
approach for the rehabilitation of people with
learning. This has been evidenced in studies
cognitive impairment, including task-specific
of people with memory problems74.
training and the use of activities which are
meaningful and familiar. – Environmental adaptation - regulation of noise
and distractions; clearing environmental
Intervention for cognitive dysfunction: clutter; and adaptations such as message
Principles of intervention for the rehabilitation of boards.
people with cognitive impairment are: – Compensation and strategy training - external
– Goal orientated: Goals should be meaningful aids and adapted methods - for example, use
and relevant. Long and short-term goals are of memory aids such as pagers, diaries and
set and they should be, as far as possible calendars.
'SMART', that is, Specific; Measurable; _ Prompts and instruction - direct instruction
Achievable (with some challenge); Realistic and guided assistance may support relearning
(within the environment and resources of skills.
available) and Timescales should be set and
Stroke 123

– Restoration/skills training - Some studies of of some use for those with mild problems, has
attention have reported improved skills when limited effect for those with severe memory
specific retraining of basic attention capacity problems. The use of adaptive (compensatory/
is offered. Retraining tends to be more effective functional) approaches and assistive devices within
when embedded in a meaningful and the context of functional activities tend to be more
functional context, targeting the specific level successful77. A combination of approaches is
of attention impairment of the individual75. recommended and these need to be selected
according to where the memory information
Attention Intervention processing system breaks down.
Attention is required for most other cognitive Some of the strategies are:
functions to take place. It is dependent on an
adequate degree of arousal and alertness and helps 1. make the information more meaningful,
us to process a large amount of information on a linking to previous learning or chunking
daily basis. Attention is commonly affected after information together.
stroke, especially in the early stages of recovery. A 2. Internal strategies and prompts - for example,
functional approach using meaningful tasks can be use of mnemonics, visual imagery.
used for intervention and it is suggested that 3. External strategies -If the patient is unable to
intervention should be focused on training specific store information, use of written and verbal
functional skills rather than the underlying prompts.
processes.
4. Compensatory aids - for example, electronic
As the patients attention improves, the challenge pagers, diaries, notebooks, calendars and
and complexity of the task can be increased to work computers.
on higher levels of attention. Repetitive tasks at the
5. work on consolidation through rehearsal and
tabletop can be used, for example, letter cancellation
practice.
and word searches, as long as the interventions are
providing an appropriate challenge and can be 6. Errorless learning in practice of tasks to
graded as attention improves. minimise performance errors and enhance
learning.
Adaptive (compensatory/functional) strategies 7. If the client has 'tip of the tongue' syndrome
The adaptive strategies recommend that patients and has difficulty recalling the information,
should be taught strategies to compensate for their give graded clues and prompts to elicit
reduced attention. This can be done by effortful but successful recall to facilitate
memory.
1. providing structure to the patient's day such
as using a diary system. 8. Support from family, carers, colleagues and
2. Minimise distraction in the patient's friends is required to implement strategies and
environment and provide prompts and support, no matter
which approaches are taken.
3. ensure the patient has a quiet place they can
go to if they become overstimulated as this Language
may manifest in agitated behaviour.
Aphasia may occur following a stroke and this may
4. Use of prompting to maintain the patient's affect a person's understanding of the spoken word,
attention during tasks can be useful (prompts verbal expression, reading and writing. It is usually
can be verbal or visual). assessed in more detail by the speech and language
These techniques should also be taught to families therapist; however, joint sessions between the
and carers to alleviate the potential emotional stress occupational therapist and speech and language
attention problems can bring to both patient and therapist may be of benefit to help to ascertain what
their carers. elements of a person's performance are due to
language difficulties or other cognitive problems.
Memory Intervention
Intervention should be individualised, goal Praxis
orientated and include psychological and emotional The principles of cognitive rehabilitation, 'goal
support. A restorative (remedial) approach, whilst orientated; individualised, educate and include
124 Neuro-Rehabilitation : A multi disciplinary approach

relevant family/carers/friends and significant functional activities' and 'verbal self-instruction,


others; focus on functional improvement; and self-questioning and self monitoring could be used
include psychological and emotional support' to promote internalisation of self-regulation
should be used and an adaptive (compensatory/ strategies'. Focusing on the functional activities
functional) approach is recommended. which are limited by specific executive skills are
encouraged, for example:
Studies have shown that people with apraxia can
improve their functional performance despite the – Goal setting - specific and meaningful for the
lasting presence of apraxia, and that adaptive person. It is helpful to encourage people to set
(compensatory/functional) strategies do not their own goals and then review the outcome
impede the recovery of the impairment78. A to develop insight into their own abilities.
proposed 'strategy training' programme for people
– Planning - activities can be graded to achieve
with apraxia can be carried out to improve
success and gradually improve the difficulty.
performance by teaching internal and external
compensatory strategies. As the assessment using – Organisation - a structured routine can be
ADL observations breaks the activity down into established with support, and responsibility
performance stages of initiation, execution and for this gradually handed over.
control, intervention targets the relevant stage – Self-initiation and self-direction - external aids
using instruction, assistance and feedback. If such as pagers and alarms can assist with
initiation is the problem, instruction is given reminders. Taped or written prompts may
verbally and if the problems are mild and if the assist with self-direction.
person is experiencing more problems initiating the
activity, then the therapist may hand items to them – Self-inhibition/monitoring and correction -
one at a time. If execution is the problem, specific develop strategies to monitor own behavior
verbal or physical guidance is given and if control and make appropriate changes. Feedback and
is a problem or performance errors are not discussion can be used to develop a person's
corrected, then appropriate feedback is given. awareness of their own performance.

Other smaller studies have shown some support – Flexible problem solving - alternating
for specific interventions: scenarios can be presented and practised to
develop a strategic approach to generating
– 'Activities in context80' - a small 3D movement alternative solutions.
analysis study suggested that motor
performance and kinematic measures Perceptual Intervention
improved when the person with apraxia was
Intervention of perceptual impairments involves a
supplied with the appropriate tools for a task
mixture of restorative (remedial) and adaptive
and the correct contextual environment.
(compensatory/functional) approaches. The
– 'Task-specific training81' - it was found that restorative (remedial) approach can be generalised,
task-specific training could restore as practice on a particular perceptual task will affect
independence for trained activities. They also the patient's performance on similar perceptual
found that skills did 'not generalise' to other tasks.
tasks and performance was retained only
when tasks continued to be 'practised'' in daily The adaptive (compensatory/functional) approach
routines. can be interpreted as repetitive practice of particular
tasks, usually activities of daily living, which will
Intervention for Executive dysfunction make the patient more independent in these
It is recommended that those with executive particular tasks.
dysfunction and activity limitation should be taught Occupational therapists use functional tasks as an
adaptive (compensatory/ functional) strategies, for intervention medium. Neglect is the most common
example, electronic organisers, written checklists; perceptual impairment suffered by stroke patients
and that family and other staff should be involved and occurs over several sensory systems; vision,
in discussions regarding the impairment and ways touch and auditory.
of supporting the person. It is recommended that82
General intervention tips for perceptual
'training of formal problem-solving strategies and
dysfunction
their application to everyday situations and
Stroke 125

– Consider the grade of the task; the complexity Restorative (remedial) strategies
of the task increases the likelihood of errors. – For the patient to become aware of midline by
– Consider the types of prompts, that is, visual, using visual feedback, place a mirror in front
verbal, physical or questioning prompts and of them and instruct the patient to self-correct
pausing before providing a prompt. themselves back to midline.
– Consider using written or visual instructions. – In all postural sets, ask the patient to identify
the position of their body and describe their
– Learning can be achieved through repetition
relationship to supporting surface86.
and practice.
– Get the patient to move between postural sets
– Reinforce positive behaviours rather than
and for them to maintain their balance.
negative ones.
– Stage components of the task, that is, break Adaptive (compensatory/functional) strategies
down the task and encourage the patient to – Place pillows on the overactive side to provide
complete one stage at a time. extra supporting surfaces to enhance the
– Use verbal rehearsal, that is, encourage the patient's feeling of security.
patient to talk through the task before – When seated in a wheelchair place the hospital
completing it, errors can then be corrected bed in a high position on the overactive side
before they are performed. to enhance feelings of security.
– Establish patterns and routines. – Teach the patient to use vertical structures
– Provide consistency in approach. within the room such as door or window
frames to adjust balance with reference to
Specific intervention strategies for perceptual these markers87.
dysfunction
Unilateral neglect
Body scheme
Restorative strategies
Restorative (remedial) strategies
– Use activities that cross midline, for example,
– Ask the patient to verbally identify parts of personal care activities.
the body83.
– During activities of daily living sessions place
– Encourage the patient to verbalise positions stimuli on the patient's affected side and
of parts of the body to improve awareness. prompt and encourage them to look over to
– Provide tactile stimulation, for example, rub a their affected side. Place necessary items in
rough cloth on the patient's arm while naming midline and to their affected side using cues
it before placing their arm through a sleeve84. to locate all items and ask patients verbalise
the location of items to practise spatial
– Identify parts of the body before washing or scanning.
dressing them.
– Practise shifting attention from left to right.
– Incorporate bilateral activities that facilitate Cue patients to target stimuli in neglected
normal movement and improve body scheme. space to assist attentional shifts.
Adaptive (compensatory/functional) strategies Move necessary items from midline to their affected
85 side, such as the knife in midline and the butter
– Provision of instructions that name parts of further into the left side.
the body, such as 'wash your arm'. – Cancellation tasks such as maze or word
– If the patient has functional awareness, searches to practise scanning left to right.
provide cues such as 'move the part of the – 2D scanning tasks, that is, paper and pen tasks
body that you use to hold things' instead of or more dynamic such as room searches.
'move your hand'.
– Computer games that require scanning from
Impaired midline awareness side to side.
126 Neuro-Rehabilitation : A multi disciplinary approach

– Tactile stimulation onto the neglected part of _ Teach the patent to place different items in
the body, using vibration, mildly hot or cold different parts of the room.
stimuli88.
_ Use of tactile kinaesthetic strategies such as
guiding the patient to the object.
Adaptive (compensatory/functional) strategies
– Place objects in midline and gradually move _ Encourage the patient to verbalise the position
objects further into the patients' affected side. of parts of the body to improve awareness.

– Approach patients from the midline. Adaptive (compensatory/functional) strategies


– When reading, anchor the page and draw a _ Organise the objects so that they are in the
red line down the affected side so that the same place.
patient becomes aware of how far across the
_ Mark drawers where key items are kept.
page to start reading89.
_ Encourage the patient to feel and describe
– Adapt the environment; remove clutter on the
objects.
affected side90.
_ Remove clutter in the environment93.
– Encourage the patient to turn their plate round
to ensure all the meal is eaten. _ Place objects on contrasting surfaces, for
example, white soap on a dark coloured cloth.
– Teach the patient to turn their heads to become
more aware of the affected side. Visual agnosia
Other intervention approaches Restorative (remedial) strategies
Constraint-induced movement therapy – Present objects in a straight position rather
than other orientation.
CIMT attempts to reverse the learnt non-use of the
affected arm; however, to be able to use this – Encourage the patient to recognise differences
technique there needs to be enough return of and similarities between items.
movement that would allow the patient to – Start with items that are very different and
functionally use their affected hand. CIMT reports gradually upgrade to items with subtle
to be a useful intervention method for unilateral variations, for example, shape, size or colour.
neglect.
– Encourage the patient to verbalise differences,
Eye patching that is, naming objects and differences between
objects.
Studies have shown that using glasses that occlude
the good (ipsilesional) side of vision in each eye,
Adaptive (compensatory/functional) strategies
the patient is forced to direct their gaze to their
contralesional side91. Compliance with this – Teach the patient to consider and think
technique can be difficult as it is the patient's natural critically.
inclination to gaze towards the occluded side. – Utilise verbal strategies where the patient
describes the perceptual and functional
Prism glasses characteristics of the object to aid retrieval of
There is evidence of the positive effects of prism the object name.
adaptation92. A 10? rightward horizontal shift of
– Use other senses to identify the object, that is,
the visual field can be achieved by wearing prism
touch, smell or sound.
glasses. Using prisms is recommended if the
unawareness is severe and persistent. – Show the object in a natural context.

Visual discrimination – Adding texture or edge orientation to objects


may assist into providing cues to
Restorative (remedial) strategies identification.
– Teach the patient to retrieve items following
– Use premorbid orientation of objects, that is,
verbal instructions with spatial concepts, for
did they keep a T-shirt kept in the drawer or
example, 'get the brush on top of the dresser
on a hanger.
behind the bed'.
Stroke 127

– If categorisation is intact ask the patient to 9. Give mental stimulation.


identify which category the object would
10. Reassess perceptual and functional abilities.
belong to.
– Provide labels for objects to maximise Equipment
independence.
The assessment for and provision of equipment to
Tactile agnosia (stereognosis) stroke patients is generally viewed as an adaptive
(compensatory/functional) method of reducing
Restorative (remedial) strategies limitations. Most equipment is issued following a
_ Exploratory hand movements for object home assessment visit completed prior to weekend
identification. Explore the object by touching leave and/or discharge. However, some equipment
the surfaces and edges of the object, holding can be used to facilitate normal movement and
the object in the hand to obtain information increase independence within the hospital setting.
on its size, shape and weight. The pros and cons of timing and type of equipment
should be carefully considered in conjunction with
Adaptive (compensatory/functional) strategies the patient, family and multidisciplinary team.
– Education of problems and how these affect
function.
Wheelchairs
– Utilise other senses, that is, vision and touch Wheelchair: wheelchairs can be prescribed to a
from the unaffected hand. stroke patients for two main reasons

– Teach the patient to focus on specific – for correct positioning and to improve sitting
properties of the object. balance and to increase stimulation during the
early management.
– Use familiar objects within functional tasks.
– Indoor and outdoor mobility during the rehab
– Use objects within context. stage.
General assessment and intervention plan for A pressure care cushion should be provided with
perceptual impairments the wheelchair and monitored throughout the day
A general plan for the assessment and intervention by nursing staff and therapists.
of perceptual impairments is shown below: A correctly fitted wheelchair provides a more active
1. Assess perceptual abilities using functional sitting posture, which encourages greater freedom
tasks and standardised assessments. of upper limb movements. When assessing any type
of wheelchair on a long-term basis, the home
2. Analyse the results and the effect of environment and local area in which the patient
comprehension, concentration, reasoning will be living should always be taken into account.
(executive function), initiation, memory, The access to the patient's home, the type of
anxiety, depression, apraxia, hemianopia/ accommodation, the width of all internal/external
eyesight, inattention, etc. doorways, the layout of the furniture and other
3. Explain the perceptual problems and their fixtures/fittings, the door thresholds and the floor
likely effects in everyday life to the patient, coverings should be considered for suitability of a
their relatives and all staff involved with the wheelchair.
patient. Pressure relief is an important consideration if the
4. Choose the intervention approach to be used, person is unable to change position without
that is, restorative (remedial) or adaptive assistance, but this still needs to provide a stable
(compensatory/functional) or both. base. Many specialist chairs are now available that
provide additional postural support such as lateral
5. Decide which intervention strategies to use.
supports, head supports, inclined seats and lap
6. Relate intervention to the patients' needs. straps (to maintain the hips at 90?).
7. Remember that not everyone likes games and Toileting
puzzles.
Raised toilet seats and frames (which are safely
8. Remember we all learn in different ways. fixed to the floor) will encourage a patient to move
128 Neuro-Rehabilitation : A multi disciplinary approach

from sit to stand in a more normal way than pulling


on grab rails fixed to a wall.

Bathing/showering
Many stroke patients with independent sitting
balance can manage transfers on and off a bath
board, but a bath seat is generally too difficult due
to the amount of effort involved. This in turn can
increase muscle tone and be too strenuous for
people with chronic heart and lung conditions and
the frail elderly. Non-slip mats should always be
provided or purchased to be used in conjunction
Upper Body dressing activity
with bath boards/seats. Chairs or seats that are
fixed across the top of the bath for use with a shower designed for one-handed use or to make heavy
or that lower into the bath require less effort for tasks lighter.
the stroke patient and carer and are much safer for
Fatigue is a major factor to consider when preparing
those with poor sitting balance. Step-in shower
a meal. The layout of the kitchen and its existing
cubicles have limited space for small stools or seats
equipment or appliances can be looked at during a
fixed to the wall and are therefore only accessible
home assessment visit. Some portable items could
to the more mobile stroke patient who can wash
be moved closer together in order to conserve the
themselves independently whilst standing or sitting
patient's energy. A perching stool could also help
on a stool.
reduce fatigue.
Dressing Eating
Clothing styles may change initially in the early During the acute stage, good positioning whilst
stages of learning a dressing technique, the patient eating will assist safer feeding and swallowing.
may wear more leisure wear which is easy to slip Plates that retain heat will keep food warmer for a
on until they become proficient in dressing slow eater. Plateguards and large-handled mugs
techniques or their motor/cognitive problems with lids reduce the risk of spillage. Dycem mats
improve, allowing the individual to dress in their will keep plates in place. Large-handled cutlery
desired style of clothing. Any change of clothing could be used with the affected hand to encourage
style must be carefully discussed with the patient further return of movement. At a later stage, the
in order to maintain the individual's autonomy and one-handed patient may require a rocker knife or
self-image. a fork with a serrated edge for cutting.
Even in the early phase of recovery, occupational Evaluation of occupational therapy effectiveness 'is
therapists can teach patients adaptive an ethical and professional imperative'.
(compensatory/functional) strategies for functional Standardised assessments are used in clinical
tasks that will improve quality of life and that are practice to identify and quantify problem areas.
considered not to have a detrimental effect on motor These assessments can also be used as outcome
recovery. Adaptations such as elastic shoe laces or measures. There are two types of tests that are
Velcro shoes/trainers are often useful, or teaching usually selected, generic activities of daily living
the patient a one-handed method of tying shoe lace and specific motor performance tests. The results
is feasible. of such assessments should be useful for planning
intervention and setting goals. The Barthel Index
Meal preparation is generally recommended as a generic activity of
Some kitchen equipment such as large-handled daily living scale while the Motricity Index, the
utensils or cutlery issued by occupational therapists Rivermead Motor Assessment and the 9 Hole Peg
could be used by patients with some return of hand Test as specific motor performance tests.
function to encourage further improvement or
At the patient level, ongoing evaluation enables the
facilitate more normal movement. These could be
appropriateness of intervention to be monitored,
used during meal preparation sessions in hospital
allowing opportunties for adjustment and to
or at home. Many other pieces of equipment are
determine if therapy has been successful.
Stroke 129

Follow-up such as a low-salt, low-fat, low-cholesterol to


prevent a recurrent stroke; they may have given
All patients with residual impairments after the up work, driving and lost contact with friends and
initial period of rehabilitation should be offered a family. At about this time, contact with most
formal 6-monthly review and appropriate rehabilitation services will have ceased. These life-
treatment recommendations. Particular areas to changing actions happen over time and people
focus on during follow-up appointments include often need support and reassurance to continue to
any changes in impairment (e.g. cognition), activity explore new avenues.
(e.g. activities of daily living), participation (e.g.
fatigue), environmental and personal (e.g. mood, Social participation
stress resistance, social support, quality of life)
People who have had a stroke often feel dependent
circumstances since discharge.
on others, lack knowledge and with one-third
feeling socially isolated and one-quarter being
Young Stroke Patients depressed it is not surprising to find that patients
Younger stroke patients may have to make massive have often lost the social networks they had before
changes to their lives and this can take a long time the stroke. Occupational therapists can initiate and
to come to terms with: changes to jobs, loss of a enable people to start participating in society but
car, changes to hobbies, breakdowns in personal over time people do not want to be always
relationships, depression, and reduction in wealth associated with professionals and they move to
as well as any physical changes. Rehabilitation of groups more normally associated with the
younger stroke patients will include a wide community such as work, family, visiting friends,
multidisciplinary team and may need to be voluntary groups such as social clubs, luncheon
extended over many months. clubs, hobbies and religious activities. There are
As well as the rehabilitation programme, vast benefits to improving social participation on
occupational therapists can help younger people quality of life, personal choice, dignity and
with stroke access this website whilst in hospital, ultimately improving health by easier access to
they can introduce them to other younger people health centres, better physical health through
and ensure they are provided with vocational exercise, reduction in falls through improved
rehabilitation. muscle strength and balance.

Lifestyle and long-term management Leisure rehabilitation


The effects of having a stroke are often ongoing and It is known that participation in leisure decreases
therefore once patients are over the immediate after stroke, even for patients with a good physical
rehabilitation phase of approximately six months, recovery95. The significance of this reduction lies
they start looking forward to resuming their lives in the fact that satisfactory leisure is related to life
as much as possible and making changes to satisfaction. Consequently, such a decrease in
accommodate their new limitations94. It is leisure activity may reflect a decrease in quality of
generally recommended that people with ongoing life.
limitations after the initial rehabilitation phase
should be offered a six monthly review and be Getting out of the house and transport
provided with further rehabilitation if clear goals Many leisure and social activities require transport
are identified. These review sessions may highlight either to get to an event or to get supplies. However,
areas that may have been previously ignored by even just getting out of the house 'for the sake of it'
the patient such as social participation, leisure, is an important rehabilitation target after stroke as
returning to work, transport and social support. the it improves psychological and functional outcomes.
main emphasis at this point is for patients to start Half of all people with stroke do not get out of the
to plan, enquire, lead and be proactive about their house as much as they would like even after
lives, about the information they need and how they rehabilitation.
are going to undertake activities without the
An occupational therapy outdoor mobility
assistance of health professionals.
rehabilitation intervention found that people who
Patients may have changed their lives completely; had received the intervention, which involved the
they may have been given specific advice on diet patient practicing outside with a therapist, were
130 Neuro-Rehabilitation : A multi disciplinary approach

twice as likely to go out afterwards as those who activity, misery and frustration. Other physical
had received the routine rehabilitation programme. changes such as incontinence, drooling, emotional
liability may be off-putting to partners and again
Vocational rehabilitation lead to a reduction in sexual activity.
it is important that people are able to undertake As well as physical difficulties, psychosocial
education, employment, re-training and voluntary impairments and depression may affect the will to
work to improve their quality of life, fulfill a role in engage in sexual relationships; however, these may
society, avoid low self-esteem and depression. not be recorded till late after stroke as patients may
There is evidence that this can be achieved by be unwilling to talk about sexual difficulties. Mood
offering vocational rehabilitation (VR) to people disorders, such as depression and anxiety are
after a stroke96. commonly observed after a stroke, frequently
Both paid and unpaid work fulfils a diversity of affecting sexual relationships and sexual function
needs for the individual, and some of the many and conversely, sexual dysfunction may lead to
benefits of work are listed below: depression.
– Increased self-esteem. After a stroke, generally both patients and partners
want to know whether resuming sexual activity will
– Maintenance of routines and habits.
cause another stroke or epileptic fit and do not
– Participation in a productive activity. know whom to approach for advice. Although there
is only a low risk of stroke from sexual excitement,
– Involvement in a socially accepted role that
people are unwilling to have sex as they believe it
provides value to the community.
may cause another stroke. Sexual activity is a
– Having 'a reason to get up in the morning'. subject which is important to both patients and
– Challenging someone to expand their partners, and should be included in stroke
horizons. rehabilitation.

Occupational therapists can still assist the patient Occupational therapists are often approached to
in establishing realistic goals and expectations discuss sexual activity whilst they are dealing with
towards work and produce a written joint plan of other personal activities of daily living. This chance
action. to contribute to part of patient's lives often ignored,
should be embraced with knowledge and
Resuming sexual activity understanding.
As rehabilitation of stroke patients constantly Patients and partners need to know that returning
improves, many new techniques develop but some to their normal sexual activity is considered
activities are often neglected because of lack of routinely as an aspect of stroke rehabilitation. They
research, lack or rehabilitation knowledge or also need to know there is the opportunity to
because patients themselves do not wish to raise discuss their sexual activity either alone or in
the subject. One such area is a return to their normal couples, at a time which is appropriate to their
sexual activity. Generally, this is not routinely needs.
discussed with patients as part of their
rehabilitation, although for many, it plays an Outcome measures
important role in their life and discussion should The consequences of stroke on an individual's
be included in the assessment process. functioning are often complex and varied in nature.
Research has observed that after stroke there is a Stroke not only effects neurological functioning, for
decline in sexuality in both genders, and partner example, movement/speech but may also lead to
dissatisfaction is high. There are many reasons for a dependence in activities of daily living and
this decline: physical changes in the brain that may cognitive and perceptual difficulties. Outcome
reduce the sexual urge; physical changes to the measures are tools or instruments used to quantify
body that may make it difficult to move; the change in a patient due to an intervention, and
psychological changes that make people not want allow for the evaluation of the effects of
to have sex. Stroke can cause physical limitations interventions to be established.
that influence body positioning and movement Therefore, the measuring of outcomes is an essential
during sex which can lead to a reduction in sexual component in determining therapeutic
Stroke 131

effectiveness and therefore is central to the • Motor Free Visual Perception Test
provision of EBP. Additionally outcome measures
• Nine-hole Peg Test
can be categorised according to the three categories;
body function/structure, activity and participation. • National Institutes of Health Stroke Scale
• Rankin Handicap Scale
Body functions and structures Activities
Participation • Orpington Prognostic Scale
• Beck Depression Inventory • Rivermead Mobility Scale
• Action Research Arm Test • Timed Up and Go
• Behavioural Inattention Test
Other considerations
• Barthel Index Standardised and non-standardised outcome
• Performance Measure measures - Standardised outcome measures have
specified, standardised procedures for completion
• Canadian Neurological Scale and scoring. These measures will usually have been
• Berg Balance Scale tested for validity and reliability to ensure
consistency in application of the measure.
• London Handicap Scale
Additionally many measures will have been
• Clock Drawing Test normatively standardised for scoring, over large
• Box and Block Test populations. This means that therapists can
compare patients' scores against a normal range and
• Medical Outcomes Study to other patients with similar conditions. Non-
• Fugl-Meyer standardised outcome measures have not been
subjected to the same rigorous testing procedure
Assessment and are therefore often of poor quality, and
• Chedoke McMaster Stroke Assessment generalisation of scores from non-standardised
Scale measures is problematic.

• Nottingham Health Profile Use of outcome measures - Outcome measures can


be used for a number of reasons.
• General Health
Outcome measures can be used to evaluate:
• Clinical Outcome
– Improvement.
Variables Scale – Maintenance, for example, of function.
• Reintegration to Normal Living Index – Reduction, for example, of pain or discomfort.
• Geriatric Depression Scale – Prevention, for example, of disability or
• Functional Ambulation Categories discomfort.
• Stroke Adapted Sickness Impact Profile – Development/maturation.
• Hospital Anxiety and Depression Scale – Recovery, for example, of function.
• Functional Independence – Delay, for example, in rate of deterioration.
Outcome measures should be routinely used by
Measure
occupational therapists not only to evaluate their
• Stroke Impact Scale own practices but also to inform and motivate
• Mini Mental State Examination patients and carers and provide feedback to the
multidisciplinary team.
• Frenchay Activities Index
• Stroke Specific Quality of Life Psychological Aspect:
• Modified Ashworth Scale Psychology is the study of behaviour and the main
goals of psychological intervention post stroke is
• Motor Assessment Scale re-gaining or improving the functioning in aspects
132 Neuro-Rehabilitation : A multi disciplinary approach

like: cognition, socialization, regaining sexual life, second only to Alzheimer's disease as a leading
vocational guidance, and psycho-educating the cause of dementia. The most common types of
caregivers and to improve the overall quality of life. cognitive deficits arising from stroke are
disturbances of attention, language syntax, delayed
Psychological Assessments: recall and executive dysfunction affecting the ability
Neuropsychological assessment following stroke is to analyze, interpret, plan, organize, and execute
undertaken for the following reasons: complex information [113, 114,115]
• To provide prognostic information, in addition Memory Loss: Memory losses post stroke is
to monitor the rate and extent of natural common and there are many ways as to how their
recovery from stroke or improvement under memory can be affected:
therapy. • Verbal Memory: Patients may have difficulty
• To provide a baseline profile of cognitive remembering names, stories and may have
functions against which to assess subsequent difficulty in forming sentences.
natural recovery and to judge the outcome of • Visual Memory: Patients have difficulty in
any intervention. remembering names, faces, shapes, directions
• To provide a basis on which to plan any and things that they see around.
cognitive remediation interventions. • Recent Memory or Short term Memory loss:
• To provide a source of advice regarding Patients may have have trouble remembering
suitable placement or long-term care following instructions, conversations, and recent events.
termination of active rehabilitation. This They may also have trouble remembering
guidance can be used to help care staff and upcoming events such as doctor's
relatives gain better understanding of the appointments, difficulty learning new things
stroke patients cognitive deficits and and new information or skills.
competencies and therefore to behave • Remote memory: Patients may have trouble
appropriately. recalling or retrieving past information or
skills.
Assessments Post-Stroke:
• Mini Mental State Examination [112] to assess Reduced Attention Span:
the cognitive functioning. Post stroke the patients may have difficulty in
• Neurobehavioral Cognition Status Exam maintaining their attention span. When their
(NCSE) to assess the cognitive and behavioural attention span is reduced, these patients may be
functioning. unable to attend to a specific task for more than a
few minutes, at a time. Another effect of a reduced
• The Clock Drawing Test to assess the cognitive
attention span is that survivors may have trouble
functioning.
dividing their attention between more than one task
• The Montreal Cognitive Assessment [113] to (such as reading the mail while listening to the
assess the cognitive functioning. news), or going back and forth between two or more
different activities.
• Hamilton's Anxiety Rating Scale to assess the
level of anxiety.
Reduced Problem Solving Skills
• Beck's Depression Inventory to assess the Difficulties with problem-solving skills can mean
presence of depression. that a stroke survivor has trouble recognizing and
solving common everyday problems, such as a car
Cognitive Dysfunction Post Stroke: that will not start or a pot that is boiling over on
Every stroke is unique and the cognitive effects and the stove. Individuals with reduced problem-
problems post stroke also differ depending on, solving skills often require supervision at home in
where the stroke injured the brain, and your overall order to avoid accidents or injury. Stroke survivors
health. Stroke can cause vascular dementia, a with impaired problem-solving skills may also have
greater decline in thinking abilities. Some experts difficulty with math and maintaining their personal
believe that 10- 20% of patients over age 65 with finances.
dementia have vascular dementia. This makes it
Stroke 133

Difficulty with Social Communication body or who has difficulty communicating or who
Some survivors of right hemisphere strokes also suffers from frequent emotional outbursts. Medical
have difficulty with social communication. This treatments have although reduced both the severity
means that they may say inappropriate things to and frequency of stroke and on the other hand the
family, friends, or strangers. They may also have rehabilitative services have made it easier to adapt
trouble understanding jokes and humour. Also, to the changes stroke effects such as physical
they may not be able to form statements and many disabilities and problems with thinking processes.
times patient's speech is restricted to few words. However, there are significant changes that take
place in the person's relationship, in their feelings
Post Stroke Depression (PSD): and in their independence. In particular the
relationship between the strokes, suffers and the
Depression is the most under diagnosed and
caregivers may unalterably change especially id it
untreated squeal of stroke in spite of it being the
becomes clear that recovery may be limited.
most common complication of stroke. The reported
prevalence of stroke widely ranges from 16 percent Common emotional effects of post stroke sufferers:
to 62 percent, with more reliable studies reporting
• Anxiety
25 percent to 40 percent of stroke patients meeting
the Diagnostic Stastical Manual, Fourth Edition, • Loss of independence
Text Revision criteria of minor depression. Most • Emotional Liability
cases develop soon after the stroke persists from 6
to 12 months, with the frequency decreasing as time • Depression
progresses, although a significant minority of • Confusion
patients with depression for 24 months or longer
after the initial stroke. • Apathy

The hindrances caused in diagnosing a stroke • Memory loss


patient with depression is the overlap that exists • Irritability
between somatic manifestations of stroke such as
Stroke suffers have different symptoms depending
alterations in sleep patter, decreased appetite, and
on the area of brain injury, that is they may
feelings of sluggishness or appetite. Language and
experience both physiologically induced emotions
cognitive impairments, emotional liability and
and psychological emotions that are a result of brain
attention disturbances further complicate the
injury and changes that has brought about it. People
diagnostic picture. Post stroke depression has been
who suffer from stroke on the right side of the brain
reported in patients who have a history of
tend to experience memory problems, time
depression pre stroke, lack of social support and
disorientation and emotional highs and lows. They
who have resulting severe disability including
may also be less able to use abstract thinking, may
cognitive dysfunction, dysphasia or altered visual
talk excessively, may have a shortened attention
perception.
span, and may be more impulsive than before the
There are a few factors that raise the likelihood and stroke. On the other hand, people who have had a
severity of depression following a stroke: stroke on the left side of their brain tend to find
• The location of the brain lesion their language and reading abilities more seriously
affected. They will also be more cautious and easily
• Previous or family history of depression frustrated. As well, left brain injuries are most often
• Pre - stroke social functioning associated with depression. Whether this is a result
of the location of the brain injury, or whether it is a
Stroke survivors, who suffer from major depressive consequence of the difficulties in communication
disorder, may be less compliant with rehabilitation, that these people experience, is not known.
more irritable and may also undergo personality
changes. Personality Changes Post Stroke:
Changes in personality following a Stroke may be
Emotional Effects of Stroke:
very disturbing to the family. Personality is the
Someone who suffers from stroke his/ her life unique combination of an individual's thoughts,
suddenly changes from an able bodied person to feelings and reactions toward themselves, others
someone who is unable to move one side of the and their environment. Post stroke the individual
134 Neuro-Rehabilitation : A multi disciplinary approach

may not be the same as he/ she was pre stroke. maintaining personal hygiene. This could
The way in which they think, feel and react may be affect the relationship as the partner would be
altered. Family and relatives need to understand deprived of his/ her sexual needs.
the new and puzzling changes. Problems and
activities once tackled easily may be difficult or Caregivers:
impossible, while other tasks are unaffected. People Common Emotional side-effects of Stroke
may become confused, self-centred, uncooperative Caregivers:
and irritable, and may have rapid changes in mood.
• Anger
They may not be able to adjust easily to anything
new and may become anxious, annoyed or tearful • Guilt
over seemingly small matters.
• Doubt
Loss of motivation: • Impatience
Decreased or absent motivation and impaired • Helplessness
ability to initiate activity may be experienced by
• Resentment
stroke. Generally, referred to as adynamia and is a
direct result of brain trauma. Stroke suffers may • Depression and anxiety
do little beyond self-care tasks and may seem lazy.
There are many good emotions that will result from
The patient may appear to have a lack of initiative
caregiving, but negative emotions about your
in activities. With extra guidance and prompting
situation are also normal. Unfortunately, negative
after time they will have a better view in engaging
emotions toward your care recipient are normal as
in familiar activities followed by trying in new
well. Rather than beating yourself up when the
activities.
frustration overwhelms you, acknowledge that
these feelings are part of caregiving - and they don't
Sexual Desires Post Stroke:
make you any less of a caregiver. Also recognize
Recovery from stroke would take a long time and that it is in everyone's best interest for you to take
would lead through many stages. If being sexually regular breaks. To be the best caregiver, you need
active was important to a person before stroke then to rejuvenate yourself from time to time. And your
there would be the same need post stroke. A few care recipient needs breaks from you too!
hindrances to having an active sexual life post
stroke would be: Even if you are the primary caregiver, your family
and friends need to understand that their support
• Fear: The common fear following a stroke is is needed - and part of that means supporting you.
that having sex will bring on another stroke. Your ability to care for your loved one is dependent
There is no reason why after a couple of weeks upon your well-being. You need many of the same
you cannot begin to have sex if you feel ready things that your care recipient needs:
to do so. Medical evidence supports this. If you
still feel unsure about having sex then arrange • Affection and love
to speak to your own doctor. • Emotional support
• Emotional changes: Both men and women • 'Away' time
experience similar emotional problems after a • Exercise and a healthy diet
stroke. How you feel about yourself and how
you perceive others feel towards you, can lead • Proper rest
to you losing confidence in yourself. It can take • Relaxation time
time to adjust to and come to terms with the How do you manage all this? Creating a care plan,
changes in your life after a stroke and many can help.
people experience anxiety and depression as
a result. This can have a knock on affect on Psychological Intervention:
your desire for sex.
Pharmacological Treatment for Depression:
• Change in relationships: Post stroke there
could be a sexual awkwardness that a patient Pharmacological treatment for post stroke
would experience. This could be especially depression is still questionable as the potential side
true if the patient needs assistance in effects of antidepressant medications is quiet
Stroke 135

evident. If a person is only mildly or moderately damaged brain must first learn or re-learn gross or
depressed, psychological treatment alone may be large-scale systems before fine and complex
effective. However, if depression is severe or systems. The gross cognitive systems include
persists, medication is often necessary as well. attention, focus and perceptual skills. And when
Medical research shows that depression is often these are redeveloped, complex intellectual activity
associated with an imbalance of certain chemicals will follow through.
in the brain. Antidepressants can help rebalance
these chemicals. Antidepressant medication can When to start cognitive therapy:
take seven to 21 days to work effectively and should Cognitive therapy should start when the patient is
not be started or stopped without medical advice. still in the hospital as time is very crucial after a
stroke attacks. Early cognitive therapy will focus
Psychological Counseling: on increasing alertness and attention. It will also
Patients with chronic physical illness and co-morbid focus on improving the stroke victim's orientation
depression may have a very negative view of their to person, place, time and situation. It will also help
situation. It could also lead to feeling helpless and the stroke victim to comprehend speech, another
hopeless about the situation. Counseling would problem associated after a stroke attack. Post
help the patients identify and change these negative Discharge, the stroke patients could be on home
ways of thinking and find ways to find hope and cognitive rehabilitation or on outpatient basis
meaning to their lives with the existing level of depending on the needs of individual patient.
functioning. Counseling session would involve
Strategies of Cognitive Rehabilitation:
usage of various psychological therapies depending
on the individual patients needs. • Form and follow a routine during the day,
which would help to improve the sequential
Cognitive Rehabilitation: memory.

Aim of Cognitive Rehabilitation: • The patient should be oriented to date, day,


time, place and person.
The chief aim of cognitive rehabilitation is to help
the stroke survivor to achieve the most independent • Break down tasks and activities into steps
level of functioning as possible. The objectives of which would help to re-learn the lost activities
cognitive rehab also vary from person to person. and functions. It would also help to learn new
Some stroke victims would want to have the ability information and recalling it faster.
to express needs verbally in simple terms while • Make note of things, activities and important
others would be happy with just pointing to events. For example: medication time or
pictures. But majority of stroke survivors want to doctor's appointment, etc.
improve their capability of defining words and to
identify the cause and effect of relationships. • Arranges the things that the patient uses in
fixed places and cupboards, this will help the
When a stroke patient undergoes cognitive patient remembering where he has to look out
rehabilitation program the first 2 steps to for particular things and to find it easily.
rehabilitation are:
• The patient should be given responsibilities
• To restore functions which, can be restored within his capacity which would help him be
• To teach new compensatory patterns of cognitively active and would also help
cognitive activities increase his confidence if he is successful. For
e.g. Making phone calls, making online
After which different cognitive methods are used
payments, etc.
to rehabilitate the patient like:
• Solving arithmetic problems Sexual Counseling Post Stroke:
• Solving logical puzzles Talking about ones feeling about one's body and
appearances to a psychologist or the partner would
• Concentrating on writing and reading skills
be very helpful post stroke. You can express your
• Playing memory games feelings in many different ways, through talking
Recovery of the brain follows a systematic pattern but also with body language and physical contact
of brain development. Just like in infants, a stroke such as kissing and cuddling. Taking the first step
136 Neuro-Rehabilitation : A multi disciplinary approach

may be the biggest hurdle to overcoming your household need to be discussed frankly [116]. The
anxiety and shyness about resuming sexual contact. family may have less time for leisure and privacy.
Retaining closeness and intimacy within your Children may also be confused by the new addition
relationship will help to overcome difficulties. It is to their home. Still, if you explain the situation to
important to keep communicating with each other. children and offer ways for them to help, they may
There can be a subtle change within a relationship volunteer to help with certain duties, like cleaning,
when a partner becomes a carer, especially when preparing dinner or spending time with your care
assistance is needed with personal care. This can recipient.
sometimes cause embarrassment or affect the way
Even if one person accepts the role of primary
you feel about each other.
caregiver, a role that may include housing and daily
care activities, they should be able to ask for
Changing Roles and Assigning Duties:
significant help from other family members. The
Caring for Caregivers: primary caregiver will also need regular breaks
Part of what makes the adjustment to a disability from time to time, including longer 'vacations'.
so difficult is the disarray it throws your life into. Other family or friends can take over care
temporarily, or perhaps the care recipient can stay
All of a sudden the old rules don't work anymore,
and new ones need to be drawn out. This can be an in a nursing home for a week or two. As well, most
extremely frustrating time, particularly for the care states have government-run programs that offer
recipient and their caregivers. You can minimize respite services. [117]
these frustrations by drawing up a care plan [115].
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142 Neuro-Rehabilitation : A multi disciplinary approach

Ch.3 Cerebral Palsy


Dr. Mamta Lohia, [Link]., F.N.R., C.B.E.(USA); Dr. Myola D'sa, [Link].;
Ms. Akshata Shetty, M.A. (Clinical Psycologist); Dr. Manasi Jani , (Speech Therapist)

Definition: enthusiasm for surgical intervention to correct


deformities, to provide stability and to improve
Cerebral palsy (CP) is a collection of motor
motor control decreased as assessment showed that
disorders resulting from damage to the brain that
deformities recurred or new ones developed.
occurs before, during or after birth. The damage to
the child's brain affects the motor system, and as a In 1932,Winthrop [Link] began developing a
result the child has poor coordination, poor balance, new approach. Phelps became aware of the
or abnormal movement patterns-or a combination necessity for including exercise, muscle training,
of these characteristics. The universally recognized and bracing in the treatment of CP. Since Phelp's
definition of cerebral palsy is "a non progressive era various non surgical approaches have evolved,
but not unchanging disorder of movement and / or emphasing Neuromuscular [Link] include
posture due to an insult to or anomaly of the Neuro Muscular Reflex therapy (Fay & Doman),
developing nervous system". CP is a static disorder Neurodevelopmental treatment ( Bobath),
of the brain, not a progressive disorder. This means Neurophysiological Approach (Rood),
that the disorder or the disease process will not get Proprioreceptive Neuro Muscular Facilitation
worse as the time goes on. However the child may (Kabat, knott & Voss) and Sensory Integration.
appear to worsen if not given proper intervention,
not because of an increase in the lesion but just Prevalence & Incidence:
because the damaged brain is not able to cope up Prevalence rates for CP vary somewhat in different
with the physical demand of the growing body and countries and regions, largely reflecting economic
the increasing demand of the environment and nutritional factors and access to good obstetric
surrounding the child. care. Because of the absence of any "test " for CP,
the vagueness and elasticity of even the best
History : definition, inaccurate ascertainment (many cases
In the late 1800's Sir William Osler, a British are never reported to physicians or public health
Physician, coined the term CP. Around the same authorities) and lack of agreement between
time, Sigmund Freud (1856-1939), a different experts in the inclusion of the individual
Neuropathologist in Austria, conducted extensive cases, precise figures are difficult to obtain. Further
research and published some of the earliest papers confusion results because many cases die in infancy
on the subject. But William John Little (1810- and others may be so mild as to be unrecognized.
1894),an English surgeon was responsible for the Prevalence rate for CP therefore should be
first clinical description, and the recognition of the presented as the prevalence at a given age per 1000
link between low birth weight and perinatal events live births of the defined population under study.
in [Link]. Little theorized that lack of oxygen Of every 2000 infants born, 5 are born with CP. This
damaged the sensitive brain tissues, which controls incidence had remained constant for over 30 years.
movement. Sigmund Freud disputed Dr. Little's Although improvement in medical care have
conclusions. He pointed out that most CP children decreased the incidence of CP among some children
suffer from additional problems like seizures, who otherwise would have developed the disorder.
mental retardation and visual disturbances. This led Medical advances have also resulted in the survival
him to believe that CP might be caused during the of children who previously would have died at a
brain's development in the womb many months young age, and many of these young children
before birth. However doctors only seriously began survive with an impairment or a disability such as
investigating CP in 1861 when Dr. William John CP. Throughout the industrialized world a
Little,published a paper describing children with prevalence of about 2 per 1000 is found. In contrast
spastic diplegia. Spastic diplegia is still sometimes estimates for India are around 45 per 1000, rather
called Little's Disease. more in rural & less in urban populations.5 to 8
percent of premature infants under 1500 grams
For many years CP was treated from a surgical
birth weight, who survive have CP. The marked
perspective that was based upon surgeon's
difference in prevalence provides a challenge for
experiences in treating poliomyelitis. The
Indian Preventive medicine to make the best
Cerebral Palsy 143

obstetric and perinatal care available to all Haemorrhage (PVH), Intraventricular


communities. Essential keys to progress include Heammorhage (IVH), Periventricular
improved education and nutrition to mothers to be. Heammorhagic Infarction, and Periventricular
Leukomalacia (PVL) Preterm infants are more
Etiology : prone to GMH, PVH, and IVH, because their
Many causative factors produce CP, infact it is the cerebral circulation is sensitive to changes of bold
multiplicity of causative factors that led Denhoff pressure (pressure passive) and they lack
and Robinault to refer to CP as a group of diseases. supporting glia in their germinal matrix.
The factor common to the group is time of
Maternal Infection Preterm Birth Pressure
occurrence of the brain dysfunction in the prenatal passive Circulation
or natal period. The brain damage that leads to CP
can be caused by : Inflammatory Cytokines Ischemia Reperfusion GMH/IVH
1. Idiopathic ( no known cause of damage to
Venous congestion
brain during pregnancy ) -still the most
common cause. Damage to Oligodendrocyte precursors Free radicals PVH
2. A viral infection during pregnancy such as
cytomegalovirus (CMV) or rubella. Failure of Nerve Cell Growth Absent or delayed myelination CP
3. Iso-immunization reactions eg : Jaundice due Events leading to CP in preterm Infant.
to immunization mechanism related to mixing
of fetal Rh positive blood with maternal Rh Prolonged Partial Dysregulation of Ischaemia -
negative blood Asphyxia cerebral blood flow Hypoxia
4. Hydrocephalous either before or after birth.
Calcium Influx Opening of NMDA Glutamate free
5. Developmental Defects. Channels radicals / NO2
6. Defects in Biochemical maturation
Mitochondrial ATP Pump Cell Necrosis /
7. Defects in Hereditary material (genetic) Dysfunction failure Apoptosis CP
8. A blood clot in the fetus brain causing a stroke
while in vitro. Events leading to Cerebral Palsy in
Term Infant
9. Haemorrhage (Bleeding into the brain ) : while
inutero this could be due to a bleeding
disorder, after birth, this can be seen as a
Classification of CP :
complication of extreme prematurity. The diverse motor and non motor associated defects
10. Prolonged period of Asphyxia (lack of oxygen) in CP, would suggest the difficulties in categorizing
for example abruptio placenta, i.e. when the this disorder. Therfore children with CP are
placenta tears away from the uterine wall categorized on the basis of the types of neuromotor
during labor, by cutting off the baby's blood deficit and topographic distribution of the deficit.
supply. According to, the following schema, which uses
only major groupings of the classification of the
11. Infections, eg : Bacterial meningitis after birth.
American Academy of Cerebral palsy.
12. Head trauma from shaken baby syndrome
(child abuse ) during the first year of life. Neuromotor Classification by Minear (1956)
13. Toxins & Poisons, eg : Lead poisoning during 1. Spastic : This type is characterized by
the first two years of life. exaggeration of the stretch reflex and increased
deep tendon reflexes in the affected parts.
Etiopathology: 2. Athetoid : The chief characteristics of this type
The underlying basis of most neurodevelopmental are the slow worm like, involuntary
sequelae in CP infant is white matter damage, uncontrollable, unpredictable and purposeless
collectively called periventricular Leukoence- motions at rest.
phalopathy. This term encompasses germinal 3. Rigidity : When the part is moved, there is a
matrix haemorrhage (GMH), Periventricular continuous resistance in agonist and
144 Neuro-Rehabilitation : A multi disciplinary approach

antagonist muscles, simulating the sensation Classification based on Severity :


of bending a lead pipe. The degree of rigidity This type of classification is used emphasing on the
may, from time to time be referred to as "lead quantification of neuromotor involvement that
pipe ", "cog wheel " or "intermittent " rigidity. limits patient's ability to perform Activities Of daily
The principal clinical finding is the living.
hypertonicity of the muscles which in some
patients is so great that no motion is present. 1. Mild: The patient needs no treatment, since he
has no secondary problems, is able to care for
4. Ataxia: The principal sign noted in the ataxic his daily needs and ambulates without the aid
patient is disturbance of balance and or any appliance.
equilibrium. The ambulation pattern has been
described as reeling, drunken type gait. 2. Moderate: The patient need treatment, since
he is inadequate in self care, ambulation, and
5. Tremor : The chief characteristics of this type had associated problems. Braces and self help
are the involuntary, uncontrollable motions appliances are needed.
that are reciprocal and regular in rhythm.
3. Severe: The patient needs treatment, but the
6. Mixed : Not all children with CP can be degree of involvement is so severe that the
diagnosed as true spastics, athetoics, or prognosis of self care, ambulation is poor. And
[Link] 1% of the total may be mixed also the associated secondary complications
cases in which there is more than one type of are major concern hampering patient's quality
the above described characteristics. of life.

Topograhic Distribution Of Neuromotor


Involvement
1. Paraplegia: involvement of the lower
extremities. Patients with paraplegia are
practically always of the spastic type.
2. Hemiplegia: involvement of an upper and
lower extremity on the same side of the body.
Patients with Hemiplegia are almost always
spastic, but occasionally an athetoid
hemiplegic patient may be seen.
3. Triplegia: involvement of three, extremities,
usually both lower extremities and one arm.
The disability is usually of spastic type.
4. Quadriplegia or tetraplegia : involvement of
all four extremities. The term diplegia is some The following list presents the medical problems
times used to indicate that the lower most often associated with CP.
extremities are more involved than the upper.
Almost all patients of athetosis have all four Neurological Problems:
extremities involved. a) Mental Retardation.
b) Learning Disabilities.
c) Attention Deficit Hyperactivity Disorder/
Behavioral issues.
d) Seizure Disorder (epilepsy ).
e) Visual Impairment.
f) Swallowing Difficulties.
g) Speech Impairment.
h) Hearing Loss.
i) Hydrocephalus.
Cerebral Palsy 145

Orthopaedic Problems: Summary:


a) Scoliosis. DYSTONIC Spastic 65% Quadriplegia (25%)
Predominantly Diplegia (20%)
b) Hip Dislocation.
abnormal tone Hemiplegia (20%)
c) Contractures of Joints. Dystonic Usually
d) Discrepancy in leg length. regarded
as Spastic
Secondary Effects: Hypotonic May eveolve
to spastic or
a) Communication Disorder. athetoid types
b) Drooling. DYSKINETIC Athetoid, 15%
c) Poor Nutrition/ growth Predominantly Choreo-
abnormal athetoid
d) Aspiration Pneumonia and gastro esophageal movement tension
reflux.
Ataxic Rare
e) Depression.
MIXED 20 % or more
f) Fragile bones and frequent fractures.
g) Cavities-Tooth decay and gum disease. A study of 1000 children with cerebral palsy in
northern India showed spastic Quadriplegia to be
h) Constipation or Bladder bowel incontinence.
the most common group (61%), followed by spastic
diplegia(22%), with dyskinetic cases amounting to
about 8 %.Mental retardation (75% )appeared to be
very much common in this population than in
industrialized nations.
Diagnosis: Early signs of CP may be present from
birth. Most children with CP are diagnosed during
the first 2 years of life. But if a child's symptoms
are mild, it can be difficult for a doctor to make a
reliable diagnosis before the age of 4 or [Link]
growth and development of infants born with CP,
may be erratic, showing normal patterns of
development in some areas but not in others. It is
at these points of perceived delay that the parents
need to become concerned, seek professional
Quadriplegic CP child with spasticity and poor advice, investigate the problem and if necessary,
voluntary control of limbs. obtain remedial help for the child. Thus, diagnosis
can be based on following broad criteria's :

A ) Concept of Normal Neurological Maturation:


Overviews of Developmental milestones
Age Gross Motor Visual/Fine Motor Languages Social
1 months Prone, Lifts head Head usually fisted; Soothes to voice Regards face
stares or objects
3 months Supports chest in Gross placed rattle; Coos/laughs Smiles easily,
prone position follows slow moving spontaneously
objects with eyes
6 months Rools and sites well, Reaches and grasps, babbles; plays Fear of Strangers;
without support transfers hand to peek-a-boo smiles at self in
hand mirror
146 Neuro-Rehabilitation : A multi disciplinary approach

12 months Walks alone Pincer grasp of raisin Say "mama," "dada", Shy, but play game,
+ 2 other words gives affection
18 months Walks up steps Stacks 3 blocks; Points to named body Helps with simple
manages spoon parts; follows simple tasks; imitates play
commands
24 months Alternates feet on Stacks 6 blocks; turn At least 50-word Washes/dries hands;
stairs; kicks balls books pages vocabulary; helps get dressed
understands 2-steps
commands
30 months jumps with both feet Holds pencil in hand, Use pronouns "I," Plays tags; asserts
not fist "ME," "YOU" personality
correctly; states
full name
36 months Balances on 1 foot, Imitates block bridge; Recognizes 3 colour Plays with children,
5 sec,; rides tricycle buttons takes turns
Note:- It is not uncommon for a child to lag behind in one area and be advanced in another. However,
there are generally accepted limits for what is considered "normal development".
Source:- Adapted from The Harriet Lane Handbook, 17th ed. (Philadelphia Elsevier Mosby, 2005).

B) Overview of Abnormal Infant Neurological Development:


Every child development involves gaining mastery of four major types of skills: gross motor, fine motor,
communication and social. Development in these areas occurs simultaneously to prepare the child to
meet physical, social, linguistic and emotional demands. Significant delays in early child development
are "red flags" that should prompt parents to discuss their concerns with the child's doctors.

Developmental Red Flags


Milestone Normal Concern if Not
Acquired by
GROSS MOTOR
Head up/chest off in prone position 2 months 3 months
Rolls front to back, back to front 4-5 months 6-8 months
Sits well un supported 6 months 8-10 months
Creeps, crawls, cruises 9 months 12 months
walks alone 12 months 15-18 months
Runs; thros toy, from standing without fall 18 months 21-24 months
Walks up and down steps 24 months 2-3 years
Alternates feet on stairs; pedals tricycle 3 years 3½-4 years
Hops, skips; alternates feet going down stairs 4 years 5 years
FINE MOTOR
Unfists hands, touches objects in front of them 3 months 4 months
Moves arms in unison to grasp 4-5 months 6 months
Reaches either hand, transfers 6 months 6-8 months
Pokes forefinger; pincer grasp; finger feeds; holds bottle 9 months 1 years
Throws objects, voluntary release; mature pincer grasp. 12 months 15 months
Scribbles in imitation; holds utensil 15 months 18 months
Feeds self with spoon; stacks 3 cubes 18 months 21-24 months
Cerebral Palsy 147

Turns pages in boos; is stready cup drinker;


removes shoes and socks 24 months 30 months
Urbuttons; has adult pencil grasp 30 months 3 years
Draws a circle 36 months 4 years
Buttons clothes; catches a ball 4 years 5 years
LANGUAGE
Smile socially after being talked to 6 weeks 3 months
Goos 3 months 5-6 months
Orients to voice 4 months 6 months
Babbles 6 months 8 months
Waves bye-bye; says"dada," "mama" indiscriminately 8-9 months 12 months
1-2 words other than dada/mama; follows 1-steps
command with gesture 12 months 15 months
7-20 words; knows 1 body part; uses mature jargoning 18 months 21-24 months
2-word combination; 20 words; points to 3 body parts 21 months 24 months
50 words; 2-words sentences; pronouns (inappropriate);
understands 2-step commands 24 months 30 months
3-word sentences; plurals; minimum 250 words 36 months 3½-4 years
knows colour; asks questions; multiple-word sentences (tells story) 4 years 5 years
SOCIAL
Regards face 1 month 1-2 months
Recognizes parents 2 months 2-3 months
Enjoys viewing surroundings 4 months 5-6 months
Recognizes strangers 6 months 7-8 months
Reciprocal games: so big, pat a cake 9 months 12 months

Primitive Reflex Patterns: 3. Midbrain: Optical Righting, Labrinthine reflex,


These are patterns seen in the early stages of Body on neck, Body on body, Neck righting
development with disappear later on or with reflex.
advanced age. These reflexes are essential for 4. Cortical reflex: Equilibrium reactions, which
normal progressive motor [Link] can be in prone, supine, kneeling, sitting and
not overcoming these primitive reflex patterns at standing. Equilibrium reaction in supine and
the right time should be definitely considered as in prone position in present from 6 months
abnormal. Initially lower centers such a s spinal onwards, in quadripud position is present
cord control these movements but later on higher from 8 months, and in sitting starts normally
centres like midbrain and cortex take control over from 10-12 months. Kneeling equilibrium
them and dominate the lower ones thus integrating occurs from 15 months onwards and finally
them for various voluntary functional task. in standing occurs from 18 months onwards.
Accordingly we have four levels at which reflexes
Automatic reflexes: Moro's reflex, Gallants trunk
are regulated:
incurvatum, Landau's reflexes and parachute reflex.
1. Spinal cord: Flexor with drawal, Extensor
thrust, Cross extension, Palmar grip, Plantar Abnormal Manifestations and Prognosis
grip, Sucking reflex, Rooting reflex, Primitive
1) A child with spinal cord dominance will not
walking.
be able to walk and carry out ADL and
2. Brainstem: Symmetrical Tonic neck reflex, therefore he / she will be bedridden
Assymmetrical Tonic neck reflex, Tonic throughout. This condition leads to a lot of
Labrinthine reflex, Positive and negative contractures and thus aim of treatment is to
supporting reaction.
148 Neuro-Rehabilitation : A multi disciplinary approach

prevent secondary complications due to lack abnormal muscle tone, unusual posture - doctors
of ambulation like preventing tightness or have to rule out other disorders that could cause
contractures, respiratory complications, etc. similar symptoms. Neuroimaging techniques like
MRI can also show a doctor the location and type
2) Child with brain stem dominance will also be
of brain damage and can hence, help in predicting
bedridden and unable to [Link] of
the severity and type of disorder and prognosis
treatment is same as above and all secondary
accordingly.
complications due to lack of ambulation need
to be prevented.
3) Midbrain dominance children will have a
relatively better prognosis, such a child can
ambulate due to and or absent equilibrium
reactions, some walking aids amy be required.
4) Cortical Dominance children will have near
mormal development.

C) Prediction of Developmental
Abnormality:
1) Biochemical:
Apgar Scoring system based on observation of
MRI Brain showing perinatal
color, respiratory effort, heart rate, tone, and reflex hypoxic brain injury
activity has been a guide to assess child's condition
at birth. Predictability is improved with the addition
of a low 5 - min Apgar score and the need for
intubation at birth. Various newborn spinal fluid
components have been shown to be effective
markers of Hypoxic ischaemic encephalopathy.
Predictive effects of newborn urine products have
also been considered since asphyxia results in injury
to the kidney. Paucity of urine flow (oliguria) and
elevated ß2 microglobulin at 36 hours have been
found to correlate with later neurological [Link]
should be kept in mind that even if these or other
biochemical markers of hypoxic ischaemic stand the
test of time an experience, they deal only with
asphyxia and ischaemic injury to the brain, which
MRI brain showing periventricular leukomalacia.
represent but one etiological basis among many for
static encephalopathy.

2) Prenatal:
Prenatal screening includes amniocentesis,
chorionic villus sampling, and use of ultrasound
are used to monitor the pregnancy and pick up
early cases of CP or developmental disabilities such
as Down's Syndrome.

3) NeuroImagining:
Doctors diagnose CP by evaluating a child's motor
skills and taking a careful and thorough look at
medical history. In addition to checking for the most
characteristic symptoms - slow development, MRI Brain showing perinatal hypoxic brain injury
Cerebral Palsy 149

Symptoms seen in CP correlating to area of brain damage seen on MRI


ANATOMICAL AFFECTION MRI FINDINGS CLINCAL FINDINGS
Bilateral white matter Periventricular Spastic diplegic,
necrosis,descending motor leucomalacia quadriplegic, visual &
fibres,optic,acoustic cognitive defects.
radiations
Focal, multifocal ischaemic Specific vascular Hemiplegic, seizures.
brain necrosis infarction, typically left MCA
Basal ganglia neuronal injury Status marmoratus Choreoathetosis or
mixed picture.
Selective neuronal necrosis Lateral geniculate, Mental retardation,
thalamus, basal ganglia seizures
Parasaggital cerebral injury Bilateral medial & Upper extremity more severely
posterior portions of cortex affected than lower.

Physical Assessment and Developmental III) Motor Assessment Instruments:


Screening Scales: 1. Alberta Infant Motor Scale (AIMS)
A large variety of rating / screening instruments 2. Early Motor Pattern Profile (EMPP)
have burgeoned within recent years, with varying 3. Gross Motor Function Measure (GMFM)
degrees of accuracy for prediction of
4. Movement Assessment Inventory (MAI)
1. Developmental delay 5. Test of Infant Motor Performance (TIMP)
2. Definite developmental abnormality
Developmental Evaluation Procedure
3. Specific Developmental Diagnosis.
A) Developmental History
Among those most commonly used at present at
the present tiem are the following : 1) Chief Complaint
2) Family and genetic history
I) Developmental Scales: a) Pregnancy
1. Amiel - Tison b) Labor / Delivery
2. Bayley c) Perinatal / Neonatal
3. Brazelton 3) Developmental Milestones
4. Dubowitz 4) Other developmental Features.
5. Haataja et al 5) Reviews of systems
6. Peabody 6) Past medical history

7. Prechtl B) Developmental Physical Examination.

II) Developmental Screening Testes: C) Developmental Neurological Examination.


1. Denver Developmental Screening Test 1) General Observation.
(Denver II) (most commonly used ). 2) Quality of general movements.
3) Tone
2. Batelle Screening Test 4) Patterns of motor behavior.
3. Clinical Adaptive Test (CAT)/ Clinical a) Primitive
Linguistic and Auditory Milestone Scale b) Postural
(CLAMS) 5) Sensation
4. Knobloch Revised Screening Inventory 6) Cranial nerves.
7) Cerebellar Function.
5. General Movement Assessment (GMs). 8) Dystonia.
150 Neuro-Rehabilitation : A multi disciplinary approach

9) Motor signs any goal will be difficult to achieve without the


a) Upper motor cooperation of the patient and relatives. The patient
b) Lower motor has to be motivated regularly to gain confidence.
10) Neurological Soft signs. Only when the motivation level of the patient is
very high will the child cooperate in the treatment
D) Developmental Screening Instruments.
session. The goal set for the patient should be
E) Laboratory Evaluation. challenging but at the same time achievable. The
goal of any therapy is to maximize each child's
Speech Affection in Cerebral Palsy: functioning to all that it can be.
Children with cerebral palsy have higher risks of
having a concomitant problem like autism, hearing Physiotherapy Management of CP:
impairment, mental retardation, learning disability Physical and Occupational therapist's approach
etc. The symptoms that the children present with overlap because their focus is to help the child
depend on the severity of problem and presence of develop motor skills. Areas in common include
the associated problems. seating assessments, early intervention therapy,
Children with cerebral palsy can have problems in and developmental testing. Physical therapists,
comprehension, delayed expressive speech i.e however focus mainly on gross motor or large
children may speak few sentences to complete muscle activities involving the legs, such as
absence of spoken language, difficulty in grasping walking, bracing using crutches, and rehabilitation
new concepts and learn normally due to mental after surgery.
retardation. Developmental dysarthria or impaired Treatment in CP has evolved from focusing on
speech clarity is also very common in children with impairments (such as spasticity and contractures)
cerebral palsy. Due to the muscle affected, the to activities (such as walking), and then to
children with cerebral palsy may present severe considerations of participation (such as child's
dysphagia and are many of the times fed on semi family role as a sibling) Thus a child can be impaired
solids and liquids. In the developmental stage of and not necessarily be disabled, and a person can
life, they fall victim to malnourishment and be disabled without being handicapped. To make
aspiration pneumonia due to which they further it clear:
deteriorate and experience frequent hospitalization. Impaired means a deviation from normal
Treatment: Disabled denotes restricted ability to perform
normal activities of daily living; and
The latest and the most effective way of treating
Cerebral Palsy children comprise of an integrated Handicapped means being unable to achieve an age
approach. The team effort is required to make the appropriate role in the society.
child independent in most ADL for his age and
thereby improve his quality of life. Treatment Interventions:
The team includes: Various interventions have been designed and
developed by various scientists and therapists to
- Parents
treat CP children. At times a combination of few
- Physicians
help to overcome problems in them. The theories
- Teachers
and the treatment interventions are explained in
- Nurses
brief :
- Physical Therapists
- Occupational Therapists I) Neuro Developmental Technique (NDT) :
- Speech Language Pathologists The most renowned of all interventions addressing
- Audiologists the movement and posture of the child with CP is
- Rehabiliattion Workers [Link] concept was developed by Karel and
- Orthotists Berta Bobath in the 1940's and it views development
- Clinical Pscychologists dynamic, sequential, cephalocaudal, proximal to
- Vocational Counsellors distal, automatic before conscious, responsive and
- Social Workers. lastly adaptive.
The therapy aims at developing rapport with Bobath's promoted the use of handling techniques
parents and patients and is extremely important as
Cerebral Palsy 151

to inhibit abnormal tone and primitive reflexes and of volitional movement present, strategy adopted
to facilitate normal movement. They believed that to treat child may contain either the facilitation
children with CP needed the experience of normal approach (absence of tone) or the inhibitory
movement. For children unable to move, a approach (presence of abnormal tone)
therapist's hands provided the experience. Quality
of movement was considered very important. Facilitation:
Bobath's also used reflex- inhibiting positions to
The use of primitive or tonic reflexes, quick
reduce the effects of the tonic reflexes. Treatment
stretching, tapping, vibration, approximation, and
progression centered on the normal developmental
weight bearing may be required to prepare the child
sequence, assuming carryover to functional tasks.
for the performance of functional activities. In
Today, NDT emphasizes functional goals. addition, facilitation can also be in terms of
Principles of treatment include weight shifting, education, mechanical, postural, manual,
weight bearing, and normalizing muscle tone. motivation, etc based on the clinical presentation
Quality of movement is still important and may of the child and goal of therapy.
reduce abnormal stresses on joints, possibly
preventing secondary impairments and deformity. Principles of facilitation:
Modalities include the use of balls, bolsters,
The most important Kinesiological parameters need
horseback riding, and swimming. Therapists
to be considered are
combine NDT principles with a variety of other
• Range of motion
approaches such as strengthening and the use of
adaptive equipment. • Alignment
• Base of support
The art of NDT: • Movement in all three planes (sagittal, frontal
and transverse plane)
The intervention process begins with the
assessment of the individual's functional Inhibition:
performance. Analytical problem solving is used Therapist also uses inhibition to restrict the child's
to develop a treatment plan. Treatment focuses on
atypical postures and movements that may prevent
increasing function by building on the individual's
the development of more selective motor patterns
strengths while addressing the impairments.
and efficient performance.
Therapeutic handling is one strategy, which is • Prevention or redirection of components of
utilized to help the individual achieve his or her movements those are unnecessary and
functional goals. Therapeutic handling is integral interfering with intentional coordinated
in NDT approach.
movement.
Therapeutic handling allows the therapist to: • Constrain the degree of freedom to decrease
the amount of force the child uses to stabilize
• Feel the child's response to changes in posture
and movement posture.
• Facilitate postural control and movement • Balance antagonistic muscles
synergies that broaden the client's options for • Reduce spasticity or excessive muscle stiffness
selecting successful actions. that interferes with moving specific segments
• Provide boundaries for movements that of the body.
distract from goal and
• Inhibit or constrain those motor patterns that,
if practiced, lead to secondary deformities
further disability, or decreased participation
in society
• The therapist's hands are purposefully and
specifically placed on the child's body during
facilitation techniques. Mrs. Bobath called this
therapeutic handling through "key points of
control".
Based on the child's motor control, presence or
absence of abnormal muscle tone and the quality Child performing quadrapud activity
152 Neuro-Rehabilitation : A multi disciplinary approach

therapist will upregulate or downregulate sensory


stimulation. Use of varying textures like cotton,
rough gauge, deep pressure are used to stimulate
kinesthetic and Proprioreceptive input.

Normalizing tone of the muscles:


In the infant with CP,spasticity is seldom significant
in the first 6 months, but sometimes between 6
months to 24 months, it starts becoming
[Link] is defined as hyperexcitability
of the stretch reflex, resulting in a velocity
dependent increase in muscle tone. Secondary to
Child prone on elbows to promote weight the brain lesion, there are also mechanical changes
bearing on the upper limbs. in the muscles of children with [Link] muscles
produce more force for a given change in length
and therefore feel stiff and resist passive
lengthening.
In children with hypertonicity slow passive
movements, sustained stretch, cryotherapy over the
muscles for 15 to 20 minutes, stimulation of
antagonist movement and vibrations are used. On
the contrary, for the cases with hypotonicity weight
bearing, joint compression, rhythmic stabilization,
vibrations, cryotherapy in brisk manner and
tapping is used.
Aaprt from therapy medications like Lioresal,
Tizanidine or Dantrium are also prescribed orally.
Child performing half kneeling activity. In severe cases Baclofen Pumps are also
recommended. A dorsal Rhizotomy, is another
Sensory integrative therapy:
surgical treatment, in which nerves as they exit
Sensory integrative (SI) therapy, was developed by from the spinal cord are identified and the ones to
Jean Ayres in the 1970's, is based on promoting the be found most involved with spasticity are cut.
organization and processing of sensory
Serial Casting and intramuscular botox injections
information. Proponents believe that information
are also recommended in few cases where spasticity
coming into the body from the proprioceptive,
is interfering with [Link] effect of botox lasts
tactile, and vestibular systems is disorganized and
for 6 -8 months and needs a good back up with
not processed well. This lack of cognitive processing
exercises.
is considered a perceptual problem. These
processing problems cause difficulties with motor
planning and motor control. Therapy focuses on
movement and environmental awareness. The use
of swings, scooters, and other moving objects helps
the child process sensory input and use sensory
information to plan movement and gain postural
control. Traditionally, occupational therapists are
more involved in the use of SI treatment.
Children with CP often have sensory processing
dysfunction (SPD) and show sensory based motor
dysfunction, Sensory processing evaluates how the
brain takes it what it receives through the [Link]
Swiss ball activities to normalize tone and
SI therapy state of arousal of child is very important
strengthen trunk muscles.
for learning [Link] on arousal
Cerebral Palsy 153

Developing postural reaction: Trigger points were used to reflexly to stimulate


creeping, rolling, or crawling activities. Proponents
Postural adjustments are essential if the child is to of Vojta suggest that very early intervention does
move freely and to adjust to various environmental not allow the development of abnormal crawling
demands rapidly. Motor milestones can be best patterns and subsequently promotes normal
achieved by good postural reactions. Postural walking patterns.
reaction consists of righting reactions, protective
extension and equilibrium reactions. Initially Strengthening Programs:
children first develop righting reactions. Righting
reaction allow the child to orient his head in space Children who cannot walk are by definition weak.
so that the eyes and mouth are horizontal regardless There is no evidence that strengthening increases
of the position of the body. It also helps in spasticity. Infact, there is evidence that weakness
maintaining the proper alignment of the head with is a problem in this population of children and
respect to the body and vice versa. Once righting strengthening is effective in reducing weakness and
reaction develop in a particular position protective improving function. Therapist need to lengthen
extension comes up. Lastly equilibrium reaction spastic agonist and strengthen the antagonist. For
emerge which helps the patient to counteract the very young children, weight training may not be
opposing force and enable them to maintain appropriate but therapists can incorporate
balance. These reactions are best developed by strengthening activities in to child's therapy
various exercises on vestibular ball and tilt board. program through games and repetition of
functional movements. eg: In Partial weight
Rood approach: treadmill training child is suspended in a
specialized harness and helps strengthening the
Rood utilized both the sensory and motor systems
to facilitate movement with her treatment weak antigravity muscles and facilitate normal gait
techniques. She felt that preventing the patterns.
development of abnormal movement patterns was
important. She used sensory input to achieve or Stretching and Mobility:
relax muscles to promote normal movement. For The muscles should be maintained at the
example, during therapy gentle stroking is used to appropriate physiological length for normal
have relaxatory effect on the muscles thereby used movement control and normal postural
to inhibit spastic [Link] the other hand brisk
adjustments. In CP because of delay or absence of
stroking, tapping, quick ice, etc, has been found to
normal movement muscles are usually in a
develop tone in the muscles, hence can be used for
shortened position hence stretching of the muscles
cases like flaccidity.
is essential to increase the neuromuscular control.
Pediatric Constraint Induced Therapy: Length of the muscles should be maintained not
only through stretching but also through various
Constraint induced therapy is used to improve the functional activities. Thus home exercises and
use of affected upper extremity in a child with activities are very important in additional to the
Hemiplegic [Link] normally functioning or therapy at the pt clinic.
stronger upper extremity is immobilized for a
variable duration in order to force the use of the Taping :
affected or weaker upper extremity over time.
Taping provides joint stability, supports weak or
The efficacy of this approach has not been over lengthened muscles. It provides sensory
established, and the adverse effects of prolonged proprioreceptive inputs. It is known to increase or
immobilization often normally developing upper decrease activation.
extremity are a significant concern.
Electrical Stimulation:
Vojta's Technique:
Neuromuscular electrical stimulation is supposed
This approach was introduced by [Link] Vojta to increase range of motion, strengthen muscle and
and used afferent sensory stimulation through bring about muscle reeducation. Therapeutic
touch, stretch and pressure for facilitation of Electrical Stimulation (TES) helps in reducing tone
movement. Reflex locomotor patterns and and strengthening of muscles, but tolerance is
proprioreceptive input are the basis for treatment. sometimes an issues with children. Therefore
154 Neuro-Rehabilitation : A multi disciplinary approach

treatment is done during sleep, and recommended hippo therapy. The underlying theory is that the
age for beginning TES is 2 years. positioning and large movements provided by
horseback riding are very helpful in establishing
Aqua therapy or hydrotherapy: balance and relaxation of spastic [Link]
Hydrotherapy is therapy performed in water. The vertical motions of horseback riding are thought to
effects of water give children a feeling of provide sensory stimulus which decreases muscle
weightlessness, which helps to reduce tone and tone. Sitting on horse helps with stretching hip
allow those children better motor control. It is also adductors and improves pelvic tilt and trunk
a good modality for gait training, especially in an positioning. This allows better movement and range
overweight child who may be able to walk in water of motion for the therapist to work with, after the
with relative weightlessness. In addition, child finishes with session.
swimming as a recreational activity is excellent in
children with CP. For many children for whom Bracing and Orthosis:
walking consumes a great deal of energy, learning The role of orthoses in CP is :
to swim, and using this a physical conditioning is
1. Improve function and efficiency.
an excellent option.
2. Improve joint biomechanics and alignment.
Theratogs: 3. Prevent Deformity.
They provide joint stability and increase body
4. Protection after surgery.
awareness. Improves posture, balance, gait and
movement skills. More information available on 5. Encourage a normal motor patterning.
[Link]. Stabilising Pressure Input
The most commonly used Orthoses are :
Orthosis (SPIOS) are flexible, provide dynamic
stability and balance thereby increasing body a. Ground reaction AFO (GRAFO)
awareness in space. b. AFO's Solid and hinged.

Hyperbaric Oxygen Therapy (HBOT) c. Supra malleolar Orthoses.

Hyperbaric oxygen therapy (HBOT) is the d. Shoe inserts.


inhalation of 100% oxygen inside a chamber that is Orthotic management using AFO is a critical part
pressurized to greater than 1 atmosphere (therefore
described as hyperbaric because the pressure is
above atmospheric pressure). HBOT is typically
administered at I-3 atmospheres of pressure.
It is not clear from a scientific standpoint how
HBOT could help overcome damages to brain tissue
that occurred years before in a child with CP. When
HBOT was studied in a scientific manner in two
group of children with CP, with a Control group
placed in pressurized room air and a treatment
group in pressurized oxygen, both groups
improved, without any difference between the two
groups. Similar results were found in a second such
controlled study of children with CP.
Ear pain/discomfort and bleeding from the ear are
by far the most commonly reported adverse events
during HBOT. In addition, there may be an
increased risk of seizures in those treated with
HBOT.

Equine therapy:
It is also known as Horseback riding therapy or Child made to walk with AFO's
Cerebral Palsy 155

of rehabilitation, controlling ankle joint motion • Biomechanical model


produces an effect on the knee during gait and by
• Canadian Model of Occupational Performance
using an AFO to manipulate the ankle rockers,
(CMOP)
therapist can increase or decrease the plantar
flexion knee extension couple. The correct selection • Cognitive Disabilities model
and prescription of lower limb orthosis is essential • Lifestyle Performance Model
to maximize a child's rehabilitation. Orthosis for
correct period of time gives the best results. • Model of Human Occupation (MOHO)
• Neurodevelopmental therapy (NDT)
Occupational Therapy Intervention
• Occupational Performance Model (OPM)
Cerebral palsy (CP) • Person Environment Occupation Performance
CP is a non progressive disorder of posture and Model (PEOP)
movement. It is often associated with epilepsy and • Sensory Integration model
abnormalities of speech, vision and intellect
(resulting from a defect/lesion in the developing Occupational therapy assessment scales
brain). It is a condition that occurs early in life and Occupational therapy services may include
is present throughout a person's lifetime. It can comprehensive evaluations of the home and other
affect all aspects of a person's development environments (e.g., school), recommendations for
throughout their life adaptive equipment and training in its use, and
guidance and education for family members and
Occupational therapy Intervention
caregivers.
Occupational therapy is the art and science of
enabling engagement in everyday living, through OT interventions can be classified into five specific
occupation; of enabling people to perform the intervention categories,
occupations that foster health and well-being; and 1. Training of sensorimotor functions including
of enabling a just and inclusive society so that all play activities to facilitate motor performance.
people may participate to their potential in the daily 2. Training of daily activities skills including
occupations of life (Townsend& Polatajko, 2007, p.
feeding, dressing, bathing, personal hygiene,
372).
writing etc.
The practice of occupational therapy in Cerebral 3. Parental counseling in which parents are
palsy means the therapeutic use of occupations, educated how to stimulate Independence in
including everyday life activities with individuals, their child.
groups, populations, or organizations to support
participation, performance, and function in roles 4. Advice and instructions regarding use of
and situations in home, school, workplace, assistive devices including provision of
community, and other settings. mobility aids like wheel chair and bathroom
devices.
Frames of Reference 5. Provision of splints such as hand orthosis to
Conceptual frameworks and Models are essential facilitate hand functions.
for determining "best practice". Best Practice The above Classification is based on the
involves imaginative problem solving, creative International Classification of Functioning
application of knowledge and research and Disability and Health (ICF) and enables the
evaluation of effectiveness of Intervention to bring categorization of all intervention possible in OT
about improved client outcomes and effect
The following are the occupational therapy tests
organizational change. Depending upon the
that can be used specifically in the assessment of
impairments in a child a suitable frame of reference
Cerebral palsy:
or combination of approaches is used
• BRUININKS-OSERETSKY TEST OF MOTOR
Examples of specific occupational therapy Frame
PROFICIENCY (BOTMP)
of Reference models which are used in evaluation
and treatment in CP include the: Purpose: Developmental motor skills
Age Range: 4.5 -14.5 years
156 Neuro-Rehabilitation : A multi disciplinary approach

Areas Tested: Balance, strength, coordination, Areas Tested: Eighty-eight items of gross
running speed and agility, upper limb motor function divided into five dimensions:
coordination (ball skills), dexterity, fine motor -Lying and rolling-Sitting Crawling and
control, visual-motor ability. kneeling-Standing-Walking, running, and
jumping. Items were selected to represent
• CANADIAN OCCUPATIONAL
those typically performed by children by age
PERFORMANCE MEASURE
five
Purpose: To detect changes in parent or child's
• GROSS MOTOR FUNCTION
self-perception of performance over time.
CLASSIFICATION SYSTEM (GMFCS)
Age Range: Any age
Purpose: To classify a child's present gross
Areas Tested: Satisfaction and disability rating motor function.
of daily activities and routines, which are,
Age Range: 12 months to 12 years
identified by the child and family as important
part of daily life Areas Tested: based on self-initiated
movement, with emphasis on sitting, transfers,
• FUNCTIONAL INDEPENDENCE MEASURE
and mobility
FOR CHILDREN (WeeFIM)
• HOME OBSERVATION FOR
Purpose: To determine the severity of a child's
MEASUREMENT OF
disability, the measurement of caregiver
THE ENVIRONMENT (HOME)
assistance needed in the performance of
functional activities, and outcomes of Purpose: A screening tool to identify the
rehabilitation quality and quantity of social, emotional and
cognitive supports available to the child in the
Age Range: Children without disabilities: 6
home environment
months to 8 years; Children with
developmental disabilities: 6 months to 12 Age Range: Infant and toddlers version birth
years; Children with developmental to three years of age.
disabilities and mental ages less than 7 years
Areas Tested: Infant and toddlers version:
Areas Tested: Eighteen items grouped into two forty-five items clustered into six subscales:
major categories of function, motor, and Parental responsivity, acceptance of child-
cognition that are divided into six domains Organization of the environment-Play
divided into sub domains: materials-Parental involvement with the child.
Motor, Self-care: eating, grooming, bathing, • ORAL MOTOR/FEEDING RATING SCALE
dressing, toileting, Sphincter control: bladder
Purpose: To document oral motor/feeding
and bowel management, Transfers: chair,
patterns and feeding function
wheelchair, toilet, tub, and shower,
Locomotion: wheelchair/crawl, stairs, Age Range: One year through adulthood
Cognitive -Communication: comprehension, Areas Tested: Two major areas of oral motor/
expression, Social cognition: social interaction, feeding behavior: Oral motor/feeding patterns
problem solving, and memory. lip/cheek movement, tongue movement, jaw
• GROSS MOTOR FUNCTION MEASURE movement. Related areas of feeding function:
(GMFM) self-feeding, adaptive feeding equipment, diet
adaptation, position, sensitivity, food
Purpose: To evaluate change in gross motor
retention, swallowing, oral-facial structures
function in children with cerebral palsy,
describe a child's current level of motor • PEDIATRIC EVALUATION OF DISABILITY
function, and determine treatment goals. INVENTORY (PEDI)
Age Range: No specific age range is Purpose: To determine functional capabilities
recommended by the authors; however, the and performance, monitor progress in
test has been validated on children between 5 functional skill performance, and evaluate
months and 16 years. Seems best suited for therapeutic or rehabilitative program outcome
children two to five years in children with disabilities
Cerebral Palsy 157

Age Range: Six months to seven years, six It predicts 12-month motor performance with
months sensitivity 92% and specificity 76% and
preschool motor performance with sensitivity
Areas Tested: Two hundred seventy-one items
72% and specificity 91% at 3 months of age
divided into three subtests in the Functional
Skill Scale:-Self care: eating, grooming, Areas Tested: 27 observed behaviors and 26
dressing, bathing, toileting-Mobility: transfers, elicited behaviors assessing the ability to orient
indoors and outdoors mobility-Social function: and stabilize the head in space and in response
communication, social interaction, household to auditory and visual stimulation in supine,
and community tasks. Also environmental prone, side lying, upright, and during
modification and amount of caregiver transitions from one position to another, body
assistance is systematically recorded in alignment when the head is manipulated,
Modification Scale and Caregiver Assistance distal selective control of the fingers, wrists,
Scale hands, and ankles, antigravity control of arm
and leg movement
• SENSORY INTEGRATION AND PRAXIS
TEST • SCHOOL FUNCTION ASSESSMENT (SFA)
Purpose: Measures sensory systems Designed to facilitate collaborative program
contributions to balance and motor planning for students with a variety of
coordination disabling conditions.
Age Range: 4-8 yrs 11 months Purpose: Used to measure a student's
performance of functional tasks that support
Areas Tested: Numerous tests of postural
his or her participation in the academic and
control, motor coordination & planning, fine
social aspects of an elementary school
and gross motor function, & sensory
program.
integration
Type of Test: criterion-referenced assessment
• THE ALBERTA INFANT MOTOR SCALE
(AIMS) Areas tested: Three parts: Participation in
school activity settings; Task supports;
Purpose: To identify motor delay and to
Activity Performance. Includes physical and
evaluate maturation over time. Fifty-eight
cognitive/ behavioural tasks.
items related to posture, movement, and
weight bearing in prone, supine, sitting, and
standing AIMS has been designed to assess
Occupational therapy strategies
gross motor maturation, to trace motor The focus of OT Treatment should be on the
retardation and to identify infants that might facilitation of Independence. The management of a
benefit from early intervention. In addition, the child with CP is done with the objective of
AIMS may also be useful in designing and optimizing functional abilities. OT focuses on
monitoring a treatment program development of skills necessary for performance
Age Range: Used during first year of life of Activities of Daily Living. These activities include
play, self care activities such as dressing, grooming
Type of Test: 58 item, performance-based, and feeding and fine motor tasks such as writing
norm-referenced, observational too and drawing. OT also addresses cognitive and
• TEST OF INFANT MOTOR PERFORMANCE perceptual disabilities especially in the visual motor
(TIMP) area. Another aspect of OT is the adaptation of
equipment and seating to allow better upper
The TIMP is a test of functional motor behavior extremity use and to promote functional
in infants independence. Parental counseling is another
Purpose: a criterion-referenced measure important aspect of the occupational therapist with
designed to evaluate motor control and regards to optimizing parental support for
organization of posture and movement for improving functional abilities of child with CP.
functional activities in infants Different approaches to treatment are taken and
considered. Since no child is the same, intervention
Age Range: 32 weeks gestational age to age 4
for each child is specific and unique and related to
months
158 Neuro-Rehabilitation : A multi disciplinary approach

the skills the strengths and limitations of the child.


To treat sensorimotor problems like spasticity,
hypotonicity, non integrated primitive reflexes,
immature postural or equilibrium reactions,
sensory dysfunction and other issues like lack of
age appropriate play behavior, difficulties in
performing ADLs, cognitive and social
impairments, Occupational therapists work on the
following :-
Stimulating back extensors to facilitate extension
1. Development of Postural Control in prone position
Postural control is the ability to maintain balance
Ocupational therapist's use Neurodevelopmental
and alignment while upright in space. The child
Treatment Approach which is aimed at facilitating
with CP has poor postural control due to delayed
and normalizing hyper- or hypotonic muscle
or incomplete motor development, decreased
reflexes using facilitation-inhibition mechanisms;
voluntary control, lack of stability, difficulty in
reaction and movement patterns; and managing the
antigravity movements, lack of dissociative
specific reactions to the treatment of equilibrium.
movements, exhibition of stereotypical movements
with compensatory mechanisms. The development
2. Development of Hand Skills
of postural control is also affected in presence of
deficits in the organization of sensory inputs. Hand function involves the following components
like reach, grasp, carry voluntary release, in hand
Therefore Interventions are aimed at:- manipulations, bilateral hand use. Often the CP
• Facilitation and development of antigravity child has impaired hand function skills due to
movements, e.g. lowering a child from a sitting problems in isolation of movements and tending
to supine position to facilitate neck flexion to use total patterns, insufficient force application,
against gravity. Neck and trunk extension can improper timing of movements, limitations in trunk
be promoted by placing the child in prone control and inability to bring hands in midline.
positions over a wedge or large therapy ball Intervention aims at

• Facilitation of automatic reactions like • Proper positioning of the child. E.g. first
Righting, equilibrium and protective reactions, consider the optimal position for eliciting the
e.g. placing the child on unstable surfaces and skills desired. Certain body positions can be
moving the surface using variations in speed, used to elicit specific hand skills like Supine
ranges and rhythms. Reach out activities while position for arm movements and visual regard
sitting on ball, bolster or balance board. of hands, prone position with forearm weight
bearing for shoulder stability, side lying
• Facilitation of sensory organization. E.g. position for unilateral movements. While
walking on different texture surfaces with sitting at table for fine motor tasks the child
various visual conditions like closed eyes or should be seated at an appropriate table height
dim lights. Reaching out while swinging on on a stable chair with foot supports, with arms
different types of swings on table surface without elevating shoulders.
• Facilitation of anticipatory control. e.g. • Improvement of postural tone and control E.g.
catching and throwing a ball. Kicking a ball. (refer to development of postural control
above) and inhibition of tone using weight
bearing or slow movement activities
• Development of hand skills by:-
Promoting isolated arm and hand movements
e.g. stabilizing proximal muscles (trunk and
shoulder) to promote opportunity for the
isolated hand movements.
Facilitation of neck flexion while going Enhancement of reach, grasp, carry voluntary
from sit to supine release, in hand manipulations, bilateral hand use
Cerebral Palsy 159

e.g. using pegs, beads or marbles of various sizes SI therapy in CP enables the child to make
and shapes, clay activities, stringing and stacking purposeful adaptive responses to sensory input in
activities the environment. It refers to a therapeutic
intervention which uses strong kinesthetic and
proprioceptive stimulation to attempt to organize
the CNS.

Guiding principles in SI:


• Sensory input can be used systematically to
elicit an adaptive response
• Registration of meaningful sensory input is
necessary before an adaptive response can be
made
• An adaptive response contributes to
development of sensory integration
• Better organization of adaptive responses
enhances the child's general behavioral
organization
• More mature and complex patterns of
behavior are composed of consolidations of
more primitive behaviors
• The more inner- directed a child's activities
Proper positioning in sitting to promote hand are the greater the potential of the activities
functions for improving neural organization.
Intervention techniques should address the
3. Sensory Integration (SI)
underlying sensory deficit and not the behavior. SI
SI is the ability of the CNS to process information uses all senses but focuses primarily on the
to make adaptive response to the environment. The Vestibular, Proprioceptive and Tactile Senses
CP child may have sensory integration problems together known as the power senses. A child's brain
which are: organizes sensory stimulation from touch and
• Sensory modulation problems movement in order to learn and respond
successfully to the environment. Vestibular
• Sensory defensiveness stimulation includes linear, circular and rotatory
• Sensory dormancy/Sensory registration movements like lying on the top of a large exercise
problems ball while receiving rocking or bouncing
movements and sitting inside an inner tube swing.
• Adaptive movement response problems
Proprioceptive stimulation includes compression
• Vestibular processing problems and traction like weight bearing activities like wheel
• Sensory discrimination and perceptual barrow walking and jumping on trampoline and
problems hanging from a trapeze. Tactile stimulation includes
different textures stimulation like sand playing,
• Tactile discrimination brushing painting activities. Different types of
• Proprioceptive perception stimuli influence a child's muscle tone and
normalizing muscle tone enables a child to move
• Visual perceptual and function well. Children with increased muscle
• Other sensory problems tone typically benefit from calming sensory stimuli
such as slow movement, rhythmic music, dim lights
• Praxis problems
and a quiet environment. Children with low muscle
Occupational therapists work with children who tone typically benefit from erratic music, bright
have sensory processing disorders, by engaging lights and irregular movement such as jumping.
them in activities that promote sensory integration.
160 Neuro-Rehabilitation : A multi disciplinary approach

Intervention focuses on visual perceptual training


involving Developmental, Neurophysiologic,
Sensory Integration and Compensatory approaches
which helps in improving skills that limit function
and also compensate for the limitation.

5. POSITIONING AND SEATING


Proper positioning can be achieved by use of
handling techniques, pillows/ wedges, bracing,
inserts etc to minimize the effects of abnormal tone
and reduce secondary complications.
Evidence supports that children with CP should be
fitted for wheelchairs that place them in a
functionally safe position, which includes; a hip-
Fig 4 Vestibular stimulation provided in the
belt, an AO, footrests, and a cutout tray, with the
form of swinging on a bolster swing
addition of a sloped forward seat to improve upper-
extremity function.
4. Development of Visual Perceptual Skills
Visual Perception is defined as the total process Adaptive Devices
responsible for the reception (sensory functions)
• Nonslip surface on chair to prevent slipping
and cognition (specific mental functions). (e.g. Dycem)
Visual receptive Functions • Bolster, rolled towel, or blocks for feet
• Acuity • Adapted or alternate chair, stander
• Accommodation • Custom fitted wheel chair or insert
• Binocular fusion
6. ACTIVITIES OF DAILY LIVING
• Steropsis- Binocular depth perception or three
dimensional vision
(ADLs)
• Convergence and divergence Activities of Daily Living-ADL involve taking care
of one's body such as toileting, bowel and bladder
Visual Cognitive Functions management, bathing personal hygiene and
grooming, eating and feeding, dressing, functional
• Visual Attention - alertness, selective attention,
mobility sleep and rest. Approaches to improve
visual vigilance and divided or shared
Performance of ADL involve Establishing,
attention
restoration, maintenance and Modification or
• Visual memory- short term and long term Adaptation and Prevention and education.
• Visual discrimination- Occupational therapists use many Behavioral
o Object (form) perception- form techniques like chaining (backward and forward),
constancy, visual closure and figure shaping and reinforcements to teach ADL skills.
ground The following are the issues to be considered while
o Spatial perception-position in space, applying the functional approach (Activities of
depth perception and topographical Daily Living-ADL) in a combined intervention of
orientation child-centered activity and structural-
developmental intervention respectively:
• Visual Imagery
1. Developing a feeling of interest in performing
CP children with Visual Perceptual problems daily activities and motivation
demonstrate many problems like in ADLs e.g.
difficulty in combing, tying laces, matching clothes 2. Experiencing learned functions by using daily
or in Play e.g. difficulty in sports, cutting, life devices
constructing, doing puzzles. 3. Practicing self-care skills
Cerebral Palsy 161

4. Developing visual-spatial skills within the • Deep and firm pressure to reduce tone
environmental setting
• The therapist can also use her hands and
5. Developing creative self-expression through fingers to provide external support e.g. using
play, artistic activity, movement and other one finger to promote chin tuck and the other
activities under jaw for support from the side or from
front
6. Developing perceptual and visual-motor
functions which are necessary for learning
7. Developing more complex play levels

• Feeding
Evaluation should encompass the patient's
visual, perceptual and cognitive skills,
physical control of head, trunk and
extremities, oral structures and ability to suck,
masticate, and swallow. Also face and mouth
sensitiveness, ability to sense temperature,
facial muscles, interference of primitive
reflexes like rooting, bite, sucking. Impaired
oral reflexes like gag coughing reflexes, Outer Oral motor stimulation to improve
oral motor assessment ( facial expression, lip oral motor control
control and jaw control) and inner oral motor
assessments (palatal functioning, tongue Adaptive devices
musculature and motor control) are
Non slip mats, wet towels, suction cups are used
conducted.
to stabilize eating utensils
The family's desires and expectations in regard
Adapted cutlery may be more suitable, e.g. rocker
to the child's feeding capacities should also be
knife, extended handle cutlery (e.g. foam handle
considered.
on utensil), Adaptive drinking devices (e.g. cup
Intervention in feeding involves positioning, with cut out rim)
handling and compensatory strategies.
Adapted chairs, tray attached to table
Proper positioning should be emphasized
while feeding to promote oral motor function. Practical tips
• Normalize child's tone as much as possible
Appropriate positioning for feeding: before beginning the feeding process
• Neutral pelvic alignment of trunk. Pelvic • Observe the child while he/she is eating.
alignment is facilitated when the child s well
• Make sure the child can see the plate, the food
supported against a flat back, on a flat seat and
which is on it and the spoon bringing the food
square on the buttocks with hip and knee in
from the plate to her mouth.
90° of flexion.
• Talk to her/him about the process and let her/
• Good head, neck and shoulder alignment in him see, feel and smell the food, feel the plate
slight neck flexion or in neutral. and the spoon
• Chin tuck with the back of the neck elongated • The feeding person should be seated directly
• Providing the child with external postural in front in order to maintain proper position
stability enhances stability, good alignment • Table should be positioned at axilla height and
and easy feeding. close to chest so distance from plate to mouth
is reduced
After positioning the child, the therapist can use
handling techniques to aid oral movements: • Chewing may need to be encouraged slowly
and patiently by very gradually increasing the
• Tapping or quick stretch, vibration to increase density of texture and later on the 'lumpiness'
tone of the food offered.
162 Neuro-Rehabilitation : A multi disciplinary approach

• Encourage her/him to pick up food (such as • Bathing


cakes, biscuits, bread or fruit) in her hands and Achieving cleanliness is essential for maintaining
bring it to her mouth. good hygiene. Bathing can be made fun and a
• Use different food temperatures and textures special bonding time with the caregiver.
to increase child's awareness of what is in his Occupational therapists can use bathing
mouth. therapeutically to enhance motor and sensory skills.

• Firm pressure is more acceptable to the child Adaptive devices


than light touch
Hand held Faucet
• Placing a mirror in front of the child during
• Soap on a string or soap tied in a lofah or liquid
mealtimes helps his feeding skills. soap
• Give the child the drink he likes best allowing • Railing, grab bars
him to make a choice when you teach him to • Adaptive chairs
drink
• Semi-inflated inner tube can be used as a
• Therapist can guide the extremity in the positioning device
correct pattern if required • Nonskid mats
• Hydraulic grab bars
• Dressing
Learning and participation in dressing is a Practical tips
major step in step in achieving independence. • Positioning the child in a such way that
Even with limited motor and sensory skills normalizes his muscle tone as much as possible
dressing can be made easy by motivating the providing a sense of security, rather than
child to actively participate and reducing challenge his sense of balance.
demands placed on the child. • Bath sponge wedge can be used to bathe an
infant in both sitting and lying positions.
Adaptations • Bathing can be time which can be used to teach
various concepts for example, arm in, out,
Adaptive clothing
through: tub full, empty, wet, warm, cold and
• Loose clothing free from unnecessary working on body parts identification. Thus
fastenings which should be limited to few bathing provides the child with many
layers opportunities to learn cause-and-effect
• Use of Velcro and elastic band clothing instead relationships, spatial relationships and
of buttons or zippers tolerance of different sensations.
• Front openings, Pullovers, large buttons, • The time following a bath can also be very
Stretchy clothing productive. For example, a fast run with a terry
towel can help the child learn to process
Practical tips sensations and can lead to better body
• Work on undressing first, as it is an easier skill awareness.
than dressing. • Toileting
• Always put the clothes on the most affected Independent toileting is very important in
part of the body first. achieving self maintenance. Toilet training can
• Positioning child on floor is safer than on a be quite challenge in a CP child as the child
chair. must be temperamentally and physically
ready to accept toilet training as well as be able
• Techniques that inhibit spastic postures will
to understand the process in order to have any
facilitate the movements in and out of clothing,
success.
If her legs are bent before putting on socks and
shoes it may help ease any stiffness in her Adaptive devices
ankles and feet and her toes are less likely to Hand held jet sprays
curl under. • Use of Toilet paper
• Encourage to stand up and hold on to • Soap on a string or soap tied in a lofah or liquid
furniture. soap
Cerebral Palsy 163

• Railing, grab bars play on different surfaces and at safe heights


• Adaptive commodes or chair with hole placed • Position child with both arms forward when
over commode playing with toys.
• Footstools to support feet • Make certain that the child can see what is
• Use of straps to maintain safe and proper happening.
posture • Talk to the child at the child's eye level.
• Nonskid mats
• Give the child ample time to respond
Practical tips • Maintain a good balance between noisy, active
• A fairly casual, nonconfrontational play and quieter, less strenuous activities.
introduction to the process will help reduce • Present toys that encourage your child to reach
any stress related to toilet training. and grasp with the hand that is more difficult
• Visual break down of task on wall to use, but allow the child to use whichever
hand he chooses.
• Habit training to go at same times to toilet
• Reinforce them for their success or for sitting • When teaching dressing to the child, put the
patiently on the toilet and trying. more affected arm or leg into the clothing first.

• Instruct caregivers to follow same routines at • When interacting with your child, take the
school more affected hand.
• Encourage bilateral activities such as rolling
MOBILITY clay or throwing a large ball,
Being mobile enhances a person's ability to learn, • Provide multi sensory input toys that have
interact with others and participate in the interesting things to see, hear and feel.
community. For children with mobility
• Avoid too noisy small play items
impairments, a variety of mobility aids and devices
are available to provide support, motion and access, • Grade level of activities, gradually increasing
as well as to enable them to lead active and fulfilling its complexity
lives. • A mirror can be a great aid in playing so that
Mobility aids include: the child can get visual feedback
• Canes • Toys that have enlarged handles or knobs to
• Crutches grasp
• Walkers
Age Appropriate toys used to enhance play
• Manual wheelchair
behaviors
• Powered wheelchair with joystick, head
0 -2 Years
switch, or sip/puff controls
• Grab rails and Railings • Play mat and frame with dangling toys
• Rocking and bouncing games
7. Play Skills • Making lots of babbling and cooing noises
Play can be an important part of the learning
• Tickling games
experience and development of motor skills for a
child with cerebral palsy. Selecting an activity • Building blocks
should incorporate the child's interests and the skill • Story books - with voice
he possess to participate in safe playing.
• Peek a Boo games
• Appropriate adapted equipment, such as
wedges, bolsters, bean bags, CP chairs, may • Banging on musical drums
be used. • Imitation and turn-taking
• Make certain your child changes positions • Playing with mirrors, press toys
frequently. Children should be encouraged to
164 Neuro-Rehabilitation : A multi disciplinary approach

• Unwrapping toys organization, problem solving, and language and


reading ability, ideation and graphomotor function.
• Lentils, rice and pasta in tubs to sit in, put your
The building blocks of early handwriting are:
hands in or just throw about
• Spatial and body awareness
1-3 Years
• Postural control
• Finger puppets
• Visual perception
• Tunnel games
• Fine motor control
• Surprise bags full of toys and interesting
objects for your child to find • Directionality
• Pulling and pushing • Handwriting
• Object identification Intervention focuses on
• Pretend games with dollies and teddies Neurodevelopmental approach: postural and limb
preparation activities like jumping on a trampoline,
• Painting, using fingers or brushes
pushups, bear walks etc to modulate tone
• Cars and trains
Biomechanical approach: sitting posture should be
• Paper tearing and crumpling with feet firmly planted on the floor, height of table
2-4 Years such that it should be 2inches above flexed elbow,
paper position should be slanted so that it is parallel
• Story books to the forearm of the writing hand, pencil grip
• Clay modeling should ideally be a dynamic tripod grasp, pencil
with a wide diameter, various writing paper (lined,
• Ball games
unlined, double lined, textured, margins)
• Obstacle courses
Sensorimotor approach: involves controlling
• Messy play (i.e. sand castle, finger painting) sensory input through selected activities to enhance
sensory systems. An inclined, vertical or horizontal
• Sticking
writing surface, writing tools like crayons, sketch
• Stamping (leaves, crumpled paper prints etc.) pens, vibratory pens, and using different textures
• Spot the difference like sand, shaving cream, talcum powder

• Shape and color matching Adaptive writing equipment


• Make believe Pencil Grippers and weighted pencils and handi-
writers (soft elastic looped around the writing
• 'Simon says'-imitation games
utensil and wrist that keeps the utensil in the
• Imaginative Play e.g. Turning boxes into toys appropriate space of the hand)
(such as castles, cars or space ships)
Adaptive writing paper that is color-coded, sticky
• Action rhymes or rough on one side or has larger lined area
• Listening games
9. Use of Assistive technology
• Making music with home-made instruments
The cerebral palsy assistive technology includes
Games involving "spotting the difference", or hardware and software which helps maximize their
pointing out which part of a picture or drawing abilities to access information and services. It has
does not belong, help the child's neurological the potential to increase abilities of person with
development. disabilities and can lead to independent living.

8. Hand writing Skills - Typing Master Software

Writing is a fine motor task required to compose - Text to Speech Software


stories, complete written examinations, copy - Voice recognition computer applications
numbers etc. It is a complex task which requires
- On - Screen Keyboard setting, Magnifier
synthesis and integration of memory retrieval,
Cerebral Palsy 165

setting, Narrator setting, Mouse Keys • Electronic or talking electronic dictionary,


Utilization and setting. thesaurus, or spell checker (e.g. Franklin
Bookman)
The objective is to see how the technology can be
used by a person with disabilities to enable him or • Word processor with spelling and grammar
her lead a more purposive life with some skills to checker
do work and at the same time too have time for
• Word processor with word prediction (e.g.
relaxing and enjoying life.
Co:Writer) to facilitate spelling and sentence
construction
Some Assistive Technology used in
• Talking word processor for multisensory
WRITING are typing
• Pencil or pen with adaptive grip
• Voice recognition software
• Adapted paper (e.g. raised lines, highlighted
• Multimedia software for expression of ideas
lines, and so on)
(assignments)
• Slantboard
Communication
• Type writer
• Augmentative and Alternative
• Portable word processor Communication (AAC)
• Computer • Communication board or book with pictures,
• Text to Speech Software objects, letters, or words
• Voice recognition computer applications • Eye gaze board (Eye gaze communication)
• On - Screen Keyboard setting, Magnifier • Simple voice output device (e.g. Big Mack,
setting, Narrator setting, Mouse Keys Cheap Talk, Voice-in-a-Box, Micro Voice,
Utilization and setting. Talking Picture Frame, or Hawk)
• Device with speech synthesis for typing (e.g.
Alternate Computer Access Cannon Communicator, Link, Write:Out Loud
• Keyboard with easy access or access DOS with laptop computer)
• Keyguard
READING, STUDYING AND MATH
• Arm support (e.g. ergorest)
• Track ball, track pad, joystick with onscreen Reading
keyboard • Changes in text size, spacing, color, or
background color
• Alternate keyboard (e.g. Intellikeys, Discover
Board, TASH) • Use of pictures with text (e.g. Picture It,
Writing with Symbols)
• Mouth stick or head pointer with standard or
alternate keyboard • Book adapted for page turning (e.g. page
fluffers, 3-ring binder, cardboard in page
• Head mouse or head master/tracer with
protector)
onscreen keyboard
• Talking electronic device to pronounce
• Switch with scanning challenging words (e.g. Franklin Bookman)
• Voice recognition software • Scanner with talking word processor
• Word prediction (e.g. Co:Writer) to reduce • Electronic books
keystrokes
Learning and Studying
Composing Written Material • Print or picture schedule
• Word cards, word book, or word wall
• Low tech aids to find materials (i.e., index tabs,
• Pocket dictionary or thesaurus color coded folders)
166 Neuro-Rehabilitation : A multi disciplinary approach

• Highlight text (e.g. markers, highlight tape, ENVIRONMENTAL CONTROL


ruler)
• Light switch extension
• Software for manipulation of objects or
concept development (e.g. Blocks in Motion, • Use of Powerlink and switch to turn on
Toy Store). Consider alternate input device electrical appliances (e.g. radio, fan, blender,
(e.g. switch or touch window) and so on)

• Software for organization of ideas and • Radio or ultrasound remote controlled


studying (e.g. Inspiration, Claris Works appliances
Outline, PowerPoint)
VISION
• Recorded material (books on tape, taped
lectures with number coded index) • Eye glasses

• Key guard to go over keyboard to help select • Magnifier


the right keys and forearm supports to help • Large print books
stabilize upper extremity.
• Screen magnifier (mounted over screen)
Math • Screen color contrast (e.g. CloseView)
• Abacus or math line • Screen magnification software (e.g. Closeview,
• Calculator, with or without print out Zoom Text)
• Talking calculator • Screen reader (e.g. OutSpoken, Jaws)
• Calculator with large keys or large LCD print • Braille Keyboard and Note taker (e.g. Braille
out N Speak)
• On screen calculator • Braille Translation Software
• Software with templates for math computation
(consider adapted input methods)
HEARING
• Tactile or voice output measuring devices (e.g. • Hearing aid
clock, ruler) • Classroom amplification
• Captioning
RECREATION AND LEISURE
• Signaling device (e.g. vibrating pager)
• Adapted toys and games (e.g. toy with
adaptive handle) • Screen flash for alert signals on computer

• Use of battery interrupter and switch to 10. Environment modifications


operate a toy
To ensure safe mobility and increase independence
• Adaptive sporting equipment (e.g. lighted or
of the CP child, the physical environment in which
bell ball, Velcro mitt)
he functions may need modifications in the form
• Universal cuff to hold crayons, markers, or of:
paint brush
Accessible Entrance/Exit - includes adding ramps,
• Modified utensils (e.g. rollers, stampers, widening doorways, making entrance locks and
scissors) door handles accessible and providing an
• Ergo Rest to support arm for drawing or emergency exit, considering lightweight doors or
painting automatic door openers

• Drawing or graphic program on computer Accessible Interior - includes widening hallways or


(e.g. Kid Pix, Blocks in Motion) interior doors, moving electrical switches and
outlets related to door widening, reinforced wall
• Playing games on the computer for grab bars, railings
• Music software on computer Accessible Bedroom - including widening
doorways, making the closet accessible (lowered
Cerebral Palsy 167

shelves and hanging rods), relocating electrical wearing schedule without the splints
switches and outlets
OT intervention in school for CP children
Accessible Bathroom - includes modifying design
of commode, sink and cabinets, tub or shower, OT in school focuses on child's ability to participate
widening entrance, moving switches and outlets, in functional school activities. A problem solving
faucet hardware approach is used in identify the difficulties the child
faces and to identify intervention strategies. Mental
Other modifications- includes reinforced ceiling if retardation and learning disabilities are some of the
need a lift, roll under sink in kitchen and bath, problems which the CP child may have to face.
assigning workspace in close proximity to school
supplies and equipment, modifying workspace or Parents may require counseling regarding the type
desk design and height of school which best suits the child. The
Occupational therapist is one of the members of a
Splinting child's IEP (Individualized Education Program)
team.
The Occupational Therapist evaluates and
Strategies used in the school:-
recommends use of Upper extremity splinting to
improve, maintain and prevent contractures and • Reframe teacher's perspective. e.g. by
deformities or to improve functional movements. explaining the issues and the underlying
Hand splints to improve thumb abduction, wrist deficit the child seems to be facing
extension and functional positioning of digits are
• Improve child's skills. e.g. use of practice
generally prescribed. E.g. resting pan splint is one
worksheets
which keeps the wrist in 20° to 30° extension, the
metacarpophalangeal joints in 60° flexion and the • Adapt the task e.g. use of keyboard to take
interphalangeal joints in extension. This type of down notes
splint is used at night and during periods of • Adapt the environment. e.g. keeping visual
inactivity with the hope of preventing deformity. distractions to a minimum
An example of a functional splint is an opponens • Adapt the routine. e.g. extra time to complete
splint to bring the thumb out of the palm of the worksheets.
hand, allowing for better grasp. This type of splint
is used in everyday activities. However, it is still Adaptive aids
unknown whether a thumb abduction orthosis
Adaptive chair (CP chair)
improves use and manual function of the affected
hand in children with hemiplegia Adaptive writing devices, aids and paper
Railings along staircase, classrooms and toilets
Precautions during splint use
The caregivers should be educated about the Adaptive commodes
wearing of the splints which includes donning and Schedule boards, checklists timers, calendars
doffing of the splint, wearing schedule and care of
Vocational and prevocational Rehabilitation:
skin and splint.
Vocational and prevocational activities are ones that
Non verbal children having poor sensations may
help to prepare students for a future job they may
not be able to report sensory problems occurring
pursue as they get older.
during wearing of the splint, so a thorough skin
inspection should be taught to the caregivers Pre-Vocational skills training: Activities are
developed that will prepare an individual for
Starting with wearing of the splint for few minutes
employment. It includes support and training in
the wearing time should be gradually be increased
behaviors related to following directions, attending
to about 8 hours a day for static splints. Use of
to task, task completion, problem solving, and
dynamic splints can be increased for additional
safety and assisting the person to adjust to the
more hours according to the child's tolerance.
productive and social relationship demands of a
Hand orthoses may inhibit the active use of the work place.
extremity. Hence it is important for the child to
In the classroom it is important to give children a
spend a certain amount of time in between the
168 Neuro-Rehabilitation : A multi disciplinary approach

chance to experience what sorts of tasks they may • After the seizure has run its course, let the child
encounter in the workplace. Some examples of these rest and be supportive.
tasks are: sorting, putting objects together,
• Although, medications are very effective in
alphabetizing, filing, data entry, and packaging
preventing or reducing seizures when given
items.
regularly, they may also produce a variety of
side effects. For example, they may cause
Vocational skills training
hyperactivity behavior, irritability, sleep
Prior to vocational skills training a Transition problems, lethargy, depression, or sedation
Programme can be conducted, in which the skills which may affect therapy.
learnt already in the pre vocational training are
transferred to vocational training. Occupational Sexuality issues
therapists specialized in Vocational rehabilitation,
Adolescents with cerebral palsy have delayed and
conduct workshops to give training in a variety of
prolonged puberty. They may develop precocious
occupations like tailoring, greeting card making
puberty as well. The therapist can guide the parents
and pot painting exercise, soap and phenyl, pickle,
in preparing the child about puberty and the bodily
card making, craft works etc.
and behavioral changes he may experience through
books or pictures. Try and recognize the timing of
Management of associated problems in sexual maturation and educating parents to provide
CP age-appropriate sexual education. Pose questions
about sexuality privately, using normalizing
Seizures Management statements and open-ended questions helps the
Almost half of children with cerebral palsy adolescent open up to discussions.
experience seizures.
Practical Things to remember in event of any child
Conclusion:
having a seizure are: Although there is no evidence that any specific
• Do not attempt to hold the child still or to approach in occupational therapy treats CP therapy
prevent physical movement; instead, make the to enhance functional skills is important. Increased
environment safe so that she cannot be Performance in all ADLs is very important which
physically hurt. is specifically addressed by OT. Also, parental
education and counseling along with home
• If the child is in a sitting position, or standing programs given by therapists help to address the
or walking when you observe the seizure, help needs of the family as a whole.
her to lie down, so that she will not fall and
get hurt. If at all possible, place the child on a Psychological Intervention:
blanket or protected surface. Position the child
on her side, supporting the head. Move all Introduction:
sharp objects out of the way, as well as any
Cerebral palsy causes a lack of muscle control and
furniture. Pad any sharp objects that cannot
motor coordination. Children with cerebral palsy
be moved, to prevent the child from getting
are at an increased risk of developing emotional
hurt. Loosen clothing, especially in the chest
and behaviour problems. They may develop a
and abdominal area.
feeling of learned helplessness and may feel socially
• Do not put anything in the child's mouth. Do isolated. Preschoolers with cerebral palsy are
not interfere with the seizure or try to stop it. unable to explore the world around them and spend
more time passively unengaged as compared to
• Allow the seizure to continue without
other preschoolers. By school age, social contact is
interruption. Check the child for breathing. If
reduced with the majority of free-playing time to
the child has stopped breathing, clear the
non-play or other activities. As these children move
airway and perform mouth-to-mouth
on to adolescence, they emphasise on activities
breathing.
planned by adults than on spontaneous activities
• Turn the child on her side so the saliva can with peers. As, adults involvement is strictly
flow out of her mouth. limited. A lack of participation can lead to the
development of physical, medical, cognitive,
Cerebral Palsy 169

emotional, or psychosocial secondary conditions in fine motor and gross motor coordination and
with adverse outcomes in health, wellness, and communication.
quality of life.
Mental Retardation: It has been estimated that
Emotional Problems in Cerebral Palsy: About two around 65 percent of the individuals living with
thirds people with cerebral palsy suffer from severe cerebral palsy also have some form of mental
emotional stress. retardation. About 50% are full mentally retarded
i.e. an IQ below 70. Because cerebral palsy and
Depression: It is often seen that adults suffering
mental retardation can be co-morbid, they can
from cerebral palsy have a sense of lack of
contribute to emotional stresses as well. Learning
emotional support, lack of coping skills and a very
disabilities may be present, depending on the area
negative view of the future. Patients may suffer
of the brain that was damaged. About a third of
from pain which may lead to depression. However,
individuals with cerebral palsy have mild
it is seen that it may not be so much due to the
intellectual impairments, a third have moderate-to-
severity of the disability but it may depend but
severe intellectual impairments, and another third
would depend on how well they cope with the
have normal intellectual functioning.
disability. Children and adults suffering from
cerebral palsy may develop depression as being
unable to control their body, embarrassment about
Behavioural Problems in Cerebral
their body in social situations and lack of Palsy:
information about their situation. Behavioural problems and cerebral palsy usually
Anxiety: As adults with cerebral palsy may age they correlate, depending on the degree of mental
may develop age related issues like arthritis, bone retardation. The child may have behavioural
fractures, chronic pain and fatigue. Due to these problems or emotional issues that in turn, may
additional problems they may develop anxiety affect psychological development and their ability
about their worsening condition and how their to have social interaction.
condition can limit their functioning. Patients may 1. Frustration: Patients suffering from cerebral
also develop sleep problems which in turn can palsy may face difficulty in completing a task,
contribute to anxiety and other emotional problems. which may lead to getting angry and
Low Self- esteem: Due to perceived physical discouraged about their condition. This
limitations, loss of body control and medical problem can be overcome by helping them
condition, patients with cerebral palsy may suffer with the task and finishing it which would
from low self-esteem. As, parents are over foster a sense of achievement.
protective about their children, this in turn may lead 2. Communication difficulties: Lack of ability to
to dependence and low self -worth in patients. communicate efficiently can cause disturbance
Parents who engage their child in conversation associated with behavioural problems. During
about other topics, such as the child's likes, dislikes, such situations the children call for a lot of
achievements and ambitions, are likely to overcome physical and mental stress to the parents.
this feeling of a lack of individuality. Excessive attention should be discouraged
whereas the child should be kept involved by
Cognitive Deficits in Cerebral Palsy: talking or just maintain eye contact.
Learning Difficulties: Children with cerebral palsy 3. Attention Deficit Disorder: Many of those who
may experience specific learning difficulties which are immobile let their attention wander. In
may include short attention span, motor planning such cases, there should be minimal distraction
difficulties, perceptual difficulties and language while teaching them such as teaching them in
difficulties. It is also seen that children suffering the corner of a room. The television sets and
from cerebral palsy who display disruptive or other modes of distraction should be kept
avoidance behaviours and low self concept may away, in order to increase their attention span.
have underlying learning issues. Students suffering
from cerebral palsy may get tired quiet easily as Employment Issues: Several studies suggest that
they need to put more effort into concentrating on about 30% to 50% of adults suffering from cerebral
their movements and sequence of actions than palsy were competitively employed. Findings of a
others. Learning may also be affected by problems study suggest that speech deficits can lead to verbal
170 Neuro-Rehabilitation : A multi disciplinary approach

difficulties and could lead to decrease in from the emotional and social needs of childhood
competitive employment. It is often seen that and adolescence. Disabled youngsters need the
individuals with hemiplegic cerebral palsy had same variety of life experiences as all other children
regular jobs as compared to those with other types to develop emotional resilience, personal
of cerebral palsy. The severity of cognitive and determination, and social skills. As the child with
motor impairment, seizure disorder and types of cerebral palsy grows older, the need for and types
cerebral palsy were predictive factors in of therapy and other support services will continue
competitive employment. Cognitive ability is a very to change.
important factor in employment.
Neuro-cognitive therapy: A new approach to
Psychosocial Factors: The development of both treating cerebral palsy from Snowdrop. It is based
intelligence and personality relies heavily on upon two proven principles. (1) Neural Plasticity.
developmental experiences and the opportunity for The brain is capable of altering its own structure
self-expression. The child may find it easier to and functioning to meet the demands of any
withdraw towards social isolation. They should be particular environment. Consequently if the child
encouraged to take independent decisions and is provided with an appropriate neurological
physical tasks. Early choices can be made by the environment, he will have the best chance of
child regarding the clothes to wear or which task making progress. (2) Learning can lead to
to do first. development. Lev Vygotsky proposed that
children's learning is a social activity, which is
Parents need to resolve their own way the
achieved by interaction with more skilled members
emotional impact of the child's disability. Most
of society.
parents feel inadequate, ignorant and relatively
helpless at being unable to remedy the situation for Counselling and behaviour therapy, for emotional
the child. They need help in feeling good about and psychological challenges may be needed at any
themselves before they can effectively guide the age, but is often most crucial during adolescence.
child towards self -acceptance as an adequate Behaviour therapy is often used to enhance child's
human being. Parents need guidance to provide ability and discourage destructive behaviours.
themselves with opportunities to rest and renew Behaviour therapy might include planning
their energies. activities that are rewarding which could provide
a sense of accomplishment; use of reinforcements
Psychological Testing: can encourage a behaviour change, enhance
learning and solidify gains. For example behaviour
Children with cerebral palsy might find it difficult
therapy might include hiding a toy inside a box to
to respond to the tests that are timed or that require
reward a child for learning to reach into the box
manipulation of objects, such as some of the
with his weaker hand. In other cases the therapist
subtests of Wechsler. Alternative tests such as
may deal with unhelpful or destructive behaviours
Pictorial Teat of Intelligence, the Columbia Mental
like biting, or hair pulling by selectively presenting
Maturity Scale or the Peabody Picture Vocabulary
a child with rewards such as praises or rewarding
Test are recommended. There are various measures
with extra play time.
of gross motor functioning amongst these are
Bruininks - Oseretsky Test of Motor Proficiency, Teaching relaxation strategies such as systematic
the McClenaghan and Gallahue Checklist and the desensitization, abdominal breathing to the client
Vulpe Assessment Battery. Raven's Coloured or the caregiver can help reduce stress and anxiety
Progressive Matrices is a fast, easy-to-administer and effect behavioural change. Token economy is
test able to obtain a measure related with linguistic, an effective way to reinforce a positive change.
visuo-perceptual, and memory cognitive Aversion therapy i.e. to reward rather than punish
functioning in persons with CP despite their motor on negative consequences can help enhance self -
and speech disorders. esteem. Expressive therapies are usually used with
people who have difficulty verbalizing their
Psychological Treatment: feelings such as art, music, poetry, etc which could
help freeing and empowering oneself. Sometimes
Education and vocational preparation come into children with cerebral palsy can become violent or
the foreground by school age. Concern with the aggressive, resorting to things such as biting or hair-
physical disability should not distract attention pulling to they can be helped to release their
Cerebral Palsy 171

aggression and frustrations, by either being vocal Websites:


or, if the child is able to control his or her hand Indian Institute of Cerebral Palsy,
enough, perhaps drawing or writing. [Link]

References: Indian Family of Cerebral Palsy,


[Link]
Physiotimes, Volume 1, Issue 5, Reg. [Link]/
A Guide to Cerebral Palsy in India,
2009/30341, March 2010."Current Concepts in the
[Link]
therapeutic management of children with Cerebral
Palsy. " Office of the Chief Commissioner for Persons
with Disabilities, [Link]
Phsiotherapy in Neuro Conditions by Glady
Samuel Raj, First Edition, 2006,Chapter 12, Cerebral Rehabilitation Council Of India,
Palsy. [Link]
Cerebral Palsy, A Complete Guide for Caregiving, Cerebral Palsy Magazine,
Second Edition, By, Freeman Miller, and Steven [Link]
[Link].
Web Centre for Social Research Methods,
Early Diagnosis & Interventional Therapy in [Link]
Cerebral Palsy, An Interdisciplinary Age - Focused
Physical Therapy Web space, [Link]
Approach, a Primer on Infant Developmental
Problems,Third Edition, Volume 11,Revised and Therapy Times- A Multi-disciplinary Online
Expanded by Alfred [Link] and Ingrid Resource, [Link]/PT
Tscharnuter. [Link]
Jane Case-Smith Occupational therapy for children, [Link]
fifth edition [Link]
Kathlyn [Link] Quick Reference to Occupational [Link]
Therapy, 2nd edition [Link]
Robert J. Palisano, Laurie M. Snider, and Margo N. [Link]
Orlin, Recent Advances in Physical and [Link]
Occupational Therapy for Children With Cerebral
[Link]
Palsy
[Link]
My Child Without Limits Advisory Committee,
[Link]
Introduction to Cerebral Palsy, My Child Without
[Link] [Link]
172 Neuro-Rehabilitation : A multi disciplinary approach

Ch.4 Head Injury


Dr. Ashok Patil, MPT(Neuro), Dr. Alok Sharma, M.S, MCh, [Link] Kukreja, M.B.B.S,
Dr. Naren Naik, M.S,MCh., Dr. Hema Biju, MOTH (Neuro),
Ms. Akshata Shetty, M.A.(Clinical Psychologist), Dr. Manasi Jani (Speech Therapist)

Head injury includes injuries of the scalp, skull, or Patients with head injury may be aymptomatic at
brain. The most common cause of head injury is the time of injury and may develop symptoms over
trauma which may be road traffic accident, fall or a few days. Symoptomatic patients may present
physical assault. with nausea, vomiting, headache, confusion,
drowsiness or seizures. There may a lucid interval
Head injuries can be classified in to closed or open
during which the patients remains conscious after
depending on whether the duramater is intact or
head injury and deteriorates later. Unconscious
breached. A closed head injury is one in which
after a head injury even for a short period is not
duramater remains intact. There may be injury to
normal. The patient may have other symptoms like
the scalp or cranial bones. An open head injury
difficulty concentrating, increased mood swings,
occurs when a penetrating object breaches the
lethargy or aggression, and altered sleep habits.
duramater.
These symptoms can occur in concussion type of
head inury and can easily be missed.
The physical examination and the history of the
exact details of the injury are the first steps in caring
for a patient with head injury. Patient should be
assessed for symptoms suggestive of severe head
injury- severe headaches, fluid draining from nose/
mouth/ears, loss/alteration of consciousness,
confusion, drowsiness, slurred speech, blurred
vision, stiff neck or vomiting, paralysis.
The Glasgow Coma Scale (GCS) should be used to
assess the level of consciousness, severity of head
injury and to determine the prognosis.
• Minor head injury: GCS 13-15; mortality
0.1%
• Moderate head injury: GCS 9-12;
mortality 10%
• Severe head injury: GCS <9; mortality
40%.
The GCS should be assessed periodically to
determine the neurological status of the patient.
Examination of pupil forms a vital part of
neurological assessment. The response of pupils to
light and their size should be examined. Anisocoria,
unequal pupil size, is a sign of serious head injury.
Pupil not reacting or reacting sluggishly to light is
suggestive of raised intracranial pressure.
Neuroimaging helps in determining the diagnosis,
A head injury may result in only scalp wounds. In
prognosis and in deciding the treatment. Non
more severe cases it may result in skull fracture and
contrast Computerized tomography (CT) scan of
direct or indirect injury to the brain. Injury to the
the head can show bleeding and swelling in the
brain may be in the form of concussion, contusion,
brain. It can also evaluate bony injuries to the skull
haemorrhage or haematoma.
Head Injury 173

and bleeding in the sinuses of the face associated poor outcome. The following ICP reduction
with basilar skull fractures. CT does not assess brain strategies should be used if the ICP is above 20-25
function, and patients suffering axonal shear injury mm Hg- hyperventilation, intravenous Mannitol,
may be comatose with a normal CT scan of the head. CSF drainage, hypertonic saline, high-dose
barbiturate therapy can be used if the intracranial
Management of head injury pressure does not respond to the above
conventional treatments. Caution is required as
Medical Management:
barbiturate may reduce blood pressure.
Acute management: In addition to reducing ICP, maintaining cerebral
Immediate resuscitation including assessment and perfusion pressure is critical in the management of
stabilization of the airway, breathing and head injury. For adequate perfusion, cerebral
circulation is a priority in the management of acute perfusion pressure should be maintained at 50-70
head injury. Cervical spine should be stabilized by mm Hg. [1] With ICP reduction strategies, the
head and neck immobilization. After resuscitation cerebral perfusion pressure may fall and should be
and stabilization, secondary brain injury can be maintained with- volume expansion and
prevented by keeping- mean arterial pressures vasopressors. Normal saline is preferred over
above 90 mm Hg and arterial oxygen saturation albumin. [2]
greater than 90%. Urgent non contrast CT scan is A systematic review has concluded that
done to assess the brain damage and hemorrhage hypothermic therapy is of no benefit in patients
(hematoma). with head injury. [3] A recent study has shown
Intracranial pressure reduction and monitoring is that very early hypothermia induction may
critical in the management of head injury. Increase improve outcomes in patients with surgically-
in ICP reduces cerebral blood flow, causes cerebral evacuated hematomas and worsen outcomes in
compression and is an independent predictor of patients with diffuse head injuries. [4]
174 Neuro-Rehabilitation : A multi disciplinary approach

Neuroprotection is the administration of some of head injury includes management of spasticity,


agent, which should reverse some of the damage cognition enhancing medications and management
or prevent further damage. [5] The majority of of psychiatric complications.
neuroprotective agents are antioxidants. Role of
Dantrolene, baclofen, diazepam, and tizanidine can
neuroprotective agents in improving the outcomes
be used orally for hypertonicity. Baclofen can also
of patients with brain injury is not well established.
be used intrathecally. Botulinum toxin has been
Agents with neuroprotective activity in brain
shown to decrease hypertonia in patients with head
innjuries include-
injuries. [19]
Nimodipine has been shown to reduce mortality
The cognition enhancing medications include
and severe disability when given acutely in patients
methylphenidate, donepezil, levodopa.
with head injuries and traumatic subarachnoid
Methylphenidate has shown promise by improving
hemorrhages. [6] Magnesium chloride did not show
motor outcomes and attention in patients with head
benefit in clinical studies with moderate and severe
injuries. [20] Donepezil has shown improved visual
head injury. [7] Intravenous progesterone has been
and verbal memory as well as attention in patients
shown to reduce 30-day mortality in patients with
with head injury. [21, 22] Levodopa has been shown
moderate and severe head injury. [8] Cyclosporin
to produce cognitive improvements in patients with
is an inhibitor of mitochondrial membrane
head injury. [23]
permeability. Dexanabinol, a weak N -methyl-D-
aspartic acid (NMDA) antagonist, showed no The management of psychiatric complications like
efficacy in outcome improvement in a recent trial, emotional lability and depression are an integral
when given within 6 hours to patients with severe part of long term management of head injury.
closed head injuries. [9] Rosuvastatin given in the Selective serotonin reuptake inhibitors have shown
acute phase of moderate head injury has been to be effective in pathologic laughing and crying.
shown to reduce amnesia. [10] Creatine has shown [24] Sertraline has shown efficacy in depression in
to reduce cortical damage primarily through mild head injury. [25]
stabilizing mitochondrial functioning in pre-clinical
studies. [11] Melatonin is a free radical scavenger, Surgical Management:
and has shown neuroprotective effect by The factors that help in decision making regarding
significantly reduced levels of lipid breakdown the need for surgery include age, neurological
products in pre-clinical studies. [12] status, mechanism of injury and pupillary reactions.
The major questions addressed by the surgeon are:
Phenytoin is efficacious in controlling early
posttraumatic seizures. Some patients may develop 1) Is the conscious level depressed?
an allergic reaction with this drug. [13]
2) Are there other signs of raised ICP
Posttraumatic seizure prophylaxis should be
(eg oculomotor palsy)?
discontinued after 1-2 weeks unless further seizures
supervene. [14] 3) Does the scan show a haematoma or contusion
with mass effect (midline shift/ ventricle
Patients with head injury are in hypermetabolic
effacement/ dilatation of contralateral
state and therefore in need of adequate nutrition
ventricle/ loss of basal cisterns/ sulcal
early. Early nutritional therapy has shown to reduce
effacement)?
mortality in severe head injury. [15]
4) Is expansion of the mass anticipated (eg
The risk of deep venous thrombosis is increased in
contusions)?
head injury as coagulation parameters are altered.
[16] Early DVT prophylaxis (with enoxaprin/ 5) Where is the mass (temporal lobe lesions are
heparin) is found to be safe in patients with head considered dangerous due to the association
injury. [17] The risk of IC Bleed extension should with uncal herniation compressing the
be balanced with the benefits of thromboembolic midbrain)?
prevention. These questions must be appropriately addressed
Steroids have found to be of no benefit in the rapidly and when required the surgery should be
treatment of acute head injury and may be performed with an appropriate sense of urgency.
associated with higher mortality. [18] 1. Extradural Haematoma: The indication of
Long-term management: Long-term management surgery for EDH include-
Head Injury 175

a. EDH greater than 30cm3 should be a. Patients with parenchymal mass lesions
surgically evacuated regardless of and signs of progressive neurological
patient's GCS. deterioration referable to the lesion,
medically refractory intracranial
b. An EDH less than 30cm3 and with less a
hypertension, or signs of mass effect on
15-mm thickness and with less than a 5-
CT scan should be treated operatively.
mm midline shift in patients with a GCS
score greater than 8 without focal deficit b. Patients with GCS scores of 6 to 8 with
can be managed nonoperatively with frontal or temporal contusions > 20 cm3
serial CT scanning and close neurological in volume with MLS of at least 5 mm
observation in a neurosurgical centre. and/or cisternal compression on CT scan,
and patients with any lesion greater than
It is strongly recommended that patients with
50 cm3 in volume should be treated
an acute EDH in coma (GCS score < 9) with
operatively.
anisocoria undergo surgical evacuation as
soon as possible. There are insufficient data to Craniotomy with evacuation of mass lesion is
support one surgical treatment method. recommended for those patients with focal
However, craniotomy provides a more lesions and the surgical indications listed
complete evacuation of haematoma. above. Bifrontal decompressive craniectomy
within 48 hours of injury is a treatment option
2. Subdural Haematoma: The indication of
for patients with diffuse, medically refractory
surgery for SDH include-
post traumatic cerebral edema and resultant
a. An acute SDH with a thickness > 10 mm intracranial hypertension. Decompressive
or a midline shift > than 5 mm on CT scan procedures, including subtemporal
should be surgically evacuated, decompression, temporal lobectomy, and
regardless of GCS score. hemispheric decompressive craniectomy, are
b. All patients with acute SDH in coma treatment options for patients with refractory
(GCS score < 9) should undergo ICP intracranial hypertension and diffuse
monitoring. parenchymal injury with clinical and
c. A comatose pt with SDH <10-mm thick radiographic evidence for impending
and a midline shift < than 5 mm should transtentorial herniation.
undergo surgical evacuation of the lesion 4. Posterior Fossa Mass Lesions: The indication
if the GCS score decreased between the of surgery for traumatic parenchymal lesions
time of injury and hospital admission by include-
2 or more points on the GCS and/or the
patient presents with asymmetric or fixed a. Patients with mass effect on CT scan or
and dilated pupils and/or the ICP with neurological dysfunction or
exceeds 20 mm Hg. deterioration referable to the lesion
should undergo operative inter- vention.
In patients with acute SDH and indication for
Mass effect on CT scan is defined as
surgery, surgical evacuation should be
distortion, dislocation, or obliteration of
performed as soon as possible. If surgical
the fourth ventricle; compression or loss
evacuation of an acute SDH in a comatose pt
of visualization of the basal cisterns, or
(GCS < 9) is indicated, it should be performed
the presence of obstructive
using a craniotomy with or without bone flap
hydrocephalus.
removal and duroplasty. In extreme cases a
"burst" lobe may be evident. Such severe b. Patients with lesions and no significant
haemorrhagic contusions may necessitate mass effect on CT scan and without signs
resection of brain in the form of a lobectomy. of neurological dysfunction may be
Brain resection requires rapid but careful managed by close observation and serial
surgery preserving the middle and anterior imaging.
cerebral arteries.
In patients with indications for surgical
3. Traumatic Parenchymal Lesions: The intervention, evacuation should be performed
indication of surgery for traumatic as soon as possible because these patients can
parenchymal lesions include- deteriorate rapidly, thus, worsening their
176 Neuro-Rehabilitation : A multi disciplinary approach

Compted Tomography brain (axial view) showing a Intraoperative picture showing large extradural
large biconvex hyperdense lesion in the right frontal haematoma below the craniotomy flap.
region suggestive of extradural haematoma.

Compted Tomography brain (axial view) showing a Intraoperative picture showing large subdural
large concavo-convex hyperdense lesion over the haematoma on opeing the dura.
right frontoparietal region with mass effect
suggestive of subdural haematoma.

Compted Tomography brain (axial view) showing a Compted Tomography brain (axial view) showing a
large hyperdense lesion in the left frontal region right frontal depressed fracture with underlying
suggestive of haemorrhagic contusion. contusion.
Head Injury 177

prognosis. Suboccipital craniectomy is the Management explained in Speech Rehabilitation


predominant method reported for evacuation Chapter.
of posterior fossa mass lesions, and is therefore
recommended. Physiotherapy In Head Injuries :
5. Cranial Fractures: The indication of surgery A Traumatic Brain Injury (TBI) is a devastating and
for cranial fractures lesions include- life changing event for the individual and his or
her family. A TBI leads to a much more wider range
a. Patients with open (compound) cranial
of conditions than from sustaining any disruption
fractures depressed > the thickness of the
to the vascular supply of the brain making it one of
cranium should undergo operative
the most challenging conditions for
intervention to prevent infection.
physiotherapists. There is a multitude of issues with
b. Patients with open (compound) these patients as affection for the different parts of
depressed cranial fractures may be brain will be responsible for their own aspects of
treated nonoperatively if there is no impairments and secondary impairments. Patients
clinical or radiographic evidence of dural with a moderate to severe TBI require extensive
penetration, significant intracranial rehabilitation during the different stages of the
hematoma, depression greater than 1 cm, recovery & physiotherapist will be involved in the
frontal sinus involvement, gross cosmetic most of them depending upon the sensory motor
deformity, wound infection, deficit & their recovery. Physical recovery does not
pneumocephalus, or gross wound always go hand in hand with the cognitive &
contamination. behavioral recovery. One of the deficits may show
c. Nonoperative management of closed very good recovery without any in other during
(simple) depressed cranial fractures is a different stages and the durations.
treatment option.
Many traumatic brain injury (TBI) survivors are left
Early operation is recommended to reduce the with significant disability. The brain is, however,
incidence of infection. Elevation and very adaptable and, with the correct physiotherapy
debridement is recommended as the surgical input, recovery can take place over a period of
method of choice. Primary bone fragment years. People often witness a rapid recovery in the
replacement is a surgical option in the absence first few weeks following a TBI, followed by a
of wound infection at the time of surgery. All slower recovery over the following years.
management strategies for open (compound)
To gain the maximum recovery, physiotherapy
depressed fractures should include antibiotic.
should be continued until patient's full potential has
been reached. With the correct physiotherapy input
Speech Affection in Head injury:
and advice patient has lot of potential to long term
Patients with head injury present with a myriad of improvements regaining as much movement and
problem which include language problems, speech function over the years.
difficulty, cognitive issues and personality disorder
depending upon the area of the brain affected. The Physiotherapy in TBI can help to:
left hemisphere damage affects language to a • prevent or improve the respiratory distress to
greater extent as compared to right hemisphere minimise/ prevent secondary brain damage
damage which affects the pragmatics of language.
• prevent the skin, soft tissues, joints dysfunction
Individuals with left sided brain damage present
while unconscious or bed ridden
with problems of understanding spoken or written
language, difficulty in expressive language, alexia, • reduce muscle spasms, pain and stiffness
agraphia and naming problems. • increase strength
An individual with a mild traumatic brain injury • retrain normal patterns of movement
may present with poor speech clarity (dysarthria) • increase affected arm and leg function
or even milder being the cognitive issues like • initiate the ability & do independent ADLs
affected attention and concentration, difficulty in from roll / move in bed / sit to stand and walk
problem solving issues, and changes in personality • improve balance and walking
which is just a tip of the ice berg.
• increase energy levels
178 Neuro-Rehabilitation : A multi disciplinary approach

• increase independence and quality of life Post traumatic amnesia (PTA)


• decrease risk of falls Declarative memory / &
Procedural memory
The physiotherapist must follow the physiotherapy
examination before the interventional planning. Behavioral : Sexual disinhibition
Interventions required will be as per the motor Emotional disinhibition
control, cognitive, behavioral or any other deficits Apathy
presented in a particular patient. The Aggressive disinhibition
interdisciplinary rehabilitation team offers a unique Low frustration level tolerance
opportunity for physiotherapist to understand the Depression
patient better and so to do the best to reach the Communication: Expressive or Receptive aphasia
maximum functional recovery. Individual tem Deficits in reading compre-
member contribute their expertise in their hension, written comprehen-
specialized area results in the enhancement of the sion, language skill
team's overall effectiveness. Communication & Dysarthria
open mindedness are the key to any team. All the
members must share their skills & findings with Visual-Perceptual: Visual impairments-
the whole team & be willing to learn from each other hemianopia, very rarely cortical
to promote optimal return of function. The blindness.
Physiotherapist must be willing to share a unique Perceptual deficits - spatial
knowledge of motor control, but be open to learn neglect, apraxia, spatial relations
from other team members which will lead to a syndrome, somatagnosia and
consistent and complete approach to care. right-left indiscrimination
Any one or some of the rehabilitation team Swallowing : Dysphagia caused by damage to
members may play more prominent role in a patient cranial nerves
depending upon the area of deficit he or she may Motor control impairments,
be having due to TBI and stages of recovery also apraxia, and poor postural
decide the dominance of roles. control.

SEQUELAE OF TBI Indirect Skin breakdown or bedsores


impairments : Soft tissue contractures
Though physiotherapists are primarily concerned Deep Vein thrombosis
with the treatment of neuromuscular impairments, Heterotopic ossification
the associated cognitive and behavioral changes Decreased bone density
become the hindrance in getting back the functions Muscle atrophy
and abilities. Decreased cardio pulmonary
endurance
Various aspects of impairments in TBI seen are
Infections
as follows:
Neuromuscular : Abnormal tone of spasticity ASSESSMENT
Primitive postures - Decorticate The first step in beginning an examination is to
Decerebrate rigidity conduct a complete review of the case paper of the
patient before actually seeing the patient as he/she
Alterations in sensation - light
may not be medically stable & to know about the
touch, pain, deep pressure and
complications as well as the precautions to be taken
temperature.
during the examination & subsequent treatment.
Impairments in proprioception Patient's medical status may be dynamic at these
and kinesthesia stages, it is important to check with the intensivist/
Cognitive : Coma / Vegetative state / anesthetist/ house surgeon / nurse before
Minimally Conscious State beginning any treatment session. Owing to the
(MCS)/Stupor / Obtunded possibility of various infections, physiotherapist
Disorientation should always observe precautions & will require
Memory deficits wearing gowns, gloves, and masking when treating
the patient.
Head Injury 179

Initially, should focus on observing the patient & Associate injuries


response to stimuli to know: Many patients with a HI have additional injuries.
• What posture is the patient in? Is there These injuries may be relatively minor & can wait
evidence of primary postures or reflexes? attention, or they may demand urgent attention, i.e.
• Are the patient's eyes open or closed? chest injuries, abdominal bleeding from damaged
viscera, fracture dislocation of the cervical spine,
• Is the patient able to respond to auditory or fractures of the face, ribs,
visual stimulation?
Often it may require many sessions to complete the
• Is the patient able to vocalize?
entire examination. Signs of progress or regression
• Does the patient exhibit any active movement? should be carefully monitored.
Is the movement purposeful or
From the examination, an assessment of severity
nonpurposeful?
of the injury can be made.
• Does the patient react to tactile/ painful
stimulation? PROGNOSIS & GOAL SETTING
• Do the patient's vital signs change when
The data gathered during the initial examination is
external stimulation is presented?
used to evaluate the patient's status & develop a
Respiratory State prognosis & plan of care. Based on the findings from
the initial examination, impairments (primary &
Respiratory distress and, therefore, inadequate secondary) contribute to the patient's functional
ventilation may be caused by: limitations.
1. Obstruction of the airway due to inhalation
Brain injury leads to a wide range of cognitive,
of blood, vomitus, false teeth, foreign body;
motor & neurobehavioral impairments, so, it is
the tongue flopping, jaw fractures
difficult to establish & predict long term outcomes
2. Depression of the respiratory centre due to & set goals for these patients. When setting goals,
damage to the respiratory centre in the the patient, family, caregivers, & entire
brainstem interdisciplinary team should be consulted. The
3. Associated injuries like primary damage to patient's potential for cognitive & behavioral
the chest wall & lung tissue improvement will greatly affect future motor ability
& social participation.
Neurological examination with Consciousness
level Though it is difficult to use general guidelines to
Consciousness assessment is very important in TBI. predict with precision for any individual patient
due to widespread neurological damage & range
Glasgow Coma Scale(GCS) is the most of resulting impairments, initial severity of injury
accepted objective examination. Any alteration as measured on GCS, duration of coma and length
in response can be seen easily if the of Post Traumatic Amnesia (PTA) may be used as
observations are plotted on a graph. predictors of outcomes.
-movement of limbs ( lateralizing & focal signs)
The majority of subjects who experience a coma of
-pupillary light reflex less than 1 week duration, get either moderate
Cardiovascular Respiratory State disability or a good recovery, whereas, when coma
is greater than 2 weeks, possibility is for moderate
Pulse & Blood Pressure: Hypotension will lead to
or severe disability.
reduced cerebral perfusion & neurological
deterioration respiratory state. The majority of subjects with PTA lasting 4 weeks
or less have a good recovery or moderate disability.
If it is not medically contraindicated, the
If it lasts more than 12 weeks, then most of the
examination should include an attempt to make the
subjects show moderate to severe disability.
patient sit on the edge of the bed with assistance.
The therapist should monitor the vital signs &
document any changes in vital signs as well as in
muscle tone, head or trunk control.
180 Neuro-Rehabilitation : A multi disciplinary approach

PATIENT CARE : nursed in a position which allows the head to be


PHYSIOTHERAPY MANAGEMENT raised at 30º & in side lying to prevent inhalation
of secretions. Regular turning will prevent
ACUTE STAGE consolidation & collapse of lungs & the pressure
sores. Positioning & turning may be difficult, if
The main priority at this stage is to ensure the there are associated injuries which require splinting,
patient is medically stable. Proactive management such as plaster casts or traction.
at this early stage, of potential secondary
complications, will enhance patient outcomes. If the patient is unable to maintain his own airway,
a cuffed endotracheal (ET) tube is required. This
At this stage, the goals & anticipated outcomes will may be sufficient to obtain sufficient oxygenation
be: & remove any retained secretions to stop any
• To handle carefully & immediately known or further deterioration. Any evidence of respiratory
suspected raised ICP inadequacy, need of artificial ventilation (IPPV) is
• To treat an unconscious patient as a whole required.
person & not a series of joints & muscles It is important to keep the chest clear of secretions
• To facilitate arousal, increase the level of as patchy collapse with sub clinical hypoxia &
alertness & physical function hypercarbia is probably more damaging to the brain
• To reduce the risk of secondary impairments than any other factor. Due to the traumatic brain a
small rise in IC volume may cause a dramatic rise
• To prevent building up respiratory secretions
in ICP. Various physiotherapeutic procedures for
and enhance oxygenation of the brain
the chest care such as postural drainage, shaking,
• To work on the response by stimulating the bag squeezing, prolonged suction & coughing may
sensory system precipitate the raise in ICP. Therefore, the patient
• To work for the normalization of the muscle must be protected during chest physiotherapy;
tone effective treatment can be carried out without
• To improve joint mobility & control provoking large increases in ICP. This can be
• To provide early family education & support- achieved by drug therapy for sedation, analgesia
by means of involvement in aspects of the & muscle paralysis.
patient's care, providing time out to discuss This is best carried out to coincide with turns &
the patient's progress & potential outcomes of before bolus feeds, a continuous feed should be
interventions, advice on support groups and switched off. The chest is vibrated on the expiratory
sources of information. phase of breathing. Tipping the patient with his
The unconscious patient is best nursed in an head down is not advisable due to the risk of
intensive care unit as the high levels of care is elevating ICP. When suction is required to remove
required. The patient may be on a ventilator, secretions, nasal suction is contraindicated if CSF
monitoring of ICP may be ongoing; there may be Rhinorrhoea is present: this indicates a CSF leakage
weight bearing precautions & movt. restrictions due through skull fracture with a risk of ascending
to musculoskeletal injuries, open wounds or infection.
presence of bone fixators. Frequent neurological The frequency of treatment depends on the
observations are required to know any individual patient, but even if there are no obvious
deterioration, if any. lung problems, treatment is given to prevent the
Chest care: Hypoxia causing further brain damage unnecessary complications of retained secretions.
is an important cause of neurological deterioration. Techniques of shaking & vibration are best used
Cerebral hypoxia may be produced by: airway while the patient is being ventilated by bag
obstruction from inhalation of blood or vomit or squeezing, i.e. off the ventilator. Careful suction via
head position; depression of the respiratory drives; the ET tube should clear secretions.
associated chest injury, infection or pulmonary
Patients having controlled IPPV usually need
edema; & epileptic fits.
continuous sedation using anesthetic agents &
Patients who are managing to maintain their own analgesics. A bolus dose of sedation is given prior
airways & adequate oxygenation may not require to physiotherapy. Total treatment time is a crucial
intubation & mechanical ventilation. They are factor & should be kept at minimum, shorter
Head Injury 181

sessions, more frequently should be given as per progress but may lead to life threatening
the requirement. Suction, in particular, is a consequences. Good positioning will help in
relatively dangerous procedure because of its effect preventing skin breakdown, contractures, improve
on ICP. pulmonary hygiene & circulation. In bed, head
should be positioned in neutral to prevent the neck
General care: Handling these patients is often made
contractures & to lessen the effects of tonic neck
more difficult from spasticity with the release of
reflexes. The hips & knees should be slightly flexed.
primitive neuromuscular activity such as reflex
Turning frequently will help in preventing the skin
patterns. Primitive postures may include those
breakdown & pneumonia. Caregivers should be
associated with decorticate (abnormal flexor
told about the turning the patient every 2 hours
response) or decerebrate (abnormal extension
when in bed. Specialized air mattresses with
response) rigidity.
electrical /pneumatic pressure relieving system are
Patients with TBI are at a high risk of developing useful for the alternate weight relieving from the
contractures owing to prolonged periods of load bearing body parts in the bed & avoiding the
immobilization & abnormal reflexing posturing. pressure sores. Splints help in positioning the head,
Techniques to preserve mobility & inhibit primitive the legs & the feet in these bedridden patients.
responses are employed as far as possible. These,
Stimulation of the sensory organs: Sensory
combined with careful positioning after treatment
stimulation is an intervention used in an attempt
sessions & every turn, should help reduce the
to increase the level of arousal & elicit movement
problems to which hypertonicity leads at a later
in individuals in a coma or persistent vegetative
stage. Never assume that these patients are unaware
state. It is proposed that, by providing stimulation
of what is happening around them. Talking to them,
in a controlled multisensory manner, with a
telling them what you are doing & why, asking
combination of stimulation and rest, the reticular
them to try & help, provide important stimuli from
activating system may be stimulated causing a
other sensory pathways along with the stimuli from
general increase in arousal. The following sensory
movement & handling. As consciousness returns,
systems are systematically stimulated: auditory,
the clinical features resulting from the head injury
olfactory, gustatory, visual, tactile, kinesthetic &
are evidently seen, but the predominant problem
vestibular. During these types of interventions, the
of spasticity will be evident early on.
patient must be closely monitored for subtle
Unless there are reasons to delay the start of an responses such as eye movts., facial grimacing, and
active physiotherapy, associated injuries such as changes in posture, head turning, or vocalization.
limb fractures, chest injuries, patients should be Overstimulation may lead to the accomodation
introduced to changes of position out of bed & making all efforts useless. Patient should be
movement early on. Aim for normal sitting posture, subjected for a single type of stimulus as
avoiding tipping chairs. multistimulii may lead to the confusion for the
The unconscious patients will also require to be patient. During this Coma stimulation,
treated for their oromotor impairments for oral physiotherapist must look for the warning signs like
hygiene, sensory motor training, chewing, flushing, perspiring, increase in respiratory rate,
swallowing, as well as speech therapy. agitation, eye closure, decreased level of arousal or
increase in muscle tone. If any of these symptoms
By Passive Range of Motion(PROM) exercises, joint are seen, coma stimuation therapy should be
mobility & integrity improvement can be achieved. immediately stopped. For the motor response,
During PROM exercises of upper extremities, care therapist should communicate with the patient by
should be taken to mobilize the scapula, otherwise, all the possible means.
impingement of glenoid fossa becomes the cause
of the pain in shoulder. PROM exercises when The patients who are slow to recover & minimally
performed, forceful or aggressive movts. should be conscious & who remain in this stage of recovery
avoided to avoid heterotopic ossification. It is more for a longer time duration are often placed in long
prone in the proximal joints & becomes the cause term rehabilitation centre/ nursing home.
of pain & hypo mobility. Proper positioning will Family members and caregivers of patients who
help in the reduction of secondary impairments have experienced a TBI play a critical role in the
such as contractures, bedsores, pneumonia, deep recovery process. Family members commonly
vein thrombosis, which not only hamper the experience a high level of stress related to worries
182 Neuro-Rehabilitation : A multi disciplinary approach

about the future, less free time, and increased who start recovering from coma with moderate to
conflict. Family education is an important severe cognitive, behavioral, and physical
component of the management. The goal of patient impairments often continue rehabilitation in either
& family education is to teach the family about the in-patient/ out-patient rehabilitation centre or a
stages of recovery and what can be expected in the multispecialty hospital. The cognitive abilities and
future. By becoming informed, the family may not behavioral presentation of individuals with TBI will
be so helpless. The family can also become involved have an impact upon their level of function. It is
in performing ROM exercises, positioning, & therefore vital that these elements be assessed and
sensory stimulation. Although it is difficult to taken into account when agreeing goals for
predict long term outcome at these early levels of interventions. Access to a neuropsychologist, who
recovery, families should be informed of possible can assess and advise on the most effective way to
outcomes. The therapist should be realistic but deal with these factors when planning treatment,
provide hope for the family. It is often beneficial to is invaluable.
have a medical social worker consult with the
As the patient begins to emerge from coma, he or
family to provide support and guidance. When the
she often experiences a period of acute post
patient begins to exhibit improved mobility skills,
traumatic agitation. The confusion, amnesia and
may lack the insight to recognize that he or she may
disorientation often result in agitation, aggression,
not yet be safe to ambulate or transfer alone. The
noncompliance and combative behavior. The
patient should be educated in how to best
patient may be markedly agitated and prone to
compensate for the residual impairments or
emotional outbursts ranging from verbally acting
disabilities. In severe cases, counseling will be
out to physically attempting to hurt themselves or
required for the patient and the family members
others or attempt sexually inappropriate behaviors.
by a medical social worker or a neuropsychologist.
The patient is always confused & with the short &
Family members should learn how to assist the
long term poor memory. Attention span is
patient with functional mobility including bed
decreased, and so, get easily distracted.
mobility, transfers, ambulation and wheelchair
mobility skills. They should be trained for the Examination at this stage would be difficult as the
proper body mechanics when assisting the patients patient is often uncooperative. The therapist must
to avoid the injury to themselves or the patient. utilize observational skills by seeing the functional
They should be taught the home exercises for the mobility, balance both in sitting and standing (if
strengthening and PROM exercises. possible), ROM, strength, motor control, tone,
sensation and reflexes. Patient's cognitive abilities
SUB ACUTE STAGE also have to be examined like orientation, attention
span, memory, insight, safety awareness and
At this stage, the goals & anticipated outcomes alertness. Neuropsychologist's help will be required
would be: to manage the patient's agitated behavior & help in
setting up behavioral modification technique such
• To preserve the integrity of the neuro
as positive reinforcement using a reward system,
musculoskeletal system, thereby preventing or
redirection and compliance training which will help
minimizing adaptive muscle shortening and
in managing inappropriate behaviors and
contractures
improving in therapy. Severe cases may require
• To mange the effects of abnormal Tone & medications too.
spasticity
Due to the confused state of the patient, it is very
• To provide an appropriate level of sensory important to maintain the consistency in the talking
stimulation as well as in dealing with the inappropriate
• To improve the motor control, postural control behaviors by all the members of the team as well as
& functional level the family members. It is important to maintain
the familiarity to the patient by keeping the same
Once the acute phase gets over & the patient with timing, place of the treatment & the same therapist.
TBI is medically stable, continuation of the care can Patient should be frequently provided orientation
be in a variety of set ups. Patients with minimally information.
conscious state or in coma may get the therapy in
At this stage, patient may require one to one staff
hospital or a long term care nursing home. Patients
supervision and assistance throughout the day.
Head Injury 183

Family support required at this stage ranges from muscles can help to decrease spasticity.
the supervision due to cognitive deficits to the Positioning is an important adjunct in the
physical assistance in all the ADLs due to motor management of tone abnormalities.
deficits. For discharge from the hospital, this factor Maintaining the head and neck in a neutral
of strong family support & ability to help is as position is important for minimizing the effects
important as the health status of the patient. that primitive postures may have in increasing
tone. Keeping the whole body in proper
The therapist will be primarily responsible to
alignment is also important.
improve the motor & postural control which will
be a prerequisite to improve the functional level of ii) Cryotherapy or air splints can also be used to
the patient. Maintaining the normal physiological reduce the hypertonia, though their effect is
length of the soft tissues is very important to temporary.
prevent or minimizing adaptive shortening and
iii) Serial casting helps in decreasing hypertonia.
contractures. This can be achieved by passive
Serial casting is often used for plantar flexors
movements and correct positioning of the patient.
or biceps contractures resulting due to either
Utmost care has to be taken to avoid the over-
increased tone or prolonged shortening of the
stimulation of the tissues and the joints. Taking care
muscle. Progressive maximal stretched
of the painful joints, especially the shoulder is very
position is achieved for the cast to maintain
important at this stage as this pain if persists,
for a week before trying to get the new more
become the cause of the inability to get the range
stretched position is tried for the casting.
even passively. If it prolongs for a longer time, to
PROM exercises are continued to have the new
get the motor control over the movements of the
increased ROM. There should be constant
upper extremities will be delayed & some times
check on the skin coming in contact with the
terminal ranges are permanently affected.
cast as it can breakdown if the patient has
Prophylactic splinting to the hypertonic muscular
sensory, communication or behavioral
body parts helps to prevent the shortening or
impairments.
contractures. Worst cases of hypertonicity may
require the medications. iv) Various medications either systemic (Baclofen)
or local (phenol or Botulinum toxin) can also
Physical & motor problems of abnormal tone in the
be used to reduce the hypertonia but their use
form of spasticity or rigidity is a common problem
should be weighed in comparison with the
throughout the recovery phase or thereafter for
side effects they can produce in a particular
many patients with TBI. More than 80% patients
patient
with TBI develop contractures due to the spastic
hypertonicity. Increased tone may be responsible v) Increased tone in lower limbs that requires
for the difficulty in personal hygiene, transfers or more than manual techniques to inhibit and
may be a source of pain or pressure sores. Increased maintain range of movement may benefit from
tone in LEs may help the patient in weight bearing the application of below knee weight bearing
and bed transfers or make it easier for the caregiver plasters. This is a skilled task and care must
to transfers. be taken to obtain correct alignment of foot and
ankle.
Physiotherapists use two basic treatment strategies
as compensatory (to improve functional skills by The tilt table can be used for periods of standing in
compensating for the lost ability) and restorative conjunction with standing with necessary assistance
(to restore the normal use of the affected part) & standing transfers. Lack of head control can
approach. impede progress to activities in sitting & standing.
Many therapeutic skills are available with Many patients with TBI get affected with ataxia,
Physiotherapist to deal with the abnormal tone and who will need the coordination & balance therapy.
in turn to avoid its adverse effects. Some of these may require a walking aids either
temporary or for a longer time. Sensory disturbance
i) Basics of spasticity management are
will involve perceptual problems including vision
therapeutic stretching-sustained & gentle,
& hearing, apraxia & agnosia. Emotional &
strengthening exercises with adjunctive
intellectual disturbance will involve memory &
modalities and functional retraining. PROM
behavioral disorders. For these patients co
and selective strengthening of the antagonist
treatments will be beneficial if done along with
184 Neuro-Rehabilitation : A multi disciplinary approach

Speech therapy, Occupational therapy & Neuro restore movement and functional mobility.
psycho therapy. Once, the patient gets the fair control over the
different parts of the body as per the
Upright sitting is very important to work towards
involvement due to their cerebral or the
the treatment goals for the early levels of recovery.
cerebellar trauma, postural training should be
Upright position is vital for the proper functioning
started on the basis of developmental sequence
of many organs like, stimulation of bowel
& the biomechanical stability basis. This may
movements and bladder emptying, improved start with the supine-side turning as a log first
ventilation due to moving down of abdominal & then by girdle initiation, cephalocaudally or
contents leading to redistribution of air flow to basal caudocephalic-prone lying- coming on the
lobes and changing perfusion/ventilation ratio. As forearm & then on hands- all four with elbow/
soon as medically stable, the patient should be forearm loading to hands loading- quadruped
transferred to a sitting position and out of bed to a position with ability to relieve the load of one
wheelchair / chair. All precautions should be or two extremities (crossed)-independent
observed. The head should be properly supported, sitting -kneel sitting-half kneel sitting-kneel
as the patient may have very inadequate neck and standing- sitting to standing- kneel standing
head control to maintain an upright posture without to standing-squatting -walking & then the
support. Often, it is beneficial to perform co training for the dynamic postures. All the static
treatments with an Occupational therapist when postures first to be trained in assisted &
first sitting & transferring the patient. Use of a tilt guarded situations. Once active control is
table is very useful as it allows early weight bearing gained, then they are made more & more
through lower extremities. The upright position in difficult so that in adverse situations they will
sitting by the side of the bed, in a wheel chair or be mastered. This can be against the resistance
standing on a tilt table improves overall level of or against the smaller base of support or
alertness. Sitting training promotes movement and against the unstable base. The gymnastic ball
learning by allowing the patient's body in functional has specific qualities which can be used to gain
position to do the tasks. Assisted movements mobility, stability & postural control. Other
provide tactile, proprioceptive and kinesthetic injuries, if any, than TBI also should be taken
stimulation while training to perform a ADL task. care of during training all these postures. As
and when, the patient masters the posture,
Constant efforts are required by the physiotherapist
ability should be used for the related possible
to improve the motor control & motor learning.
activities performance first in the therapeutic
Neuro physiotherapeutic techniques can be used
area & then in life situations.
to initiate, progress, improve the sensory motor
control and finally helping the patient to get back ii) Proprioceptive Neuromuscular Facilitation
to the as normal life as possible depending upon (PNF): This is a good technique for the
the severity of the lesion, personal as well as initiation of mass movements of the proximal
external contextual factors. Due to the newer girdles to the distal components as they are
understandings and the theories of the motor closer to the activities of the daily life. These
learning and the practices, many new skills are can be first initiated passively by the therapist
developing but still most of the older skills also hold with the commands till there is response and
true today and specifically in the initial and then assisted to learn actively by the patient.
subacute phase, they are most useful. The therapist Full range movements along with the strength
should work as per the patient's physical level of and endurance are achieved. All these
function and attempt to improve endurance. activities should be trained for the near normal
Progressing to more challenging skills which would equilibrium responses. Sufficient practice will
require new learning, should be as per the patient's give this maximum perfection to which the
capacity for new learning. patient can reach a level & with the maximal
endurance. Once this is met in the treatment
Depending upon the skills and experience of the area, this has to work equally good in the
therapist, clinical conditions and the co-morbidity actual life situational areas, where patient will
of the patient any or many of the followings are
have to be multiresponsive due to the
used. environmental demands depending upon the
i) Developmental sequence: Developmental crowd, architectural barriers & the vehicular
postures are utilized to facilitate and help traffic.
Head Injury 185

iii) Neuro Developmental Therapy(NDT): This skills & the progress each day. Patient will be
is based on Ms. Bobath's core ideas of rigorous required to give a recall each day of the previous
observation of posture and movement, careful days teaching as carryover will be difficult in many
alignment of body segments and joint position patients.
before asking patients to move, constant
It is important for the therapist to perceive the
analysis of how the patient moved throughout
patient's emotions & behavior, so that he / she
the entire treatment session, the contribution
should be able to make feel the patient safe &
of the sensory systems to movement, family
secured. Even the family members' & caregiver's
involvement in treatment, and home
attitude towards the patient will have an effect on
programming.
the behavior of the patient. Patient will not be in a
Task oriented approach is currently advocated position to understand the other people's views &
for this locomotor training, utilizing most likely egocentricity may be present. Due to
the limited attention span of the patient and the easy
iv) Body Weight Support (BWS) and a treadmill:
distractibility, concentration on the activities
Suspending the patient in a parachute like
training is going to be limited for early days of the
overhead harness which allows partial relief/
treatment.
support of the body weight. Therapist needs
to support the patient by pelvis / trunk for
the weight shifting & the forward
PHYSICAL REHABILITATION
advancement of the LEs. This can be done in At this stage the main goals of physiotherapy
parallel bars, with walker or with the treadmill. interventions are to:
Training as well as corrections can be done in
• Encourage the return of active movt. that
this system in each phase of the gait cycle.
carries over into function
Difficulty can be progressively increased by
decreasing the harness & physiotherapist's • Prevent secondary deformities
support and increasing the speed of walking • Prevent unnecessary and potentially
or treadmill as the patient's ability improves. damaging compensatory movt. strategies
v) Constraint Induced Movement Therapy(CI/ • Maximize respiratory function
CIMT): It involves promoting the use of the
most affected UE for up to 90% of waking • Encourage social and vocational reintegration
hours and reducing the use of the lesser • Provide advice to the family, caregivers and
affected UE. Intensive, task oriented training other members of the team on aspects of the
is provided for the affected UE for up to 6 hrs patient's management
per day over 2-3 weeks period.
• To educate family members on patient's
vi) Modified Constraint Induced Movement condition, management goals & outcomes
Therapy(MCIMT): A mesh is worn to the
unaffected hand for gentle restraint of the At this stage, when patient shows the signs of
actvities for several hours for 10 weeks improvement in the consciousness & the awareness
[Link] will be same as CIMT. or may be only in the sensory motor components
with respiratory independence, patient will be most
vii) Hand Arm Bimanual Intensive Training of the times either in half way rehabilitation centre
(HABIT): This is also a form of intensive task or at home & comes for the therapy on out patient
training, but the more emhasis is on the basis.
bilateral hand manipulations in ADLs.
Unaffected hand may play a major role at the The examination should be done at this level to
initial phase, but, emhasis should be given on access the status on following points:
the more and more equal participation of the • attention & cognition
affected hand during the tasks.
• cranial nerve integrity
Patient should be started training the simple daily
• balance
functional activities like bed activities, feeding &
the ambulation before expecting him/ her to learn • gait
new skills. Use of some audiovisual aids like charts/
• joint mobility
pictures/ photos will help the person to learn new
186 Neuro-Rehabilitation : A multi disciplinary approach

• motor control TBI patients have a wide variety of impairments


and functional limitations, they may be helped to
• muscle strength
compensate for the lost function by giving them
• ROM some form of adaptive device. It depends upon the
• Reflexes predicted probable residual disability as per the
opinion of the rehabilitation team, to decide how
• ADL skills & sensory integrity much help should be given. Most severe patient
• Functional abilities of the patients (better who may not be able to make to walk again will
in closed environments than an area of require a wheel chair. Depending upon his or her
multiple distractions) regained cognitive skills, wheel chair can be
advanced with the computer based controls
• Gait is generally examined by
powered one to the simple manual one which may
Observational Gait Analysis to know the
be operated by the patient or the caregiver.
deviations & asymmetries so the
impairments can be found to treat the Therapist should have more options of the activities
causative factors. Gait speed & the for the training, so after few days, different
endurance can be measured by 10 meter combinations & the options to the patient can be
walk or 6 minute walk test given for breaking the monotonicity as well as to
give choice to the patient.
• Their motor control & functional status
including the ability to perform ADL, Prior to discharge from a comprehensive
transfers, bed mobility, stairs, & interdisciplinary day treatment settings, cognitive,
locomotion can be measured by using behavioral, emotional and psychological issues
Functional Independence Measure (FIM) have to be tackled before community re-entry and
or Fugl-Meyer Assessment of Motor return to school / work. Skills in judgment,
Recovery (FAM) or Chedoke -MacMaster problem solving, planning, self awareness, health
Stroke Assessment. and wellness and social interaction should be taught
by the respective professionals with lesser and
For the task performance training, it is to be seen
lesser supervision needs. Once the patient gets the
about the balance during the performance, initiation
insight into his/ her own strength and weaknesses,
& completion, consistency, energy required, shift
they should be involved in decision making for their
of the body weight & maintenance of body
working towards reintegration back to home and
alignment.
community. Group treatment sessions and Support
Those having cognitive & behavioral deficits will groups are crucial for the patient to learn how to
have difficulty in reacquisition of the motor skills function in society with their present abilities and
due to their mental as well as physical fatigue limitations. Adaptations are required most of the
during treatment sessions. That can be known by times by the family, work place colleagues or
the signs like increased irritability, decreased schoolmates to accommodate the patient.
attention and concentration, deterioration in the
Occupational Therapy and Traumatic Brain Injury
performance of physical skills and delayed
initiation. Treatment sessions with sufficient rest Brain injury is a life-altering event which affects
periods will avoid the mental & physical fatigue every area of a person's life including his or her
and will maximize the motor relearning. relationship with family members and others close
to him or her. The effects of brain injury often
TBI patients will have many different physical,
change roles and responsibilities within the family.
cognitive and emotional impairments making the
therapist to have an individual approach as per the TBI can result in physical, cognitive, behavioral, or
patients capabilities and the difficulties due to the emotional difficulties. People with TBI may
uneven recovery of these different aspects of the experience short- term memory loss, have difficulty
brain functioning. For every patient, the emphasis concentrating or paying attention, have impaired
is given for the initiating, ability to get it actively judgment, experience headaches or migraines, have
and perfecting the motor tasks by making them slurred speech, experience seizures, become
progressively increasingly difficult and complex to fatigued, depressed, or easily agitated, or
blend them in the daily activities successfully. experience increased anxiety and impulsive
behaviors.
Head Injury 187

Half Rolling Bridging

One leg Bridging Side sitting in Quadrapud

Crawling Hip extension in Quadrapud

Unilateral arm raise in Quadrapud Kneel walking(1)


188 Neuro-Rehabilitation : A multi disciplinary approach

Ball Catching in Kneeling Position Forwar bending at the edge of bed in sitting

Long leg shifting in forward direction Long leg shifting in backward direction

Getting up from sidelying Getting up from supine

Getting up from low stool One leg Standing


Head Injury 189

Reachouts with weight bearing on affected side Coordination exercises for Upper limb

Balance board exercises Cognitive Therapy

Hip adduction on Swiss Ball Hip flexion on swiss ball

Upper abdomen on Swiss Ball Back extension on Swiss Ball


190 Neuro-Rehabilitation : A multi disciplinary approach

Post trauma manifestations may also include Interventions and Practices


occasional loss of consciousness, dizziness,
headache or vertigo provoked by sudden changes Development of Occupational Profile:
in position; confusion and disorientation about time Prior to evaluation the occupational therapist needs
and place; and emotional reactions such as to gather as much information as possible (from
combativeness. Behavioral disturbances may be varied sources such as diagnostic reports, (CT scan
blatant, particularly during intital recovery period. and MRI reports), medical notes, and interviews of
Personality disorders, ammensia and delirium may client and or care takers and other team members)
also occur. These manifestations may be regarding patients condition such as presence of
accompanied by intellectuals deficits, blindness, any co morbidities (associated fractures and or
diplopia, hemianopsia, olfactory dysfunction and pheripheral nerve injuries) and pre morbid status.
auditory deficits. Physical symptoms include The CT scan and MRI reports are invaluable in
quadriparesis, unilateral or bilateral hemiparesis, indicating the area and extent of damage caused to
initial decerebrate rigidty and speech deficits. the brain and the possible potential impairments
that the client may have. The therapist then
These deficits prevent the head injured person from
evaluates the client to develop a clear
effectively interacting with the environment and
understanding of the areas of impairment and its
may hinder rehabilitation. The patient may seem
effect on the abilities and capabilities of the patient
to have lost contact with the surroundings and to
and its impact on the occupational performance of
be able to focus only on the narrow yet
the patient. This information is then used to assist
overwhelming feelings, concerns and behaviour
in directing any assessments and identifying
imposed by the sudden disability. The clinical
underlying causes for problems in functional
picture represents a pattern of fluctuating
abilities of the client.
symptoms in these areas, subject to frequent change
evident throughout the recovery as the patient tries
Analysis of Occupational Performance:
to adjust to the deficits imposed by the injury, to
learn to decrease and eliminate handicapping Information from occupational profile informs the
effects and to renew self confidence through Occupational Therapist about the extent of
awareness and acceptance of the immediate and impairment, activity and participation limitations
future levels of function. while considering the environmental and social
contexts. In the early phases of recovery the client
Occupational therapists can help people who have may be unable to participate in purposeful and goal
sustained a TBI. Injuries can range from minor to directed activity due to the altered state of
extremely severe and call for various levels of consciousness. Hence OTs analyse the occupational
intervention and treatment. The type and duration performance on return of mental alertness and
of the intervention will also depend on the severity purposeful behavior. The occupational therapist
of the injury. should observe and anlyse the clients occupational
As part of the TBI rehabilitation process, performance in the natural or least - restrictive
occupational therapy is designed to assess functions environment. The therapist must endeavour to note
and potential complications related to the the effectiveness of the following performance
movement of upper extremities, daily living skills, skills: (i.e. the observable elements of action of an
cognition, vision and perception. Together with the occupation. )
patient and family, the occupational therapist will 1. Motor and praxis skills
help determine the best ways to perform daily
2. Sensory -perceptual skills
living skills including showering, dressing and
personal hygiene. The therapist will identify 3. Cognitive skills
equipment that can help the patient when eating, 4. Emotional regulation
dressing and bathing. 5. Communication
The occupational therapist will also address specific 6. Social skills
skills to prepare the patient for their return home.
It is also important that the occupational therapist
These skills may include activities for daily living,
gathers information about
such as Cooking, Grocery Shopping, Banking,
Budgeting, Readiness for returning to work by • The clients performance patterns e.g. habits,
assessing prevocational and vocational skills, etc. routines, rituals and roles
Head Injury 191

• The client factors like underlying abilities, • Identify potential approaches guided by best
values, beliefs, and spirituality, body functions practices and evidence and as per discussed
and body structures which influence with client and family.
individuals occupational performance.
Interventions:
• Environment and contextual factors such as
influence of cultural, personal, physical and As part of the OT process, the occupational therapist
social context on occupations and activities. develops an intervention plan that considers the
clients goals, values beliefs, health and well being,
• Activity Demands The demands of an activity the client's performance skills, performance
are aspects of the activity that include the tools patterns, collective influence of context,
needed to carry out the activity, the space and environment activity demands and client factors on
social demands required by the activity, and client's performance.
the required actions and performance skills
needed to take part in the given activity. The intervention plan outlines and guides the
therapists' action and is based on best available
The occupational therapist theoretical knowledge evidence to meet the targeted outcomes. The
and clinical expertise help in selecting specific therapist also determines the intervention approach
assessment and evaluation methods to identify and that is best suited to address the identified goals.
measure the factors affecting clients performance The Intervention approaches used by OT include
at a particular time.
Prevent: an intervention approach designed to
Occupational Therapist may elect to use client - address clients with or without disability who are
centred evaluation approach that may focus on at risk for occupational performance problems; for
possible impairments affecting performance of example, intervention to prevent development of
function. (Bottom - up approach) or an evaluation secondary impairments such as joint contractures
approach that analyses the role of individual with during the coma phase of recovery.
TBI and areas of occupation that encompass a
typical day (top - down approach) Establish and restore, an intervention approach
designed to change client variables to establish a
Occupational therapist may select dynamic skill or ability that has not yet developed or to
assessments that emphasise the process involved restore a skill or ability that has been impaired; for
in learning and change to gather information to example, restoring hand coordination to engage in
guide treatment planning and intervention or may functional activities such as cooking.
select performing evaluations that include body
structures and functions, activity and participation Modify activity demands and the contexts in which
enabling the occupational therapist to compile a activities are performed to support safe,
comprehensive view on the persons functioning. independent performance of valued activities
within the constraints of motor, cognitive, or
Prefered assessments are assessments with proven perceptual limitations.
• Validity with traumatic brain injury Create or promote a healthy and satisfying lifestyle
population that includes adherence to medication routine,
• Efficacy in detecting and quantifying the appropriate diet, appropriate levels of physical
typical pattern of impairment seen in clients activity, and satisfying levels of engagement in
with TBI. social relationships and activities by providing
enriched contextual and activity experiences that
Based on the assessment data, the occupational
will enhance performance for all persons in the
therapist then endeavors to
natural contexts of life.
• Identify factors that support or hinder clients Maintain performance and health that the
performance such as memory impairments individual with TBI has previously regained or that
affecting personal hygiene, home management neuropathology has spared.
skills, work tasks and social participation.
Types of intervention:
• Establish goals that are meaningful to the client
and family and address the client's desired The OT consider the type of intervention when
outcomes. deciding the most effective treatment plan for a
client. The types of interventions include:
192 Neuro-Rehabilitation : A multi disciplinary approach

• Therapeutic use of self (therapist's use of his/ During the early period of recovery from a TBI, the
her personality, perception and judgement). occupational therapist working with the client in
coma typically focuses the intervention on
• Therapeutic use of occupations and activities
establishing or restoring the client factors or
which include
impairments that resulted from the injury such as
– preparatory method : (e.g. Serial casting, impairment in voluntary control abnormal tone.
sensory stimulation.) Intervention also focuses on preventing the
– purposeful activity : (e.g. role playing of development of secondary impairments that occur
social situations, practicing grocery in the period of unconsciousness like prevention
shopping in stimulated environment) of pressure sores and heterotrophic ossification.
Global mental functions, such as level of
– occupation based activity: (e.g. consciousness and alertness, are often addressed
Interviewing for a job or preparing meal through a program of coma arousal or sensory
for one's family.) stimulation.
• Consultation and The focus of intervention may shift among
• Education establishing, restoring, or maintaining occupational
performance; modifying the environment and/or
I. Intervention during early period contexts and activity demands or patterns;
of recovery: promoting health; or preventing further disability
and occupational performance problems.

Occupational Therapy Intervention Approaches and Examples of Their Use with Clients at Various
Levels of Recovery from Traumatic Brain Injury
Intervention Approaches Commonly Used During the Coma Phase of Recovery (Rancho Los Amigos
Levels I-III) Interventions typically focus on state of consciousness and WHO ICF areas of impairments.
Intervention Approach Occupational Therapy Treatment Activities
Prevent Prevent loss of muscle length and joint mobility by
• performing range of motion (ROM),
• serial casting,
• tone-inhibiting techniques, and
• positioning of patient in the bed and wheelchair.
Prevent skin breakdown and postural deformities by providing
• Providing the client with proper body alignment in tilt-in-space wheelchair
with head
• rest, lap tray, gel seat cushion, and trunk inserts and
• providing the nursing staff with a splint-wearing schedule.
Establish/Restore • Restore the client's connection to the external environment by positively
reinforcing appropriate behavioral responses to sensory stimulation.
• Restore the client's ability to follow one-step demands for a motor response
in relation to sensory stimulation within 15 seconds of request or stimulus.
Modify • Modify environment to vary levels of stimulation
• prevent accommodation and attenuation to environmental stimuli (e.g.,
vary lighting, noise level, visual stimulation, temperature).
Maintain • Maintain muscle length and joint mobility by instructing caregivers in
routine stretching exercises.
Head Injury 193

Ball bouncing Tilt in space wheelchair Gel seat cushion

Attached lap tray to wheelchair Basic self care grooming activity- Wiping face

A) Object manipulation using marbles B) Object manipulation using Shape sorting activity

A) Restore cognitive skills using Stacking activity B) Restore cognitive skills by naming of objects
using flashcards
194 Neuro-Rehabilitation : A multi disciplinary approach

Neuromusculo skeletal Recovery Programs Splinting and Serial Casting: Splints are indicated
Occupational therapy intervention for when
neuromusculo-skeletal and movement-related - Spasticity interferes with movement
functions after brain injury is focused on either
- Joint range of motion limitations are
impairments that are a primary consequence of that
injury (e.g., impairments in voluntary movement, present
abnormal tone, balance) or secondary impairments - Soft tissue contractures are possible.
resulting from the immobility or excessive muscle
Splints are thought to provide elongation and
tone, such as contractures of the muscles or joints
inhibition by positioning the joint in a static position
and diffuse weakness and deconditioning. At the
with the muscle and soft tissues on stretch..
coma phase of recovery, intervention for
Splinting of elbows, wrists and hands is often
neuromuscular motor impairments is generally
implemented to maintain functional position at rest
passive in nature, using more preparatory methods
and to reduce tone. For eg. Resting hand splint or
such as passive range of motion (PROM), splinting
functional position splint. Once splints have been
and serial casting, and positioning in the bed and
fitted, then:
wheelchair to either establish and restore motor
control or prevent the development of secondary - A wearing schedule is established for the
joint and muscle contractures. Joint contractures can care givers to follow.
result in significant limitations in self-care, - Caregivers are trained in proper
particularly dressing and hygiene. application and removal of each splint.
PROM: - The client must be monitored frequently
Prolonged periods of disuse and immobility can for skin breakdown or tonal changes that
lead to change in muscle and joint postural may change the initial fit of the splint.
alignment and other impairments (such as muscle
Serial Casting:
tightness, atrophy, fibrosis, contracture, postural
deformity). Early intervention is critical in A serial casting program is a more aggressive
maintaining joint motions, tissue extensibility, intervention indicated in the presence of moderate
physical ability and function. Additional benefits to severe spasticity that cannot be managed by
include improved circulation and tissue nutrition splints. The goal of serial casting is to increase range
to the limbs and pain inhibition along with of motion and decrease tone by using progressive
decreased hypertonicity and increased sensory succession of separately fabricated casts each worn
input. continuously for a period of weeks. Casts are often
left on for five to seven days which puts the muscle
In the coma phase of recovery, when the patient is and tendons on a prolonged stretch and reduces
unresponsive, the therapist or the caregiver tone. Successive casts are designed to increase range
performs ROM exercises passively through the of motion further until a functional joint range is
patients full available range. The limbs are well achieved and maintained. The common difficulty
supported with stable positioning of the patient to that prevents the success of serial casting is skin
prevent joint trauma. Movements should be slow breakdown.
and rhythmic taking care to avoid excess force and
pain. This is especially important in clients with TBI Positioning in Bed
as they are at risk for heterotropic ossifications.
Proper bed positioning is critical in early stages of
When moving the Upper Extremity during PROM, TBI to prevent pressure sores and to facilitate
care should be taken to mobilize the scapula and to normal muscle tone. If a client exhibits abnormal
maintain proper joint mechanics at the tone or posturing a side line or semi-prone position
glenohumeral joint. Maintaining ROM at the hip is preferred as it assits in normalizing tone and
and ankle are especially important as a decrease providing sensory input. A supine position might
range of motion at these joints can greatly impact elicit tonic labyrinthine reflex and extensor tone.
functional activities like transfers, sitting, Supine position with head in lateral position may
wheelchair positioning ambulation and stair elicit asymmetrical tonic neck reflexes. Clients with
climbing. TBI generally have bilateral involvement requiring
a program for side-lying on both sides.
Head Injury 195

Pillows, foam wedges and splints may be back.


incorporated in a bed positioning program to • An abduction wedge between the lower
facilitate normal position and prevent abnormal extremities can help in reducing hip adduction
postures such as extreme elbow flexion, head and and internal rotation.
neck extension and foot drop deformity.
Management of Heterotopic Ossifications (HO)
Wheelchair Positioning:
Monitoring clients for the development of HO (e.g.,
Positioning the client in coma in a wheelchair with observation for an inflammatory reaction, palpable
adequate supports for the head and trunk also can mass, or limited ROM in joints of the limbs) in
decrease muscle tone, foster increased upright clients with abnormal tone is important. During the
motor control in a functional posture, and improve acute inflammatory stage, the occupational
awareness of stimuli within the environment. therapist should position the patient's involved
A proper wheelchair positioning helps prevent skin limb in a functional position and initiate gentle
breakdown and joint contractures. Facilitate normal PROM, monitoring the patient for signs of pain (e.g.,
muscle tone, inhibit primitive reflexes, increase facial grimace, change in vital signs). Once acute
sitting tolerance, enhance respiration and inflammatory signs have subsided, continued
swallowing and promote functioning. mobilization is indicated to maintain range. Resting
the joint appears more likely to lead to decreased
While seated on a wheelchair, the client's buttocks
joint range, whereas continued mobilization may
should bear weight evenly with both ischail
lead to formation of a pseudarthrosis.
tuberositities firmly resting on the wheelchair seat.
The knees should be positioned at 90 degreees with
Sensory Stimulation Programs
heels slightly behind the knees. It is desirable to
maintain both feet securely on the footplate to The goal of sensory stimulation program is to
provide propio-ceptive inputs and facilitate weight increase the client's level of awareness by trying to
bearing in both heels to normalize tone. The upper increase arousal with controlled sensory input.
extremity should be positioned with the scapulae Introduction of sensory stimulation in the form of
in neutral position (neither elevated nor depressed, isolated visual, auditory, tactile, olfactory and
shoulders slightly externally roated and abducted, gustatory stimulants to the individual with brain
the elbows in a neutral position of slight flexion with injury may heighten arousal. For e.g.
forearm pronation and the wrist and digits in a 1. Flash light may be used to elicit eye opening
functional position. Slightly reclining the and visual tracking.
wheelchair between 10 to 15 degrees prevents
2. Playing familiar music may cause changes in
clients head from falling forward and facilitates
respiratory rate or blood pressure.
visual interaction with the environment. A good
wheelchair positioning can be achieved by using 3. Olfactory stimulants like variety of scents may
several positioning devices as and when necessary elicit eye opening or head turning.
such as:
4. Gustatory stimulation like salty, sweet, bitter
• A firm solid seat (padded with foam and or sour taste to clients lip and tongue, etc.
covered with vinyl) facilitates neutral to
5. Kinesthetic input in the form of guided
anterior pelvic tilt and prevents internal
movements while performing simple
rotation and adduction of the hips.
movements like rolling side to side or wiping
• A wedged seat insert with downward slope the mouth.
pointed towards the back of the chair facilitates
hip flexion and inhibits extensor tone in hips The theoretical aim of functional sensory
and lower extremities. stimulation is to reactivate highly processed neural
pathways that have been established before the
• Lateral trunk supports can be used to reduce
injury.
scoliosis and lateral trunk flexion caused by
imbalanced tone of the intrinsic muscles of the
196 Neuro-Rehabilitation : A multi disciplinary approach

II. Intervention During the Acute Rehabilitation Recovery Phase (RLA Levels IV to VI)
Intervention Approaches Commonly Used During the Acute Rehab Phase of Recovery (Rancho Los
Amigos Levels
IV-VI) Interventions typically focus on motor and cognitive skills and WHO ICF areas of impairments
and activity limitations.
Intervention Approach • Occupational Therapy Treatment Activities
Prevent • Prevent aspiration during feeding by modifying the food texture and head
positioning if the client displays signs of dysphagia.
Establish/Restore • Establish the client's ability to release energy constructively during agitated
periods by providing structured and familiar activities with minimal
challenges to areas of impairments.
• Restore ability to perform self-care by engaging the client in a daily self-
care program of showering, dressing, and grooming, providing verbal
and physical cues as needed.
• Restore normal patterns of movement by engaging the client in various
functional motor tasks (e.g., grooming, self-feeding, object manipulation)
with gradual increases in the unpredictability and complexity of the
contextual and activity demands, providing tactile input to guide and
normalize movement patterns.
• Establish skills to safely and efficiently transfer from wheelchair to various
surfaces (e.g., toilet, bed, chair, car).
• Establish and restore cognitive skills by teaching cognitive strategies to
improve performance; engage in a variety of activities related to roles,
responsibilities, and interests (e.g., financial management, cooking,
parenting, leisure pursuits).
• Establish strategies and new routines to accurately use external memory
aids to recall scheduled appointments and events and to take medications.
• Establish habits to ensure accuracy of work (e.g., self-monitoring of work
for errors, timely completion, match with instructions).
Modify • Modify the client's hospital room to provide environmental cues to
minimize confusion and to provide orientation to person, time, and place.
• Modify tasks and environments to enable independence (e.g., provide
adaptive equipment to increase independence in ADLs and IADLs, such
as checklists for activity sequences and external memory aids).
Maintain • Maintain the client's postural alignment while sitting by providing
structural wheelchair supports.
• Maintain the client's maximum ROM obtained with serial casting by
providing resting cast/splint for night wear.

The acute rehabilitation phase of recovery begins spectrum of behaviors by the client that fluctuate
as the client is medically stable and emerging from with changes in situational factors such as
the coma. Occupational therapists, as part of the environmental stimulation, task demands, and time
rehabilitation team working with clients who have of day. During this phase the client is alert but often
TBI, must address the client's physical, cognitive, displays confused, agitated and inappropriate
communicative, emotional, and spiritual needs. response which might hinder his ability to
This phase of recovery from TBI encompasses a participate in the rehabilitation program.
Head Injury 197

Behaviour modification techniques such as positive Some treatment approaches utilize the
reinforcements using a point or reward system, developmental sequence and muscle facilitation
redirection and compliance training are useful in techniques. Developmental postures like prone on
managing inappropriate behavior and improving elbows, quadruped, sitting, kneeling half kneeling
participation in therapy. and standing can be used to facilitate and help
restore movement and functional mobility.
As the agitation lessens, the cognitive and motor
challenges presented to the client can be gradually
Other Considerations:
increased to address underlying impairments.
A brain injury can cause fatigue and conditions such
Addressing Motor Recovery as seizures, spasticity and swallowing difficulties
visual problems and bladder and bowel changes.
As the client with TBI emerges from coma and
performs more voluntary movement, the
Fatigue
occupational therapist begins to address
impairments seen within the sensory and Fatigue may result from the injury (and other
neuromusculoskeletal and movement-related injuries in cases of trauma) or from additional
systems. Clients with persistent spasticity resulting physical and mental effort required to do tasks that
in joint contractures interfering with performance once were performed with little or no effort.
of functional activities, who have not been Physical functioning, attention and concentration,
successfully managed with more conservative memory and communication can be adversely
rehabilitation techniques, may be candidates for affected by fatigue. In time, the person's stamina
botulinum toxin A injections, motor point or neural and energy level likely will improve, and the ability
blocks, surgical release of the contracted tissue, or to engage in activities may be increased. The
intrathecal baclofen pump placement. Occupational following strategies may be useful in helping the
therapy intervention following these procedures person with brain injury learn to manage fatigue:
can be helpful to increase functional integration of • Encourage use of a calendar or planner to help
the limb into daily activities. manage mental fatigue.
Occupational therapists apply the principles of • Set a schedule that includes regular rest breaks
practice and feedback, task-specificity, and training or naps. (For example, one nap in the morning
intensity when providing intervention focused on and one in the afternoon after some physical
motor skill recovery. Two commonly used or mental activity.) Rest breaks or naps should
approaches to address movement related not exceed 30 minutes and evening naps
impairments after TBI are motor learning and should be avoided.
constraint-induced movement therapy (CIMT).
• Gradually decrease the length and number of
Motor learning is a process of learning to produce breaks as the person's ability to tolerate
skilled movement that involves practice and activities with less fatigue improves.
experience. Occupational therapists using a motor
• Resume activities gradually, over weeks or
learning approach set up the therapeutic learning
even months.
environment, typically the occupational therapy
clinic, to promote skill acquisition by varying the • Start with familiar tasks that the person can
tasks and environment to meet the patient's current complete without fatigue.
learning abilities.
• Gradually increase the complexity of the task,
CIMT, a motor skill intervention approach based encouraging breaks as needed, and slowly
on the learning principles of shaping and increase the length of time.
preventing learned nonuse, was initially designed
• Become familiar with indicators of fatigue for
to increase the use of the impaired arm in chronic
the person such as increased inattention or
stroke patients. CIMT involves three main
distractibility, repetition of tasks or comments,
components: (1) intensive training of the affected
irritability or increased errors.
arm, (2) practice to promote transfer of therapeutic
gains from the clinical environment to real-world • Encourage breaks, every five minutes, during
situations, and (3) constraint of the less-affected arm tasks, before or as soon as signs of fatigue
during the entire period of intervention. appear.
198 Neuro-Rehabilitation : A multi disciplinary approach

• If the health care team recommends, use Driving privileges should be restricted until a
assistive aids (for example, a cane for walking) predetermined seizure-free interval has been
to conserve energy or a wheelchair for long achieved (often six months to one year). During this
distances. time, extreme caution should be taken if the person
will be working around heavy or dangerous
• Plan ahead for fatiguing activities, such as
equipment.
visitors, trips, going out.
• Schedule a nap before visitors come or before Swallowing
going out. Problems that affect swallowing after brain injury
• Consider limiting the person's time with can vary widely and may include one or more of
visitors or a rest break during visits. the following:
• Poor head or upper body control
Seizures
• Decreased lip and tongue strength, range of
Seizures can be caused by a sudden, excessive,
motion and coordination
disorderly electrical discharge of brain cell activity.
This risk of ongoing seizures is related to the • Impaired memory or concentration
severity and characteristics of the brain injury, such
• Any or all of the above may cause aspiration
as the type and location of the brain injury. Risk
(inhaling food or liquid into the lungs)
seems to be greatest in the months after injury, and
generally declines with time gradually. Exercises, treatment techniques and positioning
may help improve a person's ability to chew and
Several types of seizures may occur after brain
swallow. An occupational therapist along with a
injury. The most frequent types are generalized
speech therapist will teach the person with brain
(grand mal, tonic/clonic) and partial (partial
injury and caregivers how to perform these
complex and simple partial) seizures.
exercises and techniques. Most people regain the
Most seizures are self-limited and last only a few ability to swallow after brain injury, though it may
minutes. The person may cry out, stiffen and fall, take longer for some than others.
have jerking movements, turn flushed or blue and
have some difficulty in breathing. The OT can Visual Problems
educate the immediate caretakers on the steps that Occupational therapy practitioners are recognized
need to be taken in case of a seizure, such as: experts in addressing the vision processing deficits
• Make sure the person is in a safe area and lay that occur from traumatic brain injury (TBI).
the person's head on something soft if a fall Practitioners provide interventions to improve
has occurred. visual attention, search and speed, and efficiency
in visual processing. They work closely with
• Loosen tight clothing such as a necktie or belt optometrists and ophthalmologists to ensure that
and remove eyeglasses. clients with TBI are able to compensate for deficits
• Clear away hazardous objects that may be in acuity, visual field, and eye movements to
nearby. complete important daily activities safely and
• Position the person lying on his or her side to effectively.
keep the chin away from the chest. This will Clients with TBI either have cortical visual
allow saliva to drain from the mouth. impairment (CVI; affecting areas of the brain that
• Do not force your fingers or any object into process visual information) or ocular visual
the person's mouth. impairment (affecting the eyes). Clients with either
CVI or ocular impairment benefit significantly from
• Do not restrain the person since a seizure
coordinated and comprehensive services to enable
cannot be stopped.
them to learn to use their remaining vision more
• After the seizure, the person usually will be efficiently and learn non-visual methods to
temporarily confused and drowsy and hence complete activities.
do not offer food, drink or medication until
the person is fully awake. Someone should Bowel and bladder changes
stay with the person until full recovered has Brain injury may affect bowel and/or bladder
occurred.
Head Injury 199

function. The injured person may need help re- • Plan ahead to get to the bathroom
establishing and maintaining a pattern of regular
• Walk to the bathroom in time
bowel and/or bladder emptying.
• Recognize the sensation of bladder
Bowel management
fullness or the need to urinate
The goals of bowel management include
Premorbid conditions (such as an enlarged prostate
establishing a regular emptying pattern,
in men or a pattern of infrequent urination in men
maintaining dry, healthy skin, and avoiding
or women) may add to bladder problems after brain
incontinence, diarrhea, and constipation. Each
injury.
person is assessed by a physician and
recommendations are made as needed. The goals of bladder management include
preserving kidney function, preventing
Bowel problems can occur if the person with brain
incontinence (accidental urination), preventing
injury:
bladder overfilling and bladder infections,
• cannot control bowel emptying voluntarily establishing a regular patter of urination with
complete bladder emptying, and maintaining dry,
• cannot recognize bowel fullness and the need
healthy skin in the genital area.
to have a bowel movement
Problems with bladder management may include:
• cannot ask for help to the bathroom
• Urinary retention (an inability to void or
• cannot walk to the bathroom
pass urine)
• cannot eat enough food with fiber and drink
• Urinary incontinence
enough fluids
• Increased urgency to urinate
• cannot plan ahead and allow enough time to
get to the bathroom • Increased frequency of urination
To maintain optimal bowel function, a person with • Incomplete emptying of the bladder
brain injury should eat at regular times, focus on
• Bladder infections
eating foods with fiber, drink the amount of fluids
recommended by the dietitian or physician, and be • Skin problems because of incontinence
as active as possible. Meeting with a dietitian to If the person cannot sense the need to urinate, other
discuss a diet plan may be helpful. The person also approaches to bladder management are considered,
may be asked to follow a bowel care schedule, including:
which includes attempting to schedule a bowel
movement at the same time daily and establishing • Keeping the indwelling catheter in the
regular times for meals. bladder

At certain stages of recovery, other methods for • Intermittent catheterization (inserting


bowel emptying (fiber supplements, stool softeners, and removing a catheter several times a
suppositories, and/or laxatives) may need to be day to regularly empty the bladder)
used. However these methods are not used • Scheduling attempts at urination
regularly because they decrease the colon's natural
• Using an external condom catheter for
abilities, and these methods may be habit forming.
men
Most individuals with brain injury regain the ability
to regularly and effectively empty their bowels. • Using an adult diaper
• Other methods of bladder retraining to
Bladder management
control urination may be recommended
People with brain injury also may have a problem
with urination (bladder emptying) during the post- To maintain optimal bladder function, a person
injury period. TBI may result in the inability to: with brain injury should drink fluids as
recommended by the dietitian or physician. The
• Ask for help majority of individuals with brain injury regain the
• Control urination ability to regularly and effectively empty their
bladder.
• Recall when last urination occurred
200 Neuro-Rehabilitation : A multi disciplinary approach

Intervention Addressing Cognitive Intervention Addressing Areas of Occupation


Impairments As the client emerges from the period of agitation,
Interdisciplinary and comprehensive cognitive occupational therapy intervention can begin to
rehabilitation is an integral component of focus on the client's performance of more client-
intervention with individuals who have sustained centered areas of occupation, including ADLs (e.g.,
TBI. Even in those clients with good physical and feeding, dressing, grooming, toileting, bathing,
medical recoveries, cognitive impairments in the transfers) and instrumental activities of daily living
area of attention, memory, and executive functions (IADLs) (e.g., meal preparation, shopping, financial
can limit the person's ability to engage in functional, and home management, child rearing, caring for
social, and vocational activities. As the client pets). The occupational therapist may delay
emerges from the agitated phase of recovery and focusing on some areas of occupation (e.g., work,
can more actively engage in the rehabilitative education, leisure) until the client is transitioning
process, the occupational therapist begins to back to his or her community.
address cognitive impairments and the resulting Intervention approaches using adaptations to the
limitations to functional activity performance and task or environment require repetitive practice of
social participation more formally. the task and caregiver support and education on
how to structure tasks and the environment for
Cognitive Rehabilitation Approaches
optimal performance. Although some adaptations
Occupational therapists select cognitive to the environment may be fixed (e.g., a door alarm
rehabilitation intervention approaches that focus on to prevent wandering or color-coded labels on the
the individual, the task, or the environment, on the inside or outside of drawers and closets to reduce
basis of an understanding of the client's ability to memory demands), other modifications depend
learn and generalize information. When there is upon the consistency and reliability of another
potential for change in the client's underlying person (e.g., filling and programming an alarmed
cognitive impairments, the occupational therapist pill box to aid the client in following a medication
uses a remedial approach focused on improving
schedule). Throughout intervention focused on
and restoring the client's attention, memory, or
modifying and adapting tasks and the environment,
executive function skills.
the occupational therapist is acutely aware that
Functional approaches, which capitalize on the acceptance of these interventions requires the client
client's strengths and abilities, shift the focus of to accept a new vision of himself or herself and a
intervention from restoring underlying willingness to accept change to the pattern or
impairments to minimizing their limitations on conditions of how he or she performs activities.
engagement in activities and participation within
the community. Therapists may modify the client's If the individual with TBI demonstrates potential
environment (e.g., placing cue cards or signs in key for improvement in underlying cognitive and motor
locations, labeling closets or drawers to identify impairments, shows awareness of current
their contents) or modify the activity (e.g., pre- limitations, and shows the ability to alter
selecting and pre-arranging items needed for performance when provided cues and feedback, the
completion of the task or presenting items for only occupational therapist may choose to focus
one step of a task at a time). For clients with limited intervention on restoring underlying cognitive and
ability to learn and generalize information, a motor impairments that contribute to difficulties in
functional skill training program incorporating the performance of functional tasks. Addressing
vanishing cues and errorless learning in the natural executive-planning skills such as organization by
environment may be used. developing cognitive strategies (e.g., checklists, self-
monitoring strategies), occupational therapists may
Capitalizing on procedural memory, occupational
focus intervention on addressing underlying
therapists may teach the client to perform specific
impairments to restore ability to enable more
activities (e.g., completing morning hygiene,
consistent, independent occupational performance.
preparing simple snacks, following a medication
Application of cognitive strategies will be practiced
schedule) that would decrease caregiver burden
in performance of a variety of ADLs and IADLs
and provide some level of independence. Clients
and individual and group intervention sessions to
are trained on the same activity that they are
encourage generalization of the strategy to multiple
expected to perform in the same environment in
areas of occupation.
which it would be performed.)
Head Injury 201

A comprehensive occupational therapy safety risks resulting from motor and cognitive-
intervention program addressing ADLs and IADLs behavioral impairments; possible adaptive devices
considers multiple parameters that contribute to and compensatory techniques to improve
successful performance, including familiarity of the performance; team and family support for
environment and the items used; the client's typical implementation of the selected approaches; and the
performance patterns (i.e., habits and routines); client's ability to monitor and correct performance.

Intervention During the Community Recovery Phase


Intervention Approaches Commonly Used During the Community Phase of Recovery (Rancho Los
Amigos Levels VII-X) Interventions typically focus on higher cognitive skills and WHO ICF areas of
activity limitations and participation restrictions.
Intervention Approach Occupational Therapy Treatment Activities

Prevent • Prevent development of substance abuse and depression by educating


the client about the risks and developing healthy alternative coping
strategies.
• Prevent client injury by modifying the home environment to decrease
safety risks (e.g., installing grab bars and raised toilet seats, removing
throw rugs and potential obstacles, installing automatic turn-off switches
for stove burners and safety locks on cabinets).

Establish/Restore • Restore cognitive and social communication skills by having the client
plan and complete a community outing with family and friends; practice
social pragmatics in group activities and role-playing.
• Establish daily routines to enable the client to complete desired morning
rituals in a timely manner and prevent late arrival at work or school.
• Restore joint mobility and motor function after surgical excision of
heterotopic ossification or botulism toxin injections for muscle spasticity.
• Work with local brain injury support group to establish leisure skill
program to increase social networks for community-dwelling individuals
with TBI.

Modify • Modify home and community environments to support independent


performance of activities.

• Modify daily routines to plan physically and cognitively challenging


activities when well rested (e.g., pay bills in the morning when well rested;
perform activities requiring fine motor demands when muscles are not
fatigued).
• Modify community mobility to accommodate for nondriving status.

Create/Promote • Promote a healthy lifestyle that includes engagement in occupations that


support physical and mental health (e.g., develop exercise program, create
list of healthy food and meal selections, identify healthy social activities
to foster social relationships.

Maintain • Maintain gains made in ROM achieved by serial casting by wearing resting
elbow splint for several hours per day.
• Maintain social support systems in the community by engaging in leisure
activities with friends.
202 Neuro-Rehabilitation : A multi disciplinary approach

Note: Rancho Los Amigos levels taken from Hagen, Often, major adjustments are best made in small
C. (1998). The Rancho Los Amigos Levels of steps. Simple changes may help the person with
Cognitive Functioning: The revised levels (3rd ed.). brain injury, family and friends to find more
Downey, CA: Los Amigos Research and enjoyment in their relationships and activities.B y
Educational Institute. taking each stressful situation one step at a time,
the person with brain injury and family may feel
Adjustment to the variety of physical, cognitive, and
that life is becoming a little more "normal" again.
neurobehavioral impairments resulting from TBI
require functional coping skills from both the client
Self-esteem
and family. The ability to maintain existing
friendships and develop new friendships is Self-esteem is a person's assessment of self-worth
challenged when a person experiences a TBI. that is often adversely affected by brain injury. The
Impairments in cognitive and social skills, as well greater the self awareness the person with brain
as limitations in the ability to engage in shared injury has, the more likely are negative changes in
occupations, can result in the distancing of friends self-esteem. To counter the negative self worth felt
from the individual who sustained the injury. by the client, the therapist needs to:

Occupational therapy practitioners begin • constantly make the client express their
addressing social skills during inpatient feelings and focus on the positives.
rehabilitation, but often these impairments in social • redirect conversation to positive or neutral
skills become more evident when the individual thoughts.
with TBI is discharged home to the community and
• Express their concern and desire to understand
reassumes social roles. In the community setting,
the person's feelings.
the occupational therapy practitioners may conduct
social skills training groups to address cognitive • Point out the person's successes, even partial
components of social interaction, the pragmatics of successes.
social conversation, and tasks involved in
• Encourage as much independence as possible.
developing and maintaining friendships and
relationships. Techniques such as goal setting, • Give caring, realistic feedback.
individualized written contracts, role playing and • Help the person plan ahead to maximize
rehearsal, peer mentoring and role modeling, and opportunities for success.
videotaping social interactions with self-reflection
and supportive feedback may be used in individual • Choose activities and tasks that the person can
and group sessions. successfully complete.

Family-focused intervention may help the family Brain injury support group are vital links to
unit manage the cognitive and neurobehavioral education, life planning, and emotional support for
symptoms of their member with TBI upon return clients and families. These community groups offer
home to the community. information on life planning and real-world
problem solving. They also may offer leisure and
Intervention Addressing Social and Coping social networking opportunities in groups where
Skills the client's neurobehavioral problems are more
easily understood and accepted. Occupational
Adjustment to the variety of physical, cognitive, and
therapists encourage clients and their families to
neurobehavioral impairments resulting from TBI
connect to local brain injury support groups and
require functional coping skills from both the client
may work with the groups to provide educational
and families. Family members and others close to
information sessions or develop programming to
a person with brain injury may struggle to cope
address leisure and social needs of the members.
with behavioral changes caused by the brain injury.
The injured person also may struggle to adjust. Occupational therapy practitioners begin
Family members and others close to the person may addressing social skills during inpatient
feel stressed, burdened, even depressed by the rehabilitation, but often these impairments in social
major changes in activities, responsibilities, daily skills become more evident when the individual
schedules, leisure and support that are required to with TBI is discharged home to the community and
adjust to the consequences of acquired brain injury. reassumes social roles. In the community setting,
the occupational therapy practitioners may conduct
Head Injury 203

social skills training groups to address cognitive rehabilitation psychologists and social workers, can
components of social interaction, the pragmatics of be consulted to provide help to both the client and
social conversation, and tasks involved in to the immediate caretakers.
developing and maintaining friendships and
Family-focused intervention may help the family
relationships. Techniques such as goal setting,
unit manage the cognitive and neurobehavioral
individualized written contracts, role playing and
symptoms of their member with TBI upon return
rehearsal, peer mentoring and role modeling, and
home to the community.
videotaping social interactions with self-reflection
and supportive feedback may be used in individual Care for the Caretaker
and group sessions.
Providing companionship and emotional support
Individuals with TBI and impaired coping skills can for the person with a brain injury may be necessary,
show signs of depression and poorer outcomes. in addition to physical care. Caregivers also may
When impaired coping skills are coupled with have many other responsibilities, including
neurobehavioral symptoms such as impulsivity, the employment outside the home and caring for the
person with TBI may be at greater risk for alcohol home and children. Being a caregiver can be
and drug abuse. overwhelming, and adapting to these changes is
challenging. The OT needs to prepare the primary
Depression caretakers regarding the varied emotional and
As confusion decreases and self awareness physical challenges arising due to the differences
improves and as the person struggles to adjust to a and changes in circumstances in both the short term
temporary or lasting disability caused by TBI, he and over the long term and the ways to cope with
or she may suffer from depression. This might also the same: The occupational therapist can provide
occur if the injury has affected areas of the brain the following suggestions:
that control emotions. Depression is an illness (and
• Ask for help when needed: Caregivers
not a sign of weakness, nor is it anyone's fault)
frequently try to handle everything alone.
caused by biochemical and structural changes in
Expecting too much of oneself may add to the
the brain. Some of the symptoms of depression are:
stress. The therapist can provide suitable
• Persistent sadness options for assistance such as home health care
or respite care.
• Irritability, moodiness
• Set limits. There are only so many hours in the
• Anxiety
day and only so many things a person can do.
• Loss of interest or pleasure in life Its important to priortise and understand that
• Neglect of personal responsibilities or personal some things can wait.
care • the caretaker should plan something to look
• Changes in eating habits or sleeping patterns forward to each day for both the client and
self.
• Fatigue, loss of energy, lack of motivation
• Take time away from the person being taken
• Extreme mood changes care for. Taking an hour, a day, a weekend or
• Feeling helpless, worthless or hopeless a week away can do wonders to restore
emotional well being.
• Physical symptoms such as headaches or
chronic pain that do not improve • Maintain contact with friends and family to
discuss concerns or have fun.
• Withdrawal from others
• Take care of self. Caregivers are vulnerable to
• Thoughts of death or suicide
stress-related illnesses. Caretakers should also
Fortunately, medication and other therapies can try to exercise since it increases stamina,
help most people who have depression. Effective lessens anxiety and depression, improves or
treatments are available, including individual and maintains muscle tone and strength, and
group therapy, medication or a combination. Early increases self confidence. These benefits make
treatment can help prevent needless suffering. exercise a worthwhile use of limited time.
Mental health professionals, including
• Learn relaxation techniques such as breathing
204 Neuro-Rehabilitation : A multi disciplinary approach

exercises, meditation or progressive muscle • Feeling good about ourselves


relaxation.
• Being part of a group
• Join a support group. Support groups are an
• Competing with ourselves and with others
outlet for sharing problems and concerns.
People with similar issues can not only • Experiencing success
empathise but also provide valuable • Laughing and having fun (reducing stress)
suggestions on varied issues and coping
techniques. • Developing useful skills
• Developing friendships
Sexuality
• Strengthening social relationships
Love, affection and sexual feelings are healthy
human desires. If these desires are not understood Participation in meaningful leisure experiences is
or expressed, confusion, distress and feelings of essential to the recovery of the person with a brain
inadequacy may result. Sexuality involves the injury. Most people naturally select certain activities
expression of male or female identities through that they enjoy and help them meet some basic
sexual actions, attitudes and behavior in needs. People with brain injuries may face barriers
relationships. The ability to appropriately express to experiencing this kind of fulfillment, including:
these learned behaviors may be lost after a brain • Attitude (some people may not realize the
injury. Further, the adult with brain injury may not importance of leisure activities)
understand when it is appropriate to kiss, hug and
touch. These changes in the client, who may act • Physical disability (they may no longer be able
differently after the injury can cause feelings of to enjoy the activities they once did)
confusion, anger, fear, frustration and helplessness • Lack of cognitive skills (skills needed to
in the family members. participate in some activities - attention,
Addressing relationships between client and family concentration, initiation, planning, problem
members should be done early in the rehabilitation solving - may be impaired)
process. The occupational therapist should • Interruption of social and/or language skills
empathise that it might be difficult for the client
and or care takers to discuss sexual matters. The • Lack of knowledge (some may not know how
goals of rehabilitation for the person with brain to engage in certain leisure activities or how
injury include independence, self-reliance and to adapt them so they can participate)
healthy personal relationships. Occupational Therapist can help by:
The ability to develop and maintain social • Helping the person identify leisure interests
relationships may be the most important measure
• Assisting with structuring time and daily
of successful rehabilitation. Therefore, recognizing
schedules so that leisure balances with
and discussing concerns about love and sex are
necessary tasks and activities
important. The closest family member of a person
with brain injury is usually the most effective • Planning ahead for recreation to keep life
person to help the injured person relearn how to interesting
express sexual feelings appropriately. This family • Investigating community resources (city
member may benefit from professional support and parks/recreation departments,libraries,
guidance in addressing sexual issues. It might have churches, and other avenues for leisure
to be clarified that a person with brain injury might options)
not recognize sexual cues, and hence not respond
to a partner or would not initiate sexual activity. Intervention Addressing Occupational
Social workers, rehabilitation nurses and Performance
rehabilitation psychologists are resources for
Although acute inpatient rehabilitation typically
support in addressing sexual issues and concerns.
focuses on the performance of ADLs, individuals
Recreation and Leisure more than 10 years after their brain injury can show
clinically significant improvements in functional
Through leisure, basic human needs are met, skills when engaged in rehabilitation programs
including:
Head Injury 205

focused on retraining specific skills or training of teachers provide such accommodations. Some
new skills previously not part of the client's roles. common accommodations that may assist a person
Using elements of procedural learning in a natural with brain injury to learn in school are:
environment with no expectations for
• Extra time for tests to compensate for thinking
generalization or improvement in cognitive
or information processing that may be slower
functioning, an occupational therapist may develop
a program that incorporates errorless learning, • Taking tests privately in a distraction-free
practice of a specific task with fading cues, positive environment to accommodate for difficulties
prompts, and praise and encouragement. with attention, concentration and increased
distractibility
Occupational therapists also may need to assist the
client and family in adapting strategies taught • Placement in classrooms with less noise and
during acute inpatient hospitalization to sustain the distractions
same level of independence within the home • Tape recording lectures to compensate for
environment. The natural cues offered by the attention, concentration and memory
familiar home and community environments may problems
support greater independence, but these
environments also challenge cognitive and physical • Access to teachers' or peers' class notes to
skills due to their unpredictable nature. compensate for difficulty in dividing attention
between listening to a lecture and taking notes
Intervention Addressing Education and Work • Tutoring with a peer or professional tutor
Activities
Cell phones, smart phones, and other forms of
School reintegration and vocational rehabilitation
personal digital assistants can be used by clients
are important aspects of community recovery for
with cognitive issues to remember essential
clients with TBI.
information, navigate daily tasks, be reminded of
For children and teenagers, returning to school is appointments or times to take medication, and stay
important for both social and educational growth. focused on treatment goals. Counselors can send
At school, young people find friends and peer the client text messages or voice reminders (these
support, develop social skills, and increase their can be automated), and they can help the client
knowledge. School also provides a place to monitor program the devices to provide timer beeps, visual
young children's intellectual and social growth. cues, maps, or other cognitive aids.
Sometimes the effects of a brain injury are not
Work is defined as productive activity. It plays a
apparent in young children but become more
major role in the lives of most people. Work may
apparent in later years when the thinking and social provide many benefits such as a sense of
demands at school increase.
achievement, recognition, responsibility, financial
Clients who wish to return to academic studies need independence, social interaction and structure. A
to practice strategies that will support success in brain injury can cause many changes in behavior,
the student role. Occupational therapists may create emotions and thinking skills. This can make it
simulated classroom instructional sessions for the difficult to keep a job, even if it was the same job
client to practice taking notes and processing held before the injury. Regardless of whether or not
complex information, and review study habits and the person with the brain injury returns to work,
test-taking strategies. Cognitive orthotics such as discovering how to use his or her talents to the best
personal digital assistants (PDAs), portable tape of his or her ability will make his or her life more
recorders, alarm watches, and laptop computers rewarding. Those who return to work after brain
with scheduling software may be explored for their injury are generally healthier and have a higher self-
ability to compensate for residual cognitive esteem than those who do not work.
impairments.
Returning to work after a brain injury depends on
For those returning to high school and college, a number of factors:
developing specific accommodations can help the
• Availability of jobs
person with brain injury to be successful in school.
In most colleges, an office for students with • Health
disabilities assists in assuring that individual • Desire to work
206 Neuro-Rehabilitation : A multi disciplinary approach

• Physical abilities in a safe although virtual environment. Clients who


perform well in clinic-based and behind-the-wheel
• Ability to adjust to changes
or on-road driving assessments typically participate
• Social and behavioral abilities (for example, in a trial of driver's training to practice and reinforce
ability to control behaviors and get along with safe driving behaviors in gradually more
co-workers) challenging situations. Periodic follow-up on
• Thinking and problem-solving abilities driving skills may be warranted.

• Self-awareness of deficits and limitations For clients unable to resume independent driving,
occupational therapists can provide intervention in
• Vocational interests and capabilities use of alternatives for community mobility,
• Willingness to receive further training including community-based transportation services
for people with disabilities, taxi services, and public
• The willingness of an employer to adapt the
transportation systems.
job or workplace
Returning to work after a brain injury can be Intervention Review
challenging and rewarding. Case-coordinated early Intervention review is a continuous process of
intervention focused on vocational skills can reduce reevaluating and reviewing the intervention plan,
unemployment among clients with TBI. the effectiveness of its delivery, progress toward
Occupational therapists' unique ability to analyze targeted outcomes, and the need for future
task demands and environmental conditions and occupational therapy and referrals to other r
match these to the client's capabilities makes them professionals. Reevaluation may involve re-
well qualified to address vocational issues in administering assessments used at the time of initial
individuals with TBI. Occupational therapists can evaluation, a satisfaction questionnaire completed
provide job coaching, instruction, and education in by the client, or questions that evaluate each goal.
safe work practices. They also may recommend Reevaluation normally substantiates progress
modifications to job tasks, work hours, or work toward goal attainment, indicates any change in
positions or may recommend specialized functional status, and directs modification of the
equipment or cognitive orthoses that enable intervention plan, if necessary. Because recovery
efficient and accurate job performance. Those from TBI involves multiple stages of client
clients with TBI who are able to return to work functioning and lengthy intervention, it is important
activities may need additional coordinated for occupational therapists to periodically review
interventions and support to sustain their work the intervention plan to determine whether it
status. reflects the client or family's current priorities,
incorporates intervention approaches that meet
Intervention Addressing Community Mobility those needs, and integrates current available
When the client is discharged to his or her home, evidence.
issues of community mobility, and driving
specifically, should be addressed with the client and Discharge Planning, and Follow-Up
family. Some occupational therapists specialize in When clients with TBI are discharged from
driver rehabilitation and can assist the client and structured inpatient rehabilitation programs to their
family in determining if and when a return to homes and communities, the true extent of their
driving for community mobility is possible. Driving limitations may be revealed, often at a time when
assessments should investigate the client's driving their financial and supportive resources are
skills considering performance in clinic-based depleted. Occupational therapists' strength in
assessments as well as on-road evaluations to analyzing and adapting functional tasks can be of
determine fitness to drive. great assistance in helping clients with TBI living
Holistic, intensive, and multidisciplinary in the community in resuming meaningful roles and
neurorehabilitation can help individuals with TBI occupations.
return to safe driving. Occupational therapists may Conclusion
use driving simulators both to evaluate the client's
judgment, problem solving, and reaction times and Since individuals with TBI present with many
to practice responding to simulated driving events different physical, cognitive, and emotional
impairments, therapists should be well versed in a
Head Injury 207

variety of treatment options and not just one Level III - Localized Response: Total Assistance
approach. No matter which intervention technique • Demonstrates withdrawal or vocalization to
is chosen, an emphasis should be placed on shaping painful stimuli.
the task to the patients abilities cognitive as well as
physical to optimize success while progressively • Turns toward or away from auditory stimuli.
increasing the complexity and demands of the tasks • Blinks when strong light crosses visual field.
and relating the intervention to a meaningful • Follows moving object passed within visual
functional goal. field.
Rancho Los Amigos Scale • Responds to discomfort by pulling tubes or
restraints.
The Ranchos Los Amigos (Revised) Cognitive Scale
is used by many health care teams to can begin • Responds inconsistently to simple commands.
treatment that will develop skills and promote • Responses directly related to type of stimulus.
appropriate behavior. Health care professionals
• May respond to some persons (especially
often suggest the following simple measures to
family and friends) but not to others.
family and friends while the patient is still in coma:
Level IV - Confused/Agitated: Maximal
• Always talk as if the patient hears when you Assistance
are nearby.
• Alert and in heightened state of activity.
• Speak directly to the patient about simple
• Purposeful attempts to remove restraints or
things and frequently reassure them.
tubes or crawl out of bed.
• Explain events and noises in the surrounding
• May perform motor activities such as sitting,
area. Tell the patient what has happened and
reaching and walking but without any
where they are.
apparent purpose or upon another's request.
• Touch and stroke the patient gently. Tell the • Very brief and usually non-purposeful
patient who you are each time you approach moments of sustained alternatives and divided
the bedside. Hold their hand. attention.
• Play the patient's favorite music for them. • Absent short-term memory.
• For parents of young children, tape yourself • May cry out or scream out of proportion to
singing or reading your child's favorite stories. stimulus even after its removal.
• May exhibit aggressive or flight behavior.
Levels of Cognitive Functioning
Level I - No Response: Total Assistance • Mood may swing from euphoric to hostile
with no apparent relationship to
• Complete absence of observable change in environmental events.
behavior when presented visual, auditory,
• Unable to cooperate with treatment efforts.
tactile, proprioceptive, vestibular or painful
stimuli. • Verbalizations are frequently incoherent and/
or inappropriate to activity or environment.
Level II - Generalized Response: Total Assistance
Level V - Confused, Inappropriate Non-Agitated:
• Demonstrates generalized reflex response to
Maximal Assistance
painful stimuli.
• Responds to repeated auditory stimuli with Glasgow Coma Scale
increased or decreased activity. The Glasgow Coma Scale is based on responses to
• Responds to external stimuli with stimuli in three areas: motor, verbal performance
physiological changes generalized, gross body and eye opening. The scale assesses the depth and
movement and/or not purposeful duration of coma and impaired consciousness. The
vocalization. Glasgow Coma Scale helps to gauge the impact of
brain damage caused by traumatic and/or vascular
• Responses noted above may be same
injuries or infections, metabolic disorders, such as
regardless of type and location of stimulation.
hepatic or renal failure, hypoglycemia, or diabetic
• Responses may be significantly delayed. ketosis.
208 Neuro-Rehabilitation : A multi disciplinary approach

Glasgow Coma Scale Eye Opening Response of the brain affected and the severity of it [1]. These
• Spontaneous--open with blinking at baseline; disturbances may affect the patients availability of
4 points social contact, return to work or school and changes
in leisure activities. Patients with traumatic brain
• To verbal stimuli, command, speech; 3 points injury may also undergo personality changes and
• To pain only (not applied to face); 2 points may lack awareness of, or would have difficulty in
adjusting to post-injury outcomes.
• No response; 1 point
Acute Consequences Post Traumatic Brain
Glasgow Coma ScaleVerbal Response
Injury:
• Oriented; 5 points
A brain injury can be assumed as a head trauma, if
• Confused conversation, but able to answer it is accompanied by alteration in consciousness,
questions; 4 points neurological impairments or cognitive be deficits
• Inappropriate words; 3 points and can result from any object striking the head or
the brain after coming in contact with the skull [2].
• Incomprehensible speech; 2 points The injuries caused to the brain may be focal, multi
• No response; 1 point focal or diffuse and can often involve structures
away from the initial site of impact. The severity of
Glasgow Coma Scale Motor Response the initial impact may be predictive of outcomes
• Obeys commands for movement; 6 points depending on the factors such as age, pre-existing
conditions, psychological sequel and other factors
• Purposeful movement to painful stimulus; 5 also impact the long-term outcomes. The outcomes
points involve physical, cognitive and behavioural
• Withdraws in response to pain; 4 points impairments, which may require prolonged
hospitalization and post acute rehabilitation
• Flexion in response to pain (decorticate programmes.
posturing); 3 points
• Extension response in response to pain Consequences of Traumatic Brain Injury:
(decerebrate posturing); 2 points The deficits that arise following a brain injury where
• No response; 1 point physical deficits are visible, socially acceptable and
may very often recover quickly. Cognitive
Categorization impairments, emotional changes and behavioural
problems may be less visible and are more likely to
Coma: No eye opening, no ability to follow
limit the range of a person's activity and impacts
commands, no word verbalizations (3-8)
on the ability to reintegrate into the society. The
deficits are listed below:
Head Injury Classification
Severe Head Injury -- Glasgow Coma Scale score Cognition:
of 8 or less
The commonly affected cognitive functions post
Moderate Head Injury -- Glasgow Coma Scale score traumatic brain injuries are: memory, impairment
of 9 to 12 of attention, visual - spatial abilities and executive
Mild Head Injury -- Glasgow Coma Scale score of functions. These difficulties are compounded by
13 to 15 lack of flexibility in attending, thinking and acting
slowly and inefficiency in processing of
information, difficulty with learning, poorly
PSYCHOLOGICAL ASPECT:
organised behaviour and verbal expressions.
Introduction:
Cognitive functions affected post TBI are as follows:
A person who sustains traumatic brain injury (TBI)
can have dramatic and wide-reaching effects for Memory:
both the person who sustained the injury and his Post brain injury it is often seen that there are mild,
or her caregivers. The effects of impairment include moderate and severe brain injuries due to
physical, cognitive, emotional behavioural and impairment of all processes involved in memory
psychosocial disturbances depending on the areas
Head Injury 209

like encoding, maintenance and retrieval. Memory physical aggression, learning difficulties, shallow
disturbances has a major impact on psychosocial self awareness, altered sexual functioning,
outcomes and has been identified as an important impulsivity and social disinhibition. Mood
predictor of work status, with severe verbal learning disorders, personality changes, egocentricity,
deficits often existing ten to twenty years post the emotional liability, depression, anxiety and
injury. isolation are also prevalent after TBI [5]. Due to the
affected cognitive difficulties, there could be
Attention: problems that a person faces in social interaction.
Attention problems most often include impairment Hoofien and his colleagues (2001) reported that
of arousal, focused attention and divided attention. brain injury survivors and family members
The persons alertness is affected i.e. the person's perceived their social functioning as being the most
ability to focus on a particular stimulus and ignore problematic, as compared to other areas.
other interfering materials if often affected. The
person may experience difficulty to maintain a Emotions and Motivation:
conversation in a noisy setting, or has impairment Post TBI the patients may experience impairments
in reading complex instruction or has difficulty in in emotions and may have adaptive function of
perceiving the intentions and actions of others. providing sensations of comfort or discomfort that
indicate whether a situation is safe or threatening,
Language: while motivation provides their impetus work
Impairments of language functions include deficits towards a desirable goal. Emotions and motivations
in fluency, understanding and naming objects. may be influenced by environmental factors, pre-
Language impairments may lead to talkativeness, morbid characteristics and neurotransmitter
verbal expansiveness, frequent use of words and disturbances. Emotional reactions post injury
phrase and eventually these may lead to emotional would be mostly related to recognition of impaired
and behavioural changes. It is often seen that abilities and a changed self-concept. Apathy, which
language difficulties in people post traumatic brain is closely linked to motivation and emotion, has
injury causes frustration, anxiety and the level of been described as diminished motivation that is not
embarrassment increases. a result of diminished level of consciousness,
cognitive impairment, or emotional distress, and
Visual and Spatial Abilities: in traumatic brain injury can result from disruption
The ability of a person to represent and manipulate to a core circuit involving the anterior cingulum in
spatial information is crucial for a wide range of the prefrontal cortex, and the nucleus accumbens,
perceptual, cognitive and motor functions [3]. ventral pallidum and ventral tegmental areas (
Marin & Chakravorty, 2005).
Executive Functions:
Mental Disorders Post Traumatic Brain Injury:
Lezak (1995) [4] describes executive function as the
ability which enables a person in independent and People with neurological injuries such as traumatic
purposive behaviour. These include higher order brain injury, have elevated risk of developing
skills such as problem solving, abstraction, concept mental health disorders. The symptoms are most
formation, cognitive flexibility and self -regulation. likely to worsen during the first six months post-
Judgement of the patient may be impaired due to trauma which include variability in mood,
difficulties in scanning and assessing relevant depression, emotional withdrawal, agitation/
components of a current situation and in controlling hostility and apathy.
impulsivity. The reduced cognitive ability to
perform complex actions may lead to disturbances 1. Depression:
in initiation and abstract reasoning. Affective disorders are the most common
psychological outcomes post traumatic brain injury.
Emotional and Behavioural Changes: It is found that patients with traumatic brain injury
TBI can cause an impact on the persons emotional, are at "great risk" for developing depressive
behavioural and social functioning which leads to symptoms. The prevalence of major depressive
affecting the way a person behaves in social disorder is between 15.6 percent and 6 percent (Kin
situations. Common behavioural deficits include at al., 2007). Certain factors were significantly
reduced ability to initiate responses, verbal and related to the depression - time after injury, injury
210 Neuro-Rehabilitation : A multi disciplinary approach

severity, and post injury marital status. Therefore, 5. Suicide:


the degree of initial injury, be it mild, moderate, or The association of depression post traumatic brain
severe, can all lead to crippling depression. A injury and suicide has been investigated with an
person with traumatic brain injury may appear emphasis on identification of risk factors. Suicide
depressed, with symptoms of memory dysfunction, risk can continue for a number of years post injury.
slowed movements, apathy, lack of initiation and
blunted emotional expression in the absence of Denial of Deficits:
major depressive disorder. Usually, patients may
Altered self awareness can be due to neurological
feel worthless, helpless, frustrated and demonstrate
and non neurological origin, such as social,
loss of interest in work and family activities.
emotional and motivational factors which can
Reactions to psychosocial changes may have an
interact with organic problems to determine a
impact on prolonged or delayed onset depression.
person's level of self awareness. There can be two
factors which contribute to the person's level of self
2. Anxiety:
awareness:
Individuals with traumatic brain injury may have
reduced ability to adapt to their environment and 1. Organic Denial:
as a result, are less likely to be able to manage
Organic denial can result from damage to certain
anxiety or use it as a signal to indicate potential
areas of the brain which could disrupt the
areas of threat. Another area of concern is social
individual's self - concept and cognition.
phobia identified as the most frequently occurring
Consciousness and self awareness are the highest
phobic disorder, which may be influenced by
attributes of the frontal lobes (Stuss, 1991). Lack of
withdrawal from work and social situations.
awareness due to frontal lobe affection can have
Obsessive compulsive disorder is another symptom
an impact on the rehabilitation activities, as the
which may appear as the person tries to achieve
person will not perceive the need for rehabilitation
maximal control over his or her environment [6]
if he or she does not recognize changes from pre-
with the development of some obsessive
injury abilities.
characteristics as an adaptation to impairment in
the cognitive abilities such as memory and 2. Psychological Denial:
attention. Individuals with TBI may also experience
An emotional and protective response in the face
Post Traumatic Stress Disorder, which is
of increasing emotional distress due to recognition
characterised by sleep disturbances, social
of the disability may lead to denial. This is not
withdrawal, ruminations, and hyperirritability.
necessarily harmful as persons post traumatic brain
3. Aggression: injury should be allowed to maintain their denial
especially if it is not interfering with their
Aggressive behaviours may result as a reaction to
rehabilitation. As, denial may help the person to
irritability or rise of organic factors. These may
pace his or her recovery following trauma by
include damage to the limbic system, poor insight,
reducing excessive amounts of distress, with the
reduced impulse control, or per injury personality
need for denial, which would reduce the person's
traits. Aggression particularly involves physical
sense of self-competence and self-esteem.
assaults, which is especially directed towards the
caregivers. Social Difficulties:
4. Personality Alterations: Individuals with traumatic brain injury experience
a wide range of difficulties in terms of returning to
Individuals post injury may have difficulties with
their prior work situations, particularly those who
frustration, problem solving, self- monitoring,
were previously very successful and were holding
concrete thinking, new learning and memory,
higher positions at work. Emotional changes and
impairment of judgement and poor insight. There
disturbed behaviour were very often serious
could also be presence of inappropriate expression
problems for relatives, while loss of social contact
of affection, irritability, impulsivity and lack of
was the injured person's most disabling handicap
motivation. Premorbid characteristics of the patient
in everyday life, with families and individual's often
may frequently be exaggerated post injury, whereas
becoming isolated. A person's difficulties with
other patients may show drastic alterations.
interpretations and reaction to social situations, and
Head Injury 211

inappropriate content of speech may make social Neuropsychological assessments include sensitive
interactions generally unrewarding for others. Post tests that are used to detect subtle cognitive
the injury there may be decreased opportunities for changes, severity of injury and improvement over
establishing new social contacts and friends, and time. Neuropsychological assessment may make a
engaging in leisure activities, often due to a high contribution to the differential diagnosis of
incidence of people living at home with their neurobehavioral disorders, and the cumulative
families. [7] effect of multiple brain injuries.
Attempting to resume former social and work i. In the acute setting neuropsychological
activities prematurely may lead to failure and consultation and assessment in moderate/
rejections, which in turn may lead to increasing severe brain injury is indicated for:
reliance on family members for support and a
A) Determining emergence from post-
decline in satisfying relationships with peers. As
traumatic amnesia
people with traumatic brain injury become more
isolated, they become more vulnerable to B) Documenting the early course of
psychiatric disability. improvements in attention functioning,
memory, visual-perceptual abilities, and
Family Difficulties: language and executive functions. This
Traumatic Brain Injury affects the patient, caregiver information may be utilized in:
and the family member. Family members often
• Treatment planning and team
provide support and act as a caregiver for a
consultation
prolonged period of time, and it is necessary to
consider their psychological needs as well as those • Family education/support
of the injured persons (Kay & Cavallo, 1994). The • Education and/or psychotherapy
family members may also be in denial post trauma
and they may have an unshakable belief in the ii. During the sub acute phase cognitive/physical
injured person's potential for future recovery, stamina is reduced, availability for testing may
underestimating changes and thinking of the be limited due to medical priorities, and other
person's potential for future recover, rehabilitation commitments.
underestimating changes and thinking for the Selective neuropsychological testing may be
person they remember for before the injury, when indicated to:
they are often faced with different people. Denial
• Identify cognitive strengths and
should be taken care of when it prevents realistic
weaknesses
planning for the future and it may serve as an
adaptive function when it maintains family stability • Provide intervention such as
and role functions. Family members also undergo psychotherapy
depression post the injury and there could be • Educate individual and family about TBI
marital problems also, as the spouse may feel he or • Assess or recommend behavioural
she is with a completely different person altogether management interventions
who has aggressive or childish inappropriate
Based on an analysis of the profile of standardized
behaviour. Divorce may often follow marital
scores and trained clinical observations of the
difficulties associated with traumatic brain injury.
individual's mental processes, evaluation is made
Families may have a prolonged mourning period
regarding:
which may eventually lead to negative feelings
towards the injured person. (1) Structural brain condition,
(2) Deficiencies caused by brain trauma versus
NEUROPSYCHOLOGICAL other conditions such as pain, emotions,
EVALUATION PROCESS: personality, and pre-injury conditions that
contribute to functional status,
Neuropsychological Assessment evaluates the
cognitive processes and behaviours, using (3) Strengths in cognitive and psychosocial skills,
psychological testing to assess the central nervous (4) Comprehensive diagnostic understanding of
system function and to diagnose specific the physiological, psychological, and cognitive
behavioural or cognitive deficits or disorders. impact of the injury,
212 Neuro-Rehabilitation : A multi disciplinary approach

(5) Extent of injury and prognosis for recovery, patterns of memory performance have been found
useful in discriminating among clinical groups with
(6) Specific treatment needs along with
cerebral dysfunction or functional disorders
identification of barriers to and assets for
resulting from various neuropathological or
recovery,
psychological processes.
(7) Objectively-based prognosis for return to
work, school, and other activities, 5. MMPI-2:
(8) Foundation for life-care planning. The MMPI-2 consists of 567 items and is a self-
administered standardized questionnaire. The
Psychological Assessments Tools for Traumatic MMPI-2 elicits a wide range of self-descriptions
Brain Injury: scored to give a quantitative measurement of an
The below listed tests are used to assess the individual's level of emotional adjustment and
cognitive deficits, behavioural changes and attitude toward test taking. There are 10 major
emotional disturbances in individuals with scales, four validity measures and several
traumatic brain injury. These tests are conducted, supplementary measures. The contents for the
scored and interpreted by psychologists or majority of the MMPI-2 questions are relatively
neuropsychologists. obvious and deal largely with psychiatric,
psychological, neurological and physical
1. Mini Mental Status Exam (MMSE): symptoms. The MMPI-2 has direct relevance to
forensic applications and includes the test's ability
The MMSE was developed to standardize and
to measure various symptoms of psychopathology.
quantify the examination of an individual's
cognitive state. It is used to screen for cognitive
6. Trail Making Test:
impairment and to follow individual's progress
over time. The TMT9 is a measure of attention, speed, and
mental flexibility. It is brief, widely used by
2. STROOP Neuropsychological Screening neuropsychologists, sensitive to TBI-associated
Test: cognitive impairment, and reliable.
The STROOP NST is a test developed to predict the 7. Satisfaction With Life Scale:
probability of an individual having organic
The SWLS is a global measure of life satisfaction.
impairment. It measures what is known as "higher
The SWLS consists of 5 items that are completed
executive function". The STROOP is a neurological
screening test developed to predict the probability by the subject.
of an individual having brain damage.
8. Becks Depression Inventory (BBDI - II):
3. Weschler Adult Intelligence Scale - III: This is a 21-question multiple-choice self-report
inventory, one of the most widely used instruments
The WAIS-III is a standardized intelligence test
for measuring the severity of depression. Each
which measures 15 different aspects of cognition
answer is scored on a scale value of 0 to 3. The cut-
and gives three different IQs: Verbal, Performance
offs used differ from:
and Full Scale. The WAIS - III also offers four
additional measures of cognition by analyzing 0-13: minimal depression;
verbal comprehension, performance organization, 14-19: mild depression;
working memory and processing speed. The WAIS- 20-28: moderate depression;
III's use as a screening device for neuro- 29-63: severe depression.
psychological impairment is well documented.
Higher total scores indicate more severe depressive
symptoms.
4. Weschler Memory Scales (WMS-III
Abbreviated): 9. Stress: The Depression Anxiety Stress
The Wechsler Memory Scale-Third Edition Scales (DASS):
Abbreviated is a reliable survey of auditory and
This [8] is made up of 42 self report items to be
visual memory abilities. The WMS-III contains four
completed over five to ten minutes, each reflecting
subtests measuring auditory and visual immediate
a negative emotional symptom. [9] Each of these is
and delayed memory. This provides differences in
rated on a four-point Likert scale of frequency.
Head Injury 213

These scores ranged from 0, meaning that the client of external methods such as cue cards, watch
believed the item "did not apply to them at all", to alarms, diaries, address books and computers to
3 meaning that the client considered the item to record notes, thoughts and other data, can be used
"apply to them very much, or most of the time". It to address a number of cognitive deficits.
is also stressed in the instructions that there are no
Internal strategies may involve self-instructional
right or wrong answers.
routines that regulate behaviours using the inner
speech. Self- instructional routines can help in
10. Quality Of Life Questionnaire: The SF-36:
memory training and attentional deficits by helping
This is a multi-purpose, short-form health survey to maintain focus on specific tasks. Self instructional
with only 36 questions. It yields an 8-scale profile techniques can help with initiating actions, planning
of functional health and well-being scores as well and problem solving to patients who have executive
as psychometrically-based physical and mental function deficits due to which they demonstrate
health summary measures and a preference-based inappropriate social and work behaviour,
health utility index. particularly when they are in environment or
engaged in activities that are not routine. Patients
PSYCHOLOGICAL should learn to apply compensatory strategies in
REHABILITATION POST functional situations when they are suffering from
TRAUMATIC BRAIN INJURY: cognitive impairments as this is an important part
of cognitive rehabilitation.
Goals:
Behavioural Rehabilitation:
The goals for individuals post traumatic brain
injury are to improve the person's ability to function As the focus for individuals with traumatic brain
by enhancing his or her capacity to organise the injury is physical recovery, with less emphasis of
daily activities, to attend to and process information their ability to adapt to social behaviour and
and to interact with others in a socially appropriate changing situations. However, even after significant
manner. It would basically focus on improving the changes in the physical functions it is very difficult
overall quality of life of the patient and the to reintegrate the individuals into the community
caregivers. This could be done with the help of due to the behavioural and emotional changes.
clinical psychologists and neuropsychologists who Behaviour Modification techniques could be very
would perform assessments and depending on the helpful for patients with traumatic brain injury as
results of the tests would make recommendations the focus of this technique would be to address the
for future rehabilitation programmes. inappropriate social behaviour, attention and
Neuropsychological assessment is necessary prior motivation, lack of awareness, memory, language
to implementation of cognitive remediation, in and motor disturbances (McGlynn, 1990).
order to provide a person's areas of relative strength Professionals should come up with an
and weakness. The psychologists on the other hand individualised plan for the patient by first assessing
would provide psychotherapy services to people the behavioural issues and then apply strategies to
with traumatic brain injury and their families [10]. increase or decrease particular behaviours.

Behavioural and Cognitive Behavioural


Cognitive Rehabilitation:
Interventions:
The main aim of cognitive rehabilitation is to
Behavioural approaches can include behaviour
improve the individual's functional and integrative
modification techniques and skills' training that
performance, where this is impaired by the
addresses personality and behavioural effects from
individual's cognitive deficits. Restorative training
traumatic brain injury. These techniques may also
focuses on improving a specific cognitive function,
be incorporated within a Cognitive Behavioural
whereas compensatory training focuses on
Therapy (CBT) approach. The cognitive approaches
adapting to a deficit [11]. First the
to psychopathology regards the processing of
neuropsychologists assess the cognitive strengths
external events as biased which distorts the
and weakness and then the patient is provided with
individual's evaluation of experience which leads
learning compensation techniques or new ways of
to a variety of cognitive errors, for example, over-
doing things.
generalisation, selective abstraction and
Complementary techniques may focus on the use personalisation(Beck, 2005).
214 Neuro-Rehabilitation : A multi disciplinary approach

The main aim of cognitive behaviour technique is • Arranging things needed in one
to identify the underlying distorted beliefs that have cupboard.
been incorporated into enduring schemas or core
• Labelling, the cupboards and jars.
belief systems. The psychologist records the
patient's negative automatic thought patterns and • Setting up filling systems
conditional core beliefs. Behavioural techniques can • Getting rid of distractions.
be used in the early stages of therapy as some people
with brain injury often operate at a very concrete Managing Memory Difficulties:
level (Cicerone, 1989; Khan -Bourne & Brown, 2003),
Memory functions on the coordination of a number
while cognitive techniques help to identify the
of processes i.e. attention, encoding, storage and
person's beliefs about the current situations and
retrieval.
abilities. CBT aims to break the negative cycles that
maintain depression following the brain injury, by Strategies for Memory Difficulties: (Further
identifying the maladaptive strategies, and explained in psychological chapter).
promoting more adaptive behaviours. Post TBI, • Learning more effectively
there is a reduced control over mood shifts and
impulsive behaviour and this may have a negative • Making use of mnemonics
impact on a person's sexuality. CBT provides in • Making use of external aids like alarms,
individual therapy or group therapy to improve the tags, memory diaries, organisers, etc.
sexual and interpersonal functioning.
Managing Aggression and Irritability:
Managing Attention Difficulties: A common complaint following TBI of patients
An option to manage attention difficulties would would be the inability of the patients would be their
be to use compensatory strategies and reduced ability to manage frustration, anger and
environmental support. aggression. The psychologist working with the
patient should record his level or irritability,
The following strategies are very helpful: frustration, anger and aggression. Along with this
a note should be made as to what are the situations
1. Orienting procedure:
in which the person gets angry and the severity of
This involves monitoring an activity by checking responses in such situations.
what is involved at each step. For example things
needed to make a sandwich, listing down the items Anger management strategy:
needed to make a sandwich, the procedure used to A - Anticipate the trigger situations
make a sandwich and eventually checking it. N - Notice the signs of the rising anger.
G - Go through your rising temper by using
2. Pacing:
relaxation techniques.
As there is mental fatigue while maintaining
E - Extract yourself from the situation, if
attention for a prolonged period of time, the pacing
everything fails.
strategy suggests that one needs to develop a
realistic expectation about what can be achieved R - Record how you coped.
over a period of time.
ASPECTS OF COGNITION AND
3. Keeping Notes: PHASES OF IMPROVEMENT POST
Patients can learn to jot down the key questions or TRAUMATIC BRAIN INJURY: [12]
ideas that come to mind which can be useful to solve
a problem; it's very difficult for patient with TBI to Aspects of Cognition:
switch between tasks.
1. Attention:
4. Changing the surroundings: This is in terms of holding objects, events, words
Making a surrounding at home or workplace which or thoughts in consciousness. Cognitive
would be easy accessible: For example: components related to attention are attention span,
filtering, selectivity, filtering, maintaining, shifting
• Getting rid of clutter
and dividing.
Head Injury 215

Early Phase: During the early phase of Traumatic Late Phase: During the Late phase of Traumatic
Brain Injury: Brain Injury:
• Patient shows severely decreased arousal or • Patients could undergo subtle versions of
alertness. perceptual problems related to complexity,
rate and amount.
• Patient has minimal selective attention and has
difficulty in shifting his focus. • Patients could have inefficient shifting of
perceptual set.
• Attention would be present primarily due to
internal stimuli. • Patients usually have weak perception of
relevant feature.
Middle Phase: During the middle phase of
Traumatic Brain Injury:
3. Memory and Learning:
• Attention is generally focused on external This consists of recognition, interpretation and
events formulation of information including internal code.
• The patients attention span is short Coding is affected by knowledge, personal interests
and goals.
• Patient finds great difficulty in fixing his
attention and is usually highly distracted. Early Phase: During the Early phase of Traumatic
Brain Injury:
Late Phase: During the late phase of Traumatic
Brain Injury: • There is progression in comprehension from
minimal responses to vocal modulation and
• Patient's attention span is much reduced
stress to recognition of simple, context bound
• Concentration is relatively weak, attention is instructions.
selective and there are fluid attention shifts.
• There is however no evidence of encoding or
• The patient finds difficulty organizing things storage of new information.
or thoughts and there is absence of goal.
Middle Phase: During the Middle phase of
Traumatic Brain Injury:
2. Perception:
Perception is recognizing features and being able • Patients undergo weak encoding due to poor
to find relationship between events, thing, etc. access to knowledge base, poor integration of
Where the aspects which are taken into new with old information, or inefficient
consideration while perceiving a situation or object attention or perception.
is intensity, duration, significance, context and • Inefficiently encoded information is often lost
familiarity of the stimuli. after short delay.
Early Phase: During the early phase of Traumatic • Recognition is stronger than recall i.e.
Brain Injury: receptive vocabulary is superior to expressive
• Patient begins to recognize and use familiar vocabulary.
objects. • Patients often have difficulty in organized
• When exposed to a stimuli the patient may be search of storage system.
able to recognize only one aspect of the stimuli
Last Phase: During the Last phase of Traumatic
• Patients may start adapting to continuous Brain Injury:
stimulation
• Patients have increase in cognitive stress
Middle Phase: During the Middle phase of depending on the level of cognitive
Traumatic Brain Injury: functioning prior to TBI.
• Patient can clearly recognize familiar objects • Patients experience memory problems related
and events. to recalling information related to personal
• Patients undergo sharp deterioration as there experience or abstracted knowledge.
as an increase in the complexity of stimuli.
• Patients have difficulty in distinguishing
4. Organizing or analyzing ability:
whole form the part. We classify, integrate, and sequentially arrange
216 Neuro-Rehabilitation : A multi disciplinary approach

relevant features of objects and events; comparing • The patients have fair to good concrete
for similarities or differences by integrating into reasoning in controlled setting and
organized description. These processes are disorganized thinking in stressful or
presupposed by higher level reasoning and efficient uncontrolled setting.
learning.
• Patient's abstract thinking is deficient.
Early Phase: During the Early phase of Traumatic
Brain Injury: 6. Problem Solving and Judgement:
• There is no evidence of significant This takes place when a goal cannot be achieved
improvement in this phase. directly. Ideally goals involve identification,
consideration of relevant information, exploration
Middle Phase: During the Middle phase of of possible solutions and selection of the best
Traumatic Brain Injury: solution. Judgement is based on the way a person
• Patients have weak or bizarre association and decides over other options.
analysis of objects into features.
Early Phase: During the Early phase of Traumatic
• Patients have disorganised sequencing of Brain Injury:
events.
• No evidence of substantial improvement in the
• Patients are poor at identifying similarities and
early phase has been seen.
differences in comparisons and classifications.
• Patents can integrate concepts into Middle Phase: During the Middle phase of
propositions but they have difficulty in Traumatic Brain Injury:
integrating propositions into main idea. • The patient finds it difficult to see relationships
Last Phase: During the Last phase of Traumatic among problems, goals and relevant
Brain Injury: information.

• Patients have difficulty in maintaining goal • There is inflexibility in generating or


directed thinking. evaluating possible solutions and impulsive
trial and error approach.
• They easily get lost into details.
• Patients usually have severely impaired safety
• They can organize unstructured stimuli with and social judgement.
prompts.
Last Phase: During the Last phase of Traumatic
5. Reasoning: Brain Injury:
Reasoning means drawing inferences and • Patients are usually impulsive, disorganised
conclusions involving flexible exploration of and have difficulty in problem solving in this
possibilities and making use of past experiences. phase.
There are 3 types of reasoning: deductive, inductive
• They have inflexible thinking and shallow
and analogical reasoning.
reasoning.
First Phase: During the First phase of Traumatic
Brain Injury: 7. Long term Memory:
• There is no major evidence of improvement This contains of knowledge of concepts, words,
in these processes. rules, strategies, procedures and organizational
principles.
Middle Phase: During the Middle phase of
Traumatic Brain Injury: Early Phase: During the Early phase of Traumatic
Brain Injury:
• There is minimal inferential thinking in the
patients which may help deal with concrete • There are emerging evidences of remote
cause and effect relationship. memory, recognition of familiar objects and
persons improvement in patients with TBI in
• There is general inefficiency that the patient this phase.
experiences in abstract ideas and relationships.
Middle Phase: During the Middle phase of
Last Phase: During the Last phase of Traumatic Traumatic Brain Injury:
Brain Injury:
Head Injury 217

• Patients have recognition of strong 5. Chesnut et al., (1998). Evidence report on


associations of basic semantic relations and rehabilitation of persons with traumatic brain
common two or three event sequences. injury. Portland: Oregon Health Sciences
University Evidence- based practice centre.
Last Phase: During the Last phase of Traumatic
Brain Injury: 6. Miller, L. (1991). Psychotherapy of the brain -
injured patient: Principles and Practices.
• Patients undergo variable growth of long term
Cognitive Rehabilitation, 24- 30.
memory, depending on type and severity of
residual cognitive deficits. 7. Mortan, M. V., & Wehman, P. (1995). Review:
Psychosocial and emotional sequelae of
8. Response Systems: individuals with traumatic brain injury: A
This controls all output including speech, facial literature review and recommendations. Brain
expression and fine and gross motor activity Injury, 9 (1), 81 - 92.
including motor planning. 8. Lovibond, S.H.; Lovibond, P.F. (1995). Manual
Early Phase: During the Early phase of Traumatic for the Depression Anxiety Stress
Brain Injury: Scales (2nd ed.). Sydney: Psychology Foundation
• The patients are grossly limited and often have (Available from The Psychology Foundation,
preservative responses. Room 1005 Mathews Building, University of
New South Wales, NSW 2052, Australia.
Middle Phase: During the Middle phase of
Traumatic Brain Injury: 9. Lovibond, P.F.; Lovibond, S.H. (March 1995).
"The structure of negative emotional states:
• The patients during this stage usually speak
Comparison of the Depression Anxiety Stress
or begin with augmentative systems.
Scales (DASS) with the Beck Depression and
Last Phase: During the Last phase of Traumatic Anxiety Inventories".Behaviour Research and
Brain Injury: Therapy 33 (3): 335-343.
Patients in this phase usually have functional 10. Nelson, L.D., & Adams, K.M. (1997).
communication system. Challenges for neuropsychology in the
treatment and rehabilitation of brain injured
REFERENCES: patients, Psychological Assessment, 9 (4), 441
- 456.
1. Karol, R. L. (2003). Neuropsychosocial
Intervention: The Practical Treatment of Severe 11. NIH Consensus Conference, 1999. Consensus
Behavioural Dyscontrol after Acquired Brain Conference: NIH Consensus Conference:
Injury. Florida: CRC press. Rehabilitation of Persons with Traumatic Brain
Injury, JAMA. Paper presented at
2. Gennarelli, T. A., & & Graham, D.I. 2005 &
Rehabilitation person with Traumatic Brain
S.C. Yudofsky (Eds), Textbook of traumatic
Injury.
brain injury (pp. 27 - 50). Washington, DC:
American Psychiatric Publishing, Inc 12. Reprinted/Adapted with permission from
Functioning also related to age and pretrauma
3. McCloskey, M. (2001). Spatial representation
development and educational level. Source?
in mind and brain. In B. Rapp (Ed.), The
SF Szekeres, M Ylvisaker, AL Holland (1985).
handbook of Cognitive Neuropsychology.
Cognitive Rehabilitation Therapy: A
What Deficits Reveal About the Human Mind
Framework for Intervention. In M Ylvisaker
(pp. 101 - 132). Hove: Psychology Press.
(ed), Head Injury Rehabilitation: Children and
4. Lezak, M.D. (1995). Neuropsychological Adolescents. Austin, TX: PROED, 230.
Assessment (3rd ed.). New York: Oxford Copyright 1997 Butterworth Heinemann.
University Press.
218 Neuro-Rehabilitation : A multi disciplinary approach

Ch.5 Autism
Ms. Akshata Shetty, M.A.(Clinical Psychologist), [Link] Mishra, [Link], F.N.R.,
Dr. Manasi Jani (Speech Therapist).

History • Autism Society of America (ASA) defined


autism as: "Autism is a complex
In 1943, Leo Kanner, in his classic paper "Autistic developmental disability that typically
Disturbances of Affective Contact" coined the term appears during the first three years of life and
infantile autism and provided a clear, widespread is the result of a neurological disorder that
account of early childhood syndrome. Kanner affects the normal functioning of the brain
suspected that the syndrome was more frequent impacting the development in the areas of
than it seemed and suggested that some children social interaction and communication skills.
with the disorder had been misclassified as mentally Both children and adults with autism typically
retarded or schizophrenic. Before 1980, children show difficulties in verbal and non-verbal
with pervasive developmental disorder were communication, social interactions and leisure
generally diagnosed with childhood schizophrenia. or play activities. Autism is one of the five
Over time, it became evident that autistic disorder disorders that fall under the Pervasive
and schizophrenia are two distinct psychiatric Developmental Disorder (PDD), a category of
entities. [1 ] neurological disorders characterized by
"severe and pervasive impairment in several
Definition: areas of development".
• The Diagnostic and Statistical Manual of
Mental Disorders, DSM-IV (American
Epidemiology:
Psychiatric Association, 1994) defines autism Prevalence:
spectrum disorders as pervasive
developmental disorders characterized by: Autistic disorder is believed to occur at a rate of
about 8 cases per 10,000 children (0.08 percent) and
– qualitative impairment in social it continues to grow at an alarming rate however,
interaction because of inadequate data, these numbers may
– qualitative impairment in underestimate autisms true prevalence. By
communication definition, the onset of autistic disorder is before
– restricted, repetitive and stereotypic the age of 3 years, although in many cases it noticed
patterns of behaviour, interests and at a much older age. [1]
activities.
Sex Distribution:
It is seen that autistic disorder is four to five times
more common in boys than in girls. However, girls
with autistic disorder are more probable to have
more severe mental retardation as compared to
boys.

Socio economic Status:


Over the past 25 years, no epidemiological studies
have demonstrated an association between autistic
Triad of Impairment in Autism disorder and any socioeconomic status.

• Autism is diagnosed when a child or adult has Etiology and Pathogenesis:


abnormalities in a 'triad' of behavioural
domains: social development, communication, 1. Genetic Factors:
and repetitive behaviour/obsessive interests Current evidence supports a genetic basis for the
(APA, 1994; ICD-10, 1994). development of autistic disorder in most cases.
Family studies have demonstrated a 50 to 200 times
Autism 219

increase in the rate of autism in siblings of an index may be damaged during gestation. [ 1 ]
child with autistic disorder. Studies suggest that
even if the siblings are not affected with autism they 4. Perinatal Factors:
are an increased risk for a variety of developmental A higher than expected incidence of perinatal
disorders which are often related to communication complications seems to occur in infants who are
and social skills. Linkage analyses have later diagnosed with autistic disorder. Maternal
demonstrated that regions of chromosomes 7, 2, 4, bleeding after the first trimester and meconium in
14 and 19 are likely to contribute to the genetic basis
the amniotic fluid has been reported in the histories
of autism.
of autistic children than the normal population. It
The concordance rate of autistic disorder in the two is seen that in the neonatal period, autistic children
largest twin studies was 36 percent in monozygotic have a high incidence of respiratory distress
pairs versus 0 percent in dizygotic pairs in one syndrome and neonatal anemia. Males with autism,
study and about 96 percent in monozygotic pairs as a group have been found to be the products of
versus about 27 percent in dizygotic pairs in the
longer gestational age and were heavier babies than
second study. Approximately 1 percent of children
babies in the general population. On the other hand
with autistic disorder also have fragile X syndrome,
females with autism are more likely to be the
who tend to show gross motor and fine motor
product of post term pregnancies than babies in the
difficulties as well as relatively poorer expressive
language compared with children with autism general population. [1]
without fragile X syndrome.
Recent research was conducted where, the DNA
of more than 150 pairs of siblings with autism was
analyzed and they found extremely strong evidence
that two regions on chromosome 2 and 7 contain
genes involved with autism. The likely locations
for autism - related genes were found on
chromosome 16 and 17, although the strength of
correlation was somewhat weaker. [1 ]

2. Biological Factors:
Approximately 70 percent of children diagnosed
with autistic disorder have mental retardation.
About one third of children with autistic disorder
have mild to moderate and close to half of these Causal Factors of Autism i.e. pre natal and
children are severely or profoundly mentally posy natal effects
retarded. Children with co-morbid autistic disorder
and mental retardation display marked deficits in 5. Neuroanatomical Factors:
social understanding, abstract reasoning and verbal
The neuroanatomical basis of autism remains
tasks than in performance tasks, such as digit recall
unknown; however, recent evidence suggests that
and block design.
enlargement of the gray and white matter cerebral
Of persons with autism, 4 to 32 percent have grand volumes, but not at 2 years of age. It is seen that
mal seizures at sometime and about 20 to 25 percent the head circumference appears normal at birth;
show ventricular enlargement on computed however the head circumference growth appears
tomography (CT) scans. Various to emerge at about 12 months of age. MRI studies,
electroencephalogram (EEG) abnormalities are where the brain volume of the autistic subjects and
found in 10 to 83 percent autistic children and normal controls was compared the results revealed
although no EEG finding is specific in autistic that the brain volume was larger in those with
disorder, there is some indication of failed cerebral autism, however it seen that autistic children with
lateralization. [ 1 ] severe mental retardation generally have smaller
heads. The greatest average percentage increase in
3. Immunological Factors: size occurred in the occipital lobe, parietal lobe and
The lymphocytes of some autistic children react temporal lobe, however no difference was found
with maternal antibodies, which raise the possibility in the frontal lobe. The increased volume can occur
that embryonic neural or extra embryonic tissues from three different possible mechanisms: increased
220 Neuro-Rehabilitation : A multi disciplinary approach

neurogenesis, decreased neuronal death and cortex (OFC), and the superior temporal sulcus and
increased production of non-neuronal brain tissue, gyrus (STG). Together, she called these the "social
such as glial cells or blood vessels. Brain has been brain".
suggested as possible biological marker for autistic
disorder. The amygdale:
The temporal lobe damage is reported to be one of There are four lines of evidence for an amygdala
the critical areas of the brain abnormality in autistic deficit in autism (Baron-Cohen et al; 2000) [4]
disorders as also animal studies reveal that when
the temporal region of the animals is damaged, (a) Post-mortem evidence:
normal social behaviour is lost, there is presence of
A neuroanatomical study of adults with autism at
repetitive motor behaviour and restlessness. Some
post-mortem found microscopic pathology (in the
brains of autistic individuals exhibit a decrease in
cerebellar Purkinje's cells, which is believed to form of increased cell density) in the amygdala, in
account potentially for abnormalities of attention, the presence of normal amygdala volume (Bauman
arousal and sensory processes. & Kemper, 1994; Rapin & Katzman, 1998) [5]

(b) Similarities between autism and patients


following amygdalotom:
Patients with amygdala lesions show impairments
in social judgement [6] that have been likened to
"acquired autism" (Stone, 2000). People diagnosed
with autism tend to show a similar pattern of
deficits to those seen in patients with amygdala
lesions (Adolphs, Sears, & Piven, 2001).

(c) Structural neuroimaging:


A recent structural magnetic resonance imaging
study of autism reported reduced amygdala
volume (Abell et al; 1999) [7].This is not the only
structural abnormality in the brain (see below), but
the amygdala abnormality has some potential
relevance to the social symptoms observed. It is not
Areas of the Brain Affected in Autism yet known why this difference occurs.

An interesting study, which reveals the differences (d) Functional neuroimaging:


between male and female brains suggests the
Using single photon emission computed
capacity to predict and respond to feelings and
tomography (SPECT), patients with autism
behaviour of others by inferring their emotional
spectrum conditions show significant reductions in
states, is a stronger trait in females than in males
are a population level i.e. empathizing. On the other temporal lobe blood flow. This is not simply an
hand male population is stronger at systemizing, effect of temporal lobe epilepsy (Gillberg, Bjure,
that is, inferring rules that govern "cause and effect" Uvebrant, Vestergren, & Gillberg, 1993). In a recent
relationships of behaviour. People with pervasive functional magnetic resonance imaging (fMRI)
developmental disorders are characterized by study, adults with High Functioning Autism (HFA)
deficits in empathizing and those with high or Asperger Syndrome (AS) showed significantly
intellectual capacity have been reported to have less amygdala activation during a mentalizing task
relative strengths in rule bound thinking. (Reading the Mind in the Eyes task), compared to
normal (Baron-Cohen, Ring et al; 1999).
A neural basis of empathy or social intelligence was
first proposed by Brothers (Brothers, 1990) [2]. She
Other brain areas that might be abnormal in
suggested from animal lesion studies (Kling &
autism:
Brothers, 1992), single cell recording studies
(Brothers, Ring, & Kling, 1990) [3], and neurological Reduced neuron size and increased cell-packing
studies that social intelligence was a function of density has also been found in the limbic system,
three regions: the amygdala, the orbito-frontal specifically the hippocampus, subiculum,
Autism 221

entorhinal cortex, amygdala, mammillary bodies, Developmental Disorder (PDD) heading: Autism,
anterior cingulate, and septum in autism [8]. PDD-NOS, Asperger's syndrome, Rett's syndrome,
and childhood disintegrative disorder. PDD is an
6. Biochemical Factors: umbrella term for disorders that involve
A number of studies report that about one third of impairments in reciprocal social interaction skills
patients with autistic disorder have high plasma and communication skills, and the presence of
serotonin concentrations, however, this is not stereotypical behaviours, interests and activities.
specific to autistic disorder as children with mental The term Autistic Spectrum Disorder is used to
retardation without autistic disorder also display represent the fact that while these individuals share
this trait. In some autistic children, a high common characteristics, how these characteristics
concentration of homovanillic acid in the brospinal are manifested will differ with each individual. As
fluid (CSF) is associated with increased withdrawal a result, no two individuals are the same.
and stereotypes. Some evidence indicated that It is sometimes complicated diagnosing autistic
symptom severity decreases as the ratio of 5- disorder because there are no medical tests, blood
hydroxyindoleacetic acid (5 - HIAA, metabolite of tests or any radiological images which will
serotonin) to homovanillin acid in CSF increases. definitively indicate or diagnose a person with
The 5 - HIAA concentration in CSF may be inversely autistic disorder. Also it could be difficult as the
proportional to blood serotonin concentration, person has other issues related to medical,
which is increased in one third of autistic disorder emotional, sensory or learning difficulties. An
patients, a non - specific finding that also occurs in accurate diagnosis is made on the basis of specific
mentally retarded persons. intellectual, social and behavioural characteristics
that are listed in the DSM - IV - TR Manual. The
7. Psychosocial and Family Factors: diagnosis can be made by a physician, a
Studies comparing parents of autistic children with psychologist, or a psychiatrist.
parents of normal children have shown no
significant differences in child- rearing skills. It is The DSM - IV - TR diagnostic criteria for autistic
seen that children with autistic disorder respond disorder are as follows:
like children with other disorders respond to Diagnostic code according to DSM - IV - TR: 299.00
exacerbated symptoms like family discord, the birth
A. A total of six (or more) items from (1), (2) and
of a new sibling or a family move, etc. Some autistic
(3), with at least two from (1) and one each
children may be extremely sensitive to even small
from (2) and (3):
changes in the families and immediate
environment. While making the diagnosis the (1) Qualitative impairment in social interaction as
evaluators should consider prenatal history, manifested by at least two of the following:
perinatal history, birth complications, a) Marked impairments in the use of
developmental milestones and outlining family multiple nonverbal behaviors such as
history via interviews with the parent or relevant eye-to-eye gaze, facial expression, body
caregivers with the help of formal and informal posture, & gestures to regulate social
assessment. Autistic disorder can be diagnosed by interaction
a physician, a psychologist, or a psychiatrist.
Specific tools can be used to check for ASD. b) Failure to develop peer relationships
appropriate to developmental level
Diagnosis and Clinical Features: c) A lack of spontaneous seeking to share
Autistic disorder has been diagnosed using the enjoyment, interests, or achievements
Diagnostic Stastical Manual - IV th Edition - Text with other people, (e.g; by a lack of
Revision, this is a manual with diagnostic categories showing, bringing, or pointing out objects
approved by the American Psychological of interest)
Association and International Statistical d) Lack of social or emotional reciprocity
Classification of Diseases and Related Health
Problems, tenth revision (ICD-10) published by The (2) Qualitative impairments in communication as
World Health Organization (WHO). In 1994, when manifested by at least one of the following:
the fourth edition of DSM was published, five a) Delay in or total lack of, the development
categories appeared under the Pervasive of spoken language (not accompanied by
222 Neuro-Rehabilitation : A multi disciplinary approach

an attempt to compensate through Diagnostic code according to ICD - 10: F84.0


alternative modes of communication
A type of pervasive developmental disorder that is
such as gesture or mime)
defined by: (a) the presence of abnormal or
b) In individuals with adequate speech, impaired development that is manifest before the
marked impairment in the ability to age of three years, and (b) the characteristic type of
initiate or sustain a conversation with abnormal functioning in all the three areas of
others psychopathology: reciprocal social interaction,
communication, and restricted, stereotyped,
c) Stereotyped & repetitive use of language
repetitive behaviour. In addition to these specific
or idiosyncratic language
diagnostic features, a range of other nonspecific
d) Lack of varied, spontaneous make- problems are common, such as phobias, sleeping
believe play or social imitative play and eating disturbances, temper tantrums, and
appropriate to developmental level (self-directed) aggression.
(3) Restricted repetitive & stereotyped patterns of Autistic disorder
behavior, interests & activities, as manifested
Infantile:
by at least two of the following:
• autism
a) Encompassing preoccupation with one or
more stereotyped & restricted patterns of • psychosis
interest that is abnormal either in
Kanner's syndrome
intensity or focus
Excl.: autistic psychopathy (F84.5)
b) Apparently inflexible adherence to
specific, nonfunctional routines or rituals Atypical autism: F84.1
c) Stereotyped & repetitive motor A type of pervasive developmental disorder that
mannerisms (e.g. hand or finger flapping differs from childhood autism either in age of onset
or twisting, or complex whole-body or in failing to fulfill all three sets of diagnostic
movements) criteria. This subcategory should be used when
d) Persistent preoccupation with parts of there is abnormal and impaired development that
objects is present only after age three years, and a lack of
sufficient demonstrable abnormalities in one or two
B. Delays or abnormal functioning in at least one of the three areas of psychopathology required for
of the following areas, with onset prior to age the diagnosis of autism (namely, reciprocal social
3 years: interactions, communication, and restricted,
1) social interaction stereotyped, repetitive behaviour) in spite of
characteristic abnormalities in the other area(s).
2) language as used in social
Atypical autism arises most often in profoundly
communication
retarded individuals and in individuals with a
3) symbolic or imaginative play severe specific developmental disorder of receptive
language.
C. The disturbance is not better accounted for by
Rett's Disorder or Childhood Disintegrative Atypical childhood psychosis
Disorder
Mental retardation with autistic features
(From American Psychiatric Association.
Use additional code (F70-F79), if desired, to identify
Diagnostic and Statistical Manual of Mental
mental retardation.
Disorders. 4th ed. Text rev. Washington, DC:
American Psychiatric Association; copyright 2000) Other Pervasive Development Disorders:
[9].
The diagnostic category known as Pervasive
The International Statistical Classification of Developmental Disorders includes: Autistic
Diseases and Related Health Problems, tenth Disorder, Asperger's Disorder, Rett's Disorder,
revision (ICD-10) diagnostic criteria for autistic Childhood Disintegrative Disorder and Pervasive
disorder are as follows: Developmental Disorder Not Otherwise Specified.
All of these disorders share common features.
Autism 223

However, there are differences in some areas, such may actually be due to constipation-i.e; only liquid
as the number of symptoms, age of onset, is able to leak past a constipated stool mass in the
developmental pattern and level of cognitive intestine. Manual probing often fails to find an
functioning. impaction. An endoscopy may be the only way to
check for this problem. Consultation with a
Physical Characteristics: paediatric gastroenterologist is required.
Children with autistic disorder do not show any
physical signs indicating the disorder on the first Behavioural Characteristics:
glance. A greater than expected number of children
do not show lateralization and remain Difficulties in Social Skills:
ambidextrous at an age when the cerebral Have you ever made a mistake in social situations?
dominance is established in most children. or Told a joke that did not fit the event? Or misread
the intentions of others? Then it is easier to
Seizures: understand why individuals with autism have
It is estimated that 25% of autistic individuals also difficulty in social situations. Social situation are
develop seizures, some in early childhood and very demanding as they need us to make quick
others as they go through puberty (changes in judgements, respond to unpredictable events, read
hormone levels may trigger seizures). These facial expressions and body gestures of others. For
seizures can range from mild (e.g; gazing into space individuals with autism, these skills are missing or
for a few seconds) to severe, grand mal seizures. are functioning at a deficit.
Many autistic individuals have subclinical seizures Social difficulties are the primary that individuals
which are not easily noticeable but can significantly with autistic disorder face challenge being
affect mental function. A short one- or two-hour employed or in employment. As when young they
EEG may not be able to detect any abnormal usually have minimal interest in playing or
activity, so a 24-hour EEG may be necessary. interacting with peers, hence when social
Although drugs can be used to reduce seizure engagement occurs it is typically on their own terms
activity, the child's health must be checked or it gets very awkward. These responses reflect a
regularly because these drugs can be harmful. lack of skill in knowing how to interact rather than
a lack of desire in socialization. Also as they stick
Sleep Problems: to their ritualistic routine, they have difficulty in
Many autistic individuals have sleep problems. changing or alternating routine for which they need
Night waking may be due to reflux of stomach acid additional support and transitioning time to new
into the oesophagus. and unfamiliar experiences. [1]

Pica:
30% of children with autism have moderate to
severe pica. Pica refers to eating non-food items
such as paint, sand, dirt, paper, etc. Pica can expose
the child to heavy metal poisoning, especially if
there is lead in the paint or in the soil.

Low Muscle Tone:


A study conducted by the first author found that
30% of autistic children have moderate to severe
loss of muscle tone, and this can limit their gross
and fine motor skills. That study found that these Poor Eye contact
children tend to have low potassium levels. Autistic children do not display the expected level
of subtle reciprocal social skills that demonstrate
Chronic Constipation and/or Diarrhoea:
relatedness to parents and peers. Children with
Analyses of the ARI's autism database of thousands autistic disorder may lack social smile and may
of cases show over 50% of autistic children have have anticipatory posture as an adult approaches.
chronic constipation and/or diarrhoea. Diarrhoea They may also have less frequent or poor eye
224 Neuro-Rehabilitation : A multi disciplinary approach

contact. Autistic children display impaired social lack of motivation. Language deviance, as language
development i.e. they may display poor attachment delay, is characteristic of autistic disorder. In
behaviour towards parents or peers. They may not contrast to normal and mentally retarded children,
be able to differentiate between the most important autistic children have significant difficulty putting
people in their lives like their parents, siblings, and meaningful sentences together even when they
teachers. have large vocabularies. In the first year of life, an
autistic child's pattern of babbling may be minimal
or abnormal. Some children emit noises like clicks,
screeches and nonsense syllables - in a stereotyped
fashion, without a seeming intent of
communication. [1]
Autistic children with delays in learning to talk and
use language may not compensate by using
extensive gestures and pointing. Instead young
children with this disorder may put an adult's hand
on what they want, pull the adult over to the object
they want to access, or engage in problematic
behaviors to express their message. Individuals
with underdeveloped speech may grunt, point a
Anxiety experience by autistic child
finger, pull an adult to an area, or use a picture cue
Autistic children strictly adhere to their routine and and some may never develop a meaningful speech
when disrupted they may display extreme anxiety and may need to use singing and/or augmentative
and irritability. When autistic children have reached communication devices. Others begin their journey
school age, their withdrawal may have diminished towards developing oral communication skills by
and may be less obvious, particularly in higher- using echolalia. Some have a very restricted
functioning children. A prominent deficit is seen repertoire of use of their communication skills and
in ability to play with peers and to make friends; need direct instruction and support to expand their
their social behavior is awkward and may be skills. Even those who develop oral skills often have
inappropriate. Cognitively, children with autistic other language/communication problems in social
disorder are more skilled in visual- spatial tasks and academic situations.
requiring skill in verbal reasoning. The cognitive
Individuals with autistic disorder are very concrete
style of children with autism is that they cannot
in their understanding of the world and can have
infer the feelings or mental state of others around
significant comprehension problems and significant
them. That is, they cannot make attributions about
gaps in their store of background knowledge; hence
the motivation or intentions of others and thus,
they may see jokes and sarcasm as lies and then
cannot develop empathy. This lack of a "theory of
mistrust the speakers. [1]
mind" leaves them unable to interpret the social
behaviour of other s and leads to lack of social
Stereotypes Behaviour:
reciprocation.
Restrictive, repetitive and stereotypical behaviour
Autistic person in late adolescence, often desire
may vary with individuals, circumstance, and age
friendship, but their difficulties in responding to
and by the level of awareness about others. In an
another's interests, emotions and feelings are major
autistic child's first few years of life, the expected
obstacles in developing them. They are often
spontaneous exploratory play is absent. The toys
shunned by peers because of their awkward
and objects are often manipulated in a ritualistic
behaviour that alienate them form others. Autistic
manner, with few symbolic features. Autistic
adolescents and adults experience sexual feelings,
children generally do not show imitative play or
but their lack of social competence and skills
use abstract pantomime. The play and other
prevent many of them from developing sexual
activities of these children are often rigid, repetitive
relationships. [1]
and monotonous. The ritualistic and compulsive
Disturbance of Communication and Language: phenomenon's are common in infants and
adolescents years. Autistic children often use
Autistic children are not simply reluctant to speak
inanimate objects in a very vague manner like
and their speech abnormalities do not result from
Autism 225

spinning bottles, banging toys and may exhibit have difficulty discriminating between sounds,
attachment to particular inanimate objects like remembering directions, paying attention to a
wires, rods, etc. Autistic children generally show voice, and/or reading aloud.
resistance to transition and change like moving to
a new house, moving furniture in a room, or a Vestibular sensations:
change, such as having breakfast before a bath Vestibular sensations occur in our inner ear. The
when the reverse was routine was the routine may inner ear receptors register every movement we
evoke panic, fear or temper tantrums. [1] make and every change in head position. This
Rocking one's body for example is a repetitive encompasses messages from our neck, eyes, and
behaviour which occurs when the child is anxious, body. Rotary movements involve moving in circles
agitated at home, school and social situations. (e.g; spinning). Linear movements (i.e; back and
Repetitive behaviours might include lining things forth, side to side, up and down), especially when
up, ensuring that all cupboard doors are closed, rapid (i.e; rocking in a chair, swaying, swinging on
aligning chairs in a certain fashion, and making a tire), may cause most to become dizzy, nauseated,
certain noises. Stereotyped movements might or to get a headache. Individuals with autistic
include hand flapping, rocking, spinning, jumping, disorder may actually crave these sorts of
and other patterns. Restrictive behaviour involves movements and this means that they need a lot of
having a narrow set of interests. Some become vigorous activity in order to get started. Some
walking encyclopaedias of facts on certain topics. become distressed and show anxiety due to a fear
of falling, being picked up, standing up, or sledding
Instability of Mood and Affect: down a hill.
It is very common for autistic children to exhibit
Visual Senses:
mood changes, with bursts of laughing or crying
spells for no apparent reason. It is however, a Individual with autistic disorder may be distracted
difficult to task to learn about these episodes if the with objects hanging from the ceiling; they may feel
child cannot express the thoughts to the effects. [1] blinded by the sun or may be unable to focus in the
presence of florescent lighting. Whereas others may
Response to Sensory Stimuli: have their eyes glued on to spinning or bouncing
objects or reflection of objects.
Our senses are conditioned to organize and interact
with the world around us. It is important to realize,
Smell and Taste Sensation:
understand, and accept that some individuals on
the autism spectrum may actually feel, hear, see, Some individuals with autistic disorder may be
smell, and taste at an extreme level. They may be highly agitated by perfumes, the odour of foods or
hypersensitive (overreactive) or hyposensitive animal or hand lotions and may avoid people,
(underreactive) for example to sound and pain. places or foods so that they are not around that
odour. Whereas, individuals hyposensitive to smell
Touch Sensation: may crave odours or tastes and may lick or taste
inedible objects (e.g; clay, chalk) or prefer spicy, hot,
Touch is an important sense that is needed for social
or sour foods.
interactions with loved ones or to show care for a
person. However, some with autistic disorder don't Proprioceptive feedback helps us position our
like being touch and respond aggressively on doing bodies and move through the environment.
so, whereas on the other hand some have a high Proprioceptors exist in our muscles, joints,
tolerance for pain and may not realize a shoulder ligaments, tendons, and connective tissue.
is broken until it is swollen for several days. Proprioceptors\ work closely with tactile and
vestibular systems through body awareness, motor
Auditory Senses: control/planning, and postural stability. Autistic
Imagine how it would feel to hear a fire alarm, children may need to keep their eyes open in order
vacuum cleaner, or a room full of children at a to know how their own body is moving. Motor
birthday party at a magnified level at the same time control/planning involves coordinating one's gross
within the environment. This is what autistic and fine motor skills within the environment. Those
children go though and they have difficulty in who have difficulty in this area may bump into
blocking and auditory senses. An individual may people or obstacles or frequently fall or trip. Some
cannot regulate how much pressure to exert when
226 Neuro-Rehabilitation : A multi disciplinary approach

grasping an object. As a result they hold pencils


too tightly, and their written work is messy.
Individuals on the autism spectrum may have poor
posture, lean their head on their hands, and be
unable to balance on one foot. In all of these areas,
individuals may feel emotionally insecure due to
feeling uncomfortable or inadequate, rigid, or
intimidated by the environment. It is imperative to
accommodate individuals with sensory needs
throughout the day to help them calm their nervous
systems so they can function in home, community,
or work. These breaks should not be considered a
reward but as a necessary requirement for a
person's day. Many autistic children reportedly
enjoy music, where they frequently hum a tune or Head Banging behaviour
sing a song or commercial jingle before saying
Difficulties with Executive Function:
words or using speech.
Individuals with autism have difficulty in multi
Associated Behavioral Symptoms: tasking, completing assignments and organizing.
They are better at carrying out one task at a time.
Executive functions also include deficits in the area
of making transitions and organizing oneself.

Intellectual Functioning:
Approximately 70 to 75 percent of children with
autistic disorder fall in the mentally retarded range
of intellectual function. About 30 percent of children
function in the mild to moderate range, and about
45 to 50 percent are severely to profoundly mentally
retarded. Epidemiological and clinical studies show
that the risk of autistic disorder increases as the IQ
decreases. About 1/5 of all autistic children is have
a normal, non-verbal intelligence. The Intelligence
Quotient scores of autistic children tend to reflect
Self Injurious Behaviour most served problems with verbal sequencing and
Hyperkinesis is a common behaviour problem in extraction still, with relative strength in visuo-
young autistic children whereas hypokinesis is less spatial or rote memory skill. This finding suggests
frequent; but when present it often alternates with the importance of the effects in language related
hyperactivity. Aggression and temper tantrums are functions.
observed, often prompted by change or demands. Unusual order precocious cognitive or visuo-motor
Self injurious behaviors include head banging, abilities occur in some autistic children. The
biting themselves or others, picking their scabs, abilities, which may exist even in the overall
scratching, and hair pulling. Others may react to retarded functioning, are referred to as splinter
their environment by being physically hurting their functions or islets of precocity. Perhaps, the most
siblings, peers or family members, and such striking example is autistic savants, who have
behaviors may escalate during the adolescent years exceptional rote memories or calculating abilities,
due to the hormonal changes. Some children may usually beyond the capabilities of their normal
flap their arms, brush their hands against their face peers. Other gifted abilities in young autistic
repeatedly, or may hum as a self -stimulatory children include hyperlexia, an early ability to read
behaviour. Short attention, poor ability to focus on well (although they cannot understand what they
task, insomnia, feeding and eating problems and read), memorizing and reciting and musical abilities
enuresis are also common among children with (singing or playing tunes or recognizing musical
autism. pieces).
Autism 227

When an individual is gifted in specific areas, he or assessment process to begin. Some children may
she is often referred to as being savant. These be seen to be mildly affected with this disorder but
individuals may have exceptional talents in eventually may lead to full blown syndrome. Early
calculating numbers, playing a musical instrument, diagnosis may help to formulate an intervention
or drawing. plan which would significantly help improving the
quality of life and functioning of the child.
Early Diagnosis:
Although autism is usually seen to occur during Differential Diagnosis:
the early gestational abnormalities of brain
Autism must be differentiated from one of the other
development, but for the parent it may seem to
pervasive developmental disorder such as
begin after the first year of life. The babies are often
Asperger's disorder and pervasive developmental
"too good", very quiet and are passive or are too
disorder not otherwise specified. Further it must
irritable and intense; the eye contact may be absent
be differentiated from other developmental
from the start and the infants may seem to be
disorders, including mental retardation syndromes
unresponsive and different as compared to the
and developmental language disorders. It is
other children. The symptoms of autism in a two
sometimes difficult to make the diagnosis of autism
year old are very different as compared to a four
because of over- lapping symptoms specified
year old; hence it is extremely important to make
below:
the diagnosis depending on the age.
Schizophrenia with Childhood Onset:
Speech:
Although a wealth of literature on autistic disorder
Delayed speech and language milestones must be
is available, few data exists on children under age
taken very seriously and form the most common
12 who meet the diagnostic criteria for
presenting symptoms in the autistic spectrum.
schizophrenia.
Hearing impairment should definitely be ruled out
with the help of tests. It is often seen that autistic Mental Retardation with Behavioural
children usually lack "communicative intent" and Symptoms:
do not seem to "listen" to adult conversations.
Approximately 40 percent of autistic children are
Absolute indication of preliminary assessment
moderately, severely or profoundly retarded and
include absent babbling at 12 months of age, no
retarded children may have behavioural symptoms
single word at 12 months, no phrase at 24 months
that include autistic features. When both disorders
and at eventually may either lead to developing a
are comorbid, both should be diagnosed. The
restricted speech or absence of speech.
prominent distinguishing feature between autistic
Social Interaction: children and mentally retarded children are that
Poor eye contact and delay in social smiling within mentally retarded children usually relate to adults
the first few months of life may require urgent and other children in accordance with their mental
consideration. The one year old who ignores age, use the language they do have to communicate
gestures, does not shake or nod the head with others, and exhibit a relatively even profile of
appropriately during conversations should raise impairments without splinter functions.
suspicion. Pointing is a fundamental
Mixed Receptive - Expressive Language
communicative act which is usually seen when a
child is around 15 months of age but if there is a Disorder:
failure to point to objects at 18 months of age; this Some children with mixed receptive - expressive
would be a strong suspicion of autism. However, language disorder have mild autistic like features
absence of pointing may be seen in children who and may present a diagnostic problem.
are mentally retarded or with visual impairment.
They may also lack interest in imaginative play or Acquired Aphasia with Convulsion:
social interaction and if play is present it could be Acquired aphasia with convulsion is a rare
present in the form of repetitive behaviour, rejection condition that is sometimes difficult to differentiate
of new toys, or bizarre use of toys. from autistic disorder and childhood disintegrative
disorder. Children with this condition are normal
The critical step in early diagnosis is to recognize
for several years before losing their receptive and
the grounds for suspicion and to arrange for an
228 Neuro-Rehabilitation : A multi disciplinary approach

their expressive language over a period of weeks adult's remains severely handicapped and live in
or months. A profound language comprehension complete dependence or semi-dependency with
disorder then follows, characterized by deviant their caregivers or relatives. Only about 2 percent
speech pattern and impairment. Some children are able to lead a normal, independent life with
recover, but with considerable residual language employment and about 5 to 20 percent of them are
impairment. able to achieve a borderline status.
Congenital Deafness or Severe Hearing The prognosis of children is believed to improve if
Impairment: the environment or home is supportive and capable
of meeting the extensive need of such a child. The
Autistic children are often mute or show selective
symptoms may decrease in some cases as the age
disinterest in spoken language, they are often
increase but with others severe self-mutilation and
thought to be deaf. The prominent distinguishing
aggressiveness and aggression have been seen to
factors are as follows:
increase. About 4 to 32 per cent at a grand mal
• Autistic infants may babble once in a while, seizures in late childhood or adolescence, and
whereas deaf infants have a history of seizures adversely affect the prognosis. [1]
relatively normal babbling that then gradually
tapers off and may stop at 6 months to 1 year Treatment for Autism:
of age.
Intervention for autism is a very intensive,
• Deaf children respond only to loud sounds, comprehensive and one which involves the child's
whereas autistic children may ignore the loud entire family and a qualified team of professionals.
or normal sounds and respond to soft or low Based on the issues that the child with autism has
sounds. and a comprehensive evaluation, the therapies and
• Most importantly audiogram or auditory therapists are narrowed down for the child.
evoked potentials indicate significant hearing Treatment programs may combine therapies for
loss in deaf children. both core symptoms and associated symptoms.

• Unlike autistic children, deaf children usually Medical Management:


relate to their parents, seek affection and enjoy
being held as infants. Medical management makes an important
contribution to the overall wellbeing and outcome
Psychosocial Deprivation: in individuals with autism and to their family
Severe disturbance in the physical and emotional members, even though the primary intervention is
environment (e.g. maternal deprivation, psychological and educational in nature. The
psychosocial dwarfism, hospitalism, and failure to physician is usually the only resource for the
thrive) can cause children to appear apathetic, families in search of up- to -date and accurate
withdrawn and alienated. Language and motor information on the biology and neuroscience of
skills can be delayed. Children with these signs autism.
almost always improve rapidly when placed in a
favorable and enriched psychosocial environment, Bowel functions (diarrhoea and constipation):
but such improvement is not the case with autistic This occurs with the same frequency in children
children. with autism and other neurological disorders such
as cerebral palsy. Constipation usually reflects low
Course and Prognosis: muscle tone and immobility and deserves very
energetic treatment along conventional lines in
Autism is generally a lifelong disorder with the order to prevent fissures and pain. Family history
guarded prognosis. Autistic children who have of severe constipation should lead to the exclusion
intelligence quotient about 70 and those who use of Hirschsprungs disease. Good hydration is
communicative language by ages five to seven essential in the management of both constipation
years tend to have the best to prognosis. It is seen and diarrhoea.
that symptoms such as repetitive and ritualistic
behaviour did not seem to improve overtime. Seizures:
Studies on the outcome of children with autistic There is substantial evidence that certain nutritional
disorder reported that about two third of autistic supplements, especially vitamin B6 and
Autism 229

dimethylglycine (DMG), can provide a safer and with evening primrose oil or fish oil can do no harm.
more effective alternative to drugs, for many Non - specific or placebo like benefits seem to occur
individuals. with almost any dietary improvement or
modification.
Sleep:
Gluten Free, Casein Free Diet (GFCF): A very
Sleep is a frequent concern for parents of autistic popular dietary treatment for symptoms of autism
children and can be very difficult to treat. A strict is removal of gluten (a protein found in barley, rye,
going to sleep routine is even more important for oats, and wheat) and casein (a protein found in
these children than for others, and here their strong dairy products), in what is known as a Gluten Free,
need for routine and ritual may be used to help Casein Free diet, or GFCF. This is based on the
them. Stimulating foods and drinks should be hypothesis that these proteins are absorbed
avoided. With the exception of melatonin no drugs differently in children with autism spectrum
are recommended for insomnia in children, unless disorders and act like false opiate-like chemicals in
a simple antihistamine such as Benadryl is given the brain. There are ongoing studies to prove the
for a few nights to give the parents a break from effectiveness of this dietary intervention. However,
incessant crying or hyperactivity. Some truly many families report that dietary elimination of
hyperactive children diagnosed with autism and gluten and casein has helped to control bowel
ADHD may be helped to sleep by methylphenidate habits, sleep activity, habitual behaviour and
(Ritalin) but most will be kept awake even longer. enhance the overall progress in their children.
Placing bricks under the head of the bed may help
keep stomach acid from rising and provide better Pharmacotherapy in Autism:
sleep. Melatonin has been very useful in helping
many autistic individuals fall asleep. Other popular Current psychopharmacologic trials are under way
interventions include using 5-HTP and to investigate efficacy of a variety of classes of
implementing a behaviour modification program agents on promoting social interactions and
designed to induce sleep. Vigorous exercise will reducing disruptive behaviours in children and
help a child sleep, and other sleep aids are a adolescents with autism and other pervasive
weighted blanket or tight fitting mummy-type development disorders. Currently, no specific
sleeping bag. medications with proved efficacy in the treatment
of the core symptoms of autistic disorders are
Dental Treatment: available; however medications have been shown
As some autistic children are non- verbal they may to be promising in reducing hyperactivity,
communicate the pain of dental cavities or issues obsessions and compulsive behaviours, irritability,
by being self-abusive, hyperactive or otherwise by aggression and self - injurious behaviours.
a very difficult behaviour. The administration of antipsychotic medication has
been shown to be efficacious in the reduction of
Nutrition and Diet: aggressive and self- injurious behaviour. One early
It is often observed that autistic children are very study indicated that haloperidol reduced the
fussy feeders who incorporate their obsessions and behavioural symptoms such as hyperactivity,
need for the sameness into their feeding behaviour. stereotypes, withdrawal, fidgetiness, irritability and
Regular measurement of weight, height and head labile affect and accelerated learning. Given its
circumference will allow calorie deficiencies to be potentially serious adverse effects, haloperidol is
detected early along with clinical evaluation for no longer the antipsychotic agent of choice in the
signs of malnutrition. As sub- optimal nutrition treatment of self injurious behaviours in children
with micro-deficiencies in many vitamins and with autistic disorders.
mineral supplements should be recommended for The atypical antipsychotic agents are known to
all but those whose meal -time behaviour have a lower risk of causing extra pyramidal
approaches normal. Zinc deficiency may be adverse effects, although some sensitive individuals
associated with sensory blunting, poor appetite, cannot tolerate the extra pyramidal or
and disturbed bowel functions and perhaps with anticholinergic adverse effects of the atypical
aggressive behaviour so the supplementation antipsychotic agents. The atypical antipsychotic
should often be considered. Autistic children may agents include risperidone, olanzapine, quetiapine,
have low plasma fatty acids and supplementation clozapine and ziprasidone.
230 Neuro-Rehabilitation : A multi disciplinary approach

Risperidone, a high-potency antipsychotic with about 10 mg per day. Among olanzapine's most
combined dopamine D2 and serotonin 5 - HT2 common adverse effects are sedation, orthostatic
receptor antagonist properties, has been used to hypotension, and weight gain.
subdue aggressive or self - injurious behaviours.
Several reports have suggested that risperidone is
effective in diminishing aggressiveness,
hyperactivity and self-injurious behaviour in
children with autistic disorder. In some cases, it
reportedly encouraged socially acceptable
behaviours. The U.S. Food and Drug
Administration has approved Risperdal as autism
medication to treat irritability in autistic children
and adolescents. This is the first time the FDA
approved a drug to treat behavior-related problems
associated with autism in children. The drug can
be used to treat aggression, deliberate self-injury Olanzapine
and temper tantrums. Risperdal is considered an Clozapine has a hetrocyclic chemical structure that
atypical antipsychotic drug manufactured by is related to certain conventional antipsychotics,
Janssen Pharmaceutica N.V. in Beerse, Belgium. For such as loxapine, although clozapine carried lower
autism, lower dosages ranging from 0.5 to 4 mg risk of extrapyramidal symptoms. It is generally
per day are used in clinical practice. Extra used in treatment of aggression and self- injurious
pyramidal effects and akathisia have been reported behaviour unless those behaviours coexist with
adverse effects, as well as sedation, dizziness and psychotic symptoms. It's most serious adverse
weight gain. Drooling was reported more in the effects are agranulocytosis, which necessitates
risperidone group compared with the placebo monitoring white blood cells count weekly during
group. In this sample, extrapyramidal symptoms clozapine's use. Its use is generally limited to
were not reported more commonly in the treatment - resistant psychotic patients. Lithium can
risperidone group. The side effects that caused the be administered in the treatment of aggressive or
most concern were somnolence and weight gain. self - injurious behaviours when anti psychotic
[10] medications fail.

Risperidone 3 mg MYL,
white, round, film coated
Clozapine C11 M, 100,g, green colour,
Olanzapine specifically blocks 5- HT 2A and D2 9.00 mm, round.
receptors and also blocks muscarinic receptors. No
studies provide specific guidelines regarding the Psychotropic Medication: Psychotropic medication
use of olanzapine in children with autism. Dosages is commonly used to treat disruptive behaviors,
that have been used clinically to target aggression agitation, inattention, and hyper-activity in children
and self-injurious behaviours range from 2.5 to with ASD (Myers, et al; 2007).
Autism 231

Medication Level of Target Significant


Class (Brand name) Evidence Symptoms Potential Studies
Side Effects
Antipsych Risperidone Established Irritability, Weight gain, (Jesner, Aref-
-otics (Risperidal) Evidence hyper-activity, drooling, Adib, & Coren,
and stereotypy dizziness, 2007; McDougle,
fatigue, et al; 2005; Miral,
involuntary et al; 2008; RUPP,
muscle 2002)
movement
Haloperidol Established Aggression Tardive (Anderson, et al;
(Haldol) Evidence dyskinesia, 1989; Anderson,
sedation, et al; 1984)
irritability
Stimulants Methylpheni- Established Hyperactivity Social (Handen,
date (Ri-talin) Evidence withdrawal, Johnson, &
irritability, Lubetsky, 2000;
agitation, Quitana, et al;
stereotypy 1995)
Norepineph- Atomoxetine Preliminary Attention None (Arnold, et al.,
rine Reuptake HCI (Strattera) Evidence deficit, 2006)
Inhibitor hyperactivity
Alpha 2 Clonidine Preliminary Hyperactivity, Drowsiness, (Jaselskis, Cook,
Agonist (Catapres) Evidence irritability, low blood Fletcher, &
inappropriate pressure, Leventhal, 1992)
speech, irritability
stereotypy,
oppositionality
Guanfacine Insufficient Hyperactivity, Transient (Posey, Puntney,
(Tenex) Evidence inattention, sedation Sasher, Kem, &
impulsivity, McDougle, 2004)
aggression
Selective Fluoxetine Insufficient Repetitive Celexa: (Hollander, et al.,
Serotonin (Prozac) & Evidence behavior Hyperactivity, 2005; King, et al.,
Reuptake Citalopram (conflicting insomnia, 2009)
Inhibitors (Celexa) results) inattention,
(SSRIs) impulsivity,
diarrhea, dry
skin
Others Clomipramine Preliminary Stereotypy, Insomnia, (Gordon, State,
(Anafranil) Evidence ritualistic constipation, Nelson,
behavior, twitching, Hamburger, &
social behavior tremors Rapoport, 1993)
Valproic Acid Insufficient N/A Rash, weight (Heillings, et al.,
(Depakote) evidence gain, hair loss, 2005; Hollander,
fatigue et al; 2006)
Naltrexone Insufficient N/A Increased (Willemsen-
(Revia) evidence stereotypy Swinkels,
Buitelaar,
Weijnen & van
Engeland, 1995)
232 Neuro-Rehabilitation : A multi disciplinary approach

Role of Psychology in Autism: is an alternative for assessing verbally fluent


individuals, 6 years of age and older, with IQ scores
Diagnostic and Assessment Instruments For above 80. Questionnaire for Parents or Caregivers
Children with Autistic Spectrum Disorder: (CARS2-QPC) is an unscored scale that gathers
information for use in making CARS2-ST and
The below listed scales and tool are used by
CARS2-HF ratings. The CARS2-ST and CARS2-HF
psychologists, educators and researchers to assess
each include 15 items addressing the following
children suspected of or previously diagnosed with
functional areas like: relating to people, social and
a pervasive developmental disorder. The
emotional understanding, imitation, body use,
instruments listed below are used to measure
visual responses, verbal communication, taste,
specific dimensions of a child's development in
smell, and touch response and use, etc. It is
different domains of functioning, which would help
conducted on children 2 years and older and the
develop a proper intervention plan for the child.
approximate administration time is 5 to 10 minutes.
Diagnostic Assessment: [12]
Autism Diagnostic Interview - Revised: This is a Checklist for Autism in Toddlers (CHAT): This is
semi-structured, investigator-based interview a screening instrument designed to detect core
which is useful for diagnosing autism, having an autistic features to enable treatment as early as 18
intervention plan and distinguishing autism from months. CHAT offers psychologists a means of
other developmental disorders. The ADI-R has diagnosing autism in infancy so that educational
proven particularly effective in assessing syndrome programs can be started months or even years
boundaries, identifying new subgroups, before most symptoms become obvious. It is a test
quantifying Autistic symptomatology, and which aims to look at behaviors which, if absent at
determining the clinical needs of groups in which 18 months, put a child at risk for a social-
a high rate of Autism Spectrum Disorders might communication disorder. These behaviors are (a)
be expected, such as people with severe language joint attention, including pointing to show and
impairments. It is conducted on children and adults gaze-monitoring (e.g. looking to where a parent is
with a mental age of above 2 years. It takes pointing), and (b) pretend play (e.g. pretending to
approximately 1 1/2 to 2 1/2 hours, including pour tea from a toy teapot).
scoring. Gilliam Autism Rating Scale - Second Edition
Prelinguistic Autism Diagnostic Observation (GARS - 2): GARS-2, a revision of the popular
Schedule: The Prelinguistic Autism Diagnostic Gilliam Autism Rating Scale, assists teachers,
Observation Schedule (PL-ADOS) (DiLavore, Lord, parents, and clinicians in identifying and
& Rutter, 1995) is a semi-structured observation diagnosing autism in individuals ages 3 through
scale for diagnosing children whose speech is not 22. It also helps estimate the severity of the child's
yet developed and who are suspected of having disorder. Items on the GARS-2 are based on the
autism. This instrument provides an opportunity definitions of autism adopted by the Autism Society
to observe specific aspects of the child's social of America and the Diagnostic and Statistical
behavior, such as joint attention, imitation, and Manual of Mental Disorders: Fourth Edition-Text
sharing of affect with the examiner and parent. PL- Revision (DSM-IV-TR). The instrument consists of
ADOS scores are reported to discriminate between 42 clearly stated items describing the characteristic
children with autism and children without autistic behaviors of persons with autism. The items are
developmental disabilities. [11] grouped into three subscales: Stereotyped
Behaviors, Communication and Social Interaction.
Childhood Autism Rating Scale Second Edition Using objective, frequency-based ratings, the entire
(CARS- 2): This scale helps to distinguish children scale can be completed and scored in 5 to 10
from developmentally handicapped children who minutes. A structured interview form is included
are not autistic. It helps distinguish between mid for gathering diagnostically important information
to moderate to severe autism. Standard Version from the child's parents. [13]
Rating Booklet (CARS2-ST) is equivalent to the
original CARS; for use with individuals younger Developmental Assessment:
than 6 years of age and those with communication Psycho- educational Profile-Revised (PEP-R): This
difficulties or below-average estimated IQs. High- offers a developmental approach to the assessment
Functioning Version Rating Booklet (CARS2-HF) of children with autism or related developmental
Autism 233

disorders. It is an inventory of behaviors and skills Stanford-Binet Intelligence Scale (4th ed.): The
designed to identify uneven and idiosyncratic Stanford-Binet Intelligence Scale (4th ed.) (SBIS-IV)
learning patterns. The test is most appropriately (Thorndike, Hagen, & Sattler, 1986) is for
used with children functioning at or below the individual's ages two years to adult. It provides
preschool range and within the chronological age scores in four areas: Verbal Reasoning, Abstract and
range of six months to seven years. The PEP-R Visual Reasoning, Quantitative Reasoning, and
provides information on developmental Short-Term Memory; and a Composite Score that
functioning in imitation, perception, fine motor, is equivalent to the Wechsler Scales Full Scale IQ.
gross motor, eye-hand integration, cognitive [17]
performance, and cognitive verbal areas. The PEP-
R also identifies degrees of behavioral abnormality Academic Screening:
in relating and affect (cooperation and human Wide Range Achievement Test 3 (WRAT3): The
interest), play and interest in materials, sensory Wide Range Achievement Test 3 (WRAT3)
responses, and language. measures reading, spelling, and arithmetic in
persons from five to seventy-four years old. Two
Adaptive Assessment: equivalent forms make pre- and post testing
Vineland Adaptive Behavior Scales - Second possible. The test takes 10 to 15 minutes to
Edition (Vineland-II): This scale comes in three administer. The WRAT3 provides a good method
forms varying in degree of detail and proposed for measuring basic academic skills in children who
setting. The VABS is administered by interviewing perform below their peers.
the child's parents, teachers, or care providers. The
scales range in age from birth to nineteen years. It Behavior Assessment:
takes approximately 20 to 60 minutes for the Achenbach Child Behavior Checklist: The
conduction and scoring of the test. [14] Achenbach Child Behavior Checklist (ACBC) is for
children four to eighteen years old and is completed
Standardized Tests of Intelligence: by an adult informant. It has two major scales -
Wechsler Intelligence Scale for Children (3rd ed.): externalizing and internalizing behaviors - each of
This is used to assess intelligence as a global but which has four subscales. It has been used as a
multifaceted entity that can be inferred from a follow-up measure. The child's primary caregiver
child's performance on a series of tasks. It is (in most cases, the client's mother) serves as the
valuable for psycho-educational assessment, informant.
diagnosis, placement, and planning. WISC-III can
Analysis of Sensory Behavior Inventory (Rev. ed.):
be used to diagnose exceptionality among school-
The Analysis of Sensory Behavior Inventory (Rev.
aged children and has a strong place in clinical and
ed.) (ASBI-R) (Morton & Wolford, 1994) is designed
neuropsychological assessment and in research.
to collect information about an individual's
Like the WPPSI-R, the WISC-III is widely used and
behaviors as they are related to sensory stimuli. Six
generally regarded as the best standardized
sensory modalities are assessed: vestibular, tactile,
measure of intelligence. [15]
proprioceptive, auditory, visual, and gustatory-
Differential Ability Scales: The Differential Ability olfactory. Ratings can be made about both sensory-
Scales (DAS) (Elliott, 1990) [16] measures overall avoidance and sensory-seeking behaviors within
cognitive ability and specific abilities in children each modality. Information obtained from this tool
and adolescents. It is better suited for intellectually may be helpful in completing a functional analysis
higher-functioning children with autism. The DAS of behavior and in designing effective intervention
assesses multidimensional abilities in children ages strategies, including accommodations and
two years and six months to seventeen years and reinforces for the individual.
eleven months. It is administered individually and
takes 45 to 65 minutes for the full cognitive battery. Psychological Intervention for Autism:
The achievement test takes 15 to 25 minutes to The goals of psychological intervention for children
administer. The seventeen cognitive and three with autism is to eliminate or reduce the
achievement subtests yield an overall cognitive undesirable behaviours and target the behaviours
ability score and achievement scores. The three that will help the child integrate into schools,
achievement subtests are Basic Number Skills, develop and meaningful social communication
Spelling, and Word Reading. with peers and family members and increase the
234 Neuro-Rehabilitation : A multi disciplinary approach

likelihood of maintaining an independent living as observed in different situation as this helps to


an adult. To do this, treatment intervention aim to understand the response of the child in various
increase socially acceptable and pro-socially situations and the level of acceptability of the
behaviour, to decrease or behavioural symptoms, behavioural plan.
and to improve verbal and non-verbal
Making a Behavioural Plan and Implementing: A
communication. It basically aims at increasing the
behavioral plan needs to be formulated, where also
level of independence and increasing the quality
the strategies that are to be used are to be listed
of life. Both language and academic remediation
down and it should be implemented. Along with it
often required. In addition, treatment goal
a close track needs to be kept on whether, the plan
generally include a reduction of disruptive
benefiting the client or is it further accelerating the
behaviour that may be exacerbated especially
client's undesirable behaviours. If it is accelerating
during translation and in school. Children with
the client's undesirable behaviors then, the
mental retardation need intellectual appropriate
techniques should be replaced with one which will
behaviour intervention to be reinforcing socially
help reducing the undesirable behaviors.
acceptable behaviour and encourage self-care skills.
In addition, parents, often distraught, need support Reward System: A reward system needs to be set
and counseling. Insight oriented individual up, depending on what the child considers as a
psychotherapy has proved ineffective. Educational reward and something which in the long run would
and behavioural interventions are currently not lead to another issue. Examples of rewards
considered the treatment of choice. Structured which are safe and encouraging are praise, star on
classroom training, in combination with the hand, food items, etc.
behavioural method, is the most effective treatment
for many autistic children. BEHAVIOUR MANAGEMENT PLAN
FOR AUTISM
Well controlled studies indicate that the gain in the
area of language and cognition and diseases in the Name of the patient: Date:
maladaptive behaviour are achieved by consistent Age: Session Number:
behavioural programs. Careful training of parents
Define the behaviour problem, severity and
in the concept and skills of behaviour modification
obstacles caused by it:
and resolution of the parents' concerns me yield
considerable gain in children's language, and Hypothesis regarding the behaviour problem:
cognitive and social area of behaviour. These Identified skills to be taught to reduce the
training programs, however, are rigorous and behavioral problems:
require much parental time. And autistic child
Antecedent strategies used to put in place to
requires as much structure as possible, and the daily
prevent the behaviours:
program for as many hours as feasible is desirable.
Hierarchy of consequential strategies to use
Behaviour Therapy: when behaviours occur:
Reward system for good behavior:
Behaviour Therapy Plan:
Identification: The behaviours which are Traditional Approaches to
undesirable and which need to be either replaced Deal with Poor Behaviour:
with a desirable behavior or reduced needs to be
Aversives: Aversives are defined as the use of
identified and noted down including the level of
negative reinforcers to interrupt or stop problem
severity in it.
behaviours as and when they occur. Aversives are
Gathering Information: Information need to be response to inappropriate behaviours and not a
gathered from all the family members or caregivers, preventive measure for inappropriate behaviours.
therapists and teachers who are taking care of the For example: When a child is being very
child, teaching or treating the child to understand hyperactive and is disturbing the class the teacher
the behaviours, the pattern of the behavior and the would direct the child to stand outside the class for
situations where and when the undesirable 10 minutes.
behaviours occur.
Overcorrection: This a type of aversive technique
Observation: The child's behaviour needs to be which requires the child to complete the
Autism 235

appropriate behaviour several times because he did Behaviour Strategies: Social Language
not do it when asked, or because he continued with Difficulty:
the negative behaviour in spite of being asked to Autistic children have social language inadequacy
stop. For example: which due to which they may not be able to initiate
Restraints: Restraints are defined as techniques that a conversation or sustain it and it may lead to not
are used to "hold back" a child from doing some having peers or peer group. Social skills deficits
act which is harmful or dangerous to him. For must be addressed in individualized educational
example: If an autistic child has a self- injurious plans, as they are the core symptoms of the autistic
behaviour of banging his head hard on the table or spectrum disorder. [18]
the ground, then this behaviour can be prevented The following are few listed strategies to improve
by having a custom made helmet for the child. This social language:
would either stop the child from banging his head
or at least prevent from inflicting injury to himself. 1. Individualized training of facial expression
and behaving in a socially acceptable manner
Behavior Modification: would help grasp and understand better.
Gradually then exposure to practicing this in
Behaviour Modification is the use of a collection of a group would be really helpful.
techniques that are compiled together to increase
the number of desired behaviours and to extinguish 2. Usage of drama and role playing to teach the
or reduce the number of undesirable behaviours. voice inflection, modulation and facial
Behaviour modification takes account of only those expression is seen to be very helpful. It helps
behaviours which are observable and measureable. to reduce the fear of speaking in public and
The way to change an undesirable behaviour is to helps boosting the child's confidence.
"target the behaviour" and then break them down 3. Use videotapes and flashcards to imitate and
into tiny pieces and steps. After which the tiny practice facial expression. This would also help
pieces of behaviors need to be practiced and to improve eye contact and help increase
rewarded till it is not mastered. Punishments like attention and concentration. For example:
withdrawal of privileges or timeouts are used to Expose the child to various expressions on a
reduce the number of undesirable behaviours. It is flash card and ask him to imitate the
again important to remember that behaviour expression.
modification is a commonly used technique for
4. Peers and siblings can be trained to promote
handling behavioural problems and that it does not
help the client maintain a naturalistic kid
take into consideration the reasons for such
friendly conversation.
behaviours. For example if an autistic child is
exhibiting self-stimulatory behaviour because he is 5. Teach the child how to initiate a conversation,
stressed, then the application of overcorrection may respond and how to sustain it.
lead to replacing one self-stimulatory behaviour to 6. Also, a very important aspect that is poor in
another. autistic children is maintaining eye contact.
Help the child understand the importance and
practice this by reminding the child to
maintain eye contact while role-playing.

Behaviour Strategies: Social Behaviour


Difficulty:
Autistic children have difficulty in displaying
socially appropriate behaviours due to which they
are usually bullied or may face rejection for people
around or they themselves prefer to stay in
isolation. [19]

Behaviour Therapy
236 Neuro-Rehabilitation : A multi disciplinary approach

Behaviour Strategies: Difficulties with


Transitions and Changes:
Autistic children yearn for sameness and routine.
If there is a change in their routine, then they would
try their best to stick to their routine or would throw
temper tantrums. It's seen that children with autism
work better and learn better with people who are
more structured in their daily routine.
The following are few listed strategies to help
autistic children to accept and adjust to transitions:
1. Teach children to adjust and accept transitions
by making small changes in their routine as
and when they get very comfortable with it.
2. Dim the lights of the room, when a new
Group Social Interaction
activity is being presented to them.

The following are few listed strategies to improve 3. Give prior explanation of the transition as
getting them prepared before hand and
social behaviour:
understanding why a particular change is
1. Arrange the situation for the child in such a taking place would help the be prepared rather
way where, he needs to find clues to ascertain than having a flaring temper after the change
how someone is feeling or predict how they has occurred.
will respond. He or she needs to be taught
what various facial expression, emotions and 4. Make the transition as enjoyable and as stress
free as possible because otherwise the child's
how one's sounds in different emotions.
autistic feature will shoot up.
2. Use videotapes or television to reinforce
learning about facial expressions and sounds Behaviour Strategies: Problems with Self-
of emotions. Esteem and/ or Depression:
3. Use role play, group therapy and practice Often children with autism have a poor self-concept
scripts to respond to moments of teasing or and lack confidence. This is normally seen to be
being bullied. present by the child having a poor eye contact,
difficulty socializing or stammering. This is a very
4. Teach the child, the social rules and
common feature in autism as these children
regulations which would help them behave
undergo a lot of rejection from peers, they hear
appropriately to a situation.
people around speaking about their behaviour and
5. Use stories with a moral and explain to the problems and being bullied.
client the story step by step which would help
The following are few listed strategies to help boost
him/her understand the behaviours that are
self -esteem in autistic children:
acceptable socially.
1. Keep a track of the child's behavioural
For example: An autistic patient would have
achievements and encourage it by rewarding
trouble invading someone's personal space, by
the child in front of the family members.
positioning themselves very close to the individual
they are listening to, which would place the speaker 2. If the child is demonstrating poor or
to be very uncomfortable with the close proximity. undesirable behaviours then do not take away
To avoid this vocal reminders need to be given and his previously earned rewards.
in spite of this if there are no changes then, a 3. Reward him/ her for minor changes in the
strategy to solve this would be to tie a rope around behaviour.
the child's waist, with leaving a 3 foot tail behind.
4. Be specific about the behaviours that are liked
Practice this with peers holding the tail and
and encourage them with a reward.
demonstrating to the client the meaning of 3 feet
Behaviours that are undesirable need to be
difference.
changed by desirable ones.
Autism 237

5. Phrases and words like "good boy" or "bad


boy" should not be used rather "You followed
the direction well" should be used to help
increase the confidence.
6. The child's strengths should be motivated and
ways to enhance it should be used. For
example: A child who enjoys music should be
encouraged to learn an instrument and
excelling at playing it.
7. Encourage the client to maintain a photo book
of his achievement or good behaviours. For
example: If he/ she won some competition or
made some clay model, take a picture and keep
it in front of the child.
8. When an autistic child is using negative words, Cores Chart for managing stress
phrases or sentences referring to himself
encourage the child to make changes in his/ Behaviour Strategies: Attention Problems:
her dialogues to a more positive view about
Attention problems can lead to poor behavior. It is
himself/ herself.
often assumed that a child is non-compliant when
9. The child's grades, health concerns and short he or she does not pay attention to directions or is
comings should be kept confidential and not non-focused when a task is being performed.
publicized about.
1. The desk on which the child sits to study
should be dark in colour and should have very
Behaviour Strategies: Managing Stress and
few items on the desk. It is seen that dark
Anxiety:
colour desks are said to be able to maintain
Helping children with autism managing stress and attention for a longer time.
anxiety is extremely important in preventing
undesirable behaviours. 2. A separate room should be allotted to the
child, to sit and study. The room should have
1. Maintain a time- table for all the daily activities less furniture and the room should not be of a
and keep a reward system for it. very bright colour. While the child is studying
2. Teach the child breathing exercises, help him the windows should be closed as most of the
understand when to use it and practice it, so autistic children love to stare outside the
that the child is aware that he has to use it window.
when he is stressed and anxious. 3. Demonstrate to the client how one's boy
3. Exercise and play session is extremely should look when one is paying attention i.e.
important for the child, as it would help the modeling technique should be used.
client relieve his / her stress. 4. The child's interests should be known in term
4. Self- talk method should be taught to the client of activities with high interest and low
and he should be also taught that this should interests. The low interest activities should be
be only practiced when he is alone in the room. separated with breaks.

5. The client should have some hobby which he 5. Arrange for tests and difficult assignments to
or she enjoys and feels relaxed when taking it be completed in quiet rooms away from
up. distractions.
238 Neuro-Rehabilitation : A multi disciplinary approach

Cognitive Rehabilitation to increase attention span


and level of comprehension.

Strategies: Improve Academic Skills


Autistic children have academic problems
especially if they fall into the category of mental
retardation or if they have learning difficulties.[20]
1. Modify the homework and class work
assignments for the child. Reward
2. Use different modalities to teach in a way
which will help the child grasp and Rewards can include:
understand better like audio and visual Edible reinforcers: fruits, biscuits, chocolates, etc.
modality i.e playing nursery rhymes with Material reinforcers: toys, stickers, star on the hand,
video. etc.
3. Create motivators and rewards that are clearly Social reinforcers: smiling, patting, praising, etc.
established and known to the child.
Activity reinforcers: play time, or T.V. time, etc.
4. Have individualized and personalized session
with the client to make the child understand Reward Systems:
concepts in a better way.
Token Economy: When a child completes a task or
5. If the child is comfortable with a certain special activity assigned to him successful he is awarded
educator or a teacher see to it that he/ she is with a token for example a poker chip, which he
there with the client throughout the academic can exchange for various things like toys or T.V
year. Changing teachers or educators would time.
confuse and upset the child as autistic children
Points earned versus points lost: This is where the
if they get attached to a particular educator
child earns points on daily basis i.e. if a child's
they can be very high chances of improvement
behaviour is good then he receives a point but if
in them.
his behaviour is unacceptable then a point is
subtracted. The list of acceptable and unacceptable
Rewards and Motivators: behaviours should be specified to the child.
A very crucial aspect of any behavior modification
Sticker Card: The child here is presented with a
/ management plan is to have reinforcements listed
sticker card consisting of 20 grid rectangle. If the
down which would encourage a child to have a
child demonstrates good behaviour then he receives
desirable behaviour and eliminate or reduce a sticker and after the completion of card he can
undesirable behavior. Punishment teaches children
trade it for a reward.
with autism that they have done something wrong
and it does not teach them the right behaviour but Positive notes: A positive word communicated to
instead has a bad impact on their self-esteem and an autistic child is very encouraging but autistic
confidence. children are visual learners and if the same words
are put on papers then it would help boost the
child's confidence.
Autism 239

Above mentioned are a few reward systems for The goal of the session is to find ways to motivate
autistic children, it is very important to understand the child with the help of a number of strategies
that a meaningful reward would encourage a child and to ensure that the session is enjoyable and
to have a good behavior and maintain it. Initially productive. The purpose of the ABA program is to
the child may be very excited about the reward but improve the language skills, play, and socialization
eventually may lose out interest in the reward, at and to eliminate or reduce self-injurious or
this time the rewards should be revised. It is ritualistic behaviours that interfere with the
important that the child understands the reward learning process. It also focuses on to improve the
system. eye contact and encourage on the desire to learn.
Even if the child does not achieve a "best outcome"
Consequences: result of normal functioning levels in all areas,
nearly all autistic children benefit from intensive
Like rewards are an important factor in
ABA programs.
contributing to the child's good behaviour, in the
same way consequence is equally important for the Specific targets of the interventions are chosen
child to understand that if not behaved in an based on the child's individual issues and disorder.
undesirable manner then he will be punished. In an intensive behavioural intervention program,
goals are set and progress is continuously
Types of Consequences: monitored and evaluated. If there are any positive
• Vocal chastise (no hitting). changes in the child's behaviour then the goals are
eventually changed, and the focus is shifted on to
• Loss of pleasure activities (loss of T.V time). improving other disruptive behaviours.
• Verbal threats or warnings (If you do that Basic Principles of Behavioural and Educational
again, you will be made to sit in the timeout Intervention Approaches:
zone).
Behavioural therapies incorporate specific
• Time-out (the child should be made to sit in approaches to help the individual acquire or modify
the corner of the room, without making any behaviours. The basic theory that ABA is based on
noise for a decided time limit and should think is operant conditioning which involves presenting
about his behaviour and why he was a stimulus (request) to a child, and then providing
punished? If he argues then his time limit a consequence (a "reinforcer" or a "punisher") based
would increase). on the child's response.

APPLIED BEHAVIOUR ANALYSIS: Reinforcement: A reinforcer is anything, which


when presented as a consequence of a response,
Applied Behaviour Analysis (ABA) is based on the increases the probability or frequency of that
theory that influencing a response associated with response being repeated. Examples of possible
a particular behaviour may cause that behaviour reinforcers for young children may include verbal
to be shaped and controlled. The behavioural praise, or offering the child a desired toy.
methods in ABA are used to measure behaviour,
teach functional skills, and evaluate progress. There Punishment: A punisher is a consequence that
are various studies which suggest the success of decreases the probability or frequency of that
ABA technique in treating deficits in the behaviours response. Possible punishers for young children
of children with Autism Spectrum Disorder (ASD) may include verbal disapproval or withholding a
at any level. desired object or activity. The term "punisher" is a
technical term used in behavioural therapy and
Approaches of Applied Behaviour Analysis: does not imply the use of punishment (physical
abuse such as hitting, slapping, spanking, or
1. Discrete trial training (DTT)
pinching).
2. Pivotal Response Training (PRT)
It is necessary to keep in mind that, reinforcers and
3. Picture Exchange Communication System punishers are different for each child. Many
(PECS) different specific behavioural and educational
4. Self-Management techniques have been used as part of interventions
for individual children with autism. These
5. Social skills training techniques techniques are effective in a wide body of research
240 Neuro-Rehabilitation : A multi disciplinary approach

based on a common set of behavioural and learning respond to multiple cues, and that development of
principles. Behavioural interventions involve the these skills will result in overall behavioural
therapist controlling the activity and/or improvements.
consequences to shape the child's responses.
The primary pivotal areas of pivotal response
therapy involve:
Discrete Trial Teaching (DTT):
This is an ABA based therapy which was developed 1. Motivation
in the 1970's by psychologists Ivar Lovaas, and 2. Initiation of activities
Robert Koegel, at the University of California at Los
3. Self - Management
Angeles (UCLA). This principle makes use of the
idea that when children with autism are rewarded 4. Feeling to respond to multiple cues
for desired behaviors, they are likely to repeat that
5. Ability to respond to multiple cues
behaviour. For example: the therapist gives the
child a motivating cue, such as a request to stand The PRT training is child - directed: where the child
still or maintain eye contact, along with a correct makes the choice in which direction to direct the
response. The therapist then rewards the child by therapy session. Emphasis is also placed upon the
a praise, toys or food to reward the child for role of parents as primary intervention agents. This
completing the task. training focuses on increasing a child's motivation
to participate in learning new skills.
Discrete Trial Teaching consists of a series of distinct
repeated lessons or trials taught one- to - one. Each Pivotal response training involves specific
trial consists of a prior, a "directive" or request for strategies such as
the individual to perform an action; a behaviour, • Clear instructions and questions presented by
or "response" from the person; and a consequence, the therapist
a "reaction" from the therapist based upon the
response of the person. Positive reinforcers are • Child choice of stimuli (based on choices
selected by evaluating the individual's preferences. offered by the therapist)

Many children initially respond to, recognizable or • Intervals of maintenance tasks (previously
concrete reinforcers such as food items. These mastered tasks)
concrete rewards are faded as fast as possible and • Direct reinforcement (the chosen stimuli is the
replaced with rewards such as praise and hugs. reinforce)
Early intensive behavioural intervention such as the
Lovaas program is usually implemented when the • Reinforcement of reason for purposeful
person is young, before the age of six. Parent attempts at correct respond
training is a necessary part of an effective ABA- • Turn taking to allow modeling and
based program. A maintenance schedule allows for appropriate pace of interaction
periodic checking so the person does not regress in
Pivotal response training has a naturalistic training
mastered skills. Discrete trial training is a technique
method that is structured enough to help children
that can be an important element of a
learn simple through complex play skills, while still
comprehensive educational program for the
flexible enough to allow children to remain creative
individual with autism spectrum disorder. In some
in their play. The child can be reinforced for single
cases, a much less intensive, informal approach of
or multiple step play. The therapist has the
discrete trial training may be provided by a
opportunity to model more complex play and
knowledgeable professional to teach specific skills
provide new play ideas on his/her turn.
such as sitting and attending.
Reciprocal Imitation Training:
Pivotal response therapy (PRT):
Reciprocal imitation training (RIT) is a variation on
This therapy is also referred to as pivotal response
the pivotal response training procedure for teaching
treatment or pivotal response training, is a
play skills. This training was developed to teach
behavioural intervention therapy for autism. The
spontaneous imitation skills to young children with
main principle of this therapy suggests that
autism in a play environment; however, this
behaviour links primarily on two 'pivotal'
intervention technique has also been shown to
behavioural skills, i.e. motivation and the ability to
increase pretend play actions. This procedure
Autism 241

includes unexpected simulation in which the other individuals, typically adults or siblings,
therapist imitates actions and vocalizations of the performing target behaviours
child.
Applications of video modeling as an intervention
technique are now being extended to teaching and
Self-Management Training:
increasing play in children with autism.
This is an additional approach for teaching children
with autism to maximize in independence and Therapeutic Use of Applied Behavioural
generalization without increased reliance on a Analysis:
teacher or parent. Self-management typically
It is extremely important to have a trained ABA
involves some or all of the following components:
therapist and to give adequate training to the
self-evaluation of performance, self-monitoring,
parents or caregivers. ABA is the best method to
and self-delivery of reinforcement. Ideally, it
manage the undesirable and anomalous autistic
includes teaching the child to monitor his/her own
behaviours such as: self-injurious, repetitive,
behaviour in the absence of an adult. This therapy
ritualistic, aggressive and disruptive behaviours.
uses a self-management treatment package to train
This approach has been seen and studies to
school-age children with autism to engage in
extinguish or reduce these behaviours and promote
increased levels of appropriate play. Self-
alternative pro-social behaviours simultaneously.
monitoring procedures have also been used to
The process of ABA is successful as it breaks
increase social initiations while reducing disruptive
complex tasks into smaller parts making them less
behaviour and to increase independent interactions
daunting for the child. ABA can also be used to train
with typical peers.
a child to learn a new adaptive behaviour, such as
In a study children displayed very little dressing and toileting and to promote functional
independent appropriate play before training, and communication.
typically engaged in inappropriate or self-
ABA whether they are observed or taught they
stimulatory behaviour when left on their own. With
function on 3 aspects:
the introduction of the self-management training
package, the children increased their appropriate • Antecedent (A) - what triggered a behaviour
play in both supervised and unsupervised settings, or what happens before the behaviour,
and across generalization settings and toys. • Behaviour (B) - the behaviour itself, and
Decreases in self-stimulatory and disruptive
behaviours were maintained in the unsupervised • Consequence (C) - what happens after the
environments. behaviour.
The consequence is whatever the behaviour
Video Modeling: accomplishes, for example it can be getting
This is like vivo modeling which uses predictable attention (negative or positive) or relief of stress.
and repeated presentations of target behaviours; The consequence is not always obvious, especially
however, these behaviours are presented in video in the case of unusual behaviour (odd behaviours
format, thus reducing variations in model the child does such as arm flapping or repetitive
performance. Studies suggest that video modeling actions), which is why keeping data is helpful to
improves various skills in individuals with autism, identify what the function of the behaviours are, as
including conversational speech like verbal well as what triggers them.
responding, helping behaviours and purchasing
Applied Behavior Analysis assessment focuses on:
skills. This medium has also been claimed to
increase vocabulary, emotional understanding, • Exactly what behaviours are performed by the
attribute acquisition, and daily living skills. child
Video modeling interventions can be used in 2 • When these behaviours are performed
forms: • At what rate the behaviours are occurring
1. Self-as-model: In this individuals act as their • What happens before and after the behaviours
own models and the video is edited so that • What purpose does the behaviour serves
only desired behaviours are shown.
Skills that are to be promoted are broken down into
2. Other-as-model methods: This employs taping small sequential steps. The ABC principles of
242 Neuro-Rehabilitation : A multi disciplinary approach

behaviour intervention are used to teach the child learns to perform the trained behaviour
each step: independently.
• A (antecedent): Each instruction is given iii. It is also important to change the context of
clearly, in as few words as possible. Assistance teaching (different people giving the
is provided; for example prompting through antecedent, more people around, different
demonstration or physically guiding. situations, etc.) in order to generalize the
learned behaviours.
• B (behaviour): An appropriate behaviour is
observed. iv. When each behaviour consisting of single
sequential steps are acquired, the person is
• C (consequence): A consequence is an
taught to combine them to produce more
outcome that will reward the child and
complex behaviours.
increase the likelihood that the behavior will
be repeated again in the future, also called a v. Problematic behaviours are not reinforced;
positive reinforcer. instead the child is consistently redirected to
engage in appropriate behavior.
vi. The child's responses during each step are
meticulously recorded. The information is
later used to determine if the child is
ABC Model progressing at an acceptable rate. If progress
is not satisfactory, the learning steps are
This "ABC" process is repeated f for each behaviour
analyzed for possible flaws and the program
both in structured teaching situations and in the
modified.
course of everyday activities using PRT techniques.
i. Instructions are given to emphasize Applied Behaviour Analysis: Example of the
metacognition (learning how to learn). In this teaching procedure:
case learning how to listen, to watch, to Objective: The child will be able to make a chutney
imitate, to ask and do. cheese sandwich.
ii. As the child's learned behaviours improve, the Materials Required: Bread slices, butter, chutney,
structured guidance is systematically reduced cheese, knife and plate.
and the prompts are used less frequently and
eventually faded out. This so that the child Number of trials in set: Up to 5

Teaching Procedure:
Step Presentation Response Consequence
1. Mother making a sandwich
2. Tell the child to make the sandwich one at Child performs part of Correct = Praise
a time: each step with
1. Get the plate and the knife and assist him. assistance. Incorrect = Error
2. Get the chutney, butter and cheese from interruption,
the fridge and then assist him. 3 consecutive trial re-administer with
3. Get the bread out of the bag and assist him. increased prompts
4. Open the butter packet and take cheese
slices and assist him. No response= System
5. Spread the butter and assist him of most prompts
6. Spread the chutney and cheese slice and a Re- administer with
ssist him. increased prompts
3. Tell the child to make the sandwich The child is able to Same
Model the steps " 2 - 5" perform step 1 without
any prompt.
4. Tell the child to make the sandwich The child is able to Same
Model the steps " 3 - 5" perform step 1 and 2
without any prompt.
Autism 243

5. Tell the child to make the sandwich The child is able to Same
Model the steps " 4 - 5" perform step 1, 2 and 3
without any prompt.
6. Tell the child to make the sandwich The child is able to Same
Model step " 5" perform step 1, 2, 3 and
4 without any prompt.
Tell the child to make the sandwich Child performs step 1 - 5 Same
without any prompts.

Social Stories: Social stories are used to teach social of the brain that are used for social skills. [20] Some
skills through the use of accurate information about researchers believe that individuals with autism
the situations that the child with autism may find have trouble understanding what others believe,
difficult or confusing. Social stories can be used for know, or don't know. This difficulty is sometimes
different purposes like to prepare the child for an called the theory of mind deficit in autism. [21]
upcoming routine, or learn to communicate Recent research studies show that social stories can
appropriately in social situations. The concept help reduce problem behaviours, increase social
behind this form of therapy is that the child awareness, and/or teach new skills. In some cases,
rehearses the story ahead of time with an adult, the new behaviours were maintained and
which would help the child to act in an appropriate generalized to other situations, even after the story
socially acceptable way when the situation arrives. was faded out. Social stories are most useful for
children who have basic language skills. [22]
Social Stories use different types of sentences for
example:
Floortime (DIR):
1. Descriptive Sentence: This explains the, who, This therapeutic technique which is based on the
what, where and why about the situation, Developmental Individual Difference Relationship
which helps the child recognize the situation Model (DIR) was formulated by Dr. Stanley
as and when it occurs. For example: There will Greenspan in 1980s. The premise of floortime is that
be many shoes to choose from. an adult can help a child increase his circles of
2. Directive Sentences: This suggests the communication by meeting him at his
appropriate social response in the situation. developmental level and building on his strengths.
For example: When I decide about the shoes, I Therapy is often incorporated into play activities
will tell the grown-up. on the floor.
3. Perspective Sentences: This describes the There are six developmental milestones that
possible feelings or the response. For example: contribute to emotional and intellectual growth:
I might not know which shoes I like.
1. Emotional Ideas
4. Affirmative Sentences: This describes the laws
2. Emotional Thinking
or rules that are commonly shared. For
Example: That is okay with everyone. 3. Complex communication
5. Cooperative Sentences: Describe how other 4. Two-way communication
people will help out in the given situation. For
5. Self -regulation and interest
example: The grown-up will go get the shoes
for me. 6. Intimacy or a special love for the world of
6. Control Sentences: This helps the child human relations
remember strategies that work for him or her. The therapist or parent engages the child at a level
For Example: I can hold onto my string while where the child enjoys the activities and the
I decide. therapist then follows the child's lead. The parent
is instructed how to move the child toward more
Theory behind Social Story: increasingly complex interactions, a process known
Autistic individuals have difficulty with reciprocal as opening and closing circles of communication.
social interaction and this impairment might result Floortime does not separate and focus on speech,
from unusual activity or functioning of certain areas motor, or cognitive skills but rather addresses these
244 Neuro-Rehabilitation : A multi disciplinary approach

areas through a synthesized emphasis on emotional Progress or a successful outcome is noted through
development. The intervention is called Floortime improved performance (or adaptation), enhanced
because the parent gets down on the floor with the participation in necessary or meaningful daily
child to engage him at his level. Floortime is activities, personal satisfaction, improved health
considered an alternative to and is sometimes and wellness, and successful transitions to new
delivered in combination with behavioural situations and roles. These measures can help the
therapies. Floortime is usually delivered in a low individual, family, and team appreciate success and
stimulus environment, ranging from two to five refocus and change priorities of the intervention
hours a day by a psychologist or a special educator. plan as needed.
Occupational therapy practitioners help people
Role of occupational Therapy in Autism
with autism adjust tasks and conditions to match
Occupational Therapy their needs and abilities. Such help may include
Occupational therapy services focus on enhancing adapting the environment to minimize external
participation in the performance of activities of distractions, finding specially designed computer
daily living (e.g., feeding, dressing), instrumental software that facilitates communication, or
activities of daily living (e.g., community mobility, identifying skills they need to accomplish tasks
safety procedures), education, work, leisure, play,
and social participation. For an individual with an Occupational Therapist focuses on:
ASD, occupational therapy services are defined Evaluating an individual to determine whether he
according to the person's needs and desired goals or she has accomplished developmentally
and priorities for participation. [23] appropriate skills needed in such areas as grooming
and play and leisure skills.
Occupational therapy services for individuals with
an ASD include evaluation, intervention, and • Provide interventions to help an individual
measurement of outcomes. Throughout the respond to information coming through the
process, collaboration with the child or adult with senses. Intervention may include
autism, family, caregivers, teachers, and other developmental activities, sensory integration
supporters is essential to understanding the daily or sensory processing, and play activities.
life experiences of the individual and those with • Facilitate play activities that instruct as well
whom he or she interacts. Occupational therapy as aid a child in interacting and
services can focus on personal development, quality communicating with others.
of life, and the needs of the family and individual.
• Devise strategies to help the individual
The occupational therapy evaluation process is transition from one setting to another, from
designed to gain an understanding of the one person to another, and from one life phase
individual's skills-his or her strengths and to another.
challenges while engaging in daily activities • Collaborate with the individual and family to
(occupations). The occupational therapy identify safe methods of community mobility.
intervention process is based on the results of the
evaluation and is individualized to include a variety • Identify, develop, or adapt work and other
of strategies and techniques that help clients daily activities that are meaningful to enhance
maximize their ability to participate in daily the individual's quality of life. [24]
activities at home, school (if relevant), work, and
Assessment:
in the community environment. The evaluation
process looks at the child's development in a A combination of standardized evaluations and
number of domains including motor, perceptual, observations of behavior provides a holistic picture
communication and interaction skills; habits, and of child's ability and needs.
routines. An understanding of the child's abilities, Assessment strategies -
needs, and goals is gained through interviews with
Assessment of sensory integration for a child with
the child, parents, siblings, teachers, and/or
ASD includes:
caregivers; standardized tests; and observation of
the child during activities at school and home such • Evaluation of sensory responsiveness
as classroom tasks, mealtimes and play. (Over,under,or labile responsiveness)3
Autism 245

• Sensory preferences (likes and dislikes) [25] • Research suggests that the behaviours of
children/youth with ASD have a significant
• Ability to attach meaning to sensation and use
impact on family roles and activities (Werner
of sensation for adaptive behavior
DeGrace, 2004). The effect of having a child
• Assessment of praxis with ASD varies among family members and
• Assessment of participation in daily living depends on available community supports
activities (Galvin-Cook, 1996). Families are devoted to
the needs of the child and consequently may
Useful Assessment Tools: have difficulty engaging in positive family
experiences (Werner DeGrace, 2004).
In autism, it is important to consider both
structured and unstructured evaluation with
Treatment Effectiveness:
children in Autism Spectrum.
Research in the area of autism has developed a great
Children with high functioning ranges often are deal over the past decade. There are currently
able to be tested with structured evaluation of several studies going on within Canada which have
sensory integration such as SIPT (Sensory potential to provide valuable information for the
Integration and Praxis Test) planning and implementation of health services for
these children and their families.
Useful assessment strategies for evaluating
Sensory processing deficits: Intervention for Challenging Behaviour
• Skilled observation in school, home, and other In the past, the use of intrusive treatments, such as
environments.[26] seclusion or restraints, were once used to treat
• Sensory histories/ questionnaires such as problem behaviour (Perry et al., 2003). It is now
sensory profile the practice to use non-intrusive methods
emphasizing positive-based strategies. Intrusive
• SIPT (Ayres,1989) for those children with methods are used only as a last resort (when non-
intelligence in normal range. intrusive methods are ineffective) (Perry et al.,
• (Sensory Integration and Praxis Test) 2003).
This review concluded that the most efficacious
Sensory Intervention mainly focuses on:
strategies for problematic behavior in children with
• Helping to organize behavior autism spectrum disorder were: behavior
• Improving feedback about body enhancement strategies, behavior reduction
strategies, educational strategies, and ecological
• Helping the child attach meaning of sensation strategies (NIH 1991- Canada).
• Providing foundation for praxis The Children's Mental Health Ontario completed
• Encouraging rapport and social interaction an analysis of interventions for challenging
behaviours in children with autism (Perry et al.,
• Incorporating the child's sensory needs into
2003). Positive behavioural supports, which are
his/her daily life
non-intrusive methods for treating such behaviour,
Occupational Therapy Intervention for Children were recommended as the first course of treatment.
with Autism Several studies conducted in Ontario support its
The goal of occupational therapy interventions is effectiveness in treating symptoms of self-injury,
to enable individuals to participate in everyday aggression, and disruptive behaviour (Perry et al.,
occupations. This may be achieved through a range 2003). Horner et al (2002), in a research synthesis
of therapy approaches such as modification of tasks on problem behaviour intervention for young
and/or the environment to match individuals' children with autism, found that the most common
abilities, developing skills such as posture and problem behaviours were aggression/destruction,
coordination, or development of daily routines to disruption/tantrums, self-injury, and stereotypy.
facilitate adaptive behaviours. [27] Occupational Stimulus-based or instruction based interventions
therapists work with children in their natural are the most common form of treatment for autistic
settings; in the home with their families, in the children with problem behaviour. Horner et al.
school and in the community. (2002) also reported that early use of behavioural
246 Neuro-Rehabilitation : A multi disciplinary approach

interventions may reduce problem behaviour by 80- the aim of sensory integration therapy is to
90%. improve the ability of the brain to process sensory
information so that the child will function better
Comprehensive Programs: in his daily activities.
Intensive Behavioral Intervention (IBI) has been In the past, one of the more frequent sensorimotor
researched extensively and has been shown to be interventions in occupational therapy was sensory
an effective intervention with children with autism. motor integration. There is evidence that children
This intervention teaches children to respond to with autism do process sensory information
specific words and environmental stimuli using differently from other developing children
repetition. According to Couper and Sampson (National Institute of Child Health and
(2006), IBI is thought to provide a superior outcome Development, 2005- Canada). Therefore, the focus
since it specifically targets the deficit areas in in occupational therapy has shifted to
children with autism. Treatment needs to become understanding how and when a child is reacting
more naturalistic, and within the child's own poorly to a sensory experience and structuring the
environment. environment to accommodate or minimize such
reactions.
Occupational therapy treatment has focused on
Occupational therapists can use a mediator or
two main areas:
consultation approach to work with parents and
1. Sensory Motor Integration teachers to provide strategies to prevent reactions
Sensory processing is the normal neurological to sensory experiences from limiting daily activities.
function that all people experience when their brain By adapting the tasks and environments as well as
processes sensory information from the working with the families on how to teach new
environment around them. Most of us skills and build calming or alerting activities into
unconsciously learn to combine our senses (sight, their everyday routines, occupational therapists can
sound, smell, touch, taste, balance, body in space) make a difference in the family's day to day life. In
in order to make sense of our environment. particular, occupational therapy focuses on self care
Children with autism face difficulty while doing issues, feeding, bathing, hygiene and sleep which
this. are significant issues for children and enormous
Sensory integration therapy is a type of stressors for the family.
occupational therapy (OT) that places a child in a In the school setting, a student's occupational
room specifically designed to stimulate and performance may be impaired by sensory,
challenge all of the senses. During the session, the developmental, attentional and/or learning
therapist works closely with the child to encourage challenges (Sahagian Whalen, 2003). Occupational
movement within the room. therapists may adapt classroom tasks and the
Sensory integration therapy is driven by four key school environment to promote a child's
principles participation.
Occupational therapists can assist teaching
1. The child must be able to successfully meet
assistants and teachers with understanding the
the challenges that are presented through
impact of sensory processing difficulties on daily
playful activities (Just Right Challenge);
functioning and how they can modify what they
2. The child adapts her behavior with new and do to maximize the child's participation and reduce
useful strategies in response to the challenges behavioural difficulties.
presented (Adaptive Response);
As some children with autism find changes to
3. The child will want to participate because the routines, or unstructured time difficult to
activities are fun (Active Engagement); and comprehend and adjust to, environmental supports
4. The child's preferences are used to initiate and structures can improve the quality of life for
therapeutic experiences within the session children with autism.
(Child Directed). Common sensory integrative -related behaviors
Sensory integration therapy is based on the of children with ASD [29]
assumption that the child is either overstimulated • Difficulty intiating and maintaining social
or understimulated by the environment Therefore, interaction and relationships
Autism 247

• Communication impairments, including ranging from extreme sensitivities to considerable


language delay or echolalia lack of responsiveness. They may also demonstrate:
• Repetitive, stereotypical play Inability to cope with unexpected or intense
sensations
• Often visually focused
• Difficulty registering and attending to salient
• Sometimes good motor skills
sensory input
• Difficulty calming and regulating states of
• Many children with autism demonstrate
arousal
difficulty maintain selecting and allocating
• May have cognitive deficits attention due to poor registration of sensory
input (courchesne et al,1994)
• Confusion about the effect and consequences
of behaviors • Heightened sensitivities
• Variability in reactions to sensation
Common occupational performance difficulties
[30] • Gravitational insecurities

• Poor social participation due social isolation • Sensory- seeking and sensory- avoidance
or poor social skills behaviors in relation to movement, audition,
touch, smell and taste
• Difficulty initiating and maintaining social
interaction and relationships • Self stimulatory behaviours
• Problems in processing tactile information
• Repetitive, stereotypical play
• Strengths in visual memory and visual
• Some children having difficulty with daily life manipulation of objects
skills, such as eating a healthy selection of
foods or wearing a variety of weather • Auditory processing problems
appropriate clothing due to atypical sensory • Motor planning deficits resulting in:
responsiveness
• Poor ability to initiate new ideas for play
• Difficulty with adaptive behaviors, such as
toileting, eating and other daily life skills • Disorganized behavior

• Academic problem due to cognitive deficits • Inability to carry out daily routines
independently
• Poor sleep-wake, cycles that interfere with
daily routines • Poor initiation, especially mouth and facial
gestures (Poor oral praxix)
• Engagement in restrictive, repetitive, and
potentially self- injurious behaviours and Poor sensory processing may affect the child's
interests that limit the child's ability to learn ability to participate in his or her daily life activities,
and fit in with peers including self care activities, such as brushing his
or her teeth, eating and social activities (eg. Playing
• Delayed play skills often results in delays in with his or her peers)
learning and social skills
Many children with ASD demonstrate difficulties
• Communication deficits, including language with praxis in general, but especially ideational
delays or echolalia, limit opportunities for praxis. Ideational praxis is essential for self-initiated
social participation and play play, and thus poor ideational praxis may play role
• Difficulty carrying out daily living tasks, such in the tendency for children with ASD to prefer
as playing with other children, participating sameness and routine.
in mealtime with family, and participating in Poor sensory processing associated with impaired
family routines and outings. motor planning cause difficulty in imitation and
poor social reciprocity.
Key considerations in using a sensory
integration approach with children with ASD Intervention to Organize Behaviour
Children with ASD may demonstrate a wide Useful sensory activities help child become calmer
variety and range of sensory processing difficulties and more organized
248 Neuro-Rehabilitation : A multi disciplinary approach

Occupational therapist provides proprioceptive • Apply lotion after bath with firm pressure
and deep pressure activities, linear vestibular input
• Warm towels/cloth with blow dryer after bath
to become calmer and more organized.
• Walk barefoot outdoors on variety of surfaces
Intervention to improve Sensory Feedback from • Draw letters and shapes with finger on a carpet
the Body square and erase them with hands or feet
Many children with autism have poor body
• Have child identify shapes, letters, and
awareness because they are not obtaining adequate
numbers drawn on his or her arm, leg or back
somatosensory feedback from their body (ie, tactile
proprioceptive, vestibular and interocetive Activities to improve Proprioceptive Sensation
sensations). Occupational therapist give activities,
Proprioception is the sensation that has been
which are rich in somatosensory sensations and
observed to help children regulate their
provide foundation fpr body awareness and motor
responsiveness to sensation ( Balance &
planning needed for praxis.
Schaaf,2001). Proprioception occurs when we move
our muscles. Movement of our muscles against
Activities to Improve Tactile Sensation
gravity or against a weight increases the amount
Tactile activities help children gain awareness of of proprioceptive stimuli.
their bodies and are useful for helping children
learn to move and manipulate their body and hands Home Activities to improve proprioception
in a coordinated, planned manner.

Activity to improve tactile sensation Pushing the wall to increase


(clay dough activity) proprioception

Activities rich in tactile sensations: • Play on pull-up bars


• Go to petting zoo • Jump on trampoline
• Messy play with hands in pudding, ice creams, • Use in-line skates or roller skates
mashed potatoes, cookie dough, etc. • Ride a scooter or bicycle
• Find objects hidden in a container of a sand, • Jump or climb in and out of inner tubes
beans, macroni, rice, corn packing peanuts,
feathers and pompom balls, foam wedge, • Participate in climbing activities
buttons and beads, dirt, flour and sugar • Play running and jumping games
mixture or cinnamon and oatmeal etc.
• Swing from trapeze bar
• Water play with squirt guns, water balloons
and spray bottles • Play in sand box with damp, heavy sand

• Write and colour with vibrating pen • Use cardboard boxes with blankets and
pillows to make forts
• Textured balls, cloths, mitts
• Put heavy objects in a cardboard box and have
• Feel magic bags filled with surprises the child push it through cone- driving a car
• Finger paint- add texture with sand or rice for • Play catch with heavy ball, or bounce and roll
variety heavy ball
Autism 249

• Things to climb on • Roll, walk, or run up a hill


• Balls • Wheelbarrow walking
• Crash cushions • Open and close door for people
• Mats • Walk up a ramp or incline
• Rock wall • Animal walk (Crab walk, bear walk)
• Large bolster lying on angle
Activities Rich in Vestibular sensation:
• Tables and chairs Vestibular activities include any movements that
• Rope ladder involve the head moving through space. To
stimulate the vestibular system use activities that
• Large inflated mats/coushions
are stop and go and that occur in a variety of planes.
• Large boxes For the child who is overly sensitive to vestibular
movement, or who needs calming, use vestibular
• Push against a wall
sensations that are in the linear plane and that are
• Fill up big toy trucks with heavy blocks, slow and rhythmical like swinging or rocking.
pushing with both hands to knock things
down

Sensory Integration Room

Swinging and Sliding Activities:


• Have the child sit on a swing and drop
beanbags placed between knees or feet onto a
Swinging - Vestibular Stimulation target
• Have the child stand on a swing (with
Transition Activities to improve Proprioception:
supervision) and jump off into pillows or inner
tube
• To teach pumping, have the child extend legs
as swing moves forward so therapist can push
on bottom of his or her feet
• Hang the swing from single point so that
movement occurs in more planes, including
rotational plane
• Use sitting disc (inflated, cushioned disk) for
child during homework or mealtime
• Ride tricycle/bicycle
• Jump on a trampoline
Wheel Barrow Walking
250 Neuro-Rehabilitation : A multi disciplinary approach

• Do cartwheels and/ or somersaults • Ride tricycles and bicycles


• Use rocking horse or rocking chair • Create and complete an obstacle course
• Sit on parent's lap and rock back and forth or • Participate in imaginative play (housekeeping,
bounce up and down fireman,doctor, post office)
• Roll or bounce on big balls • Ask child to perform animal walks
• Swing in hammock- two adults and blanket
• Play "simon says" games
can form a hammock swing for a very small
child • Play balloon volleyball
• Ride a scooter • Play "mirror image" child makes a mirror
• Do "stretching" exercises with head, such as image of another child's posture
rolling head around or side to side • Play "red light/green light"- child run on green
light and stops on red light
Intervention to develop praxis- • Play "follow-the-leader"- leading and
It is very important for children with ASD to following
develop praxis skills because they are needed to
manage everyday situations that are constantly Encourage Rapport and Social Interactions
changing. Even routine activity, such as riding the The child's enjoyment and motivation during
bus to school, requires that the child adapt to new sensory-rich activities can improve a child's
stimuli and situations. (different children, different tolerance for, and satisfaction in , social arenas.
driving conditions, different seat mates, different Motivating sensory experiences can be used to elicit
seat belts). eye contact, social cuing, or social language for the
Occupational Therapist help child to develop praxis child.
by providing him or her with varied sensory
2. General Skill Building
experiences grounded the organizing adaptive
responses. Once a desired level of organization is Occupational therapists work with children with
achieved, introduce the activities that challenge the autism and their families to provide intervention
child , and scaffold sensory experiences to facilitate that improves the child's ability to participate in
skill development. For example expand activity to activities of daily living, play and school. In
include increasingly complex motor planning, occupational therapy, the focus is on task analysis,
interaction, and language skills. breaking down a task into manageable steps for the
child, teaching those steps to the key people in the
Structure the environment for simple adaptive child's environment and structuring task and/or
responses and move to more complex ones, include environment to support successful completion. For
activities that facilitate skills such as imitation, example, if a child with autism is having difficulty
sequencing, bilateral coordination, timing and dressing himself, the occupational therapist could
imitation. use a backward chaining behavioural approach
which teaches skills by breaking the tasks down
Activities to improve Praxis:
into manageable steps which are always performed
Ayes(1989) showed that there is relationship in the exact same order (CanChild, 2006).
between sensory processing and praxis, specifically Occupational therapists work as collaborative
somatosensory processing (Blanche, 2001). Sensory consultants, providing strategies for intervention
information from the body and the environment is to families, teachers, in order to develop a child's
necessary for creating ideas for movement, putting skills within their natural environments.
together the plan and providing feedback during
and following the movement activity. Many Some Proprioceptive rich activities during the
children with difficulties processing sensory course of daily activities and routines:
information also have poor motor planning or • Unpack heavy groceries
dyspraxia. Ayes described some activities which
• Carry heavy items (baskets with cardboard
provide significant somatosensory input, thus
blocks, groceries,etc.)
underscoring the relationship between body
awareness and praxis. • Climb into weighted blankets for bed.
Autism 251

• Push or pull boxes with toys or few books in children with autism was higher using sung rather
it (more resistance is provided if boxes are than spoken text [32].
pushed/pulled across a carpeted floor)
Music as a mode of intervention has yielded
• Take the cushions of the sofas, vacuum under beneficial effects for the modification of behavioural
them, and then put them back ; canalso climb problems. Orr, Myles, and Carlson (1998)
on them or jump and "crash" into them investigated the effect of rhythmic entrainment on
erratic classroom behaviours in their case study of
• Go "shopping" with a child's shopping cart
a girl with autism [33]. Rhythmic entrainment
filled with items, or have the child push the
involves the use of music to aid relaxation by the
shopping cart when you go shopping
introduction of externally produced rhythms,
• Rearrange bedroom furniture designed specifically to re-entrain the body to its
• Put large toys and equipments away natural rhythmic patterns. They found that
rhythmic entrainment helped in reducing
• Do chair push-ups problematic classroom behaviour.
The focus of therapy intervention is consultation
rather than direct intervention.

Speech Therapy:
Children with autism present with delayed speech
and language development, severe social and
pragmatic issues and behavioural problems. Speech
and language problems may range from severe
comprehension issues to inability to express their
needs and wants. They may rely on pointing and
just crying and vocalizing to express himself.
Autistic children have severe problems in social
skills and use of language. Poor eye contact, poor
social smile, solitary play behaviour and irrelevant
speech patterns are very commonly seen in these Music therapy in autism
children.
Even though music therapy has been used with
success, there is still a shortage of theoretical papers
Complementary therapies: that deal with understanding the neural substrates
Complementary therapies are typically used in and cognitive mechanisms underlying the
addition to behavioral and educational approaches. improvement in such adaptive skills [9].
Improvisational music therapy, a form of music
Music Therapy: therapy where there is interactive use of live music
Music therapy is seen to be an effective intervention for engaging clients to meet their therapeutic needs.
for emotional recognition deficits in autism. This is widely used in the treatment of children with
However, researchers have yet to propose for the autism and is gaining growing recognition as an
neurological and cognitive components that are effective intervention addressing fundamental
responsible for such improvements. Individuals levels of spontaneous self-expression, emotional
with autism show poor perception of affective cues communication and social engagement for
within the social domain and experimental individuals with a wide range of developmental
evidence suggest that such individuals fail to disorders. Within this therapeutic intervention, the
interpret and recognize vocal and facial expression music therapist identifies musical elements
of emotions. The two essential goals when music (temporal beat, rhythmic patterns, dynamics of
therapy is conducted for individuals with autism expression, pitch range and melodic contour) in the
is improving communication/ language and child's musical and non-musical behaviour, and
improving socio-behavioral skills (Kaplan & Steele, then provides a predictable, empathic and
2005). Buday (1995) observed that the number of supportive musical structure to attract and engage
signed and spoken words correctly imitated by the child
252 Neuro-Rehabilitation : A multi disciplinary approach

Art Therapy: All too often, it's assumed that a non-verbal person
or a person with limited verbal capabilities is
What Is Art Therapy? incompetent in other areas. As a result, people on
According to the American Art Therapy the autism spectrum may not be exposed to
Association, "art therapy is a mental health opportunities to use artistic media -- or the
profession that uses the creative process of art opportunities may be too challenging in other ways
making to improve and enhance the physical, (in large class settings, for example). Art therapy
mental and emotional well-being of individuals of offers an opportunity for therapists to work one-
all ages. It is based on the belief that the creative on-one with individuals on the autism spectrum to
process involved in artistic self-expression helps build a wide range of skills in a manner which may
people to resolve conflicts and problems, develop be more comfortable (and thus more effective) than
interpersonal skills, manage behavior, reduce spoken language. Group Art based therapy will
stress, increase self-esteem and self-awareness, and improve social interaction and will help the child
achieve insight." vent out his emotions.

For children and adults with autism, it can be a very


useful way to emote and express themselves.

Group Art Based Therapy

Play Therapy:
If play is a child's language, then toys can be thought
of as the words. Through play therapy the child
can work through their challenges and issues using
Art Therapy in Autism the toys that they choose, revealing their inner
dialogue. Through play the child is able to test out
Why Use Art Therapy to Treat Autism? various situations and behaviors in a supportive
One of the hallmarks of autism spectrum disorders environment. Unconditional positive regard and
is difficulty with verbal and social communication. acceptance encourages the child to feel safe enough
In some cases, people with autism are literally non- to be able to explore their inner selves without
verbal: unable to use speech to communicate at all. censorship. In this environment children are able
In other cases, people with autism have a hard time to try out different roles, work through conflicting
processing language and turning it into smooth, emotions and thoughts, and try to figure out what
easy conversation. People with autism may also the world is like. The child is able to form a
have a tough time reading faces and body language. relationship with the provider, and through this
As a result, they may have difficulty with telling a relationship they are able to develop trust,
joke from a statement, or sarcasm from sincerity. improved self-esteem, and self efficacy.
[34] In non-directive play therapy, the child is in control,
Meanwhile, many people with autism have an within some gently but firmly set limits. Children
extraordinary ability to think visually - "in pictures." often feel that they do not have control over
Many can turn that ability to good use in processing situations in their lives. Through play therapy they
memories, recording images and visual are able to work through these experiences in an
information, and expressing ideas through drawing environment that they are able to control. They can
or other artistic media. Art is a form of expression make the story be how they want it to be, they are
that requires little or no verbal interaction which in charge of the outcome. This feeling of control is
can open doors to communication. vital to their emotional development as well as
Autism 253

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Dementia 255

Ch.6 Dementia
Ms. Akshata Shetty, M.A.(Clinical Psychologist), Dr. Myola D'Sa, [Link].

INTRODUCTION:
Dementia is defined as a progressive impairment
of cognitive functions occurring in clear
consciousness (i.e. in absence of delirium). This is a
disease which has a significant global impairment
of intellect, manifested as difficulty with memory,
attention, thinking and comprehension. Other
psychological functions can be included including
mood, personality, judgment, and social behaviour.
There are specific diagnostic criteria that are found
for various dementias, such as Alzheimer's disease
or vascular dementia, however all dementias have
certain common elements that result in significant
impairment in social or occupational functioning
and cause a significant decline from a previous level
of functioning.[1]
The disorder can be progressive or static,
permanent or reversible. An underlying cause is French Physician, Philippe Pinel who coined
always assumed, although in rare cases, it is with the term "demence" i.e dementia.
impossible to determine specific cause.
Approximately 15 percent of patients with
dementia have reversible illness if treatment is
initiated before irreversible damage takes place.
to Pinel's patient. This woman was in her fifties and
HISTORY: she appeared to have the same disease Pinel
described. The woman suffered a "failure of
Western literature for centuries has described a
memory, paranoia, loss of reasoning powers,
disease process what we call as "dementia" today.
incomprehension and stupor."
"Dementia is a word which is given by a French
physician named Philippe Pinel. In 1801 he was When the German physician looked at her brain
working on a 34 year old woman who lost her however, he had a more advanced microscope, an
memory, speech, ability to walk or use common optical microscope. And in writing a research paper
objects like a fork or a hairbrush and Pinel called about his patient, Alois Alzheimer described the
this process "demence". He used the disease process for which he is known today.
words"demence" to mean an "incoherence" of Alzheimer described a brain that was (1) shrunken
mental faculties to describe her disease. Today the and (2) full of fluid, but also (3) suffered structural
word dementia indicates a person having cognitive damage in the form of neurofibrilary tangles and
impairment significant enough to interfere with (4) had bone structures growing in the brain tissues.
daily functioning and describe one of more than 48 These are the four hallmark features of a brain with
types of these diseases in the brain. Alzheimer's disease.
When Pinel's patient died, he autopsied her brain. Alzheimer sent his paper documenting his findings
Using a primitive microscope, he studied the brain to his mentor, Dr. Emil Kraepelin. When Dr.
tissue. With his microscope, he was only able to Kraepelin published his eighth medical textbook,
describe two distinct features of her disease. He Alzheimer's paper and research were included and
wrote that the woman's brain was full of fluid and the disease became known as Alzheimer's disease.
it had dramatically shrunken in size. One of the challenges for us today is the
pronunciation of Alzheimer's. Some people have a
In 1907, a German physician published a paper on
tendency to pronounce Alzheimer's like the words
a patient of his who exhibited behaviours similar
256 Neuro-Rehabilitation : A multi disciplinary approach

"Old-Timers," which continues to enforce the


thought that as we age we lose our mental abilities.
Alzheimer's is actually a disease and is not
considered a part of the normal aging process. Most
people do not develop any type of dementia. And
most people remain cognizant throughout their
lifetimes. Indeed, people learn at the age of eighty
at the same rate they learned at the age of twenty.
Dementia is truly not a part of normal aging.

DEFINITION OF DEMENTIA:
The World Health Organization (2007): "a
syndrome due to disease of the brain, usually of a
chronic or progressive nature, in which there is
disturbance of multiple higher cortical functions, Prevalence rates of different types
including memory, thinking, orientation, of dementia
comprehension, calculation, learning capacity, Other common causes of dementia, each
language, and judgment. However, consciousness representing 1 to 5 percent of all cases, include head
is not clouded. The impairments of cognitive trauma, alcohol related dementias and various
function are commonly accompanied, and movement disorder - related dementias, such as
occasionally preceded, by deterioration in Huntington's disease and Parkinson's disease.
emotional control, social behavior, or motivation
which occurs usually in Alzheimer's disease, in ETIOLOGY:
cerebrovascular disease, and in other conditions
primarily or secondarily affecting the brain." The most frequent causes of dementia in
individuals older than 65 years of age are: (1)
EPIDEMIOLOGY: Alzheimer's disease (2) Vascular dementia and (3)
Mixed vascular and Alzheimer's dementia. Other
The prevalence of dementia is rising, with the illness that account for approximately 10 percent
growing aging population. The prevalence of include Lewy body dementia, Pick's disease,
moderate to severe dementia: frontotemporal dementias normal pressure
• 5 percent - general population more than 65 hydrocephalus, alcoholic dementia, infectious
years of age dementia, such as human immunodeficiency virus
(HIV) or syphilis and Parkinson's disease. Many
• 20 to 40 percent - general population more types of dementias evaluated in clinical settings can
than 85 years of age be attributable to reversible cases, such as metabolic
• 15 to 20 percent in outpatient general medical abnormalities, nutritional deficiencies or dementia
practices syndrome caused by depression.
• 50 percent in chronic care facilities DIAGNOSIS:
Of all the other types of dementia, 50 to 60 percent Dementia is a type of chronic encephalopathy that
of patients suffer from the most common type of can have many causes, including irreversible
dementia i.e. the Alzheimer's type. Dementia of the degenerative and potentially reversible
Alzheimer's type increases in prevalence with nondegenerative causes. The most important
increasing age. For persons aged 65 years men have diagnostic step in evaluating dementias is to
a prevalence rate of 0.6 percent and women of 0.8. determine whether a chronic encephalopathy
At age 90 years, rates are 21 percent. The second results form a degenerative or other potentially
most common type of dementia is vascular reversible causes. Historical clues suggesting a
dementia, which accounts for 15 to 30 percent of reversible process include fluctuating severity,
all cases. Vascular dementia is most common in altered level of consciousness or hypersomnolence,
persons between the ages 60 and 70 and is more and visual hallucinations. Clues on mental status
common in men than in women. testing include findings the patient to be inattentive,
disoriented and somnolent but not particularly
Dementia 257

amnestic. Clues on physical examination include a Most clinical studies are in agreement that the
variety of findings that may be common in elderly earliest clinical signs of AD are memory loss, which
patients are not part of the typical picture of precedes the actual dementia. Abnormal cerebral
Alzheimer's disease, such as ataxia, hyper-reflexia metabolism, demonstrated by positron emission
and tremulousness. Laboratory evaluation should tomography (PET), may precede even mild
be directed towards. memory loss [2]. A few studies have correlated
other relative cognitive inefficiencies occurring
Dementia of The Alzheimer's Type: much earlier in life with the subsequent
development of dementia, although interpretation
In 1907, Alois Alzheimer first described the
of these findings have been a source of controversy.
condition that later assumed his name. He
described a 51 year old woman with a 4 ½ year Recent memory is easier to access reliably then
course of progressive dementia. The final diagnosis remote memory and is thought to be
of Alzheimer's disease requires a neuropathological disproportionately severely involved. Nonetheless,
examination of the brain; nevertheless, dementia remote memory is also abnormal, and there is a
of the Alzheimer's type commonly diagnosed in the gradient effect regarding recall over a retrograde
clinical setting after other causes of dementia have time interval: The oldest memories appear to be the
been excluded from diagnostic consideration. The best preserved, with proportionately greater
clinically defined syndrome, Alzheimer's dementia forgetting as the retrograde interval shortens [3].
has been considered as the paradigm of a cortical In contrast procedural memory appears to be
dementia syndrome. The hallmark of, cortical relatively spared. Alzheimer's patients are able to
dementia include not only memory loss, which is learn simple skills as easily as normal controls and
common to most dementia syndromes but also better than patients with subcortical patterns of
elements of aphasis, apraxia and agnosia. dementia or patients with various types of
sensorimotor deficits [4]. One very important skill
that needs to be addressed clinically is driving.
Despite the relative preservation of procedural
memory, patients with mild Alzheimer's dementia
have a higher rate of collisions and moving
violations than age-matched controls, although
estimates of risk vary, especially during the first 2
years of the disease. Whether this actually results
from impaired procedural memory, attentional
factors, other cognitive aspects or a combination is
unclear, although visual tracking, memory, and
Mini-Mental State score all correlate with a
laboratory-based driving score.
Aphasia, apraxia and agnosia are the other
categories of cognitive impairment that typically
occur in cortical dementia syndromes and
particularly in AD. In Alzheimer's dementia, these
aspects do not usually dominate the clinical picture,
but they can in variant syndromes. In mild to
moderate stages of dementia, anomia is prominent
and readily detectable with neuropsychological
testing using a variety of naming tests. However,
patients are fluent and may have relatively good
comprehension, so clinical detection is not always
easy.
Apraxia can be confused with impaired
comprehension in mild to moderate stages of
Alois Alzheimer after whom the condition assumed Alzheimer's dementia. Patients have difficulty in
his name Alzheimer 's disease. learning new procedures such as a new car or
258 Neuro-Rehabilitation : A multi disciplinary approach

performing tasks that they were previously adept In several well-documented cases, the disorder has
at. In moderately advanced stages, patients have been transmitted in families through an autosomal
difficulty in dressing and performing other dominant gene, although such transmission is rare.
activities of daily living. Alzheimer's type dementia has shown linkage to
chromosomes 1, 14 and 21.
Anosognosia is the failure to recognize illness, this
is a cardinal feature of Alzheimer's dementia and
this is typically present, even in mild stages of the
disease. In AD, a patient may deny significant
memory problems and will actively try to explain
away the observations of concerned family
members and friends, even to a point of becoming
hostile and accusative.
Psychiatric symptoms include both affective and
psychotic disturbances. Depression interferes with
an individual's functional status and an accurate
cognitive assessment, erroneously leading to the
diagnosis of dementia; such is the nature of
pseudodementia, which can be estimated to Genetic affect in Alzheimer's dementia
account for 4 percent or 5 percent of dementia cases
[5]. Psychotic symptoms, most commonly involves Amyloid Precursor Protein:
paranoid delusions and less commonly The gene for amyloid precursor protein is on the
hallucination. long arm of chromosome 21. Whether the
processing of abnormal amyloid precursor protein
Sleep wake cycle disturbances are common and
is of primary causative significance in Alzheimer's
may be present even during relatively mild stages
disease is unknown, but many research groups are
of their illness. They may become more common
studying both the normal metabolic processing of
and more severe during moderately severe stages.
amyloid precursor protein and its processing in
There are two aspects to the sleep cycle disturbance.
patients with dementia of Alzheimer's type in an
The first is the so called sun-drowning effect, which
attempt to answer this question.
means that the patient becomes more confused,
agitated, and difficult to manage during the Multiple E4 Genes:
evening. The second regards not sleeping at night,
One study implicated gene E4 in the origin of
waking up during very early hours or going to sleep
Alzheimer's disease. People with one copy of the
very early in the evening. As, the disease progresses
gene have Alzheimer's disease three times more
to more advanced stage, patients may become
frequently than do those with no E4gene, and
generally less active and eventually in terminal
people with two E4 genes have the disease eight
stages, are bedbound with little apparent conscious
times more frequently than do those with no E4
activity. Weight loss is common during the latter
gene. Diagnostic testing for this gene is not
stages as well. Incontinence is uncommon in mild
currently recommended because it is found in
stages but becomes increasingly frequent as the
persons without dementia and not found in all cases
disease progresses and is universal in late stages.
of dementia.
Genetic Factors:
Although the cause of dementia of the Alzheimer's
type remains unknown, progress has been made
in understanding the molecular basis of the amyloid
deposits that are a hallmark of the disorders
neuropathology. Some studies have indicated that
as many as 40 percent of patients have a family
history of dementia of the Alzheimer's type thus
genetic factors are presumed to play a part in the
Mechanism of pathology in Alzheimer's disease
development of the disorder, at least in some cases.
and associated dementia
Dementia 259

Neuropathology: Neurotransmitter:
The classic gross neuroanatomical observation of a
The neurotransmitters that are most often
brain from a patient with Alzheimer's disease is
implicated in the pathophysiological condition of
diffuse atrophy with flattened cortical sulci and
Alzheimer's disease are acetylcholine and
enlarged cerebral ventricles. The classic and
norepinephrine both which are hypnotized to be
pathognomonic microscopic findings are senile
hypoactive in Alzheimer's disease. Decreased
plaques, neurofibrillary tangles, neuronal loss
norepinephrine activity in Alzheimer's diease is
(particularly in the cortex and hippocampus),
suggested by the decrease in norepinephrine
synaptic loss (perhaps as much as 50 percent in the
containing neurons in the locus ceruleus found in
cortex) and granulovascular degeneration of the
some pathological examination of brains from
neurons.
persons with Alzheimer's disease. To other
neurotransmitter implicate in the
pathophysicological condition of Alzheimer's
disease are the neuroactive peptides somatostatin
and corticotrophin; diseases concentrations of both
have been reported in person with Alzheimer's
disease.

Other Causes:
Another theory to explain the development of
Alzheimer's disease is that an abnormality in the
regulation of membrane phospholipid metabolism
results in membranes that are less fluid - that is,
more rigid - than normal. Aluminium toxicity has
also been hypothized to be causative factor, because
high levels of aluminium have been found in the
Neuroanatomical Observation diffuse atrophy with
flattened cortical sulci and enlarged cerebral brains of some patients with Alzheimer's disease;
ventricles. but this is no longer considered a significant
etiological factors. Excessive stimulation by the
Senile plaques also referred to as amyloid plaques transmitter glutamate that may damage neurons
more strongly indicate Alzheimer's disease, is another theory of causation.
although they are also seen in Down syndrome and
to, some extent, in normal aging. The number and Familial Multiple System Taupathy with Presenile
the density of senile plaques present in postmortem Dementia: A recently discovered type of dementia,
brains have been correlated with severity of the familial multiple system taupathy, shares some
disease that affected the person. brain abnormalities found in people with
Alzheimer's disease. The gene that causes the
disorder is thought to be carried on chromosome
17. The symptoms of the disorder include short
term memory problems and difficulty in
maintaining balance and walking. The onset of the
disease occurs in the 40s and 50s and persons with
the disease live an average of 11 years after the onset
of symptoms.
As, in patients with Alzheimer's disease, tau protein
builds up in neurons and glial cells, of persons with
familial multiple system taupathy. Eventually, the
protein build up kills brain cells. The disorder is
not associated with the senile plaque seen with
Alzheimer's disease.
Neurofibrillary tangles and Amyloid Plaques in
Alzheimer's patients
260 Neuro-Rehabilitation : A multi disciplinary approach

Diagnosis: 294.10 Without Behavioral Disturbance: if the


cognitive disturbance is not accompanied by any
Diagnostic criteria for Dementia of the clinically significant behavioral disturbance.
Alzheimer's Type (cautionary statement). 294.11 With Behavioral Disturbance: if the cognitive
A. The development of multiple cognitive deficits disturbance is accompanied by a clinically
manifested by both (1) memory impairment significant behavioral disturbance. (e.g., wandering,
(impaired ability to learn new information or agitation)
to recall previously learned information) (2)
one (or more) of the following cognitive Specify subtype:
disturbances: With Early Onset: if onset is at age 65 years or
(a) aphasia (language disturbance) below
(b) apraxia (impaired ability to carry out With Late Onset: if onset is after age 65 years
motor activities despite intact motor Coding note: Also code 331.0 Alzheimer's disease
function) on Axis III. Indicate other prominent clinical
(c) agnosia (failure to recognize or identify features related to the Alzheimer's disease on Axis
objects despite intact sensory function) I (e.g., 293.83 Mood Disorder Due to Alzheimer's
(d) disturbance in executive functioning (i.e., Disease, With Depressive Features, and 310.1
planning, organizing, sequencing, Personality Change Due to Alzheimer's Disease,
abstracting) Aggressive Type).
B. The cognitive deficits in Criteria A1 and A2 Reprinted with permission from the Diagnostic and
each cause significant impairment in social or Statistical Manual of Mental Disorders, Fourth
occupational functioning and represent a Edition, Text Revision. Copyright 2000 American
significant decline from a previous level of Psychiatric Association.
functioning.
Prognosis:
C. The course is characterized by gradual onset
and continuing cognitive decline. Alzheimer's disease is a progressive disorder and
as the patients are elderly, and susceptible to a
D. The cognitive deficits in Criteria A1 and A2 variety of other disease processes, AD however is
are not due to any of the following: not the common cause of death. However, if no
(1) other central nervous system conditions other disease process supervenes, death usually
that cause progressive deficits in memory ensues approximately 6 t 10 years after the onset.
and cognition (e.g., cerebrovascular
disease, Parkinson's disease, VASCULAR DEMENTIA:
Huntington's disease, subdural The primary cause of vascular dementia is from
hematoma, normal-pressure hydroce- impaired blood flow to the brain. Formerly referred
phalus, brain tumor) to as multi- infarct dementia is presumed to be
(2) systemic conditions that are known to multiple areas of cerebral vascular disease, resulting
cause dementia (e.g., hypothyroidism, in a symptom pattern of dementia. However, the
vitamin B or folic acid deficiency, niacin name was changed to vascular dementia to reflect
deficiency, hypercalcemia, neurosyphilis, the array of conditions that can impair the blood's
HIV infection) ability to circulate to the brain. Vascular dementia
often occurs alongside Alzheimer's disease,
(3) substance-induced conditions
resulting in mixed dementia between 1% to 4% of
E. The deficits do not occur exclusively during people over the age of 65 years have vascular
the course of a delirium. dementia, and the risk of developing it increases
dramatically with age. Vascular Dementia most
F. The disturbance is not better accounted for by
commonly is seen in men especially those with
another Axis I disorder (e.g., Major Depressive
preexisting hypertension or other cardiovascular
Episode, Schizophrenia).
risk factors.
Code based on presence or absence of a clinically
significant behavioral disturbance:
Dementia 261

multiple cognitive problems, including memory


impairment and possibly aphasia, apraxia, agnosia,
or problems with executive functioning. In
maximum cases this makes it difficult for the
patients to have a successful career or hold a job,
take care of the household activities or socialize.
People with vascular dementia also experience
neurological symptoms such as exaggerated
reflexes, problems with walking and balance, and/
or weakness in the limbs, hands, and feet.
Depending on the individual and on the cause of
the dementia, delusions, confusion, agitation,
Impaired blood flow in the brain in urinary problems, and/or depression can also
Vascular Dementia
accompany vascular dementia.
Causes of Vascular Dementia: Patients have a more subcortical pattern of
dementia with psychomotor slowing, poor learning
Vascular dementia can occur either due to curve, and relative preservation of naming and
narrowing or a complete blockage of blood vessels other language skills. The degree of impairment,
in the brain, which deprives brain cells from however, correlates better with the degree of
nutrients and oxygen they need to function neurofibillary pathology than with cerebrovascular
properly. Vascular dementia often results from pathology at autopsy.
several small strokes that occur over time. It can
also occur after a single major stroke, which is Interestingly, memory loss usually occurs later in
sometimes referred to as post-stroke dementia. Not the disease compared to when it appears in
all strokes lead to dementia, but up to one-third of Alzheimer's. In vascular dementia, the first
those who have a stroke will develop dementia symptoms are often the neurological ones, such as
within six months. Conditions like high blood problems with reflexes, walking, and muscle
pressure and diabetes that don't block blood vessels, weakness. On the other hand, memory problems
but simply narrow them, can also lead to vascular and behavioral symptoms are commonly the first
dementia. issues noticed in Alzheimer's. Additionally,
vascular dementia often progresses in a step-wise
Epidemiology and Risk Factors for Vascular fashion. For example, the person will seem stable
Dementia: for a period of time, then suddenly get much worse,
Incidence and prevalence rates of vascular then continue to alternate between stable periods
dementia are affected by the definition used. The and sudden drops in functioning. On the other
prevalence of post-stroke vascular dementia ranged hand, Alzheimer's disease progresses in a more
from 11.3 percent using the NINDS- AIREN criteria gradual, downward fashion.
to 20. 1 percent using the ICD- 10 NA criteria, and
Diagnosis of Vascular Dementia:
the incidence ranged from 2.6% with the ADDTC
to 5.2 percent with the ICD - 10 - NA[5]. The As with Alzheimer's disease, a complete diagnostic
incidence rate of AD is higher among individuals workup should be performed in order to rule out
with prevelant cardiovascular disease than among other possible causes of the person's symptoms.
those without it. Vascular dementia is usually identified through
imaging procedures, which can reveal strokes and
Individuals who develop vascular dementia often
narrowed or blocked arteries. Neuropsychological
have a history of one or more of the following: heart
tests might also be conducted to determine the
attack, stroke, high blood pressure, diabetes, or high
nature and extent of cognitive impairment.
cholesterol. In particular, if an individual has a
history of multiple strokes, the risk of developing DSM-IV Criteria for the Diagnosis of Vascular
vascular dementia increases with the number of Dementia
strokes experienced over time. A. The development of multiple cognitive deficits
manifested by both:
Clinical Feature and Associated Disorders:
People with vascular dementia often display 1. Memory impairment (impaired ability to
262 Neuro-Rehabilitation : A multi disciplinary approach

learn new information or to recall Prognosis for Vascular Dementia


previously learned information). Currently, there is no cure for vascular dementia.
2. One or more of the following cognitive If the dementia was caused by multiple strokes, the
disturbances: person may get worse in a step-wise progression,
where stable periods are interrupted by sudden
a. Aphasia (language disturbance)
downward episodes. Life expectancy for someone
b. Apraxia (impaired ability to carry out with vascular dementia is highly individual and
motor activities despite intact motor depends on the nature of the cardiovascular
function) problems that are causing the dementia, along with
the person's age and other medical conditions.
c. Agnosia (failure to recognize or identify
objects despite intact sensory function)
BINSWANGER'S DISEASE:
d. Disturbance in executive functioning (i.e.,
planning, organizing sequencing, Binswanger's disease, also known as subcortical
abstracting) arteriosclerosis encephalopathy, is characterized by
the presence of many small infarctions of the white
B. The cognitive deficits in criteria A1 and A2 matter that spare the cortical regions.
each cause significant impairment in social or Atherosclerosis (commonly known as "hardening
occupational functioning and represent of the arteries") is a systemic process that affects
asignificant decline from a previous level of blood vessels throughout the body. Although
functioning. Binswanger's disease was previously considered a
C. Focal neurological signs and symptoms (e.g., rare condition, the advent of sophisticated and
exaggeration of deep tendon reflexes, extensor powerful imaging techniques, such as magnetic
plantar response, pseudo bulbar palsy, gait resonance imaging (MRI), has revealed that the
abnormalities, weakness of an extremity) or condition is more common than previously
neuroimaging evidence indicative of thought.
cerebrovascular disease (e.g., multiple
infarctions involving cortex and underlying Symptoms of the Binswanger's Disease:
white matter) that are judged to be The symptoms associated with Binswanger's
etiologically related to the disturbance.. disease are related to the disruption of subcortical
neural circuits that control what neuroscientists call
D. The deficits do not occur exclusively during
executive cognitive functioning:
the course of delirium.
• Short-term memory,
Differential Diagnosis: • Organization,
There is a broad range of cerebrovascular disorders • Mood,
that may result in cognitive impairment. Multiple • The regulation of attention,
large artery territory infarction and hemorrhages • The ability to act or make decisions, and
may result in more extensive physical disability and
• Appropriate behavior.
cortical cognitive signs such as aphasia. Non-
artherosclerotic forms of cerebrovascular disease The most characteristic feature of Binswanger's
may have their own disease associations that, in disease is psychomotor slowness - an increase in
turn, may influence cognition. Cerebral vasculitis the length of time it takes, for example, for the
may present as a rapidly progressive fulminant fingers to turn the thought of a letter into the shape
encephalopathy, a sub acute course of recurrent of a letter on a piece of paper.
cerebral infarction or a more chronic dementia - like Other symptoms include:
course.
• Forgetfulness (but not as severe as the
Evaluation: forgetfulness of Alzheimer's disease),

Considerations are the same as that for AD except • Changes in speech,


that causes of cerebral infarction also need to be • Unsteady gait,
included.
• Clumsiness or frequent falls,
Dementia 263

• Changes in personality or mood (most elsewhere in the brain. Pick's bodies are fibers that
likely in the form of apathy, irritability, look very different from the neurofibrillary tangles
and depression), and found in Alzheimer's disease. Pick's bodies are
straight rather than paired and helical.
• Urinary symptoms that aren't caused by
urological disease. Changes showing in the hippocampus gyrus and
balloned achromatic neurons and pick bodies
Diagnosis:
Symptoms of Pick's Disease:
Brain imaging, is very essential as it reveals the
characteristic brain lesions of Binswanger's disease, Many of the early symptoms of Pick's disease are
and is essential for a positive diagnosis. frontal lobe symptoms. It is these symptoms that
tend to mark out the differences between Pick's
Prognosis: dementia and the other types, such as Alzheimer's.
In Alzheimer's disease the initial symptoms tend
Binswanger's disease is a progressive disease; there
to be memory impairment. In Pick's disease because
is no cure. Changes may be sudden or gradual and
the frontal lobes of the brain are affected, the first
then progress in a stepwise manner. Binswanger's
symptoms occur in emotional and social
disease can often coexist with Alzheimer's disease.
functioning. It is the mood changes, often biased
Behaviors that slow the progression of high blood
towards euphoria, disinhibition and deterioration
pressure, diabetes, and atherosclerosis -such as
in social skills that are so noticeable.
eating a healthy diet and keeping healthy wake/
sleep schedules, exercising, and not smoking or Behavioural Symptoms Include:
drinking too much alcohol -- can also slow the • Repetitive or obsessive behavior
progression of Binswanger's disease
• Overeating or drinking to excess (when
PICK'S DISEASE: this was not previously a problem)

Arnold Pick, was the first one who described the • Impulsivity and poor judgment
disease in 1892, he reported that Pick's disease • Extreme restlessness (early stages)
causes an irreversible decline in a person's
• Lack of attention to personal hygiene
functioning over a period of years. It is most
commonly confused with the much more prevalent • Sexual exhibitionism or promiscuity
Alzheimer's disease; Pick's disease is a rare disorder • Withdrawal or decreased interest in
that causes the frontal and temporal lobes of the activities of daily living
brain, which control speech and personality, to
slowly atrophy. It is therefore classified as a • Decline in function at work and home
frontotemporal dementia, or FTD. Emotional Symptoms Include:
Pick's disease is a relatively rare form of dementia • Unaware of the changes in behavior
that causes a slow shrinking of brain cells due to
excess protein build-up. Patients with Pick's initially • Apathy
exhibit marked personality and behavioral changes, • Abrupt mood changes
and then a decline in the ability to speak coherently.
• Easily distracted; poor attention span
Pathogenesis and Pathophysiology: • Rudeness, impatience, or aggression
In Pick's disease the brain tissue changes and loss • Lack of warmth, concern, or empathy
occurs in focal areas rather than the generalized Language Problems Include:
damage of Alzheimer's. Pick's disease affects the
frontal and temporal lobes of the brain. Marked • Complete loss of speech
shrinkage, called atrophy, of the frontal lobes of the • Difficulty speaking or understanding
brain occurs that can be seen on brain scans. speech
Pick's disease is marked by the presence of • Weak, uncoordinated speech sounds
abnormalities in brain cells called Pick's bodies.
• Repeating words others say
These are found in the affected areas as well as
264 Neuro-Rehabilitation : A multi disciplinary approach

• Decrease in ability to read or write


• Trouble finding the right word
• Loss of vocabulary
Physical Symptoms Include:
• Memory loss
• Difficulty moving about
• Lack of coordination
• Increased muscle rigidity or stiffness
• Urinary incontinence
• General weakness
Pick's disease generally occurs between the ages of Lewy Bodies found in the midbrain and
forty and sixty years of age. brain stem

Pick's disease affects slightly more women than Causes:


men.
The causes of Lewy body dementia, is unknown in
majority of cases. Monogentic forms of Lewy body
Diagnosis of Pick's Disease:
disorders, where a patient inherited the disease
The diagnosis from a doctor can help to rule out from one parent, are rare and comprise about 10%
other diseases or conditions which can be treatable of cases. These familial variants are more common
or cured. A psychologist can help establish the type in patients with an earlier age of disease onset. The
of dementia. majority of cases are thought to result from complex
interactions among different "susceptibility" genes
Distinguishing Feature to Diagnose: and environmental risk factors. The primary
There are a few distinguishing feature that Pick's environmental factors that have been implicated in
disease has which distinguishes it from other increasing risk of some cases of Lewy body
disorders: (i) Onset before 65 years of age, (ii) Lack disorders include pesticide or other chemical
of inhibition, (iii) Initial personality changes (iv) exposure and head injury. There are undergoing
Loss of normal controls for example: hyper researches to study the role that genetics and
sexuality (v) Roaming behaviour. environment play in Lewy body disorders.

Prognosis in Pick's Disease: The underlying brain changes of Lewy body


dementias involve the clumping of a protein in the
The length of progression varies in individuals from
brain called alpha-synuclein. These clumps are
less than 2 years to 10 years in some.
called "Lewy bodies." The regional distribution of
these clumps in the brain influences the types of
LEWY BODY DISEASE: symptoms (e.g., cognitive, motor, psychiatric).
Lewy Body Disease is a dementia that is clinically Lewy body dementias are also associated with
similar to Alzheimer's disease and often reductions in important brain chemicals called
characterized by hallucinations, parkinsonian neurotransmitters, including dopamine and
features and extra pyramidal signs. These acetylcholine.
degenerative brain diseases are linked to abnormal
clumps of a protein called alpha-synuclein. These Diagnosis:
clumps are called as Lewy bodies, which are found An extensive neurological and neuropsychological
in nerve cells throughout the outer layer of the brain evaluation is essential for Lewy body dementia
(the cerebral cortex) and deep inside the midbrain diagnosis. Structural brain imaging for example
and brainstem. These patients usually experience MRI Brain and CT scan and laboratory tests are
progressive cognitive decline, although there is used to rule out other diagnoses. Lewy body
considerable variability between the symptoms. dementias can be difficult to diagnose because they
The common symptoms comprise of problems with can resemble and overlap with other causes of
movement, visual hallucinations, and fluctuations dementia like Alzheimer's disease, other
in thinking skills or attention. parkinsonian syndromes and vascular dementia.
Dementia 265

As in many neurodegenerative diseases, a definitive Disease Progression:


diagnosis is only available after an autopsy. The onset of the disorder usually occurs in geriatric
adults, and the disease risk increases with age.
Differential Diagnosis: There is substantial unpredictability in the course
Parkinson's disease versus Lewy Bodies: A few of the illness. In general, symptoms progress over
patients with Parkinson's disease (PD) experience several years as cognitive decline becomes more
a subtle cognitive decline and their primary prominent and psychiatric symptoms emerge or
limitation is their motor disorder. Although patients worsen. The progression of symptoms represents
with, Parkinson's disease will develop dementia as increasing pathology in the brain including more
a consequence of the disease. When dementia widespread Lewy bodies, loss of neurotransmitters
develops after an established motor disorder, we (examples: dopamine and acetylcholine) and nerve
call the disease PDD. In contrast, when the cell death.
dementia develops prior to or at the same time as
the motor disorder, we call the disease DLB. Prognosis:
Although the initial sequence of symptoms differs The prognosis for Lewy Body Dementia (LBD) can
in PDD and DLB, as the disorders progress, the be highly variable. There are no known therapies
symptoms and the underlying brain changes are to stop or slow the progression of. LDB. The average
much more similar than they are different. As such, life expectancy following the diagnosis is between
many researchers and clinicians think of PDD and five and seven years.
DLB as being on a continuum of a similar disease
process rather than as two distinct entities. HUNTINGTON'S DISEASE:
Signs and Symptoms: Huntington's disease is classically associated with
The parkinsonian motor syndrome is similar in the development of dementia. The dementia seen
PDD and DLB and can include slowed movement in this disease is the subcortical type of dementia,
characterized by more motor abnormalities and
(bradykinesia), rigidity (muscles feel still and resist
fewer language abnormalities than in the cortical
movement), tremor and gait difficulties. In terms
type of dementia. Grossly, the cortical gyri appears
of the cognitive difficulties, both disorders can
normal to slightly atrophic. Coronal sections reveal
involve progressive problems involving visual
striking caudate greater than putamen and
spatial processing, attention, executive dysfunction pallidum atrophy. Milder degenerative changes
(e.g., planning and multi-tasking) and memory. occur in the neocortex and thalamus. Evidences
Psychiatric symptoms are common and can include exist for excitotoxic damage and mitochondrial
anxiety, depression, hallucinations (usually visual) DNA mutations and oxidatives stress in the
and delusions (false beliefs). Sleep problems pathogenesis of cortical pathology in hungtington's
including excessive daytime drowsiness and disease.
difficulty staying asleep are common. Many
patients exhibit REM sleep behavior disorder (RBD) Clinical Feature and Associated Disorder:
that manifests by the patient acting out his or her Lieberman and associates [7] reported that around
dreams. 90 percent of patients were demented by a mean
Many patients experience constipation, repeated age of 48.3 years and that dementias preceded the
onset of chorea in 24 percent. Whether or not it is
falls, syncope and loss of smell. Individuals
the critical determinant of cognitive decline, and
diagnosed with Lewy body dementias often have
cognitive decline correlates to pathologically and
adverse reactions including confusion when taking
radiologically with degree of caudate atrophy and
medications that affect the brain, such as anti-
hypometabolism [8]. The area of particular interest
anxiety drugs (examples: Valium®, Ativan®), in dementia of HD are frontal lobe relate or
anticholinergic drugs (examples: Benadryl, Detrol), frontostriatal - related neuropsychiatric deficits,
and antiparkinson's drugs (examples: Sinemet®, declarative memory impairment, and motor
Mirapex). To avoid adverse reactions to learning difficulties. Regarding cognitive
medications, physicians should carefully monitor disturbances, prospective study of cognitive decline
medications, introduce medications one at a time in early cases has shown that psychomotor slowing
and prescribe minimal doses when possible. is a characteristic early feature and memory
266 Neuro-Rehabilitation : A multi disciplinary approach

impairment although present early on does not Evaluation:


deteriorate rapidly, or correlate as well with The clinical examination may provide compelling
caudate atrophy, as does psychomotor speed. evidence for HD, which can be confirmed
Huntington's disease causes memory loss but it genetically. Ancillary tests include MRI and CT to
appears to be less severe as compared with that of ascertain caudate atrophy and SPECT or PET to
Alzheimer's disease at a matched stage of severity, ascertain caudate hypoperfusion or metabolism.
although this factor has been more consistent at Neuropsychological assessment is useful to
milder stages of dementias. Most studies have document the nature and severity of cognitive
found that patients with HD have greater difficulty decline, particularly in mild to moderate stages of
learning a task, but benefit more by cued recall or illness.
recognition as compared with free or unassisted
recall. This has been thought to reflect greater Prognosis and Future Perspectives:
impairment of retrieval than storage mechanisms
Death occurs within 10 to 20 years after onset,
and it affects remote memory retrieval as well as
although suicide is more prevalent in at risk and
more recently learned material. There are many
early onset HD patients.
similarities in memory disturbances between HD
and PD, suggesting similarities between subcortial
HIV - RELATED DEMENTIA:
dementia illnesses but overlap is imperfect.
Evidence shows that the basal ganglias in patients Encephalopathy in HIV infection is associated with
with Huntington 's disease show a decrease in dementia and is termed acquired immune
activity of the mitochondrial pathway, complex II- deficiency syndrome (AIDS) dementia complex or
III. Such deficiencies are often associated with basal HIV dementia. The HIV virus will attack the nerve
ganglia degeneration [9]. This degeneration of cells in the brain, which will lead to AIDS Dementia.
striatal neurons projecting to GPe leads to So a person who carries the HIV virus may contract
disinhibition of the indirect pathway, increased AIDS Dementia. As said earlier even comparatively
inhibition of STN, and therefore, reduced output healthy people with HIV positive status can fall
of the basal ganglia [10]. prey to it. At times, the immune system has to grow
very weak for the symptoms to surface properly.
Patients infected with HIV experience dementia at
an annual rate of approximately 14 percent. An
estimated 75 percent of patients with AIDS have
involvement of the central nervous system (CNS)
at the time of autopsy. The development of
dementia in people infected with HIV is often
paralleled by the appearance of parenchymal
abnormalities in MRI scans. Other infectious
dementias are caused by Cryptococcus or
Treponema palladium.
Symptoms of HIV Dementia: lack of concentration,
withdrawal, forgetfulness, long term and short term
loss of memory, mania, altered personality,
The impact of Hungtion Disease on irritability, poor coordination, impaired judgment,
the brain structure in the basal ganglia blurred or hazy vision, reduction in attention span,
apathy and loss of bowel and bladder control.
Differential Diagnosis:
Diagnosis:
In absence of chorea, the family history is critical in
reaching a diagnosis. In the absence of both chorea, The diagnosis is done on the basis of neurological
the family history is critical in reaching a diagnosis. brain scans, coordination tests and mental activities.
In the absence of chorea and family history, the
differential diagnosis is that of other subcortical Head Trauma - Related Dementias:
dementias and chronic progressive Dementia can be a sequela of head trauma. The so
encephalopathies. -called punch- drunk syndrome occurs in boxers
after repeated head trauma over many years. IT is
Dementia 267

characterized by emotional liability, dysartharia Memory impairment is typically an early and


and impulsivity. prominent feature in dementia, especially in
dementia involving the contacts, such as dementia
Dementia Due to Other General Medical of the Alzheimer's type. Early in the course of
Conditions: dementia, memory impairment is mild and usually
The DSM IV TR lists six specific causes of dementia most marked for recently went; people Forget
that can be coded directly: HIV disease, head telephone numbers, conversation, and events of the
trauma, Parkinson's disease, Huntington's disease, day. As the course of dementia progress is, memory
picks disease and Creutzfeldt - Jakob disease. A impairment becomes severe, and only the earliest
seventh category allows clinicians to specify other learned information example of a person's place of
nonpsychiatric medical conditions associated with birth is retained. In as much as memory is important
dementia. for orientation two per cent, place, and time,
orientation can be progressively effected during the
Substance induced persisting dementia: alcohol- course of a dementing illness. For example patients
related dementia has been estimated to account for, with dementia may forget how to get back to their
approximately 4% of dementias. room after going to the bathroom. No matter how
severe the disorientation seems, however, patients
DIAGNOSIS AND CLINICAL show no impairment in their level of consciousness.
FEATURES:
Dementing processes that affect the cortex,
The dementia diagnosis in DSM-IV-TR dementia primarily dementia off the Alzheimer style and
of the Alzheimer's type, vascular dementia, vascular dementia, can affect patients language
dementia due to other general medical condition, abilities. DSM-IV-TR includes aphasia as one of the
substance induced persisting dementia, dementia diagnostic criteria. The language difficulties may
due to multiple etiologies, and dementia due to not be characterized by awake, stereo type, imprecise,
otherwise specified. The most important step in circumstantial locution and patient may also have
making the diagnosis is to determine whether a difficulty naming objects.
chronic encephalopathy results from a degenerative
or due to other potentially reversible causes. A PSYCHIATRIC AND
reversible process includes fluctuating severity, NEUROLOGICAL CHANGES:
altered level of consciousness or hypersomnolence
and visual hallucinations. Personality:
The diagnosis of dementia is based on the clinical Changes in the personality of a person with
examination, including a mental status dementia are especially disturbing for their families.
examination, and information from the patient's Preexisting personality traits may be accentuated
family, friends, and employers. Complaints of a during the development of a dementia. Patients
personality change in patients older than age 40 with dementia may also become introverted and
suggest that the diagnosis of dementia should be seem to be less concerned than they previously were
carefully considered. about the effects of their behaviour on others.
Clinicians should note patients complain is about Persons with dementia who have paranoid
intellectual impairment and forgetfulness as well delusions are generally hostile to family members
as evidence of patients evasion, denial, or and caretakers. Patients with frontal and temporal
rationalization aimed at concealing cognitive involvement are likely to have marked personality
deficits. Excessive orderliness, social withdrawal, changes and may be irritable and explosive.
or a tendency to relate event in minute detail can
Hallucinations and Delusions:
be characteristic, and sudden outburst of anger
sarcasm can occur. Patients in appearance and An estimated 20 to 30% of patients with dementia
behaviour should be observed. Liability of have had hallucination and 30 to 40% have
emotions, sloppy gloomy, uninhabited remarks, delusions, primarily of paranoid or persecutory and
silly jokes, or a dull, apathetic, or vacuous facial unsystematized nature, although complex
expression and manners suggest the presence of sustained and well systematised illusions are also
dementia, especially when coupled with memory reported by these patients. Physically aggression
impairment. and other forms of violence are common in
268 Neuro-Rehabilitation : A multi disciplinary approach

demented patients who also have psychotic • Changes of chemicals in the brain.
symptoms.
• Changes in the structure of different parts of
the brain due to dementia.
Mood:
In addition, to psychosis and personality changes, Medical:
depression and anxiety major symptoms in an
• Patients with dementia who also suffer from
estimated 40 to 50% of patients with dementia,
infections, pain, dental problems and arthritis
although the full syndrome of depressive disorder
may be unable to articulate the pain they are
may be present in only 10 to 20 per cent. Patients
experiencing and instead may express their
with dementia also may exhibit pathological
distress by vocalising or becoming aggressive.
laughter or crying that it is, extremes of emotions
with no apparent provocation. • Co-morbid conditions such as delirium,
depression, anxiety or psychosis. Individuals
Cognitive changes: with dementia are more susceptible to
In addition to appraise in patients with dementia, delirium and the illnesses/environmental
apraxias and agnosias are common, and they are stressors that lead to delirium.
included as potential diagnostic criteria in DSM IV o Side effects caused due to medication can
TR. Other neurological signs that can be associated be numerous and may have a significant
with dementia seizures, seen in approximately 10% effect on a person's behaviour
of patients with dementia of the Alzheimer style
o Hearing or vision impairment that is not
and in 20% of patients with vascular dementia and
well managed (for example,
a typical neurological presentation, such as
malfunctioning hearing aids may lead to
nondominant parietal lobe syndrome. Primitive
frustration and changed behaviours).
reflexes, such as the grasp, snout, suck, tonic foot
and palmomental reflexes may be present on o Sleep disturbances are common in people
neurological examination and myoclonic jerks are with dementia and can cause agitation
present in five to 10 percent of the patients. and restlessness during the day and
night.
Patients with vascular dementia may have
additional neurological symptoms, such as Environmental and social:
headaches, dizziness, faintness, weakness, focal
o Changes in social routine (for example,
neurological signs, and sleep disturbances, possibly
alteration in meal times or introduction
attributable to the location of the terrible or vascular
of a new care routine can cause confusion
disease.
and a feeling of loss of control for the
person with dementia, contributing to
Behavioural symptoms of dementia:
behaviours of concern).
Patients with dementia may experience behavioural
o Change in environment (for example,
and psychological symptoms and during the course
relocation to a new room or home can
of their illness. These may include:
increase agitation and disorientation).
Behavioural Symptoms: screaming, restlessness,
physical aggression, agitation, shaking, screaming, Psychosocial Determinants:
wandering, culturally inappropriate behaviours, The severity and course of dementia can be affected
sexual disinhibition, hoarding, cursing, and by psychosocial factors. The greater a person's
shadowing. premorbid intelligence and education, the better the
Psychological Symptoms: depression, delusions, ability to compensate for intellectual deficits. People
hallucinations, delirium and apathy. who have a rapid onset of dementia use fewer
defenses than do those who experience an insidious
Causes of Behavioural Symptoms: onset. Anxiety and depression can intensify and
aggravate the symptoms. Pseudodementia occurs
Biological: in depressed people who complain of impaired
• Genetic abnormalities related to the structure memory, but in fact are suffering from a depressive
of particular parts of the brain. disorder. When the depression is treated, the
cognitive defects disappear.
Dementia 269

Catastrophic reaction: A reasonably recent complete physical evaluation


Patient with dementia also exhibit a reduced ability should be obtained in patients who are suspected
to apply what Kurt Goldstein called the abstract of suffering from dementia. It may reveal evidence
attitude. Patients have difficulty generalizing from of systematic disease causing brain dysfunction,
a single instance, forming concepts and grasping such as an enlarged liver, and hepatic
similarities and differences are among concepts. encephalopathy, or it may demonstrate systematic
Furthermore, the ability to solve problems, to disease related to particular CNS processes. Patient
reason logically and to make sound judgement is should also undergo structural neuroimaging with
compromised. Goldestine also described a computed tomography (CT) or magnetic resonance
catastrophic reaction marked by agitation imaging (MRI) of the brain. When a
secondary to the subjective awareness of the nondegenerative based chronic progressive
intellectual depth received under stressful encephalopathy is suspected, electroence-
circumstances. Persons usually attempt to phalography (EEG) may be helpful in
compensate for defects by using strategies to avoid demonstrating severe dysrhythmic slowing.
demonstrating failures in intellectual performance; Cerebrospinal fluid (CSF) examination may show
they may change the subject, make jokes or evidence of a chronic meningoencephalitic process
otherwise divert the interviewer. such as an elevated IgG index and synthesis rate
and occasionally oligoclonal bands, suggesting an
Sundrowner Syndrome: intrathecal inflammatory reaction. Ultimately is
Sundrowners Syndrome is characterised by suspicion remains high based on noninvasive tests
drowsiness, conclusion, ataxia, and accidental for nondegenerative causes, cerebral angiography
faults. It occurs in older patients who are overly and meningeal and brain biopsy can be considered,
or in the absence of such invasive testing, an
sedated and inpatients with dementia that reacts
adversely to even a small dose of psychoactive empirical therapeutic trial of prednisone could be
drug. The syndrome also occurs in demented considered for a possible steroid-responsive
patients when external stimuli, such as light and inflammatory meningoencephalitis. Focal
interpersonal orientation is cues are diminished. neurological findings, such asymmetrical
hyperreflexia or weaknesses are seen more often
in vascular then in degenerative diseases.
GENERAL PATHOLOGY, PHYSICAL
FINDINGS AND LABORATORY DIFFERENTIAL DIAGNOSIS:
EXAMINATION:
Dementia of the Alzheimer type versus
A comprehensive laboratory workup must be
performed when evaluating a patient with Vascular Dementia:
dementia. The purposes of the workup are to detect Classically, vascular dementia has been
reversible causes of dementia and to provide the distinguished from dementia of the Alzheimer type
patient and family with a definitive diagnosis. The by the decremental deterioration that can
range of possible causes of dementia mandate accompany cerebrovascular disease overtime.
selected use of laboratory test. The evaluation Although the discrete, stepwise deterioration may
should follow clinical suspicion, based on the not be apparent in all cases, focal neurological
history and physical and mental status examination symptoms are more common in vascular dementia
results. The continued improvements in brain of the Alzheimer's type, as are the standard risk
imaging techniques, particularly MRI, have made factors for cerebrovascular disease.
differentiation between the dementia of the
Alzheimer type, and vascular dementia, in some Vascular Dementia versus Transient Ischemic
cases somewhat more straightforward then in the Attacks:
past. And active area of research is the use of single Transient Ischemic Attacks (TIA's) are brief
photon emission computed tomography (SPECT) episodes of focal neurological dysfunction lasting
to detect patterns of brain metabolism in various less than 24 hours. Although a variety of
types of dementia; the use or off (SPECT) images mechanisms may be responsible, the episodes are
may soon help in the clinical differential diagnosis frequently the result of microembolization from a
of dementing illnesses. proximal intracranial arterial lesion that produces
transient brain ischemia, and the episodes usually
270 Neuro-Rehabilitation : A multi disciplinary approach

resolve without significant pathological alteration Normal Aging:


of the parenchymal tissues. Approximately one Aging is not necessarily associated with any
third of the person's with untreated TIA's significant cognitive decline, but minor memory
experience a brain infarction later; therefore, problems can occur as a normal part of aging. These
recognition of TIA's is an important clinical strategy normal occurrences are sometimes, referred to as
to prevent brain infarction. benign senescent forgetfulness or age - associated
Clinicians should distinguishing episodes involving memory impairment. They are distinguished from
the vertebrobasilar system from those involving the dementia by their minor severity and because they
carotid arterial system. In general, symptoms of do not interfere significantly with a person's social
vertebrobasilar disease reflect a transient functional or occupational behaviour.
disturbance in either the brainstem or the occipital
lobe; carotid distribution symptoms reflect Other Disorders:
unilateral retinal or hemispheric abnormality. Mental retardation, which does not include
Anticoagulant therapy, antiplatelet agglutinating memory impairment, occurs in childhood.
drugs such as aspirin, and extracranial and intra Amnestic disorder is characterized by
cranial reconstructive vascular surgery are effective circumscribes loss of memory and no deterioration.
in reducing the risk of infarction in patients with Major depression in which memory is impaired
TIA's. responds to antidepressant medication.
Malingering and pituitary disorder must be ruled
Delirium: out, but they are unlikely.
In general, delirium is distinguished by rapid onset,
brief duration, cognitive impairment fluctuation COURSE AND PROGNOSIS:
during the course of the day, nocturnal exacerbation The classic course of dementia is an onset in the
of symptoms, marked disturbance of the sleep - patients 50s or 60s with gradual deterioration over
wake cycle, and prominent disturbance in attention 5 to 10 years, leading eventually to death. The age
and perception. of onset and the rapidly of deterioration vary
among different types of dementia and within
Depression:
individual diagnostic categories. The average
Some patients with depression have symptoms of survival expectation for patients with dementia of
the cognitive impairment difficult to distinguish the Alzheimer's type is approximately 8 years, with
from symptoms of dementia. The clinical picture is a range of 1 to 20 years. Data suggest that in persons
sometimes referred to as pseudodementia, although with an early onset of dementia or with a family
the term depression- related cognitive dysfunction history of dementia the disease is likely to have a
is preferable and more descriptive. Patients with Rapid course.
depression - related cognitive dysfunction generally
have prominent depressive symptoms, more The most common course of dementia begins with
insight into their symptoms than do demented a number of subtle signs that may, at first, be
patients, and often a history of depressive episodes. ignored by both the patient and the people closest
to the patient. A gradual onset of symptoms is most
Factious Disorder: commonly associated with dementia of the
Alzheimer's type, vascular dementia,
Person who attempts to stimulate memory loss, as
endrinopathies, brain tumors and metabolic
in factitious disorder, do so in an erratic and
disorders. Conversely, the onset of dementia
inconsistent manner. In true dementia, memory for
resulting from head trauma, cardiac arrest with
time and place is lost before memory for persons,
cerebral hypoxia, or encephalitis can be sudden.
and recent memory is lost before remote memory.
Although the symptoms of the early phase of
Schizophrenia: dementia are subtle, they become conspicuous the
as the dementia progresses, and family members
Although schizophrenia can be associated with
may then bring a patient to a physician's attention.
some acquired intellectual impairment, its
People with dementia may be sensitive to use of
symptoms are much less severe than are the related
benzodiazepines or alcohol, which can be
symptoms of psychosis and thought disorder seen
precipitate agitated, aggressive or psychotic
in dementia.
behaviour. In the terminal stage of dementia,
Dementia 271

patients become empty shells of their former selves- • Display inappropriate behaviour in public.
profoundly disoriented, incoherent, amnestic and
• Be confined to a wheel chair or bed.
incontinent of urine and feces.
Morbidity and Mortality associated with Dementia
With psychosocial and pharmacological treatment
Global Morbidity and Mortality:
possibly because of the self- healing properties of
the brain, the symptoms of dementia may progress • In 2000, age-standardized dementia mortality
slowly for a time or may even recede somewhat. rate was 6.7 and 7.7 for 100,000 male and
Symptoms regression is certainly a possibility in female respectively. [10]
reversible dementias once treatment is initiated. The • 24.3 million have dementia [11]
course of the dementia varies from steady
progression to an incrementally worsening • 4.6 million new cases per year [11]
dementia to a stable dementia. • Worldwide dementia contributes 4.1% of all
disability-adjusted life years (DALYs) and
Stages of Dementia: 11.3% of years lived with disability and 0.9%
of years of life lost.
1-Early stage (developed in 1-2 years):
The early stage of dementia is often overlooked India Morbidity and Mortality:
because the onset of dementia is gradual; it is often
• Age standardized death rate of 12.1 per
difficult to be sure exactly when it begins. The
100,000 reference number?
person may for example:
• 1.8 million have dementia in India and South
• Individual may have problems talking
Asia in people >60 years of age. [11]
properly (language problems).
• 400,000 new cases per year for India + South
• Individual may have significant memory loss
Asia.[11]
- particularly for things that have just
happened. • 1,034 per 100,000 DALYs.
• The individual may not be oriented.
TREATMENT FOR DEMENTIA:
2-Middle stage (developed in second to fifth
PHARMACOTHERAPY:
year):
Clinicians may prescribe benzodiazepines for
As the disease deteriorates gradually, limitations
insomnia and anxiety, antidepressant for
become clearer and more restricting. The person
depression and antipsychotic drugs for delusions
with dementia has difficulty with day-to-day living
and hallucinations, but they should be aware of
and:
possible distinctive drug effects in older people. In
• May become very forgetful - especially of general, drugs with high anticholinergic activity
recent events and people's names. should be avoided.
• Can no longer manage to live alone without Donepezil, rivastigmine, galantamine and tacrine
problems. and cholinesterase inhibitors used to treat mild to
• Is unable to cook, clean or shop. moderate cognitive impairment in Alzheimer's
disease. They reduce the inactivation of the
3-Late stage (developed in fifth year or after): neurotransmitter acetylcholine and thus potentiate
the cholinergic neurotransmitter, which in turn
This stage is one of near total dependence and
produces a modest improvement in memory and
inactivity. Memory disturbances are serious and the
goal-directed thought. These drugs are most useful
physical side of the disease becomes more obvious.
for persons with mild to moderate memory loss
The person may:
who have sufficient preservation of their basal
• Have difficulty eating. forebrain cholinergic neurons to benefit from
• Be incapable of communicating. augmentation of cholinergic neurotransmission.

• Not recognize relatives, friends and familiar Donepezil: AriceptTM is the trade name for
objects. donepezil. It is a cholinesterase inhibitor, making
it a drug from the main class of compounds now
272 Neuro-Rehabilitation : A multi disciplinary approach

used to treat people with Alzheimer's disease. Memantine: Memantine goes by several trade
AriceptTM has been used in a large number of names, or brand names, including EbixaTM,
clinical trials, including some from Canada that NamendaTM (in the United States) and AxuraTM.
have used a variety of individualized symptom- It is an NMDA, or N-Methyl-D-aspartic acid. (N-
based tests of its effectiveness. In 2006, the US Food methyl-D-aspartate) receptor antagonist, making it
and Drug Administration approved AriceptTM as distinct from cholinesterase inhibitors, the other
a treatment for severe dementia associated with class of compounds commonly used to treat people
Alzheimer's disease. with Alzheimer's disease. The reason to block the
Dosage: This is taken once a day, with food. The NMDA receptor is to block transmission of a brain
starting dose is 5 mg/day. After a month on the chemical called glutamate. Glutamate is a
drug, if it is well tolerated, most patients will be neurotransmitter, which is used widely throughout
increased to the 10 mg/day dose. Usually, at least the brain, but too much glutamate is felt to cause
two months more are needed to know whether the calcium overload in brain cells. The theory is called
drug is meeting the goals of treatment. Glutamatergic Excitotoxicity.
Rivastigmine: ExelonTM and Exelon TMPatch are Dosage: Briefly, memantine is taken twice a day,
the trade names for rivastigmine. It is a with food. The starting dose is 5 mg/day. After a
cholinesterase inhibitor for the treatment of week, this is increased to 5 mg, twice a day. If it is
Alzheimer's disease. It is also used to treat people well tolerated, most patients will be increased to
with Lewy body dementia, and with the dementia the 10 mg/ once a day and 5 mg once a day, after
of Parkinson's disease. which the dose increases to 10 mg twice a day.
Dosage: The starting dose is 1.5 mg, twice a day. Usually, after starting at the 10 mg twice a day dose,
Typically, after one month, this is increased to 3.0 at least two months are needed to know whether
mg, twice a day, which is often the lowest effective the drug is meeting the goals of treatment.
dose. Patients who tolerate that dose can have it Donepezil is well tolerated and widely used.
increased to a dose of 4.5 mg twice a day and later Tacrine is rarely used, because of its potential for
to a dose of 6.0 mg twice a day. These are the hepatotoxicity. Fewer clinical data are available for
recommended doses. rivastigmine and galantamine, which appears more
Exelon TMPatch is now available in some countries, likely to cause gastrointestinal and neuropsychiatric
including the United States and Canada. It is adverse effects than donepezil. None of these
applied to the back, chest or upper arm and medications prevents the progressive neuronal
provides continuous delivery of medication degeneration of the disorder.
through the skin over 24 hours. Treatment is
Other Treatment Approaches: Other drugs being
initiated with Exelon TMPatch 5 (9mg/5cm2) with
tested for cognitive-enhancing activity include
a release rate of 4.6mg over a 24 hour period to a
general cerebral metabolic enhancers, calcium
maximum dosage of Exelon TMPatch 10 ( 18mg/
10cm2) with a release rate of 9.5mg over a 24 hour channel inhibitors, and serotonergic agents. Some
period. studies have shown that selegiline, a selective type
B monoamine oxidase inhibitor, may slow the
Galantamine: Reminyl ER is the trade name for advance of this disease. Ondansetron, a 5 HT 3
galantamine. It is a cholinesterase inhibitor, making receptor antagonist, is under investigation.
it a drug from the main class of compounds now
used to treat people with Alzheimer's disease.. In Estrogen replacement therapy: Estrogen
addition to acting like the other cholinesterase replacement therapy may reduce the risk of
inhibitors, it also directly stimulates a class of cognitive decline in postmenopausal women;
receptors to which acetylcholine binds. however, more studies are needed to confirm this
effect. Complementary and alternative medicine
Dosage: Briefly, galantamine (extended release) is studies of ginkgo biloba and other phytomedicinals
taken once a day, with food. The starting dose is 8 to see if they have a positive effect on cognition.
mg/day. After a month on the drug, if it is well Reports have appeared of patients using
tolerated, most patients will be increased to the 16 nonsteroidal anti-inflammatory agents having a
mg/day dose, and there is also a 24 mg/day dose. lower risk of developing Alzheimer's disease.
Usually, at least two months after starting at the 16 Vitamin E has not been shown to be of value in
mg/day dose are needed to know whether the drug preventing the disease.
is meeting the goals of treatment.
Dementia 273

PSYCHOLOGICAL INTERVENTION: which is used to assess the communication


changes in the patient. This scale is completed
Psychological Intervention is very often required by staff or family caregivers and covers a range
in geriatrics but for individuals who are suffering of social behaviour and communication
from serious disorders it is very important that they variables. [16]
undergo psychological treatment. Psychological
treatment very often involves working in an 6. Cornell Scale for Depression in Dementia: This
orchestrated way with several professionals. scale is used to assess depression. This scale
consists of five broad categories using the
Intervention Plan: information from interviewers, with staff and
• Early testing and diagnosis participants [17].
• Optimization of physical health, cognition, 7. Rating Anxiety in Dementia Scale (RAID): This
activity and overall well being. scale assesses anxiety in the individual
suffering from dementia. This scale rates
• Detection and treatment of Behavioural and
anxiety in four main categories and uses
Psychological Symptoms of Dementia.
interviews with staff and participants. [18]
• Educating carer and providing long term
8. Neuropsychiatric Inventory (NPI): This test is
support to carer.
used to assess behavioural disturbances in
patients with dementia. To assess
PSYCHOLOGICAL ASSESSMENT psychopathology in the person with dementia
FOR DEMENTIA:[12] and to help distinguish between the different
1. ADAS- Cog: This test is used to measure causes of dementia. Acute, Primary,
cognition which consists of 11 tasks measuring Community and Residential Care. Includes
the disturbances of memory, language, praxis, symptoms known to be rare in Alzheimer's
attention and other cognitive abilities which disease, but are characteristic of fronto-
are often referred to as the core symptoms of temporal dementias. It also assesses the level
Alzheimer's disease. This is recommended for of caregiver distress engendered by each of the
the second stage on Alzheimer's disease. The neuropsychiatric disorders. This test assesses
estimated evaluation time of this test is 30 to 10 behavioural disturbances occurring in
45 minutes. This test is usually administered dementia patients. It takes around 10 to 20
by a neuropsychological or a clinical minutes to administer. [19]
psychologist. [13] 9. Short Form-12 Health Survey (SF-12): This
2. Quality of Life - Alzheimer's disease Scale scale is used to measure generic health
(QOL - AD): This test is used to measure the concepts relevant across age, disease, and
quality of life. This scale consists of 13 domains treatment groups. The SF-12 includes 8
to measure quality of life. [14] concepts commonly represented in health
surveys. It is a self administrative measure that
3. DEMQOL: This test assesses the quality of life provides a comprehensive, psychometrically
in persons with mild to moderate dementia. It sound, and efficient way to measure health
takes approximately 10 to 20 minutes for from the patient's point of view by scoring
assessment. standardized responses to standard questions.
4. Modified Mini - Mental Status Examination This measure is only used when a family
(3MS): The Modified Mini - Mental caregiver is available from the community
Examination is a brief widely used test to sample participants. [20]
measure the cognitive functions with good 10. Frontal Executive Function: The FAB is a brief
reliability and validity. The administration tool that can be used at the bedside or in a clinic
time taken is approximately 15 minutes. setting to assist in discriminating between
Qualified health-care professional (at least dementias with a frontal dysexecutive
trained in the Mini Mental) interviews the phenotype and Dementia of Alzheimer's Type
patient using a standard set of questions. (DAT). The FAB has validity in distinguishing
Scoring takes 5 min. [15] Fronto-temporal type dementia from DAT in
5. Holden Communication Scale: This is a scale mildly demented patients (MMSE > 24). Total
274 Neuro-Rehabilitation : A multi disciplinary approach

score is from a maximum of 18, higher scores Scoring:


indicating better performance. It takes
Score the clock based on the following six-
approximately 1o minutes to administer and
point scoring system:
5 minutes to score.
1- Perfect: No errors in the task
11. Behavioural Pathology in Alzheimer's Disease
(BEHAVE-AD): The purpose of this test is to 2- Minor visuospatial error
measure behavioural and psychological 3- Inaccurate representation of 10 after 11
symptoms of dementia in persons with when visuospatial organization is perfect
Alzheimer's disease. It takes approximately 20 or shows only minor deviations.
minutes.
4- Moderate visuospatial disorganization of
12. Clinical Dementia Rating (CDR): The purpose times such that accurate denotation of 10
of this test is to assess the severity of cognitive after 11 is impossible.
and functional impairment in dementia. The
severity of dementia is rated on the following 5- Severe level of disorganization as
severity scale: described in scoring of 4

0 - No Impairment 6- No reasonable representation of a clock

0.5 - Questionable Impairment The first step in intervention of dementia is


verification of the diagnosis. Accurate diagnosis is
1 - Mild Impairment imperative, for the progression may be halted or
2 - Moderate Impairment even reversed if appropriate therapy is provided.
Preventive measures are important, particularly in
3 - Severe Impairment
vascular dementia. Such measure might include
This is a very comprehensive test and takes changes in diet, exercise and control of diabetes and
approximately 40 to 75 minutes. hypertension. Pharmacological agents might
include antihypertensive, anticogagulant or
13. Clock Drawing Test: antiplatelet agents. Blood pressure control aim for
Instructions for the Step 1: Give patient a the higher end of the normal range, because that
sheet of paper with a large (relative to the size has been demonstrated to improve cognitive
of handwritten numbers) pre drawn circle on function in patients with vascular dementia. Blood
it. Indicate the top of the page. pressure below the normal range has been
demonstrated to further impair cognitive functions
Step 2: Instruct patient to draw numbers in
in the patient with dementia. Surgical removal of
the circle to make the circle look like the face
carotid plaques may prevent subsequent vascular
of a clock and then draw the hands of the clock
events in carefully selected patients. The general
to read "10 after 11."
treatment approach to patients with dementia is to
provide supportive medical care, emotional
support for the patients and their families, and
pharmacological treatment for specific symptoms,
including disruptive behaviour.

Treatment for Behavioural Issues in Dementia:


Treatment strategies for behavioural problems in
dementia include:
• Nonpharmacological Approach (e.g., behavior
therapy, cognitive stimulation therapy,
psychoeducation)
• Pharmacological Approach (e.g.,
antipsychotics, antidepressants, mood
stabilizers, cognitive enhancers)

Clock drawing Test


Dementia 275

PHARMACOTHERAPY FOR nonpharmacological and pharmacological


treatments are effective in decreasing the burden
BEHAVIOURAL AND
of behavioural symptoms, they usually require
PSYCHOLOGICAL PROBLEMS: sustained input from a multidisciplinary team and
Pharmacotherapy should be initiated only if the ongoing staff training to maintain superior quality
patient's symptoms have not responded adequately of care for patients with behavioural symptoms.
to nonpharmacological interventions, if there is no Medications used to treat Behaviour and
underlying medical condition causing these Psychological Symptoms of Dementia: These
symptoms, and/or if these symptoms are not medications are prescribed by a psychiatrist after
related to a medication effect. Although these unsuccessful nonpharmacological interventions.

Class of Name of Dosage Range Common Side Effects


Medication Medication (mg/day) of Medication Class
Antipsychotics Aripiprazole 2.5-15 Sedation, extrapyramidal
Haloperidol 0.5-5 symptoms, neuroleptic
Risperidone 0.25-2 malignant syndrome,
Quetiapine 25-200 metabolic syndrome, QTc
Olanzapine 2.5-15 prolongation
Antidepressants Fluoxetine 10-80 Sedation, extrapyramidal
Citalopram 10-60 symptoms, neuroleptic
Paroxetine 10-50 malignant syndrome,
Sertraline 25-200 metabolic syndrome, QTc
Trazodone 25-200 prolongation
Mood stabilizers Fluoxetine 100-400 Sedation, gait and balance
Citalopram 250-1000 issues, falls, liver
Paroxetine 300-600 dysfunction,
Sertraline hyperammonemia,
Trazodone thrombocytopenia
Cognitive enhancers
Donepezil
Galantamine
Rivastigmine
Memantine
5-10
8-24
3-12
5-20
Sedation, gastrointestinal
disturbance, confusion,
Cognitive enhancers Donepezil 5-10 Sedation, gastrointestinal
Galantamine 8-24 disturbance, confusion,
Rivastigmine 3-12 Agitation
Memantine 5-20

Current evidence indicates the efficacy of short- nonpharmacological interventions [21, 22] Evidence
term pharmacological treatment for behavioural indicates that these antipsychotic medications
symptoms and psychological symptoms. Although generally should not be continued for longer than
limited, available evidence remains in favor of using 12 weeks11-13, [22] however, longer-term therapy
atypical antipsychotic drugs, especially risperidone, may be needed in patients who have persistent
aripiprazole, and, to a lesser extent, olanzapine in symptoms, although the data for longer-term
the first-line treatment of BPSD that is resistant to treatment with antipsychotics are limited[23].The
276 Neuro-Rehabilitation : A multi disciplinary approach

possible benefits of prescribing these medications anticonvulsants, antidepressants, cholinesterase


should always be weighed carefully against the inhibitors, and memantine, although these drugs
risks. Careful monitoring and treatment of risk may have better efficacy in treating nonaggressive
factors for cerebrovascular adverse events such as behaviours such as apathy than in treating
hypertension, atrial fibrillation, hyperlipidemia, agitation, aggression, and psychotic symptoms
and diabetes mellitus should help reduce not only such as delusions and hallucinations. Despite the
the risk of cerebrovascular adverse events, but also growing evidence for pharmacotherapy for BPSD,
the risk of death [23]. Antipsychotics should also none of the aforementioned medication classes have
be prescribed within the recommended dosage been FDA-approved for these behaviors and
ranges to reduce the risk of serious adverse events symptoms. Information on common scales used to
such as cerebrovascular adverse events and death. assess the severity of dementia is listed in table an
The best pharmacological alternatives to algorithm for the treatment of BPSD.
antipsychotics in the treatment of BPSD are

Figure. Algorithm for the treatment of BPSD.


Abbreviations: AchEIs, acetylcholinesterase inhibitors; ADAS-Cog, Alzheimer's Disease Assessment Scale-
Cognitive; BPSD, behavioral and psychological
symptoms of dementia; CDR, Clinical Dementia Rating; FAST, Functional Assessment Staging; GDS, Global
Deterioration Scale; MMSE, Mini-Mental State
Examination; SIB, Severe Impairment Battery; SSRI, selective serotonin reuptake inhibitor.
Dementia 277

PSYCHOTHERAPY: feelings it produces. Clinicians can help caregivers


understand the complex mixture of feelings
Psychotherapy for older people has flourished associated with seeing a loved one decline and can
despite the now famous statement by Sigmund provide understanding as well as permission to
Freud that older people are not amenable to express these feelings. Clinicians must also be aware
psychoanalysis. According to him anyone over the of the caregiver's tendencies to blame themselves
age of 50 years lacked "the elasticity of the mental or others for patient's illness and must be to
processes on which treatment depended....old appreciate the role that patients with dementia play
people are not educatable" [24].Eventually this view in the lives of family members.
was not encouraged and now psychotherapy is
offered to the older population. Psychotherapy is Dynamics:
used to work on various psychiatric problems,
Psychotherapy is mostly based upon a dynamic
emotional issues, and adjustment problems.
view of psychiatric (and some physical) illnesses.
The deterioration of mental faculties has significant However, the word dynamic suggests activity
psychological meaning for patients with dementia. where psychiatric symptoms arise, conflict often
The experience of a sense of continuity over time leads to a deadlock. Such a conflict might be
depends on memory. Recent memory is lost before between the drive to assert oneself and to make no
remote memory is most cases of dementia, and trouble. Dynamic factors are important in old age
many patients are highly distresses by clearly psychiatry. The main aim of psychotherapy is to
recalling how they used to function while observing resolve the conflict and, in doing so, reduce
their obvious deterioration. At the most symptoms and help the person function more
fundamental level, the self is a product of brain effectively. This also focuses on improving the
functioning. Patients identities begin to fade as the patient's quality of life. This is achieved through
illness progresses, and they can recall less and less words exchanged between the patient and the
of their past. Emotional reactions ranging from therapist, and the relationship which forms
depression to severe anxiety to a catastrophic terror between them. Pitt (1982) [24] identified dynamic
can stem from the realization that the sense of self factors in three-quarters of consecutive referrals to
is disappearing. an old age psychiatric service.
Patients often benefit from a supportive and
Transference:
educational psychotherapy in which the nature and
course of their illness are clearly explained. They The dynamic theory is of the outlook that disturbed
may also benefit from assistance in grieving and relationships, past and present, contribute to illness.
accepting the extent of their disability and from Transference takes place when the patient reacts
attention to self- esteem issues. Any areas of intact to the therapist as a key figure from their past, such
functioning should be maximized by helping as a parent, sibling, child or spouse. The therapist
patients identify activities in which successful can use transference by helping the patient become
functioning is possible. A psychodynamic aware of subconscious feelings to these important
assessment of defective ego functions and cognitive figures. Although the patients are usually older
limitations can also be useful. Clinicians can help than their therapists, this does not prevent them
patients find ways to deal with the defective ego from being regarded as parent figures, as well as
functions, such as keeping calendars for orientation child (or grandchild) figures.
problems, making schedules to help structure
activities and taking notes for memory problems. Dependency:
Dependency is commonly seen in later stages of
Psychodynamic intervention with family members
life. This takes place in people who are emotionally
of patients with dementia may be of great
deprived during the earlier days of life and who
assistance. Those who take care of patient struggle
had good coping mechanisms during adulthood
with feelings of guilt, anger and exhaustion as they
but in older age feared of being neglected and not
watch a family member gradually deteriorate. A
loved as the see their useful role slipping away.
common problem that develops among caregivers
Goldfarb (1965) [25] described the frantic search for
involves their self-sacrifice in caring for patients.
help from a strong parent figure, which is frustrated
The gradual developing resentment from this self -
because the demands are too clamorous. In
sacrifice is often suppressed because of the guilt
partnership, i.e. between husband and wife or child
278 Neuro-Rehabilitation : A multi disciplinary approach

and parent, one of them is usually comes out to be There might be other problems with
stronger than the other but in fact they are communication, for example deafness or poor
dependent on each other. The 'strong' partner vision, which can compound the effects of ageing
preserves an illusion of mastery through the in reducing information processing capacity. The
wreaker's reliance on them. In these cases the overall effect is to make demanding tasks such as
therapist tries to establish a supportive relationship therapy very difficult. Physical or mental illness can
to meet the dependency needs of the patient, be used to scapegoat the older person. Conversely,
without being taken over, or turning the patient symptoms of physical illness can be accentuated
into a child. For some very old patients, supportive or become an important vehicle for the older
therapy may mean an involvement for life. person's status and power (West & Spinks, 1988).
Benbow & Marriott (1997) [27] listed the following
Confrontation: ideas as being useful in family therapy with older
Confrontation may play a very important part in adults:
therapy, in spite of the pleasant personal remarks
(a) The family life cycle: looking at how families
and warmth that a client shares with the therapist.
evolve. Key issues in later family life include
Negative reactions to the therapist such as
retirement and becoming a grandparent.
plaintiveness; exaggeration of symptoms; seeking
help elsewhere; or lateness for appointments, (b) Cross-generational interplay - life cycle
should be explored. Skilfully handled these changes in different generations may not 'fit'.
confrontations allow the patient to acknowledge One generation may be more family orientated
resentment and aggression, and find relief that (e.g. during childbirth) while others are more
therapy can continue. outward looking (e.g. early retirement).
Expectations may vary across the generations.
Group therapy:
(c) Genograms - drawing a family tree is a useful
Group therapy is a good medium of meeting other
way of collecting, organising and considering
patients with the same problem and working
family information.
through the problems including dealing with the
dependency issue. This form of therapy is very (d) Circular questions - these are in terms of
intense and than an individualized session and this relationships. Examples include, "If your
is less likely to lead to regression. Group therapy mother says this, what does your brother do?"
often helps the older isolated patient to get back to or "who in the family would this affect?"
socialization. Group therapy works as a stress (e) Reflecting teams - members of the multi-
buster and works as a ventilation for patients as disciplinary team talk about the family while
patients are quite comfortable sharing their feelings they listen, offering different perspectives. The
and emotions to people with the same problem than systemic approach derived from family
someone normal who finds it difficult to understand therapy can also be applied to the care of older
their problem. Dobson & Culhane (1991) [26] people in institutions [28] Beckenham: Croom
describe a therapeutic group run for older women. Helm.. Sometimes the problems attributed to
They emphasise the importance of having a clear one or more residents are better addressed by
purpose for a group and considering selection looking at the social network and relationships
criteria carefully. In the early stages, rules such as in the home.
not talking while others do, and valuing others'
contributions, helped to harness good intentions. Marital therapy:
Family therapy: Marital therapy is extremely important as very
often the one who cares the maximum for the
As caregivers are present with the patient for a
patient and looks after him is his/her partner.
longer period of time especially of the patient's
Throughout the life the patient and the caregivers
functioning has reduced then family therapy is
are struggling with various things like bringing up
extremely important to maintain the family
children, running the household, etc and after
dynamics or make it more fulfilling for the patient.
retirement when its now time for them to rest and
Most of the times the caregivers are totally involved
relax, either of the partner suffers from dementia
with the patients physical and emotional needs and
and this puts the other partner in a position to take
due to which very often the caregivers might be at
complete responsibility of the partner. Illness in one
a fix between their needs and those of the patient.
Dementia 279

partner may result in guilt in the other. It would Grief Counseling:


also lead to frustration and irritations but the Although bereavement is not exclusively a problem
caregiver would not be able to express it and would of later life it is more common, and can mean the
in return feel guilty about his/ her thoughts. loss of an intimate relationship which has lasted
many years. One ageist stereotype is that older
Cognitive and behavioural therapies: people are better at handling loss because it is
The most frequently reported developments have expected, or because they have had more practice.
been with younger people (Williams, 1984), but In fact older people are not only more likely to
there have been examples of applications to older experience loss, but the effects are cumulative in
people which have now been reviewed. There are terms of risk for depression (Murphy, 1986) [30].
three reasons why cognitive and behavioural Being recently widowed is a major risk factor for
therapies are particularly suited to the elderly: mortality (Rees & Lutkins, 1967).
(a) Depression in later life is prone to relapse and Losses can also reduce social support, either
there is evidence that cognitive and because friends and family have died, or because
behavioural approaches have longer-term physical illness enforces isolation. Appropriate
benefits. Patients may develop preventative interventions include bereavement therapy and
skills. practical help to improve social networks.
(b) Older people are more susceptible to adverse Important steps are recognizing that something is
side-effects from drug therapy, especially wrong and encouraging the older person to accept
those who are physically ill or taking help.
medications for other illnesses.
(c) Spontaneous remission is less likely in older Therapies for Dementia:
depressed people (Lambert, 1976; Thompson
et al, 1987). Lambert, M. J. (1976) [28] Evidence Reality Orientation:
suggests that depression often goes Reality orientation is perhaps the best known
unrecognized or untreated when it occurs with specific psychological treatment for older people
physical illness (Koenig et al, 1992). Only 10% (Holden & Woods, 1988) [31]. Reality orientation
of their sample of depressed older men in aims to help patients with dementia by directly
hospital received some form of psychological focusing on some of the deficits of the disorder,
therapy, and 44% received no treatment at all. including disorientation and impaired short-term
They suggest that behavioural and cognitive memory. It also helps to preserve skills. The
techniques are a viable therapeutic option for approach can be divided into brief sessions
the 50% of patients or more who have medical (classroom reality orientation) and a pervasive
contraindications to antidepressants. approach influencing staff-patient interactions
throughout the day (24-hour reality orientation).
Anxiety management:
Underlying both of these types of approach is the
The prevalence of phobic disorders is higher than principle that staff enhances orientation by using
might be expected, with a one month prevalence identifying names and other information. This is
of 10% (Lindesay, 1991) [29]. Disability from supported by cues such as the commonly used
phobias can be significant but specific treatment is reality orientation board, showing the date,
rare. The majority of these patients suffer from a weather, etc. (providing it is kept up to date!) and
late-onset agoraphobia following a traumatic cues to everyday behaviour such as making tea or
experience, typically an episode of serious physical visiting a pub. Activities within formal reality
illness. They have a higher than average contact orientation sessions include prompting basic
with primary care services but tend not to be information such as the names of group members;
referred for specialist treatment. While it may be looking at current events; and using tactile,
necessary to adapt anxiety management for older olfactory and other stimuli to encourage active
people, it is just as effective as in younger patients. cognition.
Older people may benefit from anxiety techniques
such as relaxation therapy with tapes. By using The enthusiasm with which reality orientation has
headphones it is possible to deliver soothing been embraced by staff has sometimes exceeded the
instructions loudly, so overcoming all but the most evidence for its effectiveness (Powell- Procter &
severe hearing loss. Miller, 1982). There is evidence that reality
280 Neuro-Rehabilitation : A multi disciplinary approach

orientation is beneficial, but the benefits tend to be Validation therapy:


modest, and only sustained with continued effort. Concerns about the specific effects of reality
There is little evidence to suggest that generalisation orientation in confronting dementia sufferers with
occurs (i.e. encouraging orientation for time does their failings have crystallised around the
not lead to gains in orientation for place). There are development of an alternative therapy which is
also doubts about the suitability of reality directed at emotional needs. There can, not
orientation for use by informal carers at home. surprisingly, be problems in reorientating people
Perhaps the biggest impact of reality orientation when the reality is upsetting. A common example
has been on staff attitudes, where it has resulted in given is of a disoriented person who wants to go
staff improving the environments in which they home to his or her spouse, not remembering that
work. When you orientate someone to their they have died. Is reorientation the best solution in
environment you become more aware of it yourself. this situation? Validation therapy focuses on the
There is little point in orientating someone to the phenomenology of dementia at an emotional,
day if all days are the same. rather than a factual level. It views the disoriented
person as struggling to cope with a complex and
Reminiscence: confusing world. It is hypothesised that the content
Thinking about the past is not exclusive to older of 'confused' talk reflects the emotional meaning of
people. Younger people reminisce, although their past events. For example, worrying about getting
memories may not extend back so far. Similarly, home in time to meet the children may reflect that
older people are not always reminiscing. Some parenting was a time of reward and security.
avoid doing so because it is painful, others because The response to the disorientation is directed at
they prefer the present. Nevertheless for many exploring what things were like for that person, and
older people looking back is an important part of how this relates to how they are feeling now. It is
making sense of themselves and their lives. It allows suggested that even the most confused behaviour
the kind of integration that Erikson (1959) suggests has some meaning for that person. Validation
is important as a developmental task of later life. therapy has begun to be reported but more
The ability to reminisce is preserved in early investigation is needed (Jones, 1997) [33]. It remains
dementia. People with dementia are better at to be demonstrated how validation therapy and
remembering what happened many years ago than reality orientation differ. Of course bad reality
what happened this week or earlier this morning. orientation can certainly be frustrating and even
The act of reminiscence therefore offers a good way unkind. However, closer scrutiny of the practice of
of engaging people with mild to moderate cognitive experienced therapists suggests that they are
impairment without reminding them of their sensitive to the emotional content of 'confusion', and
cognitive shortcomings. It is quite possible to use avoid inappropriate confrontation. We need to find
reality orientation and reminiscence in the best responses to people with dementia.
combination. Starting with reality orientation
before progressing to reminiscence appears to offer Memory therapy:
better results (Baines et al, 1987) [32]. Whether the The emergence of specific approaches aimed at
activity of reminiscence, per se, can be considered helping those with memory problems would seem
a 'therapy' is open to question. Certainly to be of particular interest to those treating the
reminiscence can have powerful emotional elderly. On the whole, however, the work has been
consequences and may be used with specific directed at younger brain damaged patients. This
therapeutic targets in mind. However, it is neither is because the conditions for memory therapy are
a panacea nor is it always appropriate for older not met so well in dementia. In particular the use
people. The overlap between reminiscence and of mnemonics or memory aids require insight into
post-traumatic stress disorder requires more careful memory loss, as well as preserved language and
study. Certainly there are older people whose psychomotor skills. Older people with mild
problem is not how to remember but rather how to memory problems can make use of simple aids such
forget memories from wartime or other past as lists, alarms and placing notices and instructions
trauma. The effects of reminiscence, like reality in key areas. A useful list of such aids is given by
orientation, are dependent on the environment. Burnside (1988) [34]. However, these are of major
There is a more obvious effect in a less interactive significance in only a few cases. These tend to
environment (Head et al, 1990). involve people with mild or relatively static
Dementia 281

Colour Recognition Problem solving with colours and numbers

Problem Solving with colours Recognition of various denominations

memory problems, such as following a stroke. For routines, eating patterns, personality traits,
those with progressive dementia prompts relationships with family friends, hobbies, etc.
supplement, rather than replace, the presence of This would help the client remember things
an alert carer. from the past and would help the family
members compare the past and present state
Resolution therapy: of the patient.
Resolution therapy has been introduced as a • Interview the patient Alone: This will help to
companion to reality orientation (Stokes & Goudie, know the patients personality, the way he
1990) [35]. It shares with validation therapy the responds to situations, his social and family
assumption that there is meaning in the behaviour structure and way of functioning.
and confused talk of patients with dementia. But,
unlike validation therapy, it looks for that meaning • Attune the environment - Wherever possible
in the 'here and now'. In other words it sees such interview the person with dementia in his/her
behaviour or speech, as an attempt to make sense own home or room. Familiarity is imperative
of what is happening now, or to communicate a to a person with dementia feeling safe and
current need. In order to try and understand these comfortable. Be sure that the environment is
hidden meanings, the therapist must use reflective free from noise, interruptions and distractions;
listening, exploration, warmth and acceptance. i.e., is quiet, pleasant and calm.

Psychological Home Care Strategies: Communication Strategies for Psychologists:


Listening to a patient with Dementia:
• Prepare a meaningful time table for the patient
i.e. brings activities that are of interest to the • Communicate interest and respect to patients
person keeping in mind their level of ability. with dementia by maintaining eye contact and
Also this will help the patient practice and posing a relaxed body language.
remember his daily routine sequentially. • Facial expressions and gestures communicate
• Historic snapshots: Gather as much as about the person's feelings and emotions in the
information about the patient and his past situation, hence learning to read these signs
experiences and memory. For example: are very important.
282 Neuro-Rehabilitation : A multi disciplinary approach

• Be calm, patient and don't interrupt while the Communication Strategies for Psychologist:
patient is speaking as this would interrupt the Speaking to Patients with Dementia
client's thought process or would stop the • Use short, simple, familiar and precise words,
emotional flow. sentences and commands.
• Enter the world with them as they remember • Take on a similar posture to theirs to develop
whatever they are expressing is actually where rapport non-verbally be on their level.
they are in time. (Their past is their present,
the present is their future, and the future • Go to their eye level and be sure that they can
doesn't exist because they can't store memory.) see you clearly when you talk and listen.

• Avoid criticism, correcting and arguing as this • Other forms of communicating include music,
can be traumatic for the person. Focus on touch, food and joint activity.
feelings, not facts, and encourage non-verbal • Treat person as an adult and don't be
communication. condescending.
• Offer best guess if you don't understand what It is important to remember that the person who is
is said by the patient as this will make the suffering from dementia and has associated
person feel secured and he will feel problems had at some point of time a life rich with
understood. history, experience, relationships, skills, hopes and
• Engage the person's "body memory" - called dreams. In your role, you may have a wealth of
the "chaining" technique - to help them initiate information about this person as he/she declines,
or sustain an activity. For example place a glass and it is important to share this information to keep
of water in the hand. continuity of care and interests alive for the person.
Be sure to inquire into the activities that have had
• Confabulating serves to fill gaps in memory. meaning to see if they are still being initiated.
Persons with dementia may make assertions
that are not true to cover for memory loss. Behavioural Strategies for Dementia
Trying to argue someone out of such beliefs is Caregivers:
usually futile because the person is not lying.
• Families and carers may find the following
• Refusal to cooperate may be due to sadness, strategies helpful when responding to the
anger, frustration, embarrassment, anxiety. behavioural and psychological symptoms of
Step back calmly to previous activity and dementia:
assure the person that he/she is safe.
• Every individual has a specific behaviour
• Recollection of old memories are key pattern, so do people suffering from dementia
ingredients to success such as humming a have. One needs to look for behaviour pattern
favorite song, talking about a pet, offering a and try to identify any triggers, as certain
familiar photo or object for a story. Smells, behaviours are displayed on at certain times
taste and touch are also strong memory or during particular activities. For example: at
triggers. a very noisy place, or very bright light which
• Offer comfort and reassurance especially may add to the confusion and restlessness.
when the person is having difficulty • Establishing a routine is very helpful as it is
expressing self; offer praise for success in helps patients with dementia be oriented, have
accomplishment (e.g., completing a thought, sequential memory feeling secure. The person
reciprocating in an activity). with dementia may become upset if they find
• Use "bridging" technique, a sensory connection themselves in a strange situation or among
that increases focus/attention and decreases unfamiliar people, and may become confused,
anxiety. This could be a touch, a light guide anxious, or agitated
on the elbow to steer, humming, stroking the • Clear and simple communication is very
skin with an object that has a unique surface important i.e. giving specific commands. The
such as satin. Be sure to ask permission before person with dementia may become agitated if
touching; tell the person what you are doing they do not understand what is expected of
as you do it. them. They may also feel frustrated with their
Dementia 283

inability to make them self understood. It is OCCUPATIONAL THERAPY


important to face the person, speak slowly in
IN DEMENTIA:
a calm and reassuring voice and use simple
sentences. Be patient and allow extra time. The World Health Organization defines dementia
Ensure the person with dementia participates as: "a syndrome due to disease of the brain, usually
as much as possible in daily activities. Make of a chronic or progressive nature, in which there
sure the task isn't too complicated or the is disturbance of multiple higher cortical functions,
person with dementia may become including memory, thinking, orientation,
overwhelmed. comprehension, calculation, learning capacity,
language, and judgment. However, consciousness
• Regular exercise is very important. Simple
is not clouded. The impairments of cognitive
exercise, like a walk outside or a game, can
function are commonly accompanied, and
reduce anxiety and depression and can
occasionally preceded, by deterioration in
minimise other behaviours such as wandering
emotional control, social behavior, or motivation
and restlessness
which occurs usually in Alzheimer's disease, in
• Distraction often helps. If possible, direct the cerebrovascular disease, and in other conditions
person with dementia away from a particular primarily or secondarily affecting the brain." (World
task or environment that seems to be Health Organization,2007). Dementia is not a
triggering the behaviour. Suggest a different specific disease, but rather symptoms caused by
activity that the person with dementia may other illness or conditions that affect the brain.
like, such as listening to a favourite song, or Contrary to what many people believe, it is not a
going outside. normal part of the aging process. It is a severe loss
• Avoid punishment. The person with dementia of cognitive ability, such as thinking, memory, and
may not remember the event, and therefore reasoning, that interferes with a person's daily
may not be able to learn from it. Try to stay functioning. Symptoms include personality
positive. changes, behavioral problems, and memory
problems.
• Be consistent. If there are strategies for
modifying behaviours that work, try to ensure Goal of Occupational Therapy:
other people who also care for the person with The primary goal of rehabilitation is to enable
dementia use the same strategies. people to achieve their optimal level of function.
• Communication is important. Always explain The challenge of occupational therapy is to create
to the person with dementia what is going to a comfortable balance between patient safety and
happen. Speak slowly in a calm and reassuring maximum independence. The focus is on the
voice, and use simple sentences. Be patient. strengths and the preserved capabilities of the
individual to help them achieve quality of life
• Involve the person with dementia as much as
regardless of level of cognitive status. Occupational
possible. Even if the person with dementia
therapists assist individuals to maintain or develop
cannot participate verbally, they can still enjoy
skills, which are needed to achieve functional
certain activities, such as reminiscing about
independence and maintain their occupational role
their past by looking at old photos, or listening
in everyday life using meaningful activities. OT
to a favourite song.
assists patients and family members with changes
• Be sensitive to the reactions of the person with needed in the living situation, different ways/
dementia. Therapies that involve techniques of performing daily activities, and/or
remembering about the past, such as tests patient's safety awareness. Occupational
reminiscence or music therapy, may prompt therapy is shown to improve quality of life for
happy memories, but may also prompt painful dementia patients and their carers.
or sad memories.
OT Assessment:
• Avoid creating discomfort for the person with
dementia. Sometimes the person with The primary focus for the dementia patient is on
dementia may not feel comfortable being habilitative, compensatory, non pharmacologic
touched, so therapies such as massage may not approaches that modify behaviors and/or the
be appropriate for these people. physical and social environment to help individuals
284 Neuro-Rehabilitation : A multi disciplinary approach

cope with the disease and continue to participate to basic self-care and instrumental aspects of
in everyday activities of living for as long as activities of daily living. Scored from 0 (most
possible. An occupational therapy assessment impairment) to 100 (least impairment).
includes
Cornell Scale for Depression in Dementia: The
• History taking Cornell Scale (Alexopoulos et al, 1988) is specifically
for the assessment of depression in dementia. The
• Cognitive and mental state examination
19-item scale is rated on a three-point score of
(include examination of attention and
'absent', 'mild or intermittent' and 'severe'
concentration, orientation, short and long-
symptoms, with a note when the score is
term memory, praxis, language and executive
unevaluable. A score of 8 or more suggests
function)
significant depressive symptoms. It is the best scale
• Physical examination available to assess mood in the presence of cognitive
Occupational Therapist evaluate present abilities impairment.
and functional performance and determine the type
of assistance, compensatory strategy, and Mini-Mental State Examination (MMSE)
environmental modification needed to successfully • It is a rating of cognitive function and takes 10
and safely complete activities. They also provide minutes to administer (Folstein et al, 1975). It
caregiver training in problem-solving, task is the most widely used measure of cognitive
simplification, communication and stress-reduction function.
techniques to ease caregiver's burden. A holistic • 26-30 (Cognitive impairment may still be
assessment of the resident's abilities and present - especially in Fronto-temporal
background is necessary to provide care and dementia and PD) - may require further
assistance that is tailored to the individual's needs. assessment.
People with dementia, which is a cognitive • 20-25 mild cognitive impairment
disability often report functional difficulties. • 10-19 moderate cognitive impairment
Therefore Functional assessments should be done
to identify impairments not demonstrated on • 0-9 severe cognitive impairment (Molloy,
formal cognitive testing. A functional assessment 2000)
by an OT provides data that enables the other team • a score of 23 or less in an individual with more
members to gather a more complete picture of the than 8 years education indicates cognitive
person and their abilities. An OT assessment impairment (Folstein, Folstein, McHugh, and
considers: Fanjiang, 2001)
• The person, • reliable and valid
• The task / activity & Clinical Dementia Rating (CDR): This scale is used
as a global measure of dementia (Hughes et al, 1982;
• The environment.
Berg, 1984). It has become one of the main methods
The purpose of an occupational therapy evaluation by which the degree of dementia is quantified into
is to design an intervention plan to increase stages. Six domains are assessed: memory;
participation, maintain occupational performance orientation; judgment and problem-solving;
or modify activity demands, or prevent community affairs; home and hobbies; and personal
deterioration in performance capability. It is care. Ratings are 0 for healthy people, 0.5 for
important to discern between what the patient can questionable dementia and 1, 2 and 3 for mild,
do and what they actually do. Ongoing evaluation moderate and severe dementia as defined in the
should be adopted to ensure that any strategy/ CDR scale.
intervention used remains appropriate.
Alzheimer's Disease Functional Assessment and
Change Scale (ADFACS) : It is used for the
Assessments Utilized in Dementia are:
assessment of activities of daily living in patients
Disability Assessment For Dementia (DAD): with Alzheimer's disease with particular reference
Designed to quantify functional abilities in activities to outcomes in clinical trials (Galasko et al, 1997).
of daily living in patients with dementia and other It is informant-based and takes 20 minutes. Consists
cognitive impairments. Contains 40 items relating of ten items for instrumental activities of daily
Dementia 285

living: ability to use the telephone; performing • Sensitive to change over time
household tasks; using household appliances;
• High inter rater reliability, testretest reliability,
handling money; shopping; preparing food; ability
concurrent validity and internal consistency
to get around both inside and outside the home;
(Cole and Dastoor, 1996)
pursuing hobbies and leisure
Assessment of Motor and Process Skills
Cognistat
(AMPS)
• It identifies basic strengths and weaknesses
• An observational assessment that is used to
• A standardized evaluation to provide a basic measure the quality of a person's occupational
cognitive profile including tests for performance objectively.
Orientation, Language, Visual Construction,
• Useful for assisting with determining return
Memory, Calculation, & Reasoning.
to independent living and guardianship
• For adolescents, and adults in three age hearings.
groups: 60-64, 65-74 and 75-84
• AMPS is designed to allow the person
• High level of reliability and validity evaluated to choose what ADL task he or she
will perform for the evaluation based on (a)
Clock drawing test the familiarity and relevance of the task to the
• It is a simple screening tool clients daily life needs, (b) the degree of
challenge that the tasks offer the client.
• It is used with people who have executive
cognitive dysfunction and a normal MMSE • 16 ADL motor and 20 ADL process skill items
the person being assessed chooses two familiar
• Measures a range of cognitive functions
and life-relevant ADL tasks to complete.
including visuospatial construction which is
a skill known to be impaired in the early stages • Fully standardized valid reliable and sensitive
of dementia (Schramm et al., 2002) assessment tool.
• A normal clock suggests that a number of Modified Barthel Index (MBI)
functions are intact and contributes to the
weight of evidence that the patient may, for • Measure dependence in ADLs
example, be able to continue independently. • Measures a person's performance in 10 ADLs
Alternatively, a grossly abnormal clock, is an with a maximum score is 100.
important indicator of potential problems
• Lower score the higher the level of dependence
warranting further investigation or resource
(Braunberger, 2001) • Good reliability and validity
• good reliability • High inter-rater reliability
• more sensitive in identifying cognitive Functional Independence Measure (FIM)
impairment than the MMSE (Flood and
Buckwalter, 2009) • Assesses physical and cognitive disability
assessment focusing on the burden of care
Hierarchic Dementia Scale (HDS) • 18 items scored 1-7 for level of independence.
• To determine a baseline and monitor a person's Scores can range from 18 to 126 with higher
cognitive function. scores indicating more independence
• To assist with the formulation of care plans. • Good reliability and validity
• Consists of 20 subscales each worth a When deficits and strengths in performance
maximum of 10, therefore the maximum components and performance areas have been
obtainable score is 200. identified, occupational therapists work with
patients and their caregivers to reduce the barriers
• 160-190 mild dementia
in daily functional activities and facilitate maximum
• 159-42 moderate dementia engagement in their environments, the human and
• 40-0 severe dementia physical context for daily living.
286 Neuro-Rehabilitation : A multi disciplinary approach

MOHO: MOHO is intended for use with any person • difficulty manipulating clothes fasteners e.g.,
experiencing problems in their occupational life and buttons, snaps, hook and eye, zippers
is designed to be applicable across the life span.
• Undressing frequently.
Focus is on
• Systemic, holistic approach for persons of Possible strategies:
varying needs and populations across the • Prompt or remind them of how to dress
lifespan through verbal and physical prompts/cues.
• Stresses the importance of the mind/body Using pictures or drawings of steps for
connection in its depiction of how motivation dressing
(internal) and performance of occupations • Be patient and allow as much time as is
(external) are interconnected necessary for the person to complete steps
• Human occupation is described as the "doing" • Encourage the person to change regularly. If
of work, play, or activities of daily living the patient wants to wear the same clothes
within a temporal, physical, and socio cultural every day, make duplicates available so that
context. favorite clothes can be laundered.
• Interactive nature between the person and his • Use simple one step instructions
environment and how this relationship
• Arrange closets and drawers systematically
contributes to one's source of motivation,
separating individual clothing
patterns of behavior, and performance.
• Break task down into manageable steps
OT intervention:
• Remove unnecessary clutter from the
Through assessments there is clarification of the cupboard
presence or absence of skills, the person strengths
and weaknesses. The present skills are then fully • A mirror maybe useful for providing visual
utilized and developed. Absent skills are feedback while dressing
compensated through the development of various • Ensure environment provides adequate light
strategies. And this helps to minimize the impact and temperature
of the condition on daily life and promote functional
• Limit number of choices provided
independence. Involving the person with dementia
and the family members in the decision making • Utilize color contrast techniques for people
process helps in easy acceptance of therapy. The with visual problems e.g., light color clothing
plan, then developed should reflect client and on dark bedcover
caregiver needs.
• Select non crease clothing so ironing is not
required
ADLs
• Clothes with Velcro tape or elastic waist bands
Dressing: are preferred
Dressing is a very complex task with numerous
steps involved which can be overwhelming for
people with cognitive impairment.
Some of the problems encountered in area of
dressing are:-
• forgetting how to dress, to change clothes or
dressing in the incorrect order (e.g., underwear
over trousers)
• wearing extra layers as judgment and
sensation of temperatures may be impaired
• may recognize the item of clothing but forget
which body part goes into that clothing item
• difficulty with clothes selection Examples of labeling on drawers
Dementia 287

Toileting and Continence: • Once a routine is established try to avoid


A number of difficulties experienced by the person unnecessary changes
when using the toilet including perceptual • Use simple one step instructions
difficulties, falls, hygiene difficulties, anxiety or
• Use positive reinforcement to promote
behavioral problems are:
independence
• Difficulty locating and transferring to the toilet
• Distance to the bathroom Incontinence:
Incontinence is the loss of control of the bladder or
• Difficulty undressing/ maintaining hygiene
bowel function. Being in control of these functions
• Urinating in places other than toilet depends on having an awareness of bodily
sensations and the memory of how, when and
• Falls while going to toilet
where to respond. Incontinence is always a
• Difficulty sitting still to use toilet symptom of an underlying problem. The cause of
Possible solutions: the incontinence should always be investigated as
it may be due to numerous medical reasons such
• Simplify clothing. Use Velcro/elastic instead as infection, constipation, hormonal changes and
of buttons and zippers. prostate enlargement.
• Select clothing that is easy to wear like elastic Dementia can impact on a person's continence by
waistbands for trousers and wrap around interfering with their ability to:
skirts and also easy to wash to wash
• Recognize the need to go to the toilet
• Use visual cues to assist with locating the toilet.
Place a sign or label on the toilet door such as • Short term memory loss resulting in frequent
a picture or a photograph of the toilet in a urination
prominent position. Leave the toilet door open • Be able to wait until it is appropriate to go to
and close all other doors leading to the toilet the toilet
to discourage urinating in other rooms.
• Knowing when the bladder/bowel is empty
• Paint the bathroom/toilet door/ door knobs/
• Locate the toilet
toilet seat/floor tiles & walls a contrasting
color e.g., dark blue to make it stand out in a • Recognize the toilet
lighter colored hallway.
• Use the toilet correctly
• Using large arrows to direct to the toilet from
• Manage clothing
the living room or bedroom
• Attend to hygiene
• Utilizing sensor lights or night lights to avoid
having to enter a dark Suggestions for managing incontinence:
• Glow in dark strips placed around light • Be sure the person is drinking adequate fluids,
switches or in hallway to assist in finding the preferably water, 5-8 glasses. Try to establish
light switch or direct the person to the a regular routine for drinking fluids
bathroom • Reduce the person's caffeine intake by using
• Keep from passageways or stairways free decaffeinated beverages
from clutter • Observe the person's toileting pattern and
• Stick contrasting tape on rails to help locate suggest they use the toilet
them • At regular times that follow their established
• Install grab rails or equipment to assist with pattern
transfers • Utilize protective garments or disposable pads
• Consider keeping a voiding diary (frequency • Utilize suitable aids or appliances.
and amount) which can assist with
establishing a voiding routine e.g., every two • Ensure person consumes a high fiber diet
hours. • Regular exercise
288 Neuro-Rehabilitation : A multi disciplinary approach

Eating:-
Eating habits and behaviors change during the
course of dementia, and may be caused by
physiologic or psychological factors.
• Straws with one way valves
• Modified eating utensils, plates with lips or
rims
• Minimal reflections/glare from polished
surfaces
• Reminders or prompts for meals and
medications e.g., alarm clock or phone call Use of contrasting colours in eating utensils
allow and encourage finger feeding when Bathing and Personal Hygiene
person is no longer able to manipulate cutlery.
Present finger foods on a flat plate at a Personal care is often a sensitive issue for people
comfortable reaching distance with dementia. Tasks associated with hygiene were
once completed independently and privately.
• Reduce clutter: avoid lots of cutlery, crockery
etc. As dementia progresses the person may require
physical assistance with the tasks resulting in a lack
• Keep background noise and activity to a of privacy and autonomy. By focusing on the
minimum, turn off TV person rather than the task the person with
• Serve only one plate of food at a time dementia may be more willing to accept assistance.
Identifying potential triggers and interpreting what
• Allow time for the persons memory to respond
the person is trying to communicate can assist in
• Physical prompts initially to use cutlery making personal care activities positive
experiences. Being prepared and familiar with the
• Modeling eating so patient can follow
person's usual routine and normal level of
• Offer meals at regular times involvement will also assist in positive hygiene
• Encourage physical exercise to promote a experiences.
healthy appetite
Possible problems or concerns:
• If assistance is required ensure the carer
• Difficulty with manipulating taps
utilizes appropriate feeding techniques such
as allowing sufficient time to chew and • Forget to turn off taps
swallow, not over loading the person's mouth, • Change in sense of perception of hot and cold
using hand over hand techniques
• Unable to regulate water temperature risk of
• For chewing difficulties try light pressure on scalds
the lips or under the chin, tell the person when
to chew, demonstrate chewing, offer small • Fear of water
bites • Fear of drowning particularly if water is being
• For swallowing difficulties verbally propmt poured over their head
the person to swallow, stroke throat gently, • Fear of falls
check mouth to see food has been swallowed,
• Access to potentially dangerous items e.g.,
avoid foods that are hard to swallow, moisten
razors, mouth wash, electrical items
foods
• Overwhelmed by complexity of the task
• Cut food into small pieces if over stuffing is
(undressing, showering, drying, dressing etc.)
an issue
• Difficulty shaving, combing hair, applying
• Monitor food temperatures
makeup
• Poor oral hygiene, forgetting to brush teeth,
forgetting how to use toothbrush or nail cutter
Dementia 289

Possible solutions:
• Automatic taps that turn off if user forgets
• Put a few drops of blue food colouring in the
water to strengthen its visual impact
• Non skid floor tiles which contrast with wall
tiles
• Hand held shower level floor surfaces
• Non-slip floor tiles
• Grab rails, powder coated provides more grip
(Calkins, M 2001)
• Keep access ways free from clutter
• Wide entry doorway with outward swinging
or sliding door or hinges to allow removal of
door/easy emergency access to bathroom use
laminate signs or posters of bathing/grooming
steps and hang them where the person can see
them during the different stages of each task.
If reading is difficult, use pictures or drawings.
• Soft calming music may be helpful in the
background
• Choose the best time of day for the person for
bathing i.e. when they are most relaxed or the
time they used to bathe earlier
• Establish a routine
• Break down the task to manageable simple
steps. Gently explain each step
• Lay out items that are required for task for
example soap, shampoo, towel in the order
they will be required Examples of contrasting colours's
• Use simple clear one step directions use in the bathrooms.

• Place items within easy reach to accommodate


reduced mobility
• Use brightly colored soap, towels etc.,
• Model the action or give visual or verbal
prompts when necessary
• The visual prompt could be a picture of the
person performing the task, drawing or
picture of another person performing the task,
a drawing of the objects or a label specifying
the task or objects
• Put grooming items out in the sequence they
will be used
• Schedule regular visit to the dentists, saloons
to maintain personal hygiene
• Use positive reinforcement and provide
Anti- skid mats
compliments
290 Neuro-Rehabilitation : A multi disciplinary approach

Sleep: Possible problems or concerns:


Sleep problems are often regarded as one of the • forgetting to take medication or taking twice
most difficult symptoms of dementia. The person or more times
with dementia, their families and carers all require
• incorrect use or storage of medication
adequate sleep.
• using expired medication or taking medication
Possible problems or concerns: that is not required
• Disturbance to the person's biological clock i.e. • difficulty opening bottles or containers.
being awake and restless during the night and
sleeping during the day Possible solutions:
• No longer able to distinguish between day and • Medical/nurse review re - need for
night medication, dose and frequency
• Going to bed too early or sleeping too much • Leave medications in a visible location (if safe)
during the day • Reminders in the form of Alarm system,
• Overtiredness impacting on a person's ability notepads
to fall asleep
• Link medication times to routine activities e.g.,
• Reduced activity resulting in reduced need for taking medications at meal times
sleep
• Count and keep medications according to date
• Glare or reflections from glass or mirrors can and dosage in separate packs
be disturbing
• Temperature of bedroom: maybe too hot or Attention and concentration:
cold Strategies to improve attention and concentration:
• Nightwear that is too restrictive, hot or cold • Remove distractions whenever possible (turn
• Bed wetting due to incontinence off the TV and radio during conversations)
• Difficulty in locating bed • Talk to the person one at a time
• Concentrate on relevant material
Suggestions:-
• Establishing a sleep routine that is familiar and • Simplify information and written instructions
predictable • Reduce the amount of information that is
• Avoid having day clothes on display at night presented at one time
as this may trigger a response to get up • Get the person to repeat back information to
• Try to incorporate exercise into their daily ensure he or she has understood what was told
routine • Get the person to focus on one task/step at a
• Listening to relaxing music, radio or television time
themes associated with preparing for bed
• Keep the person focused by breaking down
• Water proof covers tasks into manageable parts
• Keep the environment as consistent as possible • Allow enough time to process information
and free from distractions
• Utilizing a contrasting top and bottom sheet Memory:
may assist the person to locate their bed Due to the cognitive deficits the person with AD
• Gently remind the person it is night time and struggles with memory losses, increasing
time to sleep forgetfulness disorientation and confusion. The
following ideas serve as memory aids to help the
• Reduce time duration of sleep during day time
person with dementia:-
Medication Management: • Labeling cupboards and drawers, maybe
Medication should be reviewed by a doctor using pictures rather than words - for example,
regularly. a photo of a cup and jar of coffee
Dementia 291

• Keep a large calendar showing the day, month


and year in every room
• Visual schedule of activities and Alarms
• Signs, line drawings or arrows to help identify
rooms or objects
• Keep a notepad/board by the phone for
messages
• Use an answering machine for phone
messages
– Decide on a consistent place to keep
handbags, keys and wallets
– Attach important items so they cannot be
mislaid (using a neck cord for glasses,
tying keys to a belt)
– When a person is trying to recall an event,
assist them by providing a meaningful
context (who was there, what the event
Examples of labeling different rooms
was for, how long ago it occurred);
of the house
providing cues and prompts to aid recall
• Write down all appointments in a clear and Speech and communication:
simple diary or wall-calendar.
Can be improved by:-
• Only use one diary/ whiteboard or wall-
calendar; use it consistently and encourage the • Allowing the person more time to
person to refer to it every day communicate

• Set an alarm to remind the individual of things • If the person is struggling to find a word in
that he or she needs to complete - if the person conversation, offer assistance after allowing a
is used to using a mobile phone or electronic reasonable amount of time
daily planner these are ideal tools. • Stand in front of the person and minimize
• Make sure the person has his or her name, distractions
address and contact in his or her wallet/purse • Place yourself at eye level
and consider an identity bracelet if
• Speak gently and clearly using statements
disorientation is becoming a problem.
rather than questions
• Use of remembering techniques e.g.
• Wait for a response from the person with
Mnemonics
dementia before continuing
If the ability to communicate through spoken word
or written language is lost, then other forms of
communication can be used. Encourage and use lots
of Nonverbal communication like Facial
expressions, Gestures and Eye contact.

Assistive Technology:
Assistive technology is any item or object that
enables a person to complete a task that they would
otherwise be unable to do. Independence in task
completion impacts positively on a person's sense
of self worth, self esteem and self reliance. Assistive
Games to improve memory technology should not replace human contact.
292 Neuro-Rehabilitation : A multi disciplinary approach

When considering assistive technology • Strategically place sturdy furniture for use
interventions a simple approach should be utilized when mobilizing
and only changing what needs to be changed.
• Eliminate glare from all areas where the person
Caution should be utilized as every person's
is transferring or mobilizing
reaction to technology will be individual and in
some cases it may have a negative impact. Involving • Give time for the care recipient's eyes to adjust
the person with dementia in the decision making to changes in light when moving from one area
process and trialing of items will lead to greater to another.
acceptance and use of the technology solutions. • Avoid use of furniture with sharp edges,
Examples of some of the technology currently carpets with turned edges
available: • Use single word commands when directing
• Electronic assistive technology: automated transfers or ambulation
door openers, alarms, computers, smart stove • Training may be required to teach safe transfer
tops etc. and handling techniques. The carer may also
• Mobile phones: features vary on each phone benefit from back protection or energy
but can include speed dialing, call blocking, conservation techniques.
larger buttons and screen, calendar with • Provide positive reinforcement when the
reminders/ to do lists, medication/schedule person displays safe behaviors
reminding software, GPS tracking etc
• Use visual, verbal or physical prompts
• Computers: larger keys or keyboard; touch
screen; screen enlarger or magnifier, speech • Use antiskid mats and floorings
and voice recognition programs; screen
readers that read all screen contents; software Leisure and social Activities:
programs that use speech synthesizers etc. Engaging in leisure activities promotes physical,
social and emotional health. People with dementia
• Use of a Universal remote.
gradually withdraw from leisure and social
activities and become socially isolated. Although
Mobility and Falls:
Simple games can provide enjoyment for people
Falls in dementia can occur for a wide variety of with dementia, the person should not be treated as
reasons. Even though people with cognitive decline a child, when planning recreational activities.
are three times more likely to fall, mobility should Gardening or other hobbies, arts and crafts, or pets
always be encouraged. can all be enjoyable sources of stimulation.
Daily mobility helps to: Activities should be tailored to the individual's
personality.
• maintain strength and endurance
• improve balance Some solutions to improve leisure skills and social
interaction are
• maintain joint mobility
• Place one interesting activity, game or item in
• promote cardiovascular health
a prominent position where it will be noticed.
Tips to assist with mobility and falls prevention: Once interest declines replace with another
• Make movement/ daily physical activity item of similar skill level and interest
enjoyable • Choose activities that are familiar, repetitive,
• Ensure furniture that the person transfers from require one step
is a suitable height, has a firm base, where
• Incorporate former interests or hobbies
possible use chairs with arm rests
• Ensure suitable footwear is worn • Choose movies and music that are era specific
for the person
• Minimize clutter by arranging furniture
simply and leave it in the same place • Plan leisure as a part of the daily routine
• Keep the floor free from hazards such as rugs • Encourage exercise and activities that require
or items that can be walked into or tripped no strenuous gross motor actions. Walking
upon with another person is an excellent activity.
Dementia 293

• Avoid competitive activities. Choose activities 3. Beatty WW, et al, 1988, Retrograde amnesia
that meet the person's capabilities in patients with Alzheimer's or Hungtington's
disease. Neurobiol Aging; 9 : 181 -186.
• Simplify the rules of games to encourage
success 4. Heindel WC, et al., 1989, Neuropsychological
evidence for multiple memory systems: A
• Utilize talking books if reading has become
comparison of Alzheimer's Hungtions and
difficult
Parkinson's disease patients J Neurosci ; 9 : 582
• Make a family history book/family photo - 587.
boards. Use captions to assist with memory/
5. Clarfeild AM 1988: The reversible dementias:
orientation
Do they reverse? Ann Intern Med; 109:476 -
• Talking photo albums: available from specialty 486.
photographic stores
6. Rasquin SM, et al., 2005: The effect of different
• Compile photos/videos to make DVD's which diagnostic criteria on the prevelance and
could be viewed during passtimes incidene of post troke dementia.
• Utilize easy cookbooks i.e. large font, spiral Neuroepidemiology 24: 189 - 195.
bound with step by step instructions 7. Benjamin James Sadock and Virginia Alcot
• Encourage the person to watch the activities Sadock, 2007, Pervasive Developmental
of the neighborhood from a window or Disorders in Kaplan and Sadock's 10 -edition,
veranda Synopsis of psychiatry, 1191 - 1205, New
York., Lippincott Williams and Wilkins.
• Traveling is encouraged if the person can
tolerate the changes. Try to preserve the daily 8. Berent S, Giordani B, Markel D, et al: 1988,
routine, plan frequent rest breaks, take a third Positron emission tomographic scan
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Conclusion: data sources. World Health Organization. Ref
Thus an occupational therapist uses a combination Type: Generic.
of education, setting up of feasible goals,
11. Ferri CP, Prince M, Brayne C, Brodaty H,
adaptations in physical environment, training of
Fratiglioni L, Ganguli M et al. Global
compensatory skills, training supervision skills, and
prevalence of dementia: A Delphi consensus
also changing dysfunctional cognitions on patient
study. Lancet 366(9503)()(pp 2112-2117), 2005
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Date of Publication: 17 Dec 2005 2005;
366(9503):2112-2117.
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296 Neuro-Rehabilitation : A multi disciplinary approach

Ch.7 Cerebellar Ataxia


Dr. Nancy Thomas, MPTh (Neuro), [Link] Mishra, [Link], F.N.R.,
Ms. Akshata Shetty, M.A.(Clinical Psychologist), Dr. Manasi Jani( Speech Therapist).

Introduction to both internal and external forces, then it is easy


to consider walking as a task requiring coordination
Normal motor movements are achieved in human as well as balance (Crutchfield et al. 1989). Since
beings with constant neuro-developmental ataxia is resistant to medical treatments, physical
maturation following birth and gets completed treatment plays an important role which involves
when normal postural mechanisms become proprioceptive training, balance exercises,
localized, followed by the ability to maintain stabilization techniques for the extremity ataxia and
balance in different positions and lastly by the vestibular exercises for accomplishing functional
formation of muscular coordination. improvement and restoration of the ataxic patients.
Normal postural tone helps in keeping posture erect Compensatory techniques can be used by adaptive
in standing against gravity, adaptation to changes devices thereby improving quality of life(1)
on support surface and proximal stabilization.
Balance can be defined as postural adaptation to Anatomical Structures Responsible for Balance
changes in gravitational center with the Development and Coordination
contribution of normal postural tone. Muscular Normal motor control is the output of normal
coordination on the other hand, is the functioning postural tone, muscle coordination, and balance
of all muscles active during the voluntary motor working together. Postural control within the
movement in appropriate rhythm, velocity and normal functioning of the nervous system is
amplitude. A person may perform daily life maintained by the different sensory-motor
activities through normal motor movement formed subsystems working together within a circular
by the above mentioned three components. network. The vestibular, visual, somatosensory
Nervous system diseases and/or injuries usually systems and cerebellum work in a coordinated
affect postural control mechanisms where in some manner to produce postural orientation depending
of these diseases, balance problems are more upon the goal of the movement and environmental
dominant and in others coordination problems conditions. Each subsystem may be dominant at
related to extremities are prominent. different times. For example, whenever the type or
quality of the surface on which the patient is
Ataxia meaning 'disorderly' (Bastian 1997) is standing changes, the changes will be registered
defined as "the in-coordination of movements" by the somatosensory system whereas when the
(Bastian 1997, Mariotti et al. 2005) and where both information in the visual area changes, the visual
insufficient postural control and incoordination system will register changes. The subsystems
of multi-joint movements are observed. (DeSouza collecting the information at that particular moment
1990) This results in postural instability leading to will dominate thereby determining the appropriate
imbalance and walking disturbances. It has three motor response (Crutchfield et al. 1989).
subcategories, which are sensory, cerebellar and
vestibular ataxia. Some researchers has given
frontal ataxia as the 4th category whereas 5th
category termed as mixed ataxia which involves
symptoms of at least two basic types of ataxia
together have been put forward.. In some cases
incoordination is usually accompanied by balance
dysfunction and gait problems whereas in other
cases, balance dysfunction is observed without the
existence of in-coordination of movements which
includes muscle weakness. For example, if normal
walking is considered to be the perfectly timed
sequence of muscle activity that occurs in response
Cerebellar Ataxia 297

Somatosensory System (proprioceptive and and possibility for fall. This finding
superficial senses): Sensation from lower differentiates sensory ataxia from the other
extremities, position of cervical (Treleaven 2008) ataxia.(Bannister 1992).
and lumbar area, length of muscles and positions
3. Loss of vibration sense in the extremities and
of joints are transmitted through proprioceptors
loss of deep tendon reflexes.
located in joints, ligaments, muscles and tendons.
The contribution of proprioceptive senses (position 4. Worsening of the finger-pointing test with the
and kinesthesia) is particularly important in the eyes closed.
formation of normal motor function (Sherrington
1907, Bear et al. 2001). Sensory ataxia are generally observed in:
1. Hereditary types of ataxia such as Friedreich's
Position and kinesthetic senses are transmitted via
ataxia and spinocerebellar ataxia.
fibres with thick myelin which convey messages
rapidly and are transmitted to central nervous 2. Other diseases like diabetic or alcoholic
system through two paths. The first one is the dorsal neuropathy, vitaminB12 inadequacy
column- medial lemniscal system through which neuropathy, tabes dorsalis,
conscious sensations are conveyed to sensory areas
3. Tumoral conditions found in the posterior
3,1,2 of cerebral cortex (Bear et al. 2001).
cord of the medulla spinalis,
Proprioception and the visual system, provides
information about speed, form and size of the 4. Multiple sclerosis (Edwards 1996).
movement that motor cortex has to generate.
(Scneider et al. 1977). Vestibular System:
Vestibular system has two pathways:
Ventral and dorsal spinocerebellar tracts are the
second path through which proprioceptive 1. Central Pathway.
information is transmitted from spinal cord to brain. 2. Peripheral Pathway.
The sensory input which is carried by these tracts
The central vestibular pathways coordinate and
comes to an end in the area called spinocerebellum
integrate information about head and body
within the cerebellum, and does not reach
movement and use it to control the output of motor
consciousness. In other words, the sensory input
neurons that adjust head, eye, and body positions.
carried by these tracts is the subconscious
proprioceptive sense. This sensory input enables Primary vestibular axons from cranial nerve VIII
the cerebellum to correct the faulty motor make direct connections to the vestibular nucleus
commands that the motor cortex may send by of the same side of the brain stem and to the
informing the cerebrum and cerebellum cerebellum. The vestibular nuclei also receive
simultaneously about the size, speed, form and inputs from the cerebellum, visual and somatic
timing of the movement before the movement is sensory systems, thereby combining incoming
performed (Guyton 1976, Ramnani et al. 2001). vestibular information with data about the motor
system and other sensory modalities.
Dysfunction of the dorsal columns of the spinal
cord results in loss of proprioception which The vestibular system, is composed of otolithic
generally carries the proprioceptive information to organs (utricle and saccule) and semicircular canals.
the brain leading to condition called Sensory ataxia. 1. Vestibulo-spinal reflex: Axons from otolith
At times it may be due to dysfunction of various organs project to the lateral vestibular nucleus,
brain parts which receives those information, which then projects via the vestibulospinal
including the thalamus, and parietal lobes. tract to excite spinal motor neurons controlling
leg muscles which helps to maintain posture
Sensory ataxia shows the following features: even on unstable surface.
1. High steppage gait and postural instability
2. Axons from semicircular canals project to the
due to lack of proprioception which cannot be
medial vestibular nucleus, which sends axons
compensated by visual input.
via medial longitudinal fasciculus to excite
2. Romberg's Test is positive when patient's motor neurons of trunk and neck muscles that
stands with his feet together and eyes closed orient the head. These pathways help the head
resulting into instability with wide oscillations to stay straight even if the body moves around.
298 Neuro-Rehabilitation : A multi disciplinary approach

One important function of the vestibular system is The medial zone is involved in adaptive control of
to keep eyes fixed in a particular direction, which somatic and autonomic reflexes and compound
is actualized by vestibulo-ocular reflex which works movements such as locomotion and saccadic eye
by sensing rotations of the head and immediately movements commonly seen in vertebrate species.
commands a compensatory movement of the eyes
The intermediate zone is developed in relation to
in the opposite direction. The movement helps to
voluntary movement in mammals.
keep gaze fixed on a visual target. (Guyton 1976,
Bear et al. 2001). The lateral zones are related to higher functions of
the cerebral association area. In humans, it is likely
Vestibular ataxia: develops as a result of peripheral
to be associated with cognition.
or central diseases which directly affects the
vestibular nuclei or the afferent and efferent Each zone receives afferents from discrete areas
connections of the vestibular nuclei. entering the cerebellum via mossy or climbing
fibers. These two fiber systems transmit various
A patient with vestibular ataxia shows :
types of information and influence the efferents
1. Symptoms of balance disturbances in standing cerebellar Purkinje cells to relay the information to
and sitting. Patient represents stagger, has a cerebellar or vestibular nucleus. The mossy fiber
broad base support and may tend to lean system carries afferent information from the spinal
backwards or towards the side of the lesion cord, brain stem, and cerebral cortex via pons and
while walking. is responsible for moment-to-moment, rapid firing
2. Head and trunk motion along with arm swing of Purkinje cells and then modulates ongoing
is often reduced (Borello-France et al. 1994). movements. The climbing fibers relay information
The patient is limited particularly when to the cerebellum from the inferior olivary nucleus
crossing the street and shopping at the market which results in slow firing of Purkinje cells that
seems to be important for motor learning.
3. Balance is disrupted when performing a head
or eye movement. The input into the cerebellum is from all 3 peduncles
with the ascending input through the inferior and
4. It may be accompanied by vertigo, nausea,
the cortical input through the middle cerebellar
vomiting, blurred vision and nystagmus due
peduncle. The superior cerebellar peduncle is
to the vestibular system's role in sensing and
responsible predominantly for the output from the
perceiving self-motion and stabilizing gaze via
cerebellum. The afferents received by the
the vestibulo-ocular reflex (Horak&Shupert
cerebellum have specific functions in terms of
1994). Extremity ataxia is by no means
locomotion, postural control, voluntary
observed in vestibular ataxia.
movements, and finally cognition within the
5. Deep tendon reflexes are normal. cerebellum.
Vestibular ataxia can seen in :
Functions of cerebellum:
1. Central factors such as medullar stroke and
multiple sclerosis. The cerebellum has a crucial role in balance and
locomotion where different zones are responsible
2. Peripheral vestibular diseases such as for different functions. In humans. cerebellar
Menier's, benign paroxysmal vertigo, or
vestibular neuronitis.

Cerebellum:
Anatomy and Physiology of Cerebellum:
The transverse lobular arrangement of cerebellum
shows neuro anatomical presentation and 7
longitudinal mediolateral parallel zones on each
side of midline and are termed as functional units
of the cerebellar [Link] zones are apparently
formed via developmental mechanisms and with
evolution the cerebellum has expanded
mediolaterally.
Cerebellar Ataxia 299

damage results in postural sway with difference Functions: 1. It helps in voluntary modification
seen in their amplitude, frequency, direction and of motor activities and the
movements in relation to lesion affecting the zone. locomotion.
• The medial zone of cerebellum : This zone 2. Lateral cerebellum is especially
integrates spinal and vestibular inputs and active in novel walking conditions
subsequently projects out to vestibulospinal where precise limb placement is
and reticulospinal tracts through the fastigial necessary.
nucleus. 3. It modulates visually guided
motor activities because of the
Functions :
robust projection it receives from
1. They exert modulatory control of the the visual cortex.
rhythmic flexor and extensor locomotor
• A lesion in this region leads to limb ataxia and
pattern generated by vestibular and
locomotion problems in normal walking and
reticular nuclei.
challenging situations with slight postural
2. They also control extensor tone to instability or sway.
maintain upright balance and stance.
In normal walking, balance deficits is seen more in
A lesion in this zone leads to a significant balance cerebellar gait ataxia (medial and intermediate
problem and impairment of postural tone with low zone) whereas visually guided leg control deficits
frequency, high amplitude postural sway, without are seen in the lateral zone.(2)
a preferred direction and without intersegmental
movements. Voluntary movements controlled
Intermediate zone (paravermal region): receives by cerebellum:
input from the spine (via spinocerebellar tracts) and Cerebral cortical association areas plan voluntary
projects out to the red nucleus and cerebral cortex. movements and the plan is executed by the motor
through the globose and emboliform nuclei It cortex. But there is a robust cerebellocerebral loop
integrates spinal and cortical inputs and influences that modulates these motor functions. These loops
locomotion through projections to motor cortical connect the intermediate part of the cerebellum to
areas. the association cortex and the motor cortex. It helps
in planning and monitoring the movements..
Function of the intermediate zone in relation to limb
placement includes : Cognitive function of cerebellum
1. Timing of limb placement. A closed cerebellocerebral loop is found in the
2. Elevation of limb placement prefrontal cortex and thus the cerebellum provides
a forward model for mental functions in the cerebral
3. Trajectory of limb elevation. cortex which is analogous to cerebellocerebral loop
4. Descent of limb placement. concerned with motor functions. A primary
cerebellar injury in premature infants has shown
Lesion to this region leads to gait ataxia and swing
to be associated with contralateral decrease in
phase overshoot of legs with no change in balance
cerebral volume.[7] This show the evidence of the
or postural tone.
importance of the cerebellocerebral connections
It includes increased postural sway of high velocity responsible for important cognitive functions.
and low amplitude in anteroposterior direction,
A mental model of image, idea, or concept is formed
increased postural tremor, increased
in the temporoparietal association cortex which are
intersegmental movements of the head, trunk, and
manipulated by the prefrontal cortex. After
legs.
repeated exercise, the cerebellum copies a mental
• Lateral zone: This area receives input model to form an internal model through cerebello-
primarily from cerebral cortical area via cerebral loop. Because of this internal model formed
pontine nucleus (corticopontocerebellar fibers) by the cerebellum, we are able to conduct
and projects out to the thalamus and cerebral movements and thoughts unconsciously
cortical areas via the dentate nucleus through
Clinical phenotypes show considerable overlap;
the red nucleus.
however, the genetic, molecular, and biochemical
300 Neuro-Rehabilitation : A multi disciplinary approach

causes for these disorders are often distinct. In It results : 1. With a wide-based gait,
dominant ataxias phenotypes show considerable characterized by uncertain start
genetic heterogeneity. These phenotypes may and stop,
manifest with pure ataxia or involve multiple levels 2. Lateral deviations.
of the nervous system (including dementia,
3. Unequal steps.
seizures, disturbance in proprioceptive function,
movement disorders, and polymyoclonus). 4. Abnormal inter-joint coordination
patterns.
Thus, from the proprioceptors in the periphery,
When this part of the cerebellum is damaged, gait
cerebellum learns the position of the body in space.
ataxia or walking in-coordination occurs.
It receives information about balance from the
vestibular system as well, and through cortico 1. Cerebro-cerebellar dysfunction indicates a
ponto cerebellar tract about the features of the lesion of the deep pontine nuclei connections
intended motor movement. Based on this with the cerebellum. The cerebro-cerebellum
information, the cerebellum facilitates coordinated contributes to planning and monitoring of
and balanced movement by making the appropriate movements and any lesion results in
adjustments. (Guyton 1976, Young&Young 1997, disturbances in performing voluntary,
Herdman 1998). planned movements.
Symptoms associated with cerebellar ataxia
Cerebellar ataxia include:
Cerebellar ataxia develops as a result of lesions to
the cerebellum or the afferent and efferent
connections of the cerebellum.
1. Vestibulo-cerebellar dysfunction corresponds
to lesion in the flocculonodular lobe (flocculus
and nodulus) and involves problems
regulating balance and controlling eye
movements thereby resulting with postural
instability, which worsens when standing with
the feet together irrespective of whether the
eyes are open or closed: this is a negative
Romberg's test. (Liao et al. 2008, Morton &
Bastian 2004).
2. Spino-cerebellar dysfunction corresponds to
the vermis and paravermis.
Cerebellar Ataxia 301

1. Dysmetria: This refers to inaccuracy in 7. Dysarthria: This occurs due to in-coordination


achieving a final end position (hypermetria between tongue and lip muscles. The patient
equals overshoot; hypometria equals speaks like he is drunk.
undershoot). This clearly is demonstrated by 8. Nystagmus: Abnormal eye movements that
the patient attempting the finger-nose test. develop in horizontal and vertical directions
2. Tremor: Kinetic tremor, which is oscillation mostly as nystagmus at the end point
that occurs during the course of the (Edwards 1996).
movement. 9. Deep tendon reflexes are maintained in
cerebellar lesions, and gain a pendular
• Intention tremor: Increase in tremor
characteristic.(5)
which is seen towards the end of the
movement. Cerebellar ataxia can be observed:
• Postural tremor: occurs when holding a 1. Spino-cerebellar ataxia among hereditary
limb in a given position. ataxias.
• Titubation: Tremor affecting the head and 2. Friedreich's ataxia.
upper trunk showing lesion in the vermis. 3. Chronic alcoholism
• Postural truncal tremor: affecting the legs 4. Paraneoplastic cerebellar degeneration.
and lower trunk, which is seen in anterior 5. Pontocerebellar angle tumors.
cerebellar lobe lesions. 6. Multiple sclerosis.
3. Dyssynergia: is particularly seen while doing As the underlying mechanisms of disease begin to
multi-joint movements especially when: be understood, the inherent challenges are
1. Agonist-antagonist and synergistic apparent; for instance, several ataxias are caused
muscles may not be able to contract in by defects in DNA repair, while others may result
correct order during voluntary from protein folding and chaperoning defects.
movement; Advances in genomics, proteomics,
transcriptomics, and metabolomics are paving the
2. Antagonist muscle may be failing to
way towards understanding of gene function,
control eccentric contraction during the
protein synthesis and transcription, and gene-gene
concentric contraction of agonist muscle.
and protein-protein interactions.(6)
With the combination of these two
factors, the extremity undergoes a Frontal ataxia
sudden velocity resulting in Frontal ataxia (also known as gait apraxia) is
inappropriate and uncontrolled motor observed when tumors, abscesses, cerebro vascular
movement. accidents and normal pressure hydrocephalus
4. Dysdiadockokinesia: This is the inability to effect the frontal area. It shows the following
perform rapidly alternating movements such features like:
as alternately tapping with palm up and palm • Patient has difficulties standing erect
down. The rhythm is poor and force of each
• Even with use of support, patient tends to lean
tap is variable.
towards hyperextension
5. Hypotonia: This occurs in acute cerebellar • Patient's legs are in scissors-cross position
lesions, but it is rarely seen in chronic lesions. during walking and there is incoordination
Hypotonia is seen in proximal and antigravity between the legs and trunk
muscles.
• Ataxia is accompanied by frontal dementia,
6. Weakness and fatigue: A generalized non- urinary incontinence, frontal release signs and
specific weakness as a feature of cerebellar perseveration ([Link]/Neuro Guide-
dysfunction often observed with extensive and maps/gait).
deep lesions and is most apparent in the
proximal musculature. Fatigue has also been Mixed ataxia
noted as a common feature of cerebellar Mixed ataxia refers to the type of ataxia when
dysfunction. symptoms of two or more types of ataxia are
302 Neuro-Rehabilitation : A multi disciplinary approach

observed together, such as occurrence of sensory observed together; whereas in cases of spino-
and cerebellar ataxia symptoms. cerebellar ataxias, cerebellar and sensory ataxia may
In some diseases, mixed ataxia may be observed be seen.
frequently. For instance, in Multiple Sclerosis, Features of basic types of ataxia are briefed in Table
cerebellar, vestibular and sensory ataxia may be 1 below:

Table 1: Clinical Differences Between Basic Types of Ataxia


Type Cerebellar Ataxia Sensory Ataxia Frontal Ataxia Vestibular Ataxia
Head Upright and Leans forward Leans Upright and
posture sometimes fixed forward definitely fixed
Trunk Stooped-leans Stooped-upright Upright Upright
posture forward
Stance Wide-based Wide-based Wide-based Wide-based
Initiation Normal Normal-wariness Start Normal
of gait hesitation
Postural +/- Intact May be +/-
reflexes absent
Steps Stagger-lurching High-stepping Small-shuffling Normal
Stride length Irregular Regular Short Normal
Leg Variable, ataxic Variable - hesitant Stiff, rigid Normal
movement and slow
Speed of Normal-slow Normal-slow Very slow Normal-slow
movement
Arm swing Normal, exaggerated Normal Exaggerated Normal
Turning Veers away Minimal effect Freezing Dysequilibrium
corners - shuffling
Heel-toe test Unable +/- Unable Unable
Romberg's +/- Increased unsteadiness +/- –
test
Heel-shin Usually abnormal +/- Normal Normal
test
Falls Uncommon Yes Very common Common

Genetic-biochemical basis for classification 2. Mode of inheritance also varies such as


Initially on the basis of anatomical localization of Autosomal dominant, recessive, and
pathological changes, inherited ataxias were nonmendelian inheritance patterns where
classified. nonmendelian inheritance patterns refers to
disorders of inheritance for which the rules of
In 1993, Harding introduced another classification
Mendelian genetics do not apply.
in which the ataxias were placed into 3 categories:
Disorders of triplet repeat expansion and certain
1. Congenital.
mitochondrial defects are examples of
2. Inherited metabolic syndromes with known nonmendelian inheritance.
biochemical defects.
Causes of Ataxia:
3. Degenerative ataxias of unknown cause.[8]
The last category was subdivided further into early 1. Idiopathic or Sporadic Ataxias.
onset (< 25 y) and late-onset types 2. Vitamin B12 deficiency: with other
In ataxia: neurological abnormalities can cause
overlapping cerebellar and sensory ataxia.
1. Presentation can be variable (eg, static vs
progressive, intermittent vs chronic, early vs 3. Inherited Ataxias: caused by a genetic fault
delayed). inherited either from the mother or father or
Cerebellar Ataxia 303

both. A faulty gene that is passed down family history for inheritance can help in reaching
through generations can result in cerebellar upto diagnosis.
ataxia. At times, the severity worsens from one
The following tests are performed:
generation to the another with age of onset
getting more younger group. This type of 1. Magnetic resonance imaging (MRI) or
worsening inherited ataxia is known as Computerized Tomography (CT) scan to
anticipation. determine whether there is any brain damage.
2. Genetic tests: to determine whether the patient
For getting inherited ataxia, the chances depends
has inherited ataxia.
on the type of ataxia that parent has.
3. Urine tests: Urinalysis may suggest specific
To develop Friedreich's ataxia, the faulty gene abnormalities that are associated to some types
would need to be carried by both the mother/father of ataxias.
and the father ( recessive inheritance).
4. Blood test: to rule out any specific type of
Spinocerebellar ataxia: Faulty gene requires one ataxia.
parent to carry the defective gene(dominant
Assessment :
inheritance) and each of his/her offspring would
have a 50% risk of developing the condition. 1. Age of onset.
3. Non-inherited Ataxia: Without any family 2. Sex
history. 3. Mode of onset (ie, acute, subacute, chronic)
The following are the: 4. Clinical History
1. Brain Surgery. 5. Natural history (ie, nonprogressive/static,
2. Head injury. episodic, progressive)
3. Alchol abuse. 6. Associated symptoms/signs that provide
localizing information such as:
4. Drug abuse: can cause ataxia as they have a
depressant effect on central nervous system 1 Presence of dystonia or chorea suggesting
function. E.g Ethanol is capable of causing involvement of the striatum
reversible cerebellar and vestibular ataxia. 2. Proprioceptive dysfunction suggesting
Antiepileptic drugs produces cerebellar ataxia involvement of spinocerebellar pathways
as a adverse effect.
3. Visual deficits (retinitis pigmentosa),
5. Infections such as Chicken pox. auditory involvement (Refsum disease)
6. Brain tumour. 4. Cognitive dysfunction possible early
7. Exposure to toxic Chemicals: Inability to and/or late
excrete copper from the body is seen in Examination1,3 of cerebellar Signs:
Wilson's Disease which is autosomal-recessive
gene disorder. Copper accumulates in the 1. Evaluate muscle power, tone and reflexes:
nervous system and liver causing ataxia as In cerebellar disorders, Tone remains mild
well as other neurological and organ hypotonia and reflexes shows hyporeflexia.
impairments.
• Evaluate upper limbs for limb ataxia :
8. Multiple sclerosis, Cerebral palsy and some 1. Rebound of outstretched arms.
other neurological conditions.
2. Finger-nose test
9. Malformation of the cerebellum while the 3. Dysdiadochokinesis.
baby is still in womb.
• Evaluate co-ordination in leg with heel-shin
10. Radiation Poisoning: Ataxia can be induced test.
as a result of severe acute radiation poisoning
with an absorbed dose of more than 30 grays. • Ask patient to sit up with arms crossed to
check for truncal ataxia.
Diagnosis: With patient's medical history for
causative factors such as brain injury as well as • Ask the patient to walk heel-to-toe (Tandem
Walking) to check any gait ataxia.
304 Neuro-Rehabilitation : A multi disciplinary approach

striatum, brainstem, etc), and patterns of


involvement.
• Other investigations like MR spectroscopy,
cytogenetic studies and metabolic screening
tests are also useful.

MEDICAL TREATMENT
Several drugs used to control ataxia including
muscle relaxants like diazepam which also reduces
muscle spasm.

Speech Affection in Cerebellar Ataxia:


Cerebellum along with the other parts of central
nervous system forms a control circuit to refine and
fine tune speech. Individuals having cerebellar
affection have typical ataxic dysarthria. Their
speech may sound "drunkard" type with lot of
slurring. The rhythm and stress pattern of speech
is affected. Language is usually normal in these
patients as there is no higher cerebral involvement.
• Ask the patient to stand with feet together
The therapist can guide them about how to make
where in cerebellar disorders, patients will be
the voice sound clearer by :
unsteady with eyes open or closed - not a true
Romberg's positive. 1. Changing the posture thereby improving the
quality of voice.
• Ask the patient to take their tongue out and
move it from side to side (movement slowed). 2. To strengthen the muscles with exercises
which are used for talking.
• Ask patient to repeat "baby hippopotamus"
thereby looking for dysarthria and abnormal 3. Teaching to speak slowly to make it more
speech rhythm and syllable emphasis. emphatic.
• Evaluate eye movement for ophthalmoplegia 4. Breathing techniques used to improve speech.
or nystagmus.
5. If dysarthia worsens, speaking aids, such as a
• Evaluate fundi for papilloedema. laptop computer that is connected to a voice
synthesizer can be used as modification.
• If unilateral signs - check V, VII, and VIII
(cerebellopontine angle pathology)(8) 6. In case of dysphagia, therapist can teach the
exercises which will stimulate the nerves
• Other systemic features like:
which will trigger the swallowing reflex and
1. Dysmorphic features and associated strengthen the muscles used during
congenital malformation suggesting of a swallowing.
specific association or clinical syndrome.
7. Physical techniques like tucking the chin
2. Cardiac (Friedreich ataxia), renal forward during swallowing will prevent any
(NPCA), and cutaneous (xeroderma food from entering the airways.
pigmentosa) features are examples.
Nutritionist: will guide about what should be
• Family history and pedigree analysis provides incorporated to have a healthy and balanced diet
diagnostic clues and information on possible like more of fibre type of food and liquids which
patterns of inheritance, which are useful for will be easier to swallow and digest.
planning investigations and genetic
For e.g mashed potatoes are a good source of
counselling.
carbohydrates, and scrambled eggs and cheese are
• MRI is useful to identify the location of the high in protein and calcium.
lesion (e.g cortex, white matter, cerebellum,
Cerebellar Ataxia 305

Physical therapy and rehabilitation of ataxia and equipment should be employed whenever
The goal of the physiotherapist is to improve the necessary.
functional level of the patient through restorative 7. Treatment should be supported by an
techniques. When this is not possible, the therapist appropriate home exercise program and
makes use of compensatory strategies to make the sports activities.
patient perform as independent as possible within
the present functional level. The goals can be briefly Measurement and assessment
described as: Evaluation of a patient with cerebellar lesion should
1. Improving balance and postural reactions include the following:
against external stimuli and gravitational 1. Bed mobility and posture.
changes.
2. Ability to sit up from a reclining posture.
2. Improving and increasing postural
stabilization following the development of 3. Maintenance of sitting posture on surfaces
joint stabilization. normally used by the client.

3. Developing accuracy of limb movements 4. Ability to stand up from a sitting position and
incorporating placement of both upper and transfer to and from a commode as if the person
lower extremity when performing all was within the home environment.
functional activities. 5. Maintenance of standing posture.
4. Developing independent and functional gait 6. Ambulation and the environment within
including clearance of obstacles, which the person will ambulate.
5. Increasing the patient's independence while
7. Ability to dress, groom and eat as normal daily
performing activities of daily living.
living activities.
Main principles of training Performance can also be measured by:
1. Whatever be the pathology, treatment requires 1. Assistance required to perform the activities.
repetitions of a task and task sequence which
will help in execution of both slow and rapid 2. Level of effort involved.
movements. 3. Time required to complete the activity.
2. Exercises should be practiced consciously at 4. Potential Hazards to the client.
first, and in later stages should be followed by
automatic exercise activities. 5. Unusual accompanying movements.

3. Exercises should progress from simple to Although the observational methods and scales
complex activities. mostly designed to assess balance are easy to
use,their ability to provide standardized
4. Activities should be practiced first with the measurements is limited, and the results can vary
eyes open and later with the eyes closed. depending on the person who has done the
5. After achieving proximal tonus and observation.(9) Though computerized systems are
stabilization, the coordinated movement of the highly reliable, they are costly systems which
distal segments should be taken into require working within the laboratory
consideration. environment. Balance assessment tools frequently
6. Compensation methods and supportive aids used by physiotherapists are shown in Table 2.

Table 2: Methods of Balance Assessment

Assessment Tool Purpose of Tool


External Perturbation Test - Push and Release test (Jacobs et al.2006, Static balance
Valkovic et al. 2008) External Perturbation
Test - Pull test (Hunt&Sethi 2006, Static balance in different
Munhoz et al.2004, Horak et al. 2005) sensory conditions
306 Neuro-Rehabilitation : A multi disciplinary approach

Clinical Sensory Integration Test (Smania et al. 2008, Dynamic balance in


Chaudry et al.2004) different sensory
conditions
Sensory Integration Test of Computarised Dynamic Posturography Static and dynamic
(Mirka&Black 1990, Jackson et al. 1995, Cham et al.2006) balance
Static and Dynamic Posturography (Mohan et al. 2008, Static balance
Federica et al. 2008, Buatois et al. 2006)
Single Leg Stance Test (Soyuer et al. 2006, Mann et al. 1996) Static balance
Functional Reach Test (Martin et al. 2006, Jacobs et al. 2006) Functional static and
dynamic balance
Berg Balance Scale (Yelnik&Bonan 2008, Ryerson et al. 2008, Functional static and
Enberget al. 2008) dynamic balance
Five Times Sit to Stand Test (Buatois et al. 2008) Functional dynamic
balance and gait
Time Up and Go Test (Zampieri& Di Fabio 2008, Vereeck et al. 2008) Gait and functional
dynamic balance
Dynamic Gait Index (Herman et al. 2008, Chang et al. 2008) Dynamic balance
and gait
Tandem Walking (Ravdin et al. 2008) Dynamic balance
Four Square Step Test (Blennerhassett&Javalath 2008) Dynamic balance

Measurements such as gait duration, step length, Index can be employed to assist in assessment
step width can be used apart from these tests. methods (Wrisley & Pavlou 2005).
Moreover, self-perception scales filled in by the There are a limited number of scales which have
patient such as Dizziness Handicap Inventory, been developed to assess truncal ataxia and
Activity Specific Balance Confident Scale and scales extremity ataxia together, and tested for validity
for daily living activities such as FIM™ and Barthel and reliability. (Table 3)

Table 3: Scales of Ataxia


Assessment Tool Purpose of Tool
International Cooperative Ataxia Evaluating truncal and extremity ataxia, gait ataxia,
Rating Scale (D'Abreu et al. 2007) nystagmus and talking
Scale for Assessment and Rating of Evaluating truncal and extremity ataxia, gait ataxia
Ataxia (Yabe et al. 2008) and talking
Ataxia Functional Composite Scale Evaluating gait speed, upper extremity ataxia and
(Assadi et al. 2008) visual acuity
Nine Hole Peg Test (Lynch et al.2005) Evaluating upper extremity ataxia
Computer Graphics Tablet Evaluating upper extremity ataxia
(Erasmus et al. 2001)
Brief Ataxia Rating Scale Evaluating truncal and extremity ataxia, gait ataxia,
(Schmahmann 2009) nystagmus and talking
Friedreich's ataxia impact scale Speech, upper limb functioning, lower limb
(Cano 2009) functioning, body movement, complex tasks,
isolation, mood, self perceptions
Composite cerebellar functional Upper limb functions
severity score (du Montcel 2008)
Cerebellar Ataxia 307

Physical therapy approaches Biofeedback can be used to promote upright head


A physical therapy program is tailor made from position in severely affected patients for example,
the interpretation of the measurement and wearing helmet provides visual and auditory clue
assessment results. The contents of the treatment which can be used when the vertical position of the
program can vary depending on the type and head is not maintained.
characteristics of ataxia. For instance, while Stretch reflex activation may be used to increase
approaches which improve proprioception and muscle contraction in the initial phases of treatment
incorporate visual aids are used more commonly as it is required to maintain muscle contraction in
in patients with sensory ataxia, stabilization the core trunk muscles.
training is more important to reduce truncal and
• Firstly, the proximal muscles and stabilization
extremity ataxia in patients with cerebellar ataxia.
of the trunk should be improved (Edwards
The patient with vestibular ataxia should be given
1996). It is appropriate to use the mat activities
habitation exercises in order to reduce vertigo, and
of the PNF techniques.
also vestibulo-ocular, vestibulo-spinal reflexes
should be stimulated to improve balance. In some • Bridging
cases, a problematic condition which requires the • One leg bridging.
use of a number of approaches, such as mixed
• Abdominal and back strengthening exercises.
ataxia, may arise. In such cases, Physiotherapy
helps in improving the gait, balance and trunk • Long leg shifting in circles and in forward
control of people with ataxia and reduce activity backward directions.
limitations and increase participation.(10) • Quadrapud
Cerebellar signs are seen in many pathologies like • Wt shifting in Quadrapud.
multiple sclerosis, head injury, cerebellar stroke,
• Hip extension in Quadrapud.
brain tumour, cerebellar degeneration, central
vestibular dysfunction and Friedreich's ataxia and • Side sitting in Quadrapud.
interventions are to be tailor made for individuals • Crawling
varying in terms of type, intensity, duration and
• Kneel standing.
frequency.
• Trunk rotation in kneeling.
Interventions like PNF, Frenkel's exercises, dynamic
training of postural stability with task and activity • Kneel walking.
focus, gait and balance training; along with • Perturbation (pushing and pulling in different
strengthening and flexibility can be included. directions).
Therapeutic equipment was often provided to • Weight transferring and functional extension
support function.
• Sitting balance: When the patients can hold
Dynamic task practice for Strength and flexibility their heads up and can hold some trunk
would improve stability and gait. (11) control in a static position then they can be
The use of orthotics and devices along with challenged to various sitting postures which
movement retraining reduces the degree of will challenge their balance which includes
freedom but it enables the patient to do the ADLs treatment on the edge of the mat table or in a
better. chair without back or arm support like able to
get up from sidelying position and side
The classification of treatment applications can be
shifting in bed,
briefly described as follows:
• Joint approximation has been used to promote
Head and trunk control: trunk stability.
A patient with postural instability needs to be • Rhythmic stabilization for trunk rotation can
assisted in maintaining posture in all activities in be used to sustain contraction of the trunk
which the patient engages. Learning to sit on muscles where it is to give more of sensation
noncompliant surfaces versus compliant surfaces of stability
requires the cerebellum to process more • Rotation of the trunk also may be valuable in
proprioceptive input in the former and more gaining stability.
vestibular input in the latter.
308 Neuro-Rehabilitation : A multi disciplinary approach

Bridging One leg bridging

Long leg shifting in forward direction Long leg shifting exercises

Quadrapud Weight shifts in Quadrrapud

Side sitting in Quadrapud (a) Side sitting in Quadrapud (b)


Cerebellar Ataxia 309

Crawling

Trunk rotation in kneeling (1) Trunk Rotation in kneeling (2)

Getting up from sidelying (2) Getting up from sidelying position (1)

Side shifting at edge of bed in sitting Side shifting at edge of bed in sitting
unsupported unsupported (1)
310 Neuro-Rehabilitation : A multi disciplinary approach

Side shifting at edge of bed in sitting


unsupported (2)

Ball Bouncing

Forward reachouts in sitting

If a patients cannot sustain a contraction which coordination exercises were introduced.(Edwards


requires co-activation of the trunk muscles, a 1996, Danek 2004). Actively repeated contractions
pattern of slow-reversal -hold over a steadily similar to PNF can be utilized on their own or by
decreasing range of trunk rotation can be used. combining them with Frenkel's coordination
• Push-pull activities with hands joined or with exercises (Armutlu et al. 2001). While these two
the cane or pole are helpful as well in types of exercise are effective in cases with mild
promoting coactivation needed for stability. extremity ataxia, they can be insufficient in severe
cases. In such cases, rhythmic stabilization and
• Therapeutic Ball is also a good activity as combination of isotonic techniques are more
gentle bouncing on the ball may promote effective than PNF (Adler et al.2000).
activity in the small trunk extensors thereby
regaining awareness of being vertical. Use of proprioceptive neuromuscular facilitation
(PNF) patterns of rhythmic stabilization or slow-
• Weight shifts in all directions while sitting
reversal-hold for the lower extremities will allow
with both the hands support progressing to
clients to ambulate with better control.
no support from the hands further with arms
overhead and the trunk rotated as these Functional tasks involving the arms can be
positions are used in our daily activities. preceded by PNF patterns for the arms.

Techniques to improve Limb Ataxia Frenkel's exercises are effective for some patients
and can be performed in the supine, sitting or
Exercises are designed in a manner to provide
standing positions. Each activity is to be placed
fixation by establishing balance between the
slowly, with the patient watching the extremity
eccentric and concentric contractions within the
carefully. When the client has gained reasonable
multi-joint movements of lower extremities and the
control of one activity, she or he should proceed to
upper extremities in particular. During the
the next. The patient starts with moving a limb with
performance of these exercises, it is important to
support, to moving without support at one joint of
establish slow, controlled and reciprocal multi-joint
a limb, to moving the limb as a whole. Fig 6.18
movement and stabilization, thus Frenkel's
Cerebellar Ataxia 311

Frenkel's exercises in Lying Frenkel's exercises in lying (1)

Frenkel's Exercises in Standing Frenkel's exercises in Standing (1)

Frenkel's exercises: 3. Sit steady for a few minutes.

1. Supine: 4. Rise and sit with knees together.


a) Flex and extend one leg, heel sliding Standing:
down a straight line on table.
1. Place foot forward and backward on a straight
b) Abduct and adduct hip smoothly with line.
knee bent, heel on table.
2. Walk along a winding strip.
c) Abduct and adduct leg with knee and hip
extended, leg sliding on table. 3. Walk between two parallel lines.
d) Flex and extend hip and knee with heel 4. Walk, placing each foot in a tracing on floor.
off table. The sequence used will depend upon the way the
e) Place one heel on knee of opposite leg and patient sleeps as well as the client's weight, side of
slide heel smoothly down shin toward involvement, underlying muscle strength, age and
ankle and back to knee. comorbid conditions. The patient should be
f) Flex and extend both legs together, heels provided with a variety of ideas for rising from the
sliding on table. bed. Methods for rising from the floor should be
taught and fall can also be practiced with lying to
g) Flex one leg while extending other leg. sitting.
h) Flex and extend one leg while abducting
and adducting other leg. Independent Transfers :
If the clients have adequate sitting balance but are
2. Sitting: not considered candidates for safe ambulation, they
1. Place foot in therapist's hand, which will should be taught as many independent transfers
change position on each trial. as possible. Transfers can be taught with the
2. Raise leg and put foot on traced footprint caregiver to increase follow-through and safety at
on floor. home. A sliding transfer from a wheelchair to
312 Neuro-Rehabilitation : A multi disciplinary approach

another chair or bed will be safest. Swivel sliding two narrow lines, tandem gait, backward gait,
boards may assist the caregivers for transfers in and slowed down gait (soldier's gait), stopping and
out of a car. A trapeze over a bed or bars in the turning in response to sudden directions, flexion,
bath may increase the level of independence if the extension and left-right rotations of the head and
accuracy of limb movements allow such activity. balance board exercises.
It is hoped that the patient can learn to come to
Progressing towards for Ambulation:
and maintain standing without pulling on the bars,
If the goal is to progress the patients toward however, for some people this will be impossible.
ambulation, a series of preliminary activities would Those individuals who rely on the bars will not
be beneficial before they attempt to stand. These become independent in ambulation but may with
activities may include exercises such as bridging. assistance of another person or an assistive device,
The patient may practice transferring from sit to be able to get up and walk.
stand many times through the day. A simple way
to increase strength and practice in this activity is Once standing and stable, the patient needs to
to have the patient stand after sitting one to five practice walking on a level surface as well as
times, she or he sits down or stands( expect for walking over obstacles and uneven surfaces to be
when the patient is transferring to the toilet for considered as functionally independent in
hygiene. ambulation.(21)

Moving from sit to stand and stand to sit can be Ambulation:


further progressed by using their hands on the
When the patient begins to walk within the parallel
table( before and after meals) or with the use of a
bars, he or she will need precise verbal feedback as
walker or wheelchair.
to step length, body rotation, accessory movements
If the patient has been in a wheelchair or has been and trunk position. Ambulation over the rungs of
in bed for a long time, the therapist may need to a ladder or lines on the floor can be used to increase
prepare the person's cardiovascular system for visual clues and feedback regarding foot placement.
being upright by placing him or her on a tilt table.
A visual clue will be needed to prevent the patient
Standing activities may be started in the parallel
from walking too far into the walker and falling
bars.
backward. A four-wheeled walker may be easier
When standing up from sitting, the patient need to to manipulate and does not need to be lifted just
slide forward in the chair and flex their trunks pushed forward.
considerably, placing the center of gravity over the
feet. The trunk and legs should be extended only Crutches or canes may be used but require
after gaining balance on the feet. This may be the reciprocal movement of the arms and legs with
most difficult step for an ataxic individual, who will appropriate timing and placement. The patient may
either lean too far forward or extend the trunk too actually do better using a walking stick for support.
early and drop back into the chair. One can measure the patient progress in
ambulation by the number of times the patient loses
Once upright, the person should practice balancing his or her balance in a treatment sessions, frequency
which can be reinforced by approximation through of a specific error, The distance ambulated or the
the hips and shoulders. Weights have been used to level of assistance needed.
increase stability in standing and walking. A study
by Widener indicated that small weights carefully The more closely the treatment activities mirror the
placed for the individual was effective in improving patient's life, the more likely the treatment will be
standing balance. successful.

In the standing position, weight transferring onto Relaxation and Biofeedback


the front, back and sides, narrowing the support
Guercio et al (1997, 2001) describe two case studies
surface and balance training in tandem position,
of individuals with severe ataxic kinetic tremor
balance training on one leg should be performed
following a TBI that used behavioural relaxation
thereby challenging their stability. Fig 6.19-6.26
training with EMG auditory biofeedback to reduce
The best indicator of dynamic stabilization/balance tremor severity and improve functional
is gait. Therefore, gait training should be given performance. Findings reported benefits in
including the following applications : walking on functional performance and reduction of tremor.
Cerebellar Ataxia 313

Sit to stand from the chair

Getting up from low stool

Getting up from sidelying

Balancing exs in standing


314 Neuro-Rehabilitation : A multi disciplinary approach

Tandem Walking

Crossing the Obstactles Balance board exercises.

Baram and Miller (2007) investigated the effect of 2. Intention tremor, which occurs during target
auditory biofeedback for 14 people with MS and directed, visually guided movements (e.g.
gait dysfunction due to cerebellar ataxia. Positive finger-nose test), and worsens at the terminal
results were reported in terms of speed and phase of the movement as the target is
steplength but not maintained for doing ADLS approached (Deuschl et al, 1998).
In addition to affecting activities of daily living
Interventions used to improve tremor in upper
(Feys, et al, 2004) the psychosocial consequences
limb:
of upper limb tremor can be significant (McGruder
Lesions affecting the cerebellar hemispheres give et al, 2003).
rise to ipsilateral limb symptoms including tremor
in addition to dysynergia, disdiadochokinesia and The use of weights and the carryover when the
rebound phenomenon. weights are removed will require more careful
investigation may indeed be patient or disease
An action tremor occurs during movement i.e. is specific..The clinician must analyze whether the
produced by voluntary contraction of muscle. weights are increasing joint approximation in a
Postural tremor occurs when voluntarily closed chain or increasing joint distraction in an
maintaining a position against gravity e.g. holding open chain. Closed chain enhancement may help
an arm out straight. the cerebellum, while open-chiar distraction may
Kinetic tremor occurs during any type of voluntary increase the imbalance between agoinst and
movement and further can be subdivided into: antagonist during a movement pattern.(12)

1. Simple kinetic tremor which occurs during Wrist weight cuffs


voluntary movements that are not target-
Wrist weighting is an intervention used to reduce
directed ([Link]/extension or pronation/
upper limb tremor, however mixed effects are
supination).
reported. Use of supportive devices enables the
Cerebellar Ataxia 315

Coordination exercises for upper limb Picking the Beads

Picking the Beads1 Picking the beads 2

Rolling the theraband

Cutting the page Hand functions-Picking up Putting the beads


316 Neuro-Rehabilitation : A multi disciplinary approach

patient to function more easily within his present 1. Endurance/aerobic Training


functional level. In cases of severe ataxia, Fillyaw and Ades (1989) investigated the
suspending weights from the extremities and the physiological adaptation to aerobic training in
use of weighted walkers can be preferred (Gibson- Friedreich's Ataxia where training took place over
Horn 2008). a period of nine weeks and consisted of 27 ECG
monitored, bicycle ergometer sessions of 20-25
Cold Therapy
minutes of continuous cycling at training level
It has been reported that cold therapy has [Link] to commencement of training the
significant beneficial effect in the cases of tremors participant, who had no history of cardiac
in MS in upper limbs. symptoms, underwent a cardiovascular
Interventions used to improve proprioception: examination and echocardiogram that confirmed
normal cardiac size and function. Increases in
The aim is to increase proprioceptive input by
cardiorespiratory and work measures
mechanically stimulating the joint surfaces, muscles
demonstrated clinically important physiological
and tendons, and decreasing postural instability by
adaptations to aerobic conditioning suggesting that
improving body awareness. There are many
people without cardiomyopathy, aerobic training
approaches that can be used for this purpose. These
offers a means of promoting activity and reducing
are: Proprioceptive Neuromuscular Fascilitation
deconditioning which may provoke functional as
(PNF), rhythmic stabilization, slow reversal
well as other health benefits.
techniques (Adler et al. 2000, Gardiner 1976),
resistive exercises (DeSouza 1990, Arai et al. 2001), 2. Hydrotherapy and Swimming
use of Johnstone pressure splints (Armutlu et al.
Cook (2007) advocates the use of hydrotherapy and
2001), gait exercises on different surfaces (hard, soft,
swimming for people with ataxia because water
inclined surfaces) with eyes open and closed and
activities offer risk and challenges providing
balance board exercises.
freedom of movement and may be beneficial for
Vibration has been a frequently used application speech. Hydrotherapy is also considered to offer
where it is directly applied to the muscle and beneficial effects on health related quality of life.
tendon, and also it can be applied by exposing the No studies directly evaluate the efficacy of
whole body to vibration (Schunfried et al. 2007, hydrotherapy for people with ataxia and can be
Hatzitaki et al. 2004, Semenova 1997). taken as a form of exercise.
In addition yoga, and body awareness exercises can
be included in the program. 3. General fitness training, Yoga and Pilates
Theraband exercises can be used to maintain Benefits of general fitness training, yoga and Pilates
resistance and improve joint approximation. for people with ataxia can help in maintaining
strength, flexibility and balance thereby resulting
Interventions used to improve vestibular in overall improvements in psychosocial aspects.
dysfunction:
Since dizziness accompanies balance dysfunction
ROLE OF OCCUPATIONAL
in vestibular problems, repetitive head movements THERAPY IN CEREBELLAR ATAXIA
and Cawthorne and Cooksey exercises (Dix 1979) Occupational therapist form an important part of
are of great importance. A vestibular exercise the multi-disciplinary care team in ataxia. OT is an
program consists of repetitive, progressively more important intervention for patients with
difficult, eye, head and body movements designed progressive neurological conditions in maintaining
to encourage movement and facilitate sensory independence and quality of life and enable people
substitution. to perform simple tasks that are meaningful to
them. (13)
Exercises
Occupational therapist should use assessment and
Exercises should be encouraged as part of health outcome tools that measure the persons satisfaction
[Link] should be tailored by exploring with the performance of an activity, since use of
several different options as well as building tools that measure only impairment would not
motivation and sustainability into the exercise demonstrate the effectiveness of OT
prescription (Dean, 2009;Rhodes and Fiala, 2009)
Cerebellar Ataxia 317

intervention(14). Evidence suggests that people of unweighting and resistance to movement. This
with ataxia may have a lower quality of life in the can be quite effective in reducing hyperkinetic
early and end stage of the condition. It is therefore movements and enhancing postural stability. For
important to recognize that even at the early stage example, a patient who demonstrates significant
of the condition, difficulties with roles and activity ataxia may be able to sit or stand in the pool with
engagement may benefit from support. (15)At most minimal assistance while these same activities
ataxias are progressive an important consideration outside the pool are not possible.
is proactive planning for future needs.
Although active movement is the goal, assistance
Patients with ataxia present with insufficient may be required during initial movement attempts
postural control and incoordination of multijoint for both the dynamic movements' as well as the
movements resulting in postural instability leading stabilizing body segments. Specific task- oriented
to balance and mobility dysfunction. Some patient training (eg. Reaching, stepping) are more
may also present with hypotonia and hyperkinesia motivating, especially if the task is important to the
i.e tremors, titubation which interferes in all their patient.
functional activities. Hence it is essential that
Therapy ball activities are effective in developing
occupational therapist addresses these issues
dynamic stability control. For example, the patient
sits on a ball and gently moves the ball side-to-side,
Management for Hypotonia:
forward-backward, or in a combination (pelvic
Intervention techniques to increase tone for patients clock motions or the patients sits on ball while
with hypotonia (flaccidity) can include quick performing voluntary movements of the arms or
stretch, tapping, resistance, approximation, and legs alternate legs or arm raises)
positioning. Patients typically also demonstrate
weakness and at times it is difficult to differentiate
between two. Strengthening exercises that do not
overload the weak, hypotonic muscles are
indicated. Postural instability is common problem.
Intervention should be designed to improve
postural stability in functional positions

Management to improve postural control


In patients with ataxia instability is associated with
excessive postural sway, wide BOS, a high guard
hand position or handhold, and loss of balance. The
therapist can provide training through postures
Reachouts in standing
that demand increasing amount of
upright(antigravity) postural control eg. prone- on- E.g. reaching for pegs/kicking a football.
elbows to quadruped and finally sitting. As trunk Progression is from unilateral to bilateral and finally
becomes more stable, the patient is expected to to reciprocal limb movements. Voluntary trunk
assume active control in stabilizing the posture. For motions can be practiced while sitting on the ball
patients with ataxia, the PNF technique of (e.g. head and trunk rotations are forward/
stabilizing reversals (slow reversals) is appropriate backward leans). Resistance can be introduced by
The therapist can have the patient stabilize while using elastic resistance bands or weight cuffs on
seating on a therapy ball. Gentle bouncing provides the ankle or wrists. Difficulty can also be increased
joint approximation through the verbal joints, by adding a second task (dual task training) such
facilitating extensors and an upright posture. For as catching and throwing a weighted ball, balloon
patients requiring more assistance, sitting control volleyball, or kicking a ball. Group activities can
can first be practiced on a complaint surface placed be introduced when patients can safely perform
on a platform mat or sitting on a ball with a ball each of the activities individually.
holder. Task difficulty can be increased by reducing
the BOS. Management for Ataxic Movements (16):-
• Control of ataxic movements can be achieved
Aquatic therapy can also be used to enhance
through proprioceptive loading and light
proprioceptive loading. The water provides degree
318 Neuro-Rehabilitation : A multi disciplinary approach

resistance. Resistance can be provided by pressure sores while waiting for help to arrive. OTs
application of elastic resistance bands/ light should consider joint assessment with or referral
weights to stabilize movements. Weight cuffs, to a physiotherapist, and referral to a falls
weighted boots or weighted jacket/ belt can programme/group locally. If there is a family
reduce tremors of limbs/ trunk. member or carer involved, the occupational
therapist and physiotherapist should consider the
• The extra weight will also increase energy
safety of the carer.
expenditure and must therefore be carefully
balanced against increased fatigue it might Fall Prevention Strategies: Modifying the Home
cause. Environment
• Weighted canes/walkers can be used to • Adequate lighting is essential. Both low light
decrease ataxic lower extremities movements and glare can be hazardous, particularly for
that interfere with use of assistive device the elderly. Glare can be reduced with
during ambulation. translucent shades or curtains.
• Weighted spoons and fork can be used to • Light switches should be positioned at the
enhance eating for patients with significant entrance to a room and fully accessible.
tremor, these devices may mean the difference
• Carpets with loose edges should be tacked
between dependent and independent
down. Scatter or throw rugs should be
function.
removed.
• A soft neck collar can be used to stabiles head
• Furniture that obstructs walkways should be
and neck tremors.
removed or repositioned.
• All these strategies however are temporary
• Chairs should be adequate height and
and compensatory.
firmness to assist in sit-to stand transfers.
Chairs with armrests and elevated seat heights
Strategies to improve Safety (16):
may be required.
Prevention of falls for the patient with balance
deficiency is an important goal of therapy. Lifestyle • Stairs are the site of many falls. Ensure
counseling is important to help recognize adequate lighting. Contrast tape using bright
potentially dangerous situations and reduce the warm colors (red, orange, or yellow) can be
likelihood of falls. For example, high risk activities used to highlighting steps. Handrails are
likely to result in falls include turning, sit to stand important for safety on stairs and, if not
transfers, reaching and bending over. Patients present, may need to be installed.
should also be discouraged from early hazardous • Grab bars or rails reduce the incidence of falls
activities such as climbing on step stools, ladders, in the bathroom. Nonskid mats or strips in the
and chairs, or walking on slippery surfaces. The bathtub along with a tub or shower seat can
education plan should stress the harmful effects of also improve safety. Toilet seat can be elevated
a sedentary lifestyle. Patients should be encouraged to facilitate independent use.
to maintain an active lifestyle, including a program
When ADL activities are difficult to manage or
of regular exercise and walking. Medications
perform OTs support people by changing and
should be reviewed and those medications linked
to increase risk of falls (e.g., medications that result adapting their relationship with their physical and
in postural hypotension) should be addressed. social environment to develop new valued activities
and role. Focus of occupational therapist on
Assistive devices should be used assist to balance engagement in activity, rather than the disorder is
when necessary. important in progressive conditions. OT
intervention should focus on goals that support the
Falls management person and carers and improve their quality of life.
Falls may occur in any area that a person mobilizes.
The main focus of occupational therapist is
At home, the person should be taught fall recovery
participation in activities rather than focus on
techniques and where appropriate, consider the use
the progressive condition of disorder thus
of community care alarms such as pendant alarms,
enhancing the present lifestyle and improving
and techniques to avoid further injury such as
quality of life.
Cerebellar Ataxia 319

Evidence suggests that people with ataxia may have Food preparation
a lower quality of life in the early and end stages of Preparing food is one of the common concerns in
the condition (15). As most ataxias are progressive the early stages of ataxia due to poor imbalance and
an important consideration is proactive planning Inco-ordination. OTs should carry out an activity
for future needs. analysis of food preparation tasks and suggest a
Common Occupational Therapy management for variety of methods and aids that may compensate
Cerebellar Ataxia for difficult or unsafe aspects of tasks. Kitchen
platform should be on wheelchair level so cutting
Self care and toileting vegetables, cooking and doing other work will be
Treatment planning includes reducing the impact easier and independent.
of excessive movement and helping the patient to
increase their independence. Mainly people with Household management
ataxia face difficulty in balance, so support bar can In ataxia, people are cognitively able to manage the
be used to provide stability during transfers. home but may have difficulty in physically doing
Dressing and undressing to toilet and personal heavy housework such as cleaning floor and
hygiene are other problems encountered during washing clothes. Fatigue is also main concern, so
toileting people should use energy conservation techniques
organization and adaptive devices.
Practical Suggestions
• Encourage bath in seating position with Practical Suggestions
support for the back and arms. • Use Kettle tipper devices can help making
drinks safer
• Use of hand held shower instead of using taps.
• Using a travel mug with a lid can sometimes
• Use of rail bars in toilet to minimize risk of
assist with carrying a drink
fall
• Waist height ovens; use of full-length oven
• Instead of Zip or button use of Velcro.
gloves; sliding food to a level surface (or level
trolley) rather than lifting
Eating and drinking
Due to multiple difficulties faced by ataxic patients • A microwave oven can provide a safer
Feeding needs to be considered for effective eating alternative to standard ovens
and drinking. Due to multiple impairments • Chopping boards with an attached cutting
impacting on safe and effective eating and drinking blade can be safer than a separate knife
feeding needs to be considered. Proper positioning
• A food processor can help with slicing or
helps to maximize posture and support core
chopping vegetables.
stability, which helps to reduce the impact of
excessive limb movements. Occupational therapist
Bed, chair and toilet transfers
works in tandem with the speech and language
therapist to find appropriate feeding solutions. (20). Ensure that the height of the chair and bed should
be on same level for safe transfer and the hip and
Feeding Assessment (Use of oral -motor/ feeding knee angle is at 90 degrees and that the feet are flat
rating scale) on the ground. While transferring it should be in
noticed that the chair is stable and armrest is of
Practical suggestions suitable height and the patient should be in a
• All the utensils and necessary things while position to do a push up. Educate the patient and
eating should be organized to increase care giver on sit to stand techniques and adequate
independence. training of transfers should be taught. In particular,
• Use of Plate guards, and non-slip matting to provide the most support possible for safety
prevent unnecessary movement of plate. reasons.
• Use lidded/insulated cups or cups with straws
for drinking, especially hot liquids - such as
Indoor Mobility
tea and coffee Indoor mobility should be ideally assessed in the
• Use of weighted spoon or fork while eating environments that the person uses. According to
320 Neuro-Rehabilitation : A multi disciplinary approach

patient needs OTs should modify the home as well Practical Suggestions
as work place environment. Use of railings, grab • Public transport and rail providers offer
bars, walking frames should be considered to subsidized fares and can provide a meet and
increase independent mobility in daily living. OTs greet service
should consider the use of walking aids in the home
and other environments. eg a walking frame to the • Outdoors motorized scooters or wheelchairs
toilet and then the use of hand rails inside the toilet. can maximize independence
If wheelchair mobility is considered the
environment should be modified accordingly to
ensure access to the areas they need to. For eg.
Modify door widths, arrangement of furniture,
ramps/ lifts, work table height.

Practical Suggestions
• Consider the height of the bed and location
within the room
• Mattress variators, or profiling beds may be
of benefit
• Ensure surfaces are the optimal height to
ensure the most efficient and safest transfers
• Firmer mattresses will aid bed mobility
• If patient is wheelchair bound or bedridden
and mobility is severely restricted, pressure
care needs should be considered.
• Wear tight fitting clothing instead of loose
clothing and avoid carrying items while
walking to avoid imbalance while walking.
• Use of trolleys to carry items, especially food,
drinks and heavy items at work or in the home
• Use of nonskid mats at floor and remove loose
electrical cables to avoid risks to mobility in
the home environment Handwriting
• Good lighting will help optimize performance
Handwriting is another area of concern in
of tasks and decrease the chance of further
progressive ataxia
complications.
Practical suggestions
Outdoor and community mobility
Use of weighted wrist cuffs and weighted pens may
In ataxia, incoordination and imbalance while be helpful in reducing the tremors.
walking is main problem so mobilising outdoors is
• Proper posture is advisable to maximize
often difficult for the person with ataxia. Educate
independence while writing
the carer and the person with ataxia about energy
conservation techniques like rest regularly while • Use of Dictaphones or voice-activated
walking outdoors and use of walking aids, use of computer software to compensate for
wheelchair for outdoor can help to reduce fatigue • handwriting and speed difficulties
or maximize safety. If patient is using wheelchair,
proper assessment for optimal seating position Computer use
should be done, like use of cushions and back
Patients with Cerebellar Ataxia gradually find
support should consider. Ongoing assessment for
difficulty in using computers. If the patient has
seating position can be useful in all the stages. A
dysarthria, consideration of voice-activated
compromise between optimizing function and
software should be carefully thought about.
providing adequate support is important.
Cerebellar Ataxia 321

Practical suggestions to be therapeutic.


• Keyboard and mouse modifications can be
made to adjust the sensitivity and speed of Practical suggestions
response • Educate the person and carer on allowing the
car door to be opened fully and to consider
• Alternative mouse such as a Tracker ball can
the height of the transfer being undertaken
be helpful
• Smaller keyboards or key guards may help- • Ensure the person sits their bottom down first
check it before moving their legs into the car

• Location and setup of computer equipment • Use of transfer board with transfer mat and if
should be easily accessible. the car seat is low, use firm foam

Work Cushions for safe transfer


Patient should consider continue to work as long • Choose a model of car that optimizes transfers,
as possible. When faced with difficulties. door access and storage space
Occupational Therapist should conduct onsite Activity Analysis & Synthesis
evaluation and suggest or modify environment
accordingly. Work hours, Transportation, seating A key skill of an OT is to analyze the component
arrangement, type of equipment etc should be parts of an activity in order to use it purposefully,
considered. meaningfully and therapeutically (Finlay, 2004).
Activity Summary of Expected Performance
Practical Suggestions-
This activity analysis format is taken from Lamport,
• Adequate rest periods should be provided Coffey, Hersch (2002).
between working hours.
1. Name of activity:
• Ergonomically appropriate seating
2. Brief Description of the activity:
• Use of lift instead of stairs
3. Tools/Equipment (non-expendable), Cost and
• Educate patients about safe use of equipments
Source:
Leisure 4. Materials/Supplies (expendable), Cost and
If the patient has loss leisure role such as Source:
participation in sport, gardening, painting then 5. Space/Environmental Requirements:
occupational Therapist should consider alternate 6. Sequence of Major Steps
leisure activities like Watching on television, 7. Precautions:
painting with adaptive devices such as stencils,
8. Special considerations:
block stamping, care of potted plants. Active
participation in hobbies should be encouraged. 9. Acceptable criteria for completion:
Outings, get-togethers with loved ones can prove 10. Performance Components
322 Neuro-Rehabilitation : A multi disciplinary approach

Components Required ? Required for Synthesis

A. Sensorimotor

Sensory

Sensory Awareness

Sensory Processing

Tactile

Proprioceptive

Vestibular

Visual

Auditory

Gustatory

Olfactory

Perceptual Processing

Stereognosis

Kinesthesia

Pain Response

Body Scheme
Cerebellar Ataxia 323

Components Required ? Required for Synthesis

R-L discrimination

Form constancy

Position in space

Visual-Closure

Figure Ground

Depth Perception

Spatial Relations

Topographical Orientation

Neuromusculoskeletal

Reflex

Range of motion

Muscle Tone

Strength

Endurance
324 Neuro-Rehabilitation : A multi disciplinary approach

Components Required ? Required for Synthesis

Postural control

Postural Alignment

Soft tissue Integrity

Motor

Gross coordination

Crossing the midline

Laterality

Bilateral Integration

Motor control

Praxis

Fine coordination/ Dexterity

Visual-Motor Integration

Oral-Motor Control
Cerebellar Ataxia 325

[Link] Integration and cognitive components

Components Required ? Required for Synthesis

Level of arousal

Orientation

Recognition

Attention Span

Initiation of activity

Termination of activity

Memory

Sequencing

Categorisation

Concept formation

Spatial Operations

Problem Solving

Learning

Generalisation
326 Neuro-Rehabilitation : A multi disciplinary approach

C. Psychosocial Skills and Psychological Components

Components Required ? Required for Synthesis

Psychological

Values

Interests

Self-concept

Social

Role performance

Social Conduct

Interpersonal Skills

Self-expression

Self-management

Coping skills

Time management

Self-control
Cerebellar Ataxia 327

Psychological Aspects in neurodegenerative disorders are more prone than


CEREBELLAR ATAXIA other people to become confused if drug doses are
too high or are advanced too fast. After 1 or 2
Problems due to Cerebellar damage: months of no response to one antidepressant at a
typical dose it is reasonable to switch to another
When the Cerebellum is affected it leads to the agent. Helpful environmental interventions often
following impairments: in executive function, involve establishing a structure for the patient,
visual-spatial analysis and selected deficits in including setting regular hours for sleeping, eating,
language skills as well as changes in personality exercising and getting out of the home. Meanwhile,
and behavior. There may be major problems in the patient can be freed from burdensome
multi tasking, planning and organizing. The responsibilities, which then can be gradually
cognitive flexibility, which the patient used to reinstated as the patient improves. As always,
perform earlier but after the onset of the disease treatment must be individualized. Unlike
they may require conscious effort and new depression, the goal for management of personality
strategies. The patients may suffer from mood change or impaired cognition is not elimination of
changes like depression, apathy, irritability and the condition, but rather adaptation to it. Medicines,
limited frustration tolerance. typically antipsychotics drugs (such as haloperidol,
Over 65% of individuals with cerebral ataxia resperidone, or olanzapine) or mood stabilizers
develop mood disorders at time after the onset of (such as valproate or gabapentin) may prove useful
their neurological illness, usually a form of in minimizing moodiness or irritability. Apathy
depression. While low mood is usually a part of may improve with stimulants (such as
clinical depression, some or all of a long list of other amphetamines) or other agents affect the chemistry
symptoms are also present and may be more of the dopamine system (such as amantadine).
prominent than low mood: loss of interest in usual Establishing routines that emphasize the strengths
activities, excessive anxiety or agitation, irritability of the affected individual often prove very helpful
or changes in sleeping patterns, changes in appetite in managing personality change and cognitive
r sexual drive, etc. Episodes of depression typically impairment. For instance, a set schedule of regular
last weeks to months, but may be longer or shorter. daily activities, minimizing change from day-to-
This type of depression is caused by abnormalities day, is often helpful for patients who react with
in brain functions and is not a psychological anger, suspicion, or confusion to change. New
reaction to adverse events in a person's life. routines with increased stimulation may be of some
value in overcoming apathy.
Treatment: All treatment efforts must first start with
education. It is essential that individuals with the Learning the proper response to irritability
disease and their family members must be aware (ignoring it, switching to another topic of
of the psychiatric and the cognitive problems that conversation, or distraction by introduction of a
may arise during the course of their illness. Just like different activity) may help prevent escalation of
they experience trouble with balance or temper into verbal or physical violence. Patients
coordination, psychiatric and cognitive problems may partially compensate for mild cognitive
are a part of the disease and are not the fault of the impairment by keeping lists and allowing
affected individual, family members or health care themselves more time for tasks. The stress on
providers. patients and families of more severe cognitive
impairment or personality change may be
The recognition of depression is essential, since
decreased by removing the responsibility of the
among all the other complications of the disease,
patient for performing tasks which are beyond their
this may be the most treatable. The approach to the
capacity (maintaining finances, supervising small
treatment is multipronged and this includes
children, driving, employment), while maintaining
antidepressants, support and environmental
the patient's responsibility for tasks within their
changes. Antidepressants are very effective in
capacity. Such interventions require considerable
treating depression i.e. Serotonin specific reuptake
delicacy. Developing, implementing, revising, and
inhibitors (SSRI's), of which Prozac (fluoxetine) is
maintaining treatment for cognitive and psychiatric
the most famous example. It is prudent to start with
complications of cerebellar disease requires an
a relatively low dose and advance the dose
accurate assessment of the patient's strengths and
relatively slowly, since individuals with
weaknesses, individualization of treatment, and
328 Neuro-Rehabilitation : A multi disciplinary approach

flexibility to change the plan as needed. 7. Mariotti C, Fancellu R, Di Donato S:An


Neurologists and primary care physicians with overwiev of the patient with ataxia. Journal of
expertise in depression and personality change may Neurology, 2005.
assume overallmanagement of the treatment. In
8. Yabe I, Matsushima M, Soma H, et al.
more complicated cases, or with neurologists or
Usefulness of the Scale for Assessment and
primary care physicians who are less comfortable
Rating of Ataxia (SARA). Journal of the
with these issues, consultation with a psychiatrist
Neurological Sciences 2008.
with experience in the complications of
neurodegenerative diseases may be necessary. 9. Yelnik A, Bonan I: Clinical tools for assessing
Neuropsychological testing can provide precise balance disorders, 2008.
information on cognitive strengths and weakness, 10. Patten J; Neurological Differential Diagnosis,
and occupational therapists can assess an 2nd Ed, Springer 1996,
individual's capacity to perform both routine and
more complicated tasks of daily living. Involvement 11. Kumar P; Clarke M; Clinical Medicine, 6th Ed,
of the family is essential in every step of the process (2005). WB Saunders: London.
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To summarize, diseases that result in cerebellar Holland: Management of the Ataxias: towards
degeneration are commonly complicated by best clinical practice, 2009.
cognitive impairment and psychiatric disorders, 13. Hanks et al. The role of Therapy in Rett
especially depression. These complications are syndrome; American Journal of Medical
probably more frequent and more severe when Genetics 2005
other brain regions in addition to the cerebellum
14. Fogel and Perlamn. An approach to the patient
are also involved. Based on our current knowledge
with late - onset cerebellar ataxia
of neurodegenerative diseases affecting other brain
regions, both pharmacologic and non- 15. Wilson et al: Quality of life in Friederiech
pharmacologic interventions may prove helpful to Ataxia, Eur J Neurol 2007.
patients with cerebellar degeneration and their
16. Susan B O'Sullivan: Physical Rehabilitation.
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to the strengths, weakness, and environment of therapy and physiotherapy in multiple
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Multiple Sclerosis is an inflammatory relapsing or and rest of Australia which are closer to the equator
progressive disorder of CNS white matter and is a are medium frequency areas, with incidence
major cause of disability in young adults and affects reported of 10 to 15 per 100,000 population. Asia,
between the ages of 20 and 40 years.(1) Africa and South America are low frequency areas,
with incidence reported of less than 5 per 100,000.
Perhaps the most incriminating evidence for the
role of environmental factors in the development
of MS is the changing risk with migration and the
occurrence of MS clusters and epidemics.
Immigrant populations tend to acquire the MS risk
inherent to their new place of residence. Migration
from high to low prevalence before the age of 15
lowers the risk, whereas migration after this age
does not affect risk. Migration from high to low
prevalence areas increases the risk of MS, but the
effect of age is less clear.
Other environmental factors associated with the
development of MS include cigarette smoking
(odds ratio of 1.81), animal fat intake, and deficiency
It was identified and established by Dr. Jean Martin of vitamin D.
Charcot in 1868 who described it as Sclerose en It has been observed that, place where person
Plaques.(2) spends the first 15 years of life may determine to a
greater or lesser likelihood of developing MS as
Epidemiology opposed to where he or she lives at the time of
diagnosis.(3) Minor respiratory infections relapses
MS is not a rare disease and it affects millions
27% of relapses in patients with established MS.(4)
worldwide. Symptoms usually begin during
adulthood, with peak onset around the age of 24. A multitude of other environmental factors have
Approximately 0.3% of MS cases are diagnosed been suspected to alter the risk for MS (cold climate,
before age 15. Multiple sclerosis (MS) affects women precipitation, amount of peat in the soil, exposure
more than men. to dogs, and consumption of meat, and dairy
products), but none has been verified to be an
MS is most predominant in whites of Northern independent risk factor.
European ancestry and is rare in some races like
African blacks and Eskimos. By studying people Etiology
who move from one area to another, researchers The exact cause of MS is not known though it is
have learned that individual risk changes based on believed to be caused by damage to the myelin
location. They have concluded that some exposure sheath, the protective covering that surrounds
in the environment increases the risk of for MS. nerve cells. When this nerve covering is damaged,
The incidence of MS is seen more in places which nerve signals slow down or stop. The nerve damage
are farther from the equator, MS occur more is caused by inflammation which occurs when the
prominently over there. Studies have shown that body's own immune cells attack the nervous
distribution of MS depend on areas of high, system. This can occur along any area of the brain,
medium and low frequency. optic nerve, and spinal cord.
The presence of increased immunoglobulin (IgG)
The temperate zones of the northern United States, and Oligoclonal bands in CSF of 65 to 95% of MS
northern Europe, southern Canada, New Zealand, patients suggestive of a precipitating infection
and Southern Australia are high frequency areas; eliciting an autoimmune response with resulting
with incidence reported of 30 to 80 per 100,000 pathological changes.(5)
populations. Southern United States and Europe
330 Neuro-Rehabilitation : A multi disciplinary approach

It is believed that viruses such as Epstein-Barr fatigue rapidly. With severe disruption, conduction
(mononucleosis), varicella zoster, and the hepatitis block occurs with resulting disruption of function.
vaccine may be the cause of MS to till date. 13
However, this belief has not been proven.(6)
The definitive genetic association in MS is with the
Clinical Course of Multiple Sclerosis
serologically defined human leukocyte antigen Clinical course of MS is unpredictable. Patients,
(HLA) DR15, DQ6. This is one of the DR2 usually have one of the following clinical courses:-
haplotypes, also known as Dw2 in cellular
Benign MS: Some patients get only rare relapses
terminology and DRB1*1501, DQA1*0102,
of their disease, in which patients' remains
DQB1*0602 in molecular nomenclature.7, 8, 9
functional till 15 years after disease onset. It affects
Other susceptibility genes likely contribute, 20% of MS patients
possibility the T-cell receptor variable ? region and
Benign multiple sclerosis" is often temporary as
the IgG heavy-chain variable region (especially the
apparently benign disease often becomes
VH2-5 gene)
disabling.14
Fine mapping data suggests that the DRB1 gene
Malignant MS: In this type there is rapid
itself is responsible for a significant portion of this
progression of the disease which sometimes cause
risk.(10)
significant disability or death within short period
Another theory suggests that, the immune system after the onset
mistakes a portion of myelin protein for a virus that
Relapsing/Remitting type: It is the most common
is structurally similar and targets it for destruction,
type and is seen among 70% of MS patients and
known as Molecular Mimicry.(11)
occur frequently, one to several times per year.
Genetically identical monozygotic twins are more
Primary Progressive MS: In the primary
often concordant for MS than dizygotic twins (26%
progressive form continuous worsening occurs
and 2.4% respectively), indicating a genetic
with steady progression but not interrupted by
component; however, even after following
distinct relapses. It affects 10% of MS patients
monozygotic twins past age 50 or using MRI data,
less than 50% are concordant, suggesting a role for Secondary-Progressive MS: In this type relapsing
environmental factors. remitting disease followed by progression with or
without occasional relapse, minor remissions and
Smoking is being implicated as risk factor in MS as
plateaus.
it has been associated with increased blood-brain
barrier disruption, and greater atrophy in multiple
sclerosis.(12)
Progressive-relapsing
Progressive disease from onset but with clear, acute
Pathophysiology: relapses that may or may not have some recovery
or remission; Periods between relapses are
In patients with MS, the immune response triggers
characterized by continued progression.
the production of T-Lymphocytes, macrophages,
& immunoglobulin's (antibodies). In turn, a That mainly seen in people, whose disease onset after
command or trigger protein, the antigen is 40 years.(15)
activated, producing autoimmune cytotoxic effects
within the CNS. Signs and Symptoms
The blood brain barrier fails and myelin sensitized Signs and symptoms of MS vary depending on the
T - Lymphocyte cells enter and attack the myelin location of specific lesions. Symptoms can develop
sheath that surrounds the nerve. Myelin serves as quickly, within hours, or slowly over several days
an insulator, speeding up the conduction along or weeks.
nerve fibres from one node of ranvier to another
termed salutatory conduction. It also serves to
conserve energy for the nerve because
depolarization occurs only at the nodes. Disruption
of myelin sheath produces active demyelination,
slowing neural transmission & causing nerves to
Multiple Sclerosis 331

From the study of 697 patients with MS


Most Frequent Symptoms of MS
1. Fatigue 85%
2. Walking problems- 87%
3. Bladder problems- 65%
4. Pain and other sensations- 60%
5. Visual disturbances- 58%
6. Cognitive problems- 44%
7. Tremors- 41%
It has been concluded that-
• 50 to 70% of people with MS shows
evidence of some cognitive impairment
• Majority have mild impairment
• 10% to 20 % have significant dysfunction

Common Symptoms in Multiple Sclerosis

Sensory Symptoms: Hypoesthesia,numbness,


Paraesthesia(tingling)
Dysesthesias(burning)

Motor Symptoms : Weakness or Paralysis


Fatigue
Spasticity
Incoordination
Intentional Tremor
Impaired Balance
Gait Disturbances.

Pain: Lhermitte's Sign: Tingling in spine with limbs


on neck flexion.
Chronic Pain.

Visual Symptoms: Blurred or double vision


Diminished acuity or loss of vision
Scotoma
Nystagmus.
Optic or trigeminal Neuritis

Bladder Symptoms: Urinary Urgency, Frequency


Nocturia
Incontinence
Urinary Hesitancy, Dribbling
Bowel Symptoms: Constipation
Diarrhoea
Incontinence
Speech & Swallowing: Dysarthria
Diminshed verbal Fluency
Dysphonia
Dysphagia
332 Neuro-Rehabilitation : A multi disciplinary approach

Cognitive Symptoms: Memory or recall problems


Decreased attention, concentration
Diminished abstract reasoning
Diminished problem solving, judgment.
Diminished speed of information processing.
Diminished visual -Spatial abilities.

Emotional Symptoms: Depression


Pseudo bulbar Affect
Anxiety
Cardiovascular Dysautonomia Very rare.

Sensory Symptoms: This is the most common simultaneous optic neuritis is uncommon in adults,
presenting manifestation in MS (21% to 55%) and but formal visual field testing reveals unexpected
ultimately develope in nearly all the patients. The defects in the clinically normal eye in a substantial
symptoms like Hypoesthesia, numbness, number of patients.
Paraesthesia (tingling) Dysesthesias(burning) may
Examination shows an afferent pupillary defect,
occur in one or more limbs, trunk, face or in
diminished visual acuity, subdued colour
combinations. The more distinctive sensory
perception, and often a central scotoma.
relapses of MS consist of the sensory cord syndrome
and sensory useless hand syndrome. The sensory There may be persistent visual blurring, altered
symptoms may ascend to the trunk, producing a colour perception, or Uhtoff's sign. MS patients
sensory level, or may involve the upper extremities. without a clinical history of optic neuritis often have
In most of the MS patients it has seen that they evidence of optic nerve involvement on
develop persistent sensory loss, usually consisting funduscopic examination or visual evoked
of diminished vibratory and position sensations in potentials. Recurrent optic neuritis can occasionally
distal extremities. be seen without evidence for dissemination to other
areas of the CNS.
Motor Symptoms: Motor symptoms like
Weakness, Spasticity or Paralysis, Fatigue, Patients with frequent and severe optic neuritis
Incoordination, Intentional Tremor, Impaired events in the first 2 years were more likely to
Balance, Gait Disturbances occur due to Pyramidal convert to NMO; they also had a higher likelihood
tract dysfunction. It can occur acutely or in a chronic of significant persistent vision loss.
progression with weakness of one or more limbs Cerebellar Symptoms: Cerebellar pathways are
and facial weakness, leg stiffness that impairs gait frequently involved during the course of MS, but a
and balance, or extensor or flexor spasms. Exercise predominately cerebellar syndrome is uncommon
or heat frequently worsens subtle deficits. at onset. The manifestations include dysmetria,
Muscle atrophy is usually due to disuse, but lesions dysdiadochokinesia, action tremor with terminal
of lower motor neuron fibers or of the anterior horn accentuation, dysrhythmia, breakdown of complex
itself can cause pseudoradiculopathy with motor movements, and loss of balance. Patients
segmental weakness, atrophy, and diminished with long-standing MS may develop a "jiggling"
reflexes. Motor symptom manifestations of MS in gait and an ataxic dysarthria with imprecise
32% to 41 % of all cases and their prevalence is articulation, scanning speech, or varying inflection,
higher than 60% in long-standing MS. giving it an explosive character.
Visual Symptoms: The initial symptoms of MS Bladder Symptoms: Urinary urgency, frequency,
includ optic neuritis (ON) in 14 % to 23% of patients, and urge incontinence (due to detrusor hyper-
and more than 50 % experience a clinical episode reflexia or detrusor-sphincter dyssynergia) result
of optic neuritis during their lifetime. The most from spinal cord lesions and are frequently
common manifestation is visual loss in one eye that encountered in MS patients. The combined
evolves over a few days. Periocular pain, especially incidence of bowel and bladder dysfunction in MS
with eye movement, usually accompanies and may is more than 70%. Symptoms of bladder
precede the visual symptoms. Bilateral dysfunction may be transient and occur with an
Multiple Sclerosis 333

exacerbation but are commonly persistent. fibers is responsible for these symptoms
Impaired vesicular sensation causes a high capacity (pseudobulbar effect).
bladder and may lead to bladder atonia with
Fatigue: It is a pervasive symptom among MS
thinning and disruption of detrusor muscle. Due
patients that is not related to disability or
to incontinence, there is constant dribbling of urine
depression. Over 75% of MS patients experience
in this irreversible condition. Interruption of brain
fatigue during their disease course. A diurnal
stem mictuirition center input sometimes lead to
pattern is characteristic and follows the normal
cocontracture of the urinary sphincter and detrusor
circadian pattern of body temperature fluctuations,
muscles (detrusor=sphincter dyssnergia). If it is
with the worse symptoms occurring in afternoon
untreated, high pressure may lead to
hours (peak core body temperature) often giving
hydronephrosis and chronic failure.
way to improvement in the late evening.
Bowel Symptoms: Constipation is a common
Pain: Intense pain and ipsilateral or crossed sensory
problem, occurring in 39% to 53% of MS Patients,
symptoms may accompany them. Paroxysmal
especially with limited activity and spinal cord
weakness occurs, but it is uncommon. Paroxysmal
involvement. Fecal incontinence is a socially
sensory symptoms like tingling, prickling, burning,
devastating symptom that is often associated with
or itching may occur in MS patients. Sharp
perineal sensory loss in MS patients.
neuralgic pain is also common. Trigeminal
Sexual Dysfunction: It is a frequent problem in MS. neuralgia may appear in patients with MS. The
Nearly two thirds of patients report diminished occurrence of trigeminal neuralgia in a person
libido. One third of men have some degree of younger than age 40 is suggestive of MS.
erectile dysfunction, and a similar percentage of
Lhermitte's sign (transient sensory symptoms
women have deficient vaginal lubrication. Besides
usually precipitated by neck flexion)- It is an
direct neurological impairment, sensory loss,
electrical or tingling sensation that travels down the
physical limitations, depression, and fatigue
spine or into the extremities. Although quite
additionally contribute to sexual difficulties in MS
common in MS, Lhermitte's sign can also occur with
patients and sometimes the partner's attitude and
a wide variety of other disorders, such as vitamin
psychological factors dealing with self-image, self-
B12 deficiency, spondylosis.
esteem, and fear of rejection may also lead to
impotence or loss of libido. Seizures: It has seen that seizures occur in larger
proportion of MS cases compared to normal control
Intense vertigo assosciated with nausea and emesis
subjects. Cortical and juxtacortical lesions may be
is an occasional manifestation of MS relapse.
responsible for the increased incidence of seizures
Speech and Swallowing: Dysphagia is often due
in MS patients. Focal motor seizures, possibly with
to impairment of cranial nerves IX, X and XII and
secondary generalization, are the most frequent.
generally appears late in the course of some
The occurrence of seizures usually follows one of
patients.
two patterns. Some times focal onset seizures begin
Cognitive Symptoms: Cognitive symptoms are early in the course of MS and become a chronic
present in 40% to 70% of MS patients. The pattern problem that may be difficult to control.(16)
of cognitive decline is typified by decrease of
episodic memory, processing speed, verbal fluency Diagnosis
and difficulty with abstract concepts and complex
MS is a disease disseminated in time and
reasoning. To a lesser extent, executive functioning
disseminated in space (Harrison's)
and visual perception, semantic memory and
attention span may also decrease. General Diagnosis of MS is largely clinical. The diagnosis
intelligence is not typically affected. of MS is generally done by a neurologist on the basis
of history, clinical findings, and supportive clinical
Emotional Symptoms: Anxiety and depression are
tests, including magnetic resonance imaging(MRI),
more frequent in MS patients than in the general
cerebral spinal fluid(CSF), and evoked
population. In long-term studies, the incidence of
potentials(EP)
depression in MS patients is close to 75%. Patients
sometimes experience uncontrollable weeping or Magnetic resonance imaging (MRI): The brain
less commonly laughter incongruent with their MRI is the most sensitive test for detecting
mood. Interruption of inhibitory corticobulbar structural abnormalities due to MS-related disease
334 Neuro-Rehabilitation : A multi disciplinary approach

MS cannot be made without an abnormality in the


spinal fluid. The spinal fluid analysis may also be
useful in excluding an infection that may be difficult
to distinguish from MS.(17)
Prognosis: The prognosis for visual recovery after
each episode of optic neuritis is good, and most
patients regain normal visual acuity. Profound
visual loss, recurrent optic neuritis, and age older
than 35 are associated with a higher risk for poor
recovery.
Factors that are associated with an increased risk
of developing MS as a disseminated illness are the
presence of venous sheathing, recurrent optic
neuritis, family history of MS, white race, previous
vague or non-specific neurological symptoms, and
the presence of oligoclonal bands (OCBs), elevated
IgG index, or IgG synthesis rate in CSF. The severity
of acute transverse myelitis is inversely related to
the risk of acquiring further symptomatic
activity. MRI scans show focal brain abnormalities demyelinating lesions. Complete transverse
in more than 90% of patients with clinically definite myelitits with profound loss of motor, sensory, and
MS. MRI with gadolinium scan distinguish between sphincter function imparts a relatively low risk of
new or old lesion. Because the imaging 3 to 14 for the later diagnosis of MS.
abnormalities seen in MS patients can also be seen
in other medical conditions, a diagnosis of definite Partial transverse myelitis with preservation of
MS cannot be based solely upon the MRI. significant motor function at peak is associated with
a much higher incidence of MS.
Evoked Potentials: In 90% of MS patients shows
90% of abnormal evoked potentials Evoked Prognosis depends on the subtype of the disease;
potentials reflect changes in the electrical activity the individual's sex, age, and initial symptoms; and
that occurs within the CNS due to sensory input (a the degree of disability the person experiences.
stimulus). Visual evoked Potentials (VEP), Brain Female sex, relapsing-remitting subtype, optic
Stem auditory evoked potentials(BAEP), and neuritis or sensory symptoms at onset, few attacks
Somatosensory evoked potentials provide evidence in the initial years and especially early age at onset,
of altered nerve conduction. These tests are are associated with a better course
abnormal in 70-90% of patients with clinically The life expectancy of people with MS is 5 to 10
definite MS and often detect abnormalities that are years less than that of unaffected people. Almost
not apparent on neurological examination. Because 40% of patients reach the seventh decade of life.
these tests measure function within the brain or Nevertheless, two-thirds of the deaths in people
spinal cord, they complement the information about with MS are directly related to the consequences of
brain structure provided by the MRI. the disease. There is higher prevalence of suicide
Lumbar Puncture (Spinal Tap): Cerebrospinal fluid in MS than in the healthy population, while
abnormalities are detected in 80-90% of patients infections and complications, depressions are
with clinically definite MS. These abnormalities especially hazardous for the more disabled ones.
include an increase in the number of cells and Patients with sensory or visual symptoms as a
immunoglobulin proteins suggesting an dominant neurological sign have a more positive
inflammation or a heightened immune response. prognosis.(18)
This test may be used to establish a diagnosis in
patients who have experienced a slowly progressive Although most patients lose the ability to walk prior
decline in function without exacerbations (i.e; to death, 90% are still capable of independent
patients with so-called primary progressive MS) walking at 10 years from onset, and 75% at 15
and who have no abnormalities seen on the brain years.(19)
MRI scan. In such instances, a diagnosis of definite
Multiple Sclerosis 335

Medical Management Surgical Procedures- Phenol blocks and


tendon release for severe spasticity
Disease -Modifying Agents 2. Fatigue -
There are three disease- modifying agents, which
First line treatment for fatigue- Amantadine
are recognised by the U.S Food and Drug
Hydrochloride (effective in 40% cases of MS)
Administration (FDA) for relapsing-remitting MS.
It has seen that these drugs are effective on Primary Sometimes CNS stimulants like-Pemoline
and secondary progressive disease. (cylert) and methylphenidate (Ritaline) are given
but there are some side effects (Anorexia,
Drugs are:-
Irritability, and Insomnia)
• Interferon beta1a (Avonex)
3. Bladder and Bowel Symptoms-
• Interferon beta1b (Betaseron)
Bladder symptoms-
• Glatriramer acetate (Copaxone)
• Neurogenic bladder:- Urinary tract
These drugs have shown approximately one third infection, should be taken care off.
reductions in relapse rate.
• Smooth Muscle relaxants or nerve
blockers - Propantheline (Pro-Banthine)
Management for Relapses and Oxybutynin (Ditropan) reduce
Natural improvement occurs in 4 to 12 weeks and urinary frequency and urgency.
degree of improvement varies from person to • Bethanechol (Urecholine) or
person Phenoxybenzamine - For urinary
For Acute Relapse- High dose intravenous retention
corticosteroids • Crede techniques and intermittent
Eg- Methylprednisolone (Depomedrol) catheterization to empty the bladder.
Given over 3to5days, gradually followed by • If problem cannot be solved with
tapering does of Oral corticosteroids (eg. medication or intermittent
Prednisone) catheterization then continuous
catheterisation (Foley Catheterisation)
Sometimes Oral corticosteroids may be used to treat
a mild or moderate relapse, and intravenous • Sometimes antibiotics given to treat the
methylprednisolone used to treat Optic Neuritis bladder infections.
and retrobulbar neuritis. There are some side effects • Dietary recommendations include
of having long term corticosteroids, such as drinking 8 glasses of fluid per day
Osteoporosis, Hypertension, Cataracts, Muscle
wasting etc. Bowel Constipation/ Incontinence
• For Stool Softeners- Docusate (Colace)
Management for Symptoms
• Laxatives - Milk of magnesia
1. Spasticity- Most commonly used medications
are • Suppositories- Glycerine or Bisacodyl
Baclofen (Lioresol), Tinzanidine (Zanaflex) (Dulcolax)
Other drugs are- • Adequate intake of fluids and fiber in
Sodium dantrolene (Dantrium) - induce diet.
weakness
Diazepam (Valium)- Spasms (in Night) 4. Pain- It is not common in MS
Cyclobenzaprine (Flexoril)- Back spasms But many people with MS suffer from-
Carbamepazine (Tegretol)- Tonic Spasms • Paroxysmal Pain
There are possibilities of some side effects with
Medications - Carbamazepine (Tegretol),
overdose of these drugs including sedation,
Amitriptyline (Elavil), Phenytoin
weakness and fatigue but reduction in
(Dilantin), Diazepam (Valium)
spasticity.
336 Neuro-Rehabilitation : A multi disciplinary approach

• Dysesthesias - Low doses of 9. Transfers


Amitriptyline (Elavil), Imipramine
10. Fatigue
(Tofranil) or Desipramine ( Norpramin)
11. Cardiovascular and Respiratory status
• Pain and Numbness - Corticosteroids
12. Visual Impairments
5. Tremors 13. Bowel/Bladder /Sexual impairment
• Medications - Hydroxyzine (Atarax,
14. Speech
Vistaril), clonazepam (klonopin),
propranol (Inderal), buspirone (Buspar), 15. Swallowing
ondansetron (Zofran), and primodone
16. Sensory Status (Deep and Superficial)
(Myosoline) reduce the tremors
17. Cognition
• Dizziness and Vertigo- medications like
- Meclizine 18. Activities of daily living (BADL, IADL)
19. Vocational and Avocational Status
6. Sexual Dysfunction
• Erectile difficulties, orgasmic dysfunction 20. Psychosocial Status
and decreased libido
Specific Measures for MS
Medications - sildenafil (Viagra),
alprostadil (muse)
Scales and Assessment tools:
Injections - prostaglandin for erectile 1. Expanded Disability Status Scale (EDSS)-
dysfunctions Measures clinical impairments
Sometimes, sexual aids and prosthetic 2. Functional Independence Measures(FIM)
devices also advised. and Barthel Index- For functional Status

7. Cognitive Impairments and Emotional 3. Multiple Sclerosis Quality of Life


Problems (Depression) Inventory (MSQLI)
For Cognitive Impairments- Retraining and 4. Modified Fatigue impact scales (MFIS)
teaching of compensatory techniques 5. MS functional Composite (MSFC)-
Depression - In studies it has been seen that in measures leg function and ambulation,
MS Depression is 7.5 times higher than general arm and Hand function and cognition. It
populations.(20) can be used for periodic baseline
function.(21)
Rehabilitation Management 6. Assessment of motor and process skills-
A standered assessment for ADLs
Examination :
1. Detailed history including current chief Speech affection in Multiple Sclerosis:
complaints and functional status, family
history, medical and surgical history. Multiple sclerosis affects various parts of the
central nervous system. Along with
2. Strength ambulatory problems, individuals with
3. Tone multiple sclerosis experience a lot of problem
in speaking and swallowing if the affected
4. Range of Motion areas are the speech and swallowing areas.
5. Balance (Static and Dynamic Sitting and This affects the normal tone and strength of
Standing Balance) the muscles of jaw, tongue, lips, soft palate and
the pharyngeal constrictors.
6. Coordination
The commonest speech problem seen in
7. Ambulation
individuals with multiple sclerosis are slurring
8. Bed Mobility of speech due to imprecise consonants and
scanning. Scanning problems lead to problems
Multiple Sclerosis 337

in rhythm and suprasegmentals of speech. Exercising to the point of fatigue is


Slurring of speech leads to affected clarity of contraindicated so frequent rest intervals
speech. Swallowing problems in multiple are advised as time to fatigue varies
sclerosis are not uncommon. The problems can greatly among individuals with MS. So
range from weak oral musculature to respect patients desire to rest and allow
disordered swallowing due to reduced him to rejuvenate himself between
pharyngeal peristalsis. sessions.
Management is explained in detail in chapter d) Type of Exercise: Circuit training in
speech rehabilitation. which improved work capacity is
developed through the use of various
Physiotherapy Management in different stations that alternate work
Multiple Sclerosis between upper and lower extremities,
distributes the load among muscles and
Physical therapist assesses an MS patient with may prove best for reducing the
emphasis on measures of voluntary movement, likelihood of fatigue.
sensory appreciation, ROM, strength, balance,
fatiguability, mobility, gait and functional status. Symptomwise Management :
1. Spasticity : Apart from oral medications,
Framework for Physical therapy management in
MS: a) Topical cold or hydrotherapy can
temporarily reduce spasticity by
According to the National MS Society's Medical
decreasing tendon reflex excitability and
Advisory Board, rehabilitation referral should be
clonus and by slowing conduction of
initiated whenever there is an abrupt or gradual
impulses in nerves and muscles.
worsening of the function or an increase in
impairment that has significant impact on the b) Intermittent static stretching held for
individual's mobility, safety independence and / minimum of 30 to 60 seconds be applied
or quality of life. ideally for 5 to 10 repetitions.
2. Coordination and Balance training : Frenkel's
Specific Measures for MS
Exercises including upper and lower extremity
Scales and Assessment tools: Items in these are coordination exercises. Also exercises on
included to provide information about the disease balance board are recommended in patients
process and outcomes and ideally document who are able to stand, but have poor standing
clinically meaningful change over time. balance and coordination.
Expanded Disability Status Scale (EDSS) and 3. Tightness: Flexibility exercises and ROM
Functional Independence Measures(FIM). exercises, to ensure adequate joint ROM.
Mainly stretching advised for hip flexors,
Goals of PT in MS :
adductors,hamstrings and heel cords in lower
1. To Improve muscle performance in terms of limbs.
strength, power and endurance:
In upper limbs pectoralis major / minor and
Prescription is based on four interrelated lattismus dorsi as these are likely to develop
elements (the FITT Equation ) shortness due to slumped posture.
a) Frequency of exercise: Daily exercises 4. Pain: Use of TENS (Transcutanious Electrical
sessions should be scheduled, preferably Stimulation), multidisciplinary stress pain
in the morning, when body core clinic, stress management are adviced.
temperature tends to be lowest and
before fatigue sets in. 5. Urinary incontinence: Apart from
medications, patients are also taught Kegel's
b) Intensity of exercise :Submaximal exercises to strengthen pelvic girdle muscles,
Exercise intensities (50 to 70 % of MVC- so as to assist in improving stress incontinence
Maximal Contraction) due to poor muscular control.
c) Time or Duration of exercise :
338 Neuro-Rehabilitation : A multi disciplinary approach

Occupational Therapy Management: Occupational therapist may initiate coordination


training with neuromuscular education and
Role of Occupational Therapy progress to repetitions activities requiring desired
coordinated movement patterns. Placing blocks,
Primary objectives and aims
marbles, cones, paper cups or pegs, are enabling
The primary objective of occupational therapy is activities that demands repetition pattern of non-
to enable individuals to participate in self-care, resistive movements.
work and leisure activities that they want or need
to perform, thereby optimising personal fulfilment, Activities should be structured to enable the client
well-being and quality of life. The occupational to use precise movement pattern and to work at
therapist evaluates whether people with MS are speeds consistent with the maintenance of
limited in the life domains that are important to precision. Therapist should avoid
them Figure and determine strategies for constantrepetition of an incoordinated pattern as
overcoming these difficulties. Possible strategies this reinforces incoordination. Practice that increase
include restoration, compensation, adaptation and incoordination such as fear, poor balance, faulty
prevention. posture, pain, too much resistance, too much
fatigue, strong emotions, prolong inactivity and
Interventions excessively prolong activity should be avoided or
Evaluation is the first step in the occupational dealt with prior training.
therapy programme. The therapist assesses the over Purposeful activities such as leather lacing, mosaic
all performance areas like ADL, IADL, work leisure, tile work, needle craft and house hold task such as
social participations, participation skills like Motor, wiping, sweeping and dusting also provide
process and communication skills. repetitions in movement along with psychological
A home and work environment assessment is benefits such as alleviating feelings of guilt and
generally completed to evaluate potential need for reinforcing feelings of self worth.
modifications and a customised occupational Fatigue is one of the most common symptoms in
therapy rehabilitation program. As Multiple MS(reported by at least 75% of MS patient) which
sclerosis has an unpredictable cause, periodic re- impaired quality of life among MS patients,
evaluation may be necessary. Patients endurance/ independent of depression or disability. Fatigue
fatigue, sleep, patterns, visual abilities, perceptual also imposes significant socioeconomic
processing and cognitive emotional and balance consequences, including loss of work hours and in
should also be reassessed periodically. some instances, loss of employment.
Multiple sclerosis is a progressive condition, hence Role of occupational therapist is to teach energy
OT focuses on the need of the patients to adapt to conservation techniques, so individuals can
their deteriorating/disabling condition. It is participate in self-care, work and leisure
essential to educate the patients and their families activities.(23)
about the condition and to obtain their support for
making long term realistic plans and for Occupational therapy management to improve
consideration, modification or adaption as and Coordination, strength and endurance
when required
Activities that can be given to improve balance
Programme improves the individual's ability to and co-ordination, strength and endurance
perform daily activities within his or her own
• Reach outs in all fours position like pegs
unique situation, thus, often requiring a
reach outs to improve gross motor and
combination of different techniques for making
eye hand coordination.
long term realistic plans.(22)
• Reach outs in kneeling position in
In patients with multiple sclerosis, occupational
different directions to improve balance
therapy can be used to develop strength, stamina
and overhead peg transfers to improve
and coordination. Occupational therapist
bilateral hand coordination
emphasizes on active and purposeful occupations,
which have an advantage of engaging the clients • Ball throwing in kneeling and standing
attention and interests as well as help in position to improve balance
psychological upliftment.
Multiple Sclerosis 339

• Wand exercises to improve gross motor Managing Heat during exercise in Multiple
coordination Sclerosis Rehabilitation.
• Beads stringing and picking small beads Some patents with MS are affected by heat, but
by cloth pins improve fine motor some are particularly sensitive. Hot weather, an
coordination over-heated room and exercise can all make MS
symptoms worse. This is a temporary effect but
Management for Spasticity - when the body cools down again, symptoms return
Active and passive ROM exercises to the level they were before. If patients are sensitive
to heat, keeping cool during or shortly before
• Weight bearing exercises
exercise may help to do exercise for longer, or more
• Hydrotherapy strenuously, without bringing on heat-related
symptoms. Patient can take ice drinks, cooling
• Serial casting in inhibitive postures has
garments, or give regular breaks to prevent
been seen to be effective in tone
overheating. Research showing benefits for these
reduction.(25)
cooling techniques is not conclusive, and they may
• Stretching exercises not help everyone, but they are unlikely to be
The aims of stretching in spasticity are to improve harmful. Sometimes lowering the body's
muscle extensibility, reduce muscle stiffness, and temperature, with cold baths or cooling garments,
improve function. Clinically, a range of stretching cold packs reduce muscle stiffness temporarily. In
techniques are used including static, dynamic, contrast, some people with MS find that cool
Proprioceptive Neuromuscular Facilitation (PNF), temperatures make their spasms or stiffness worse.
as well as prolonged and ballistic stretching.(26) For these people, exercising in a warm swimming
pool may help with stretching and relaxing
When sitting for long periods in a wheelchair, there muscles. Precaution is needed while taking hot and
is a greater risk of tone increase and muscle cold therapies. When applying cold directly to the
shortening (for example of hip adductors and hip skin, or when using cooling garments or cold water
flexors). Rehabilitation should then include to cool the body, care should be taken not to damage
instruction on sitting correctly in a tone inhibiting the skin. MS can cause changes in the way the
posture (for example, with knees separated). Tone- patient experience temperature, distorting the
inhibiting postures enable long-term stretching of feeling that would normally tell them when
the spastic muscles and are effective in preventing something is too hot or too cold.
muscle shortening.
Strategies to manage fatigue:
Use a tilting table to stretch hip flexors and calf
• Frequent, short rests can be beneficial.
muscles using body weight. For the arms, one can
consider individually adapted splints, for example • Balance between periods of rest and activity.
to maintain an open hand position, which is Regular rest periods should be built into the
important for hand hygiene.(27) daily schedule. Rest should be taken before
fatigue sets in. The balance can be difficult to
Management for Anxiety and Depression- Deep find -- too much exercise can induce fatigue,
breathing and relaxation techniques. but decreased physical activity can also lead
to tiredness and lack of energy.
Regular relaxation can lead to decreased tension in
muscles, lower blood pressure and slower heart • Organize the tasks Priorities: most important
rate. Relaxation can help with fatigue as it promotes and valued activities should be done first. A
good sleep patterns; increases benefit from rest valued leisure activity can boost motivation
periods during the day and can be used to manage and well-being, better equipping the person
stressful situations. to take on the next task.

Deep breathing is the basis for many relaxation • Because of fatigue, full time work may not be
techniques. However, focusing on how you breathe possible hence part time work should be
and creating a slow, deep and even pattern helps considered.
to feel calmer and more relaxed and can create a • Ergonomics are important. Good, supportive
distraction from the causes of stress. seating posture should be encouraged.
340 Neuro-Rehabilitation : A multi disciplinary approach

Ball throwing in sitting to improve balance Ball throwing in standing to improve balance and
coordination

Weighted resistive therapeutic exercises to decrease


tremors in upper limbs

Rearrange the environment to avoid Occupational therapy in the home: - Meeting the
unnecessary lifting, carrying or stooping. Use individual and the family in their environment can
proper lifting techniques -- bend with the provide the occupational therapist with valuable
knees instead of the back. Use a trolley, if information that may not be readily available when
necessary, to avoid unnecessary carrying. assessing the person within a healthcare setting.
The assessment includes an evaluation of the
• Simplify tasks where possible. Hard tasks can
individual's current functional status in relation to
be done with help of another person, to reduce
the performance of activities of daily living.
fatigue.
An assessment of the home situation includes the
• Smoking can increase fatigue as can infection.
evaluation of the need of any modifications For
• A healthy, balanced diet can help -- involve a example:- Kitchen modification or need for assistive
dietician. Unnecessarily hefty meals can equipment and training.
invoke fatigue as can an inadequate diet.
Finally, the occupational therapists visit to the home
• Extremes of temperature can exacerbate can serve to determine whether additional paid
fatigue -- for example. Hot bath assistance, e.g. Part time paid caretaker may be
• Stress can contribute to fatigue, so explore useful.
methods of minimizing stress. Kitchen Modifications - Use pull-out shelves; install
• Always set realistic goals, Unrealistic goals can these just below waist height or at horizontal reach.
cause stress. Relaxation techniques may be Use lightweight dishes and pans, and for larger and
helpful. heavier items use a trolly.
Multiple Sclerosis 341

Use of assistive devices- Assistive devices can help Vocational Rehabilitation:


the patient to be independent. Sometimes
People with MS constitute a valuable labour
individuals have poor balance and difficulty in
resource for the societies in which they live. They
overhead functions, so tools like long-handled shoe
are often well-educated and skilled workers with
horns, reachers, and dressing sticks can be helpful
extensive employment histories, which is not
• Use of dressing and grooming aids surprising given that MS typically manifests itself
(demonstrating button hooks, suggesting in early-to-middle adulthood (after people have
replacing zippers and small buttons with begun and, in many cases, established their careers).
Velcro, making closets easier to use) Too often, however, these highly trained,
• Use eating and drinking devices (helping productive, and experienced workers leave the
work force shortly after MS diagnosis, usually of
clients use assistive devices, such as double-
their own choosing and usually before the illness
handled cups, lightweight or weighted cups)
has rendered them unable to work.
• Helping patients learn how to use assistive or
Experts do not know exactly why so many people
mobility devices (wheelchairs, canes, scooters,
walkers) efficiently in their home (transferring with MS leave the work force prematurely, but
some of the most commonly reported barriers to
to/from the devices and beds, toilets, chairs;
continued employment among people with MS
maneuvering them in various spaces;
include
installing ramps to make using devices easier)
• The unavailability of transportation to and
• Computers open a world of recreation and job-
related activities, but MS symptoms may from work,
require special adaptations. For example, • Difficulties in obtaining on-the-job
people with vision problems can use accommodations
computers with enlarged keyboards,
• Lack of awareness of assistive technology
alternative mouse such as a Tracker ball can
be helpful or text-to-speech programs that • Limited awareness of employment issues on
read aloud the material onscreen. Location and the part of treating physicians, disincentives
setup of computer should be easily assessable. to work in government assistance
programmes
Electronic remote controls can be used for virtually
all the appliances in an environment-lights, radios, • The unpredictable and sometimes progressive
telephones, televisions, air conditioners, An OT can nature of MS
help the patient to determine which devices are
most appropriate. Patients with MS with memory Studies of people with MS show that men,
problems may find a day times very useful to people with
schedule their daily activities. It will help them to • Higher levels of education
remember things to do and also conserve their
• People who do not experience cognitive
energy.
impairments
Patient should try to do the hardest or necessary
• Workers whose jobs require little physical
tasks for times when they have the most energy
exertion or exposure to heat
and save the easier tasks for less energetic times.
• Management or professional-level workers,
Use mobility aids whenever required. Occupational
therapists can help the patient to find the right ones. • Employers who have formal disability related
Canes, walkers, wheelchairs and scooters may be personnel policies are the most likely to retain
warranted. employment over time.
Occupational Therapist can help the patient learn Vocational rehabilitation provides a mechanism for
to use all these equipments efficiently in their home people with MS to make adjustments in their
(transferring to/from the devices and beds, toilets, careers and to continue working as long as they
chairs; maneuvering them in various spaces; wish to. The purpose of vocational rehabilitation is
installing ramps to make using devices easier). to provide services, supports, and training that
(28,29,30) enable people with disabilities to obtain, maintain,
and advance in jobs that are compatible with their
342 Neuro-Rehabilitation : A multi disciplinary approach

interests, abilities, and experience. Early chronic illness can trigger off psychological issues
intervention is emphasised. Occupational therapist such as depression and anxiety. When behavioural
can assess and direct patients to get job placement. changes are addressed, the changes are described
Career counselling services offered to people with simply as emotional reaction to the life situation or
MS who have left the work force but wish to return poor adjustment to chronic illness. Patients with
to it. Occupational therapist conduct vocational multiple sclerosis don't go through a definite
evaluation of vocational interests, limitations, orderly set of stages culminating in adjustment.
strengths, work behaviours, transportation, Rather, adjustment is an ongoing, life process that
identification and implementation of workplace flows through the unpredictable changes brought
accommodations, training in the use of assistive about by the disease.
technology, self advocacy training, and
consultation with employers on a wide range of Psychological Process and Problems:
disability-related matters.
Emotional Issues:
For people with MS, the workplace
Major Depressive Disorder: Depression is the most
accommodations that have proven effective in
common psychological symptoms in multiple
helping them stay in the work force include
sclerosis. The incidence rate in multiple sclerosis is
Schedule modifications (the most common form of three times that of the general population. The 12-
workplace accommodation implemented on behalf month prevalence of major depressive disorder
of people with MS), among persons with multiple sclerosis is 15.7%,
nearly double the prevalence of major depressive
Memory aids to combat cognitive impairments,
disorder in persons without multiple sclerosis
Motorised scooters to combat fatigue and mobility (7.4%). Reports of the lifetime risk for major
problems, climate control in the work station, depressive disorder in multiple sclerosis
• Low vision aids (e.g. magnification machines, populations have ranged from 27-54%. It could be
voice output software), very difficult to diagnose multiple sclerosis as there
are many symptoms that are seen in both like
• Accessible parking, building renovations to fatigue, weight loss, etc. Also, suicidal ideations are
allow for wheelchair access, commonly seen in patients suffering from multiple
• Cooling vests, sclerosis, occurring7.5 times higher than in general
in the population. The point prevalence of anxiety
• Ergonomic keyboards in multiple sclerosis ranges from 19% to 34%. In
• Voice-activated computer programs order to slower the progression of multiple clerosis
patients are put on various medications such as
• Telecommuting or home-based employment.
Baclofen, Dantrolene, Betaseron and Avonex etc.
Occupational therapy focuses on learning strategies these could cause changes in mood in either
for managing daily life, based on the person's direction. Also, as some of them are on steroids, it
physical, social and psychological needs. could also lead to periods of hyperactivity and
euphoria, which could be followed by a low feeling
PSYCHOLOGICAL ASPECT IN period. Also certain steroids can cause or worsen
MULTIPLE SCLEROSIS depression. Causes of depression in multiple
sclerosis patients is due to psychosocial challenges,
Multiple sclerosis is an extremely unpredictable and maladaptive coping, brain lesions resulting from
complex disease as no two patients suffering from multiple sclerosis disease in process and immune
the disease have the same psychological effect. deregulation associated with multiple sclerosis
Numbness, fatigue, pain, visual disturbance, exacerbation.
parenthesis, muscle weakness, muscle spasm - these
troubling symptoms are invisible to other people Studies suggest that 13% patients with multiple
as compared to a cane or a wheelchair. This makes sclerosis also have bipolar disorder. Bipolar may
multiple sclerosis like an iceberg with a huge be an initial symptom of multiple sclerosis,
hidden part that often causes intense mental preceding other neurological symptoms. While
anguish. manic episodes in multiple sclerosis may be
precipitated by steroid therapy, there is increasing
The significant stress of being diagnosed with evidence that affective disturbance may be due to
Multiple Sclerosis 343

organic changes in the brain. Both euphoria and functioning; typically causing deficits in speed of
pathologic weeping or laughing in multiple information processing, attention, executive
sclerosis patients may be due to lack of emotional functions, verbal fluency and memory were clearly
control in multiple sclerosis. associated with less brain volume, and with higher
lesion loads, in particular at frontal and occipital
Treating depression in MS is often rewarding and
areas. There has been research that between 45%
drugs in the Specific Serotonin Reuptake Inhibitor
to 65% of all people, with multiple sclerosis
(SSRI) group are probably safest. Common side
experience problems with memory, attention,
effects, usually transient, include nausea, sexual
problem -solving and other cognitive functions,
dysfunction and gastrointestinal disturbance.
which may vary from one patient to another.
Withdrawal from these drugs should be gradual,
especially with paroxetine (Seroxat). Problems in neurocognitive functioning in multiple
sclerosis are mainly modulated by speed and
Euphoria: This is a mental condition where there
stability of speed processing; in particular when
is a feeling of happiness, confidence, or well-being
attention-demanding controlled information
sometimes exaggerated in pathological states as
processing is required. Cognitive difficulties can be
mania. It can often lead to having unrealistic
particularly stressful for people because they are
expectations and thought process. It is seen that
changes that nobody sees. Understanding the type
patients in this state lack insight. Cognitive
and the source of cognitive problems is the first step
problems like inattention, disturbed thinking, etc
for patients and their family members to begin
usually trigger euphoria.
coping with these symptoms effectively. The
Emotional Liability: Emotional liability means that cognitive issues may vary from patient to patient.
the patient may easily burst into tears, both at The range of cognitive dysfunctions which can be
happy and sad events or might become suddenly associated with multiple sclerosis:
very angry or aggressive which may be
Cognitive Fatigue: Research shows that patients
inappropriate or exaggerated for the situation. This
with multiple sclerosis even if they have no other
is different from pathological crying or laughter.
cognitive problems, they can get fatigued from
In patients with multiple sclerosis, the symptom of
doing tasks that are mentally challenging work,
emotional liability may vary from mild to severe.
even if they aren't exerting themselves physically.
Lack of Insight: Patients with multiple sclerosis Patients with multiple sclerosis once they are
may lack insight about their condition and this cognitively fatigued they are more likely to work
could cause a lot of problems. Lack of insight may slowly and make errors than people who do not
lead to problems in day-care or rehabilitation. For suffer from multiple sclerosis.
example, when a patient suffering from multiple
Attention and Concentration: Problem arises when
sclerosis may try to regain walking, when
patients suffering from multiple sclerosis are asked
everybody around would be aware that he or she
to focus on more than one thing. Often patients as
may not be able to walk again. This is when the
their disease progresses they feel that they can work
caregivers and the therapists may face a problem
on one thing at a time, in a quiet and distraction
as they would be in a soup whether to encourage
free place whereas, before they could multi-task
or discourage the patient.
quiet easily.
There could be a number of emotional aspects that
Processing Speed: Cognitive slowing in individuals
a patient may go through like anger, isolation, loss
with multiple sclerosis (MS) has been documented
of control and dependence, self doubt and dislike
by numerous studies employing explicitly timed
which is discussed in the chapter of psychological
measures in which speed of responding is an
rehabilitation.
obvious focus of task performance. Often the
patients experience an overall slowing the way they
Cognitive Deficits:
process information as compared to before.
As multiple sclerosis is a disease that causes damage
Memory: Memory is a complex neuropsychological
to nerve fibers in the brain and spinal cord because
function. There are two separate systems for
of which demyelination occurs in the brains of most
memory:
patients with multiple sclerosis. A
neuropsychological point of view suggests multiple 1. Procedural memory is the memory for how
sclerosis often affects higher order cognitive to do things. Procedural memory guides the
344 Neuro-Rehabilitation : A multi disciplinary approach

processes we perform and most frequently to dealing with cognitive changes is to recognize
resides below the level of conscious awareness. that they have been changes. Usually patients with
When needed, procedural memories are multiple sclerosis do not take these changes quiet
automatically retrieved and utilized for the seriously. Adequate assessments of cognitive
execution of the integrated procedures deficits need to be carried out by a psychologist or
involved in both cognitive and motor skills. a neuropsychologist with the help of standardized
In multiple sclerosis, this almost always neuropsychological tests. It is also important to
remains intact and unaffected, so although you evaluate depression, stress, anxiety which may be
may forget to do something, you will not a contributing factor to cognitive problems with the
forget how to do it. help of scales like Beck's Depression Inventory,
Hamilton Anxiety Rating Scale, etc. An overall
2. Semantic memory needs several steps to occur
assessment would help the psychologist formulate
before a memory can be stored or recalled, and
a good rehabilitation plan like vocational
interruption at any stage, such as may be
rehabilitation, improved family life, cognitive
caused by the inability to concentrate, can
rehabilitation, etc.
interfere with the ability to store or retrieve
information. This memory can be affected in
multiple sclerosis. It's often seen that patients
Treatment for Cognitive Deficits:
with multiple sclerosis have trouble recalling There is as such no "pill" which could improve the
recent everyday events since the diagnosis. cognitive functions in multiple sclerosis. However,
Some patients could also experience difficulty there is a class of drugs called potassium channel
recalling things that they see but have no blockers (4-aminopyridine (4-AP and 3, 4
trouble recalling things that they have heard, diaminopyridine) have been studied for several
while others can remember things that they years. The potassium channel blockers increase the
see with clarity but are unable to remember speed of nerve conduction in demyelinated nerve
what they have heard. In very advanced cases, fibers, suggesting that they might improve
memory problems could be as severe as to neurologic function and cognition. However, these
completely disrupting the learning and recall drugs lead to high risk for seizures as they speed
process. up the nerve conduction. A recent study of 69
Language: In patients with multiple sclerosis they patients suffering from multiple sclerosis with
could be issues related to language fluency. memory deficits showed that Aricept had modest
Especially, there could be difficulty in finding benefits for verbal memory i.e. the ability to
words i.e. accidentally saying the wrong word and remember a list of words. Five drugs are now
problems with naming i.e. problems with recalling approved by FDA of which Avonex has
uncommon words or the names of acquaintances. demonstrated to be the most positive benefit on
Patients with multiple sclerosis can also have cognition. However, none of the medications have
problems with dysphagia or dysarthria, which refer shown dramatic benefits, on slowing the
to problems coordinating the movements of the lips, progression of cognitive dysfunctions or reversing
mouth, tongue, and throat. They also suffer from the effects.
slurred speech or physical difficulty saying certain Cognitive Rehabilitation: Cognitive rehabilitation
words or syllables, without any cognitive problems is a systematic program designed to improve
at all. cognitive functions. There are a few stratergies
Problem Solving: At times, people with multiple mentioned which are used during a cognitive
sclerosis have difficulty solving problems in new rehabilitation session:
situations or they may have difficulty making • If the patients complain of weak memory then
decisions because it is hard to identify which of a substitute it for an organizer for example
number of choices is the best. maintain a dairy where one needs to write
down everything down, or maintain planners
Visual Spatial Skills: Patients may find themselves
and set up sections for appointments,
getting lost more frequently, losing items, or having
addresses, phone numbers, driving directions,
trouble understanding visual-spatial information
etc.
like maps, diagrams, and charts.
• Maintain particular place for everything as this
Evaluating the Cognitive Changes: The first step would help to avoid misplacing things.
Multiple Sclerosis 345

• Try to focus more attention on things that are • Low self esteem
important and keep repeating it to yourself so
• Fertility and pregnancy issues
that it registers in the long term memory.
Meditate regularly to improve attention and • Genetic factors
concentration. How do couples regain their sex life?
• Work slowly and the focus should be on 1. Communication: The first step is to accept that
accuracy rather than speed as a patient with there is a problem and that to verbalize it with
multiple sclerosis suffers from poor your partner or the person you are able to
processing. confide in. By being open about the situation
would reduce the level of embarrassment and
Sexual Issues: doubt about the situation.
Sexuality is an integral part for each one of us and 2. Consulting a Doctor: Discuss the side effects
it has an impact on our identity, personality, of medication that one takes for multiple
interpersonal relationship and our life span. sclerosis and medications taken for muscle
Multiple sclerosis is a disease which can target any relaxants and pain as this could lead to being
part of the nervous system like if it affects the nerves intimate more readily.
servicing the reproductive organs, which in turn
could lead to changes in sexual functioning. 3. Alternatives: Doctors could suggest various
Multiple sclerosis can cause problems for the alternatives.
patients on two aspects: neurologically and 4. Sexual Counseling is necessary as it helps the
psychologically. Neurologically, the sexual couple to come in terms with the challenges
excitement and response arises in the central of multiple sclerosis and also come up with
nervous system which is normally affected. While different alternatives. Sexual Counseling is
the brain sends messages to the sex organs through further discussed in the psychological
spinal cord but due to multiple sclerosis the affected rehabilitation chapter.
area is the nerve pathways which is caused by
demyelination. This can cause impairment in sexual Family Issues:
functioning like:
Families have their own style of functioning, coping
• Loss of libido or sexual interest and communicating. If anyone the family suffers
• Slower arousal time from multiple sclerosis eventually the whole family
• Difficulty masturbating would be involved physically, psychologically and
financially. It can lead to a lot of stress and challenge
• Changes in genital sensation to the coping mechanisms of everyone in the family.
• Decreased vaginal lubrication and muscle tone A person who was once there for the family as
• Difficulty in erectile functioning and financial, social and psychological support to the
ejaculation family members, after diagnosis may have to reduce
• Difficulty attaining climax working hours which may lead to reduces family
income. Also, the person who may have been very
Psychologically, the patient may go through active socially may now avoid social functions and
problems like bowel and bladder incontinence, interaction and may also need emotional support
weakness, fatigue, decreased sexual desire and loss as a result of his deteriorating condition. Family
of interest. Also, as physical contact and sexual members may be expected to take on additional
positioning may become uncomfortable due to responsibilities of the family member who is
sensory changes this could lead to further impact suffering from multiple sclerosis. This leads to
psychologically like: change in functioning and family role, also people
• Depression, stress or anxiety might feel that this change would be temporary
which is not true, it would be a lifelong change and
• Performance anxiety
responsibility.
• Altered body image or sexual self-image
Often the family members may stop spending time
• Fear and worries about the future of the on leisure activities that they would enjoy doing
relationship previously. Family members should spend some
346 Neuro-Rehabilitation : A multi disciplinary approach

time on their hobbies or what they enjoy doing, Hamilton's Anxiety Rating Scale - To assess anxiety
otherwise this would lead to caregivers burnout.
Wechsler's Memory Scale - To assess cognition
Multiple sclerosis can be very challenging to a
Psychological Intervention for Multiple Sclerosis:
married life as when the patient is diagnosed as
The below listed are discussed in detail in
multiple sclerosis the couple is usually planning
psychological rehabilitation chapter.
and working towards future goals. The couple
needs to have a strong bond which would help • Individualized Psychotherapy: This allows
them to come out with effective coping the patient with multiple sclerosis to deal with
mechanisms. As the course, of the disease could be emotional difficulties, lack of motivation,
very unpredictable with high chances of relapse. negative impact of the disability and inability
The best possible way to deal with the situation is to handle change in functional skills typical of
to forget the old ways of doing things and adapting this condition.
to the progression of the disease and finding out
alternatives to old ways of doing things. Work out • Group Therapy: A new study at The
a specific plan for determining how practical needs University of Nottingham has revealed that
are handled and who will be responsible for what, offering emotional support to Multiple
when. Patience, compromise and enlisting help Sclerosis sufferers through group therapy
from other family members could prove to ease out sessions could improve their quality of life.
the situation. • Sexual Counseling
Hiding the diagnosis from the children would not • Cognitive Rehabilitation
be an idle way to deal with the situation as the
children would sense something wrong. Also, they • Family Therapy
would not be able to understand the behaviour of
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Multiple Sclerosis 349

Section 3
Muscle and Nerve Related
Disorders
Ch.9 Muscular Dystrophy
Dr. Nancy Thomas, [Link] (Neuro), Dr. Hema Biju, MOTH (Neuro),
Ms. Akshata Shetty, M.A. (Clinical Psychologist)

Sir Charles Bell, in 1830 was the first one to note which causes series of events resulting in muscle
the progressive weakness seen in boys which was contraction.
studied further by another scientist who reported
During contraction and relaxation of muscle,
generalized weakness, muscle damage &
muscle membranes are protected by group of
replacement of damaged muscle tissue with fat&
protein named dystrophin- glycoprotein complex
connective tissue on two brothers and was thought
which are inbuild in membranes which contains
to be tuberculosis. By 1850s, these boys grew
protein creatine kinase needed for the chemical
progressively weaker, unable to walk & later on
reactions which produces energy for muscle
died at an early age and made a marked impression
contractions. When this protective membrane gets
on medical field. Later in 19th century French
damaged, there is a leakage of protein creatine
neurologist Guillaume Benjamin Amand Duchenne
kinase leading to excess calcium influx in muscle
did pioneering work & gave a comprehensive
fibers, which causes further harm and eventually
account of 13 boys with the most common and
death thereby resulting to progressive muscle
severe form of the disease( which now carries his
degeneration.
name-Duchenne muscular dystrophy) & revealed
of the disease having more than one form, and that All the muscular dystrophies are inherited and
these diseases are affected in people of either sex result from different mutations in the gigantic gene
and of all ages. Duchenne was the first to do a that encodes dystrophin and which is required to
biopsy to obtain tissue from a living patient for maintain muscle integrity. The body cells don't
microscopic examination & used his biopsy needle work properly when a protein is altered or
on boys with DMD and concluded to have this produced in insufficient quantity ( or sometimes
disease of muscle origin. missing completely. Many cases of MD occur
spontaneous mutations that are not found in the
genes of either parent, and this defect can be passed
to next generation. There are 79 exons in DMD gene
spanning at least 2,300 kb. Deletions cause
deficiencies in 1 or more of these as a result of which
mental retardation and cardiomyopathy sometimes
accompany DMD, which is seen in clinical
presentation depending on what the deletions was
removed from the dystrophin gene.
Dystrophin protein is found in muscle fibre
membrane, acting like a spring. It joins the
membrane actin filiments. The protein is rod shaped
Guillaume Benjamin Amand Duchenne
around 150nm in length, 3684 amino acids long,
Muscles are composed of many muscle fibers 427kDa molecule weight. It is hydrophobic (does
which are separated by each other by connective not like water). Conformation is alpha-helical,
tissues called endomysium and arranged in bundles allowing protein to act as a shock absorber,
called fasciculues where individual fibers are preventing overstress. Dystrophin links actin
arranged parallel to each other. Each fasisculus has (cytoskeleton) and dystroglycans of the muscle cell
a outer connective tissues membrane called plasma membrane, known as the sarcolemma
perimysium and muscle has a whole consists of all (extracellular). Dystrophin functions in two ways;
these fasisculues together with outer layer called mechanical stabilisation and regulated calcium
epimysium levels. Cycles of degenerating and regenerating
When an impulse travels from brain down to occur in presence of dystrophin deficiency. It's a
neuromuscular junction through the peripheral mystery as how these cycles are related to altered
nerves, a chemical named acetylcholine is released strength & stability of cell membrane, increased
cell permeability of cell membrane, altered
352 Neuro-Rehabilitation : A multi disciplinary approach

Dystrophin Gene

Dystrophin Protein
Muscular Dystrophy 353

mechanism of Ca+2 regulation, calpain activity, 3. X-linked (or sex-linked) recessive inheritance
ischaemia, mast cell infiltration, satellite cell occurs when a mother carries the affected gene
function & proliferative potential, basic fibroblast on one of her two X chromosomes and passes
growth factor activity, activity of platelet derived it to her son (males always inherit an X
growth factor receptors, endomysical & perimysical chromosome from their mother and a Y
fibrosis& finally replacement of muscles by chromosome from their father, while
fibrofatty connective tissue. daughters inherit an X chromosome from each
parent). Sons of carrier mothers have a 50
In Muscular dystrophy, integrity of the muscles
percent chance of inheriting the disorder.
fibers are predominately affected leading to muscle
Daughters also have a 50 percent chance of
degeneration, progressive weakness, fiber death,
inheriting the defective gene but usually are
fiber branching and splitting, phagocytosis and at
not affected, since the healthy X chromosome
times leading to chronic or permanent shortening
they receive from their father can offset the
of tendons and muscles which results in overall
faulty one received from their mother.
decreased or absent in muscle strength and tendon
Affected fathers cannot pass an X-linked
reflexes due to replacement of muscle by connective
disorder to their sons but their daughters will
tissue and fat.
be carriers of that disorder. Carrier females
(Genes are like blueprints containing coded occasionally can exhibit milder symptoms of
messages that determine a person's characteristics MD.
or traits; arranged along 23 rod-like pairs of
chromosomes,*- with one half of each pair being
inherited from each parent. Each half of a
chromomsome pair is similar to the other, except
for one pair, which determines the sex of the
individual. )
Muscular dystrophies can be inherited in three
ways:
1. Autosomal dominant inheritance occurs when
a child receives a normal gene from one parent
and a defective gene from the other parent.
Autosomal means the genetic mutation can
occur on any of the 22 non-sex chromosomes
in each of the body's cells. Dominant means
only one parent needs to pass along the
abnormal gene in order to produce the Types of Dystrophies:
disorder. In families where one parent carries There are nine types of the muscular dystrophies
a defective gene, each child has a 50 percent which are classified on the basis of the :
chance of inheriting the gene and therefore the
disorder. Males and females are equally at risk 1. Extent and distribution of muscle weakness.
and the severity of the disorder can differ from 2. Age of onset
person to person.
3. Rate of progression.
2. Autosomal recessive inheritance means that
both parents must carry and pass on the faulty 4. Severity of symptoms
gene. The parents each have one defective gene 5. Family history (including any pattern of
but are not affected by the disorder. Children inheritance).
in these families have a 25 percent chance of
Some forms of MD appears in infancy or childhood
inheriting both copies of the defective gene and
while others appear in middle age or later. Even
a 50 percent chance of inheriting one gene and
though incidence rates and severity varies, each of
therefore becoming a carrier, able to pass along
these dystrophies causes progressive skeletal
the defect to their children. Children of either
muscle deterioration, where some types affect
sex can be affected by this pattern of
cardiac muscle also.
inheritance.
354 Neuro-Rehabilitation : A multi disciplinary approach

muscle strength and size) caused by degenerating


muscle fibers before spreading into the upper arms.
Other symptoms include loss of some reflexes, a
waddling gait, frequent falls and clumsiness in
movements especially while running or jumping,
difficulty when rising from squatting position, low
chair, sitting or lying position or while climbing
stairs, early fatigue, postural imbalance, impaired
breathing, lung weakness, and cardiomyopathy
(heart muscle weakness that interferes with
pumping ability).The muscles are wasted and are
replaced by an accumulation of fat and connective
tissue, causing them to look larger and healthier
than they actually are hence called
pseudohypertrophy). As the disease progresses, the
respiratory muscles (diaphragm) which assist in
breathing and coughing weakens resulting into
breathing difficulties, respiratory infections, and
Infancy or childhood onset Muscular dystrophy
swallowing problems. Bone thinning and scoliosis
are classified into four types:
are common complications with slight impairment
1. Duchenne Muscular dystrophy. in cognition. Between ages 3 and 6, children may
2. Becker Muscular dystrophy. show brief periods of physical improvement
followed by progressive muscle degeneration and
3. Congential Muscle dystrophy. becomes wheelchair-bound by age 12 and usually
4. Emery- Dreifuss Muscular dystrophy. die in their late teens or early twenties from
progressive weakness of the heart muscle,
1. Duchenne Muscular Dystrophy (DMD) is the
respiratory complications, or infection.
most common and severe childhood form of
muscular dystrophy, affecting one in 3,500 male 2. Becker Muscular Dystrophy is closely related
births. DMD results from an abnormal gene which to DMD with less severity. It is slowly progressive
is seen on X-chromosome at band Xp21, which X-linked recessive disorder with partial but
encodes for dystrophin, a427-kD cytoskeletal insufficient function of the protein dystrophin. The
protein in membrane. Since it has X-linked onset of weakness is manifested between the age
recessive inheritance, it primarily affects boys, 10 and 15 years, and patients generally live upto
whereas girls and women who carry the defective the middle age or later. The rate of progression,
gene may show some symptoms. About one-third symmetrical pattern, muscle atrophy and weakness
of the cases reflect new mutations and the rest have varies greatly among affected individuals. Most of
family history where sisters of boys with DMD have the patients are able to walk until they are in their
a 50 percent chance of carrying the defective gene. mid-thirties or later, while others are unable to walk
past their teens where as some doesnt need any
In most of cases, DNA analysis is normal and
wheelchair. As in Duchenne MD, muscle weakness
diagnosis gets confirmed by immunoblotting or
in Becker MD is typically noticed first in proximal
immunostaining techniques where complete
muscles like the upper arms and shoulders, thighs
absence of dystrophin from muscle tissue is seen.
and pelvis.
Pathological tests show creatine Kinase (CK)
elevated more than 100 times in early stages of Early symptoms of Becker MD include tip toe
disease and these levels decrease over a period of walking, frequent falls, and difficulty rising from
time with loss of muscle mass. Elevated CK is the floor. Calf muscles may appear large and
evident at birth long before symptoms are evident healthy as damaged muscle fibers are replaced by
fat, and muscle activity at times cause cramps in
DMD usually becomes apparent when a child
some individual. Cardiac and mental impairments
begins to walk with onset of muscle weakness
are not as severe as in DMD.
typically seen in between ages 2 and 5. Progressive
weakness and muscle wasting is seen in proximal 3. Congenital Muscular Dystrophy refers to a
group of muscles in lowerlimbs (a decrease in group of autosomal recessive muscular dystrophies
Muscular Dystrophy 355

that are either present at birth or become evident Onset of Emery-Dreifuss MD is usually apparent
before age 2. They affect both boys and girls with by age 10, but symptoms can appear as late as the
hypotonic type of [Link] degree and mid-twenties. This disease causes slow but
progression of muscle weakness and degeneration progressive wasting of the upper arm and lower
vary with the type of disorder. Weakness may be leg muscles and symmetric weakness. Early onset
first noted when children show delayed milestones of contractures before the onset of any significant
in motor function and muscle control. Muscle weakness is classic presentation of this disorder.
degeneration may be mild or severe and is restricted Contractures may cause elbows to become locked
primarily to skeletal muscle. Most of the patients in a flexed position. The entire spine may become
has no sitting or standing balance and some may rigid as the disease progresses. Other symptoms
never achieved walking ability. include shoulder deterioration, toe-walking, and
mild facial weakness. Serum creatine kinase levels
There are three groups of congenital MD:
may be moderately elevated. Nearly all Emery-
• merosin-negative disorders, where the protein Dreifuss MD patients have some form of heart
merosin (found in the connective tissue that problem by age 30, often requiring a pacemaker or
surrounds muscle fibers) is missing; other assistive device. Female carriers of the
• merosin-positive disorders, in which merosin disorder often have cardiac complications without
is present but other needed proteins are muscle weakness. Death results in mid-adulthood
missing; and due to progressive pulmonary or cardiac failure.

• neuronal migration disorders, in which very Youth/adolescent-onset muscular dystrophies are


early in the development of the fetal nervous classified in two types:
system the migration of nerve cells (neurons) 5. Facioscapulohumeral MD (FSHD) is an
to their proper location is disrupted. autosomal dominant with progressive weakness
Defects in the protein merosin is mostly the main initially seen in muscles of the face (facio), shoulders
cause seen nearly in half of all cases of congenital (scapulo), and upper arms (humera). It is also
MD. known as Landouzy-Dejerine disease. Life
expectancy is normal, but some individuals become
Contractures (chronic shortening of muscles or severely disabled. Disease progression is typically
tendons around joints, which prevents the joints very slow, with intermittent spurts of rapid muscle
from moving freely), scoliosis, respiratory and deterioration. Onset is usually in 15 years of age
swallowing difficulties, and foot deformities are the but may occur as late as age 40. Muscles around
complications seen in congenital muscular the eyes and mouth are often affected first, followed
dystrophy. Some patients have normal intellectual by weakness around the lower shoulders and chest.
development while others become severely A particular pattern of muscle wasting causes the
impaired depending upon central nervous system shoulders to appear to be slanted and the shoulder
affection, resulting in vision and speech problems, blades to appear winged. Although reflexes are
seizures, and structural changes in the impaired only at the biceps and triceps, muscles in
[Link] failure is also seen due to the lower extremities may also become weakened.
weakness in diaphragm. Some children die in Changes in facial appearance may include the
infancy while others may live into adulthood with development of a crooked smile, a pouting look,
only minimal disability. flattened facial features, or a mask-like appearance.
4. Emery-Dreifuss Muscular dystrophy is X- Some patients cannot pucker their lips or whistle
linked recessive primarily affects boys with and may have difficulty swallowing, chewing, or
classic triads of: speaking. Other symptoms may include hearing
loss (particularly at high frequencies) and lordosis,
1. Early contractures especially of elbows,
an abnormal swayback curve in the spine.
Achilles tendon, and posterior cervical
Contractures are rare. Some FSHD patients feel
muscles.
severe pain in the affected limb. Cardiac muscles
2. Cardiac conduction defects are not affected, and the pelvic girdle is rarely
significantly involved. An infant-onset form of
3. Slowly progressive weakness and
FSHD can also cause retinal disease and some
atrophy in a humeroperoneal
hearing loss.
distribution.
356 Neuro-Rehabilitation : A multi disciplinary approach

6. Limb-girdle MD refers to inherited conditions difficulty in performing fine motor activity with
marked by progressive loss of muscle bulk and difficulty in straightening the fingers. As leg
symmetrical weakening of proximal muscles, muscles become affected, walking and climbing
primarily those in the shoulders and around the stairs become difficult and some patients may be
hips. At least three forms of autosomal dominant unable to hop or stand on their heels., The cause in
limb-girdle MD (known as type 1) and eight forms one of distal MD is a muscle membrane protein
of autosomal recessive limb-girdle MD (known as complex called dysferlin which is suppose to be
type 2) have been identified. Some autosomal lacking.
recessive forms of the disorder are due to a
Although distal MD is primarily an autosomal
deficiency of any of four dystrophin-glycoprotein
dominant disorder, autosomal recessive forms have
complex proteins called the sarcoglycans.
also been reported in young adults. Symptoms are
The recessive limb-girdle muscular dystrophies similar to those of DMD but with a different pattern
occur more frequently than the dominant forms, of muscle damage. An infantile-onset form of
usually begin in childhood or the teenage years, and autosomal recessive distal MD has also been
show dramatically increased levels of serum reported with slow progressive weakness noticed
creatine kinase. The dominant limb-girdle muscular around age 1, when the child begins to walk, and
dystrophies usually begin in adulthood. Rate of continues to progress with weakness throughout
progression disease depends upon earlier his adult life.
appearance of clinical signs affecting both the
8. Myotonic MD, also known as Steinert's
genders. Some forms of the disease progress
disease and dystrophia myotonica, is the most
rapidly, resulting in serious muscle damage and
common adult form of Muscular Dystrophy,
loss of the ability to walk, while others have very
affecting both men and women with autosomal
slow progression over the years causing minimal
dominant inheritance. Myotonia, or an inability to
disability, leading to normal life expectancy. At
relax muscles following a sudden contraction, is
times, the disorder appears to halt temporarily in
only seen in myotonic muscular dystrophy. People
some cases, but later symptoms resume back.
with myotonic MD has long life expectancy, with
Weakness is typically noticed first seen in proximal variable but slowly progressive disability. Onset of
group of lower limbs affecting around the hips disease is seen between ages 20 and 30, but can
before spreading to the shoulders, legs, and neck. develop earlier. Myotonic MD affects the central
They have waddling type of gait and has difficulty nervous system and other body systems, including
in getting up from squatting position or low chair, the heart, adrenal glands and thyroid, eyes, and
climbing stairs, or carrying heavy objects. They gastrointestinal tract. Muscles in the face and the
have a history of frequent falls andare unable to front of the neck are the first to show weakness and
run. Contractures are rare but can develop in the may produce a haggard, "hatchet" face and a thin,
back muscles, which gives them the appearance of swan-like neck. Wasting and weakness can be
a rigid spine. Proximal reflexes are also affected. apprecipated in forearm muscles with other
Cardiomyopathy and respiratory complications symptoms including cardiac complications,
have been seen in limb girdle muscular dystrophy difficulty swallowing, ptosis, cataracts, poor vision,
with intelligence remaining normal. They become early frontal baldness, weight loss, impotence,
severely disabled within 20 years of disease onset. testicular atrophy, mild mental impairment, and
increased sweating. Patients may also have
Adulthood onset of Muscular dystrophy are
tendency to feel drowsy and have an excess need
classified into three types:
to sleep.
7. Distal MD, also called distal myopathy,
Females may have irregular menstrual periods and
consists of a group of at least six specific muscle
may be infertile. The disease occurs earlier and is
diseases that primarily affect distal muscles like in
more severe in successive generations. A childhood
the forearms, hands, lower legs, and feet. Onset of
form of myotonic MD appears at age between 5
distal MD is between the ages of 40 and 60 years
and 10 years. with symptoms of generalized
affecting both genders. Distal dystrophies are less
muscle weakness particularly in the face and distal
severe, with slow progression, and involve only
muscles, lack of muscle tone, and mental
fewer muscles. Distal MD can affect the heart and
impairment.
respiratory muscles, and patients may eventually
require the use of a ventilator. Patients finds
Muscular Dystrophy 357

An expectant mother with myotonic MD can give Stage 1: Early Pre-symptomatic :


birth to an infant with a rare congenital form of the During Presymptomatic stage, diagnosis remained
disorder. Symptoms at birth may include difficulty unnoticed unless there is some positive or strong
swallowing or sucking, impaired breathing, family history or unless for some reasons any blood
absence of reflexes, skeletal deformities (such as investigations are done. Symptoms of delay in early
club feet), and noticeable muscle weakness, developmental milestones like crawling, walking
especially in the face. Children may also experience and in speech remained unnoticed with child been
mental impairment and delayed motor flaccid and less physically active than normal
development. This severe infantile form of children. Given a new diagnosis like Muscular
myotonic MD occurs almost exclusively in children dystrophy shatters the families and there comes
who have inherited the defective gene from their sensitive counselling and support which can
mother, who may not know she is a carrier of the influences the family's ability to manage the child
disease. in the future.
The inherited gene defect that causes myotonic MD
is an abnormally long repetition of a three-letter Stage 2 : Early Ambulatory (Walking)
"word" in the genetic code. In unaffected people, Boys shows classic traids like Gower's Manoeuvre
the word is repeated a number of times, but in in which the child gets up from the floor by using
people with myotonic MD, it is repeated many more his arms to crawl up his own legs,Has waddling
times. This triplet repeat gets longer with each type of gait and has toe walking with difficulty in
successive generation. The triplet repeat mechanism activities such as running, hopping and climbing
has now been implicated in at least 15 other stairs.
disorders, including Huntington's disease and the
spinocerebellar ataxias.
9. Oculopharyngeal MD (OPMD) generally
begins in a fourth or fifth decade of life and affects
both men and women. The disease is most common
in families of French-Canadian descent and among
Hispanic residents of northern New Mexico.
Patients complaints of symptoms of drooping
eyelids, followed by weakness in the facial and
pharyngeal muscles in the throat, causing difficulty
in swallowing. The tongue may atrophy and
changes to the voice may occur. Eyelids may droop
so dramatically that they compensate by tilting back
their heads. At times they have double vision and
problems with upper gaze, while others may have
retinitis pigmentosa (progressive degeneration of
the retina that affects night vision and peripheral
vision) and cardiac irregularities. Muscle weakness
and wasting in the neck and shoulder region is Stage 3: Late Ambulatory (going off feet )
predominately seen with limb muscles also being Mobility becomes labourous with difficulty in
affected. Persons with OPMD may find it difficult climbing stairs and walking distances. They present
to walk, climb stairs, kneel, or bend and with those with frequent falls and have difficulty or unable to
persons most severely affected will eventually lose getting up on their feet without assistance. Fractures
the ability to walk. are the main cause because of frequent falls
resulting into loss of mobility. Also early
Stages of Muscular dystrophy: involvement of scapular stabilizers results in
As some muscular dystrophies are progressive in decrease in arms and hands movements during
their disease course while others are very slow; reachout activities. Classic test of scapular stability
these disease can be divided into stages according called Meryon sign where child slips from
signs and the pattern of involvement. examiner's grip as the child is being lifted from
under the arms.
358 Neuro-Rehabilitation : A multi disciplinary approach

Life span of DMD.


Wrong pattern of lifting.
Functional Transitions in patient with Muscular
Dystrophy.
1. Walks with mild waddling gait with lordosis.
Running becomes strenuous but can ascend,
descend steps, curbs.
2. Walking with moderate waddling gait with
lordosis. Running becomes impossible.
Climbing stairs and curbs becomes difficult.
Uses Gower's maneuver while getting up from
floor but can rises from chair independently.
3. Walks with moderately severe waddling gait
and lordosis. Can raise himself from chair
independently but ascending descending
curbs or stairs or rise from floor becomes
Meryon's sign. totally dependent.
4. Walks with assistance or with bilateral KAFOs.
Stage 4 : Early Non-Ambulatory Can have surgical release for contractures.
Boys are able to sit up of their own independently Can propel manual chair slowly and
but are unable to walk. In this stage, wheelchair wheelchair propulsion for community
becomes a main aid for propulsion where they can mobility. Independent in bed and self -care
self propel for some period of time. Good postural even though some help is needed in dressing
management is required as most of time they will and bathing because of time constraints.
be maintaining sitting posture. Since there is poor 5. Unable to walk independently but can bear
trunk control because of muscle imbalance, there and shift weight to walk with orthoses and can
is increased risk of developing scoliosis and transfers from wheelchair independently. Can
management of same is mandatory. propel self in manual chair but with limited
endurance whereas motorized wheelchair
Stage 5: Late Non-Ambulatory becomes more functional. Independent in self
Postural imbalance and malalignment is the main care with transfer assist for bath or shower.
concern seen in this stage with decrease in upper 6. Independence in motorized wheelchair but
limb function which results in decline on requires trunk support or orthosis. Assistance
performing ADLS like dressing, feeding and is needed in bed and with major dressing, is
difficulties with oro-motor function can lead to independent for performing self -grooming
nutritional problems. Because of no mobility but dependent for toileting and bathing.
happening, there is impairment seen in respiratory
7. Independence in motorized wheelchair but
and cardiac functions and require interventions for
need to recline intermittently while in chair.
same.
Needs assistance for hygiene and most self
Stage 6: Palliative Care/ End of Life. care requiring proximal upper limb control.
Muscular Dystrophy 359

8. Use both hands for single hand activities, can Investigations:


perform simple table- level hand activities, and Laboratory tests such as:
can perform self-feeding with arm support.
1. Blood and urine tests can help in identifying
9. Can sit in wheelchair only with trunk support defective genes which are responsible for
and intermittent reclining or transfer to supine identify specific neuromuscular disorders. For
position. May require nighttime ventilatory example:. Serum aldolase level, an enzyme
support or intermittent daytime PPV. Some involved in the breakdown of glucose, is
hand control can be achieved if arms are measured to confirm a diagnosis of skeletal
supported and needs help for turning at night. muscle disease.
10. Totally dependent and cannot tolerate upright Creatine kinase is an enzyme that leaks out of
position and needs home ventilatory support damaged muscle and its elevated levels may
if needed for prolonged ventilation, then indicate muscle damage, before physical
tracheostomy is needed. symptoms become apparent in the early stages
of Duchenne and Becker MD. Testing can help
Diagnosis: in identifying whether a young woman is a
To diagnose any muscle disease thoroughly medical carrier of the disorder or not.
and a complete family history should be reviewed
• Myoglobin is an oxygen-binding protein found
to determine whether it is secondary to a disease
in cardiac and skeletal muscle cells and
affecting other tissues or is an inherited condition
measured when injury or disease in skeletal
or it is result of prior surgery, exposure to toxins,
muscle is suspected. High blood levels of
or current medications that may affect the patient's
myoglobin are found in patients with MD.
functional status.
• Polymerase chain reaction (PCR) /Molecular
The first suspicions are usually raised by one of the
diagnosis or Genetic testing: can detect
following three signs (even when there is no history
mutations in the dystrophin gene method for
of DMD in the family):
generating and analyzing multiple copies of a
1. Cluminess in Movements : fragment of DNA.
Boys who have DMD walk slower and are less • Serum electrophoresis is a test to determine
active than other boys of their age group. They quantities of various proteins in a person's
have pseudohypertrophy of calf muscles and DNA.
have difficulty in running, jumping or • Electron microscopy: can identify changes in
climbing stairs. They have frequent falls and subcellular components of muscle fibers that
have a tendency of toe walking. They may characterize cell death, mutations in muscle
also have a speech delay. The classic signs cell mitochondria, and an increase in
likethe "Gowers'" manoeuvre or sign, where connective tissue seen in muscle diseases such
the boy uses his hands and arms to "walk" up as MD.
his body in order to push himself to an upright
position. This is due to weakness in the hips Changes in muscle fibers that are identified in a
and thigh muscles and Meryon sign in which rare form of distal MD using an electron
the child slips from examiner's grip as the chld microscope.
is being lfted from under the arms. Exercise tests can detect elevated rates of certain
2. High levels of the muscle protein creatine chemicals and are used to determine the nature of
kinase (CK) in a blood test : the MD or other muscle disorder following exercise.
It also assess muscle strength by measuring muscle
Serum creatine kinase is elevated more than function against gravity and detecting slight muscle
100 times normal in early stages of weakness. If weakness in respiratory muscles is
[Link] CK is evident at birth long suspected, respiratory capacity may be measured
before symptoms are evident and CK levels by having the patient take a deep breath and count
decrease over time with loss of muscle mass. slowly while exhaling if respiratory muscles are
• Some have delay in their speech involved.
development also. Genetic testing identifies genes which are
360 Neuro-Rehabilitation : A multi disciplinary approach

responsible for the cause or associated with Neurophysiology studies can identify physical
inherited muscle disease. DNA analysis and and/or chemical changes in the nervous system.
enzyme assays can confirm the diagnosis of certain • Electromyography (EMG) can record muscle
neuromuscular diseases, including MD. Genetic fiber and motor unit activity. Increase in
linkage studies can identify whether a specific insertional activity is seen in early stage of
genetic marker on a chromosome and a disease are disease which can decrease later as fibrotic
inherited together. They are particularly useful in tissue replaces muscle and is characteristic of
studying families with members in different MD. Patterns of low-amplitude, short-
generations who are affected. duration polyphasic motor unit action
Genetic counselling can help parents who have a potentials is seen in EMG with patients of
Muscular dystrophy affection.
family history of MD determine if they are carrying
one of the mutated genes that cause the disorder. • Nerve conduction velocity studies measure
Two tests can be used to help expectant parents find the speed with which an electrical signal
out if their child is affected. travels along a nerve. Sensory NCSs are
normal and Motor NCSs have normal
• Amniocentesis: is usually done at 14-16 weeks latencies,conduction velocities and F-wave
of pregnancy, where a sample of the amniotic latencies, but amplitude of compound muscle
fluid in the womb for genetic defects is taken action potential (CMAP) decreases as disease
as the fluid and the fetus have the same DNA. progresses.
• Chorionic villus sampling or CVS: involves • Repetitive stimulation studies involve
the removal and testing a small sample of the electrically stimulating a nerve 5 to 10 times,
placenta during early pregnancy. at a frequency of 2-3 shocks per second, to
study muscle function.
Magnetic resonance imaging (MRI) is a
noninvasive, painless procedure,used to examine Medical Management:
muscle quality, any atrophy or abnormalities in size, There is no specific treatment that can stop or
and fatty replacement of muscle tissue, as well as reverse the progression of any form of MD. All
to monitor disease progression. forms of MD are genetic and cannot be prevented.
Treatment is aimed at keeping the patient
Other forms of diagnostic imaging for MD include
independent for as long as possible and preventing
phosphorus magnetic resonance spectroscopy,
complications that result from weakness, reduced
measuring cellular response to exercise and the
mobility, and cardiac and respiratory difficulties.
amount of energy available to muscle fiber, and
Treatment may involve a combination of
ultrasound imaging (also known as sonography), approaches, including physical therapy, drug
uses high-frequency sound waves to obtain images therapy, and surgery.
inside the body and can be used to measure muscle
Assisted ventilation is required to treat respiratory
bulk.
muscle weakness especially seen in the later stages.
Muscle biopsies are used to monitor the course of Oxygen is fed through a flexible mask to keep the
disease and treatment effectiveness. The muscle lungs inflate fully. some patients may need
specimen is examined to determine whether the overnight ventilation when respiration becomes
patient has muscle disease, nerve disease more strenuous. A mask worn over the face is
(neuropathy), inflammation, or another myopathy. connected by tube to a machine that puts out
Muscle biopsies also assist in carrier testing. With intermittent bursts of forced oxygen. Obese patients
the advent of accurate molecular techniques, muscle with Duchenne MD may develop obstructive sleep
biopsy is no longer essential for diagnosis. In apnea and require nighttime ventilation. Patients
muscular dystrophy, degeneration with gradual on a ventilator may also require the use of a gastric
loss of fiber, variation in fiber size & proliferation feeding tube.
of connective and adipose tissue is seen. Drug therapy may be prescribed to delay muscle
degeneration.
Immunofluorescence testing can detect specific
proteins such as dystrophin within muscle fibers. Corticosteroids such as prednisone can slow the
rate of muscle deterioration in Duchenne MD and
Histochemical studies indicate loss of subdivision help children retain strength and prolong
into fiber types, with a tendency toward type 1 fiber independent walking by as much as several years.
predominance. However, these medicines have side effects such
Muscular Dystrophy 361

as weight gain and bone fragility that can be


especially troubling in children. Immuno-
suppressive drugs such as cyclosporin and
azathioprine can delay some damage to dying
muscle cells.
Drugs that may provide short-term relief from
myotonia (muscle spasms and weakness) include
mexiletine; phenytoin; baclofen, which blocks
signals sent from the spinal cord to contract the
muscles; dantrolene, which interferes with the
process of muscle contraction; and quinine.
Anticonvulsants, also known as antiepileptics, are
used to control seizures and some muscle activity.
Commonly prescribed oral anticonvulsants include
carbamazepine, phenytoin, clonazepam, Standing posture.
gabapentin, topiramate and felbamate.
Respiratory infections may be treated with
antibiotics.

PATTERNS OF WEAKNESS AND


COMPENSATION ACCORDING
TO STAGES WISE:

Waddling gait.

Progression of Weakness.
Early stage:
Weakness seen in hip extensors, ankledorsiflexors,
hip abductors, hip adductors, abdominals, neck
flexors, shoulder depressors, extensors and
abductors and elbow extensors.

Compensation :
1. Increased lumbar lordosis to keep force
line behind hip joint.
2. Lack of heel strike.
3. Foot may be pronated and everted.
4. Hip waddling gait
5. Decreased in Cadence.
6. Gower's Maneuver. Normal standing vs Muscular dystrophy.
362 Neuro-Rehabilitation : A multi disciplinary approach

Functional Losses: All activities against gravity like


ability to rise from floor, stair climbling and rising
from chair.
Compensated Gait: Standing and toe walking with
wide base of support and extreme lumbar lordosis,
extreme lateral trunk lurch and upper limb
abduction.
Prescription of long leg braces like KAFO with or
without surgery should be done to prolong
ambulation.

Winging of scapulae. Late or Non-Ambulatory Stage :


Weakness in upper limb interferes functional,
elbow extensor, forearm supinator, wrist and finger
extensor with above mentioned muscles in two
stages. Distal hand functions are preserved.
Compensation: Here in this stage compensation are
used to maintain upright posture and facilitate
ambulation, achieve support and stability in sitting
and upper limb function.
Tightness is seen elbow flexors, pronators, wrist
and finger flexors and neck extensors results in
development of upper limb contracture.
Scoliosis results as there is more of sitting posture
with loss of ambulation.
Loss of functional activity is seen in upper limb
activities, sitting ability and in doing ADLS.

Most comfortable position.

Middle stage:
Weakness seen in quadriceps and ankle evertors
in addition to muscles mentioned above in early
stage.

Compensation:
1. Line of gravity infront of knee joint and behind
in hip joint.
2. Base of support widens to maintain balance
and due to tightness in iliotibial band
Talipes equino varus position.
3. Increase ankle plantarflexion and equinus
posture to avoid knee flexion. COMPLICATIONS:
4. Frequent falls. 1. Scoliosis can be explained into two phases:
5. Strong action of tibilias posterior results in one in ambulatory phase and non-ambulatory
inversion attitude at ankle. phase.

Tightness develops in iliotibial bands & tensor During ambulatory stage, scoliosis is flexible,
fascia lata, hip flexors, hamstrings, gastrosoleus and functional and is minimized by protective spinal
posterior tibialis. hyperextension and lateral trunk lurching.
Muscular Dystrophy 363

Factors that influence whether or not scoliosis 2. Factors that initate asymmetry.
appears prior to final loss of ambulation: Compensatory movement pattern used for
1. Age at which walking ceases. stability, upper limb function, pelvic position
and lower extremity position. The deforming
2. Intervention used or not used to prolong
force of gravity on vertebral column increase
ambulation.
in presence of asymmetrical spine and pelvic
3. Final gait pattern. alignment that compromise the simple
mechanical ability of vertebral column to
In non-ambulatory phase, scoliosis becomes more
withstand the force of gravity. Inaddition,
prominent as there is constant use of wheelchair
resultant unequal distribution of weight on
and no ambulation. It can lead to decline in
epiphyseal growth plates increased the
pulmonary function, upper extremity function and
potential for an initial flexible scoliosis to
sitting ability.
become structural.

Sign of scoliosis X-Ray showing scoliosis

2. Obesity:
Since the muscles are replaced by the fat and
fibrous tissues in later stage of disease with
the loss of ambulation, there is less calorie
expenditure resulting into obesity.

Scoliosis.

Factors that results in the development of


scoliosis are as:
1. Factors that make spine vulnerable
Severe symmetrical weakness in trunk
musculature. Rapid vertebral growth during
adolescent coincides with loss of ambulation.
Loss of protective spinal hyperextension. Obesity.
364 Neuro-Rehabilitation : A multi disciplinary approach

3. Osteoporosis: Physical Therapy Assessment:

Pathokinesiology :
Imbalanced muscle weakness, compensatory
movement patterns, postural habits and influence
of gravity add to weakness progression in a pattern
of proximal to distal. As weakness increases,
postural alternations and movement which
produced mechanically locked joints thereby
substituting for adequate muscle strength. The
substitutor are responsible for optimizing function
eventually leading to muscle tightness, contracture
and deformity thereby increasing disability.
In Muscular dystrophies, assessment is ongoing
process where specific type of weakness and
Osteoporosis. tightness and compensation are identified and
interventions should be designed inorder to
It is more common in vertebral column than in long
maximize strength, prevents deformity and provide
bones, worse in lower extremities than in upper
effective adaptive functioning.
extremities and is aggravated by steroids.
1. Postural alignment.
4. Respiratory Affection.
2. Range of motion
It is compromised by no. of factors:
3. Manual muscle testing
1. Decrease in thoracic and spinal mobility due
to progressive muscle weakness leading to 4. Girth Measurement
replacement into fibrous tissue and restricted 5. Respiratory status
pattern of breathing.
6. Activities of Daily living
2. Asymmetrical breathing pattern due to muscle
7. Gait
weakness.
8. Functional Status.
3. Total lung capacity, vital capacity and forced
inspiratory and expiratory abilities decreases 9. Transfers
and residual volume increases.
10. Orthosis/Casting/Bracing
4. Breathing becomes strenuous from initial
11. Mobility status:
shallow breathing to more rapid breathing to
less chest or lung volume/expansion leading 12. Wheelchair mobility: Manual
to decline in breathing volume. Motorized
Decrease in lung expansion leads to areas of 13. Physical environmental and Accessibilty.
lung collapse and weakness in abdominals and
in muscles of forced expiration results in Outcome Measures:
decreased coughing. 1. Vignos Functional Rating Scale.
5. Cardiac Involvement : 2. Brooke's clinical protocol.
Cardiac muscles are too affected by
3. WeeFIM.
dystrophin. Cardiac involvement can also
compromise by scoliosis and respiratory 4. Pediatric evaluation of disability inventory.
status. It is so progressive that it shows ECG 5. Muscle, Pulmonary and ROM testing.
abnormalities, hypertrophic cardiomyopathy
and dilated cardiomyopathy. Cardiac 6. GSGC Assessment which includes noting
abnormalities may also include AV block, timed tasks in 10 m timed walkng, Stair
atrial paralysis, atrial fibrillation or flutter, climbling, sit to stand from chair and rising
ventricular arrhythmia, conduction defects from floor.
and reduced ejection fraction.
Muscular Dystrophy 365

Physiotherapy Interventions for 7. To maintain sitting ability.


Muscular Dystrophy: 8. To maintain functional, independent mobility
throughout all phases of disease progression.
Physiotherapy in Early stage:
9. To provide active respiratory program and to
1. Educating the family about the condition and establish & maintain most effective breathing
coping strategies pattern.
2. Prevention of contractures and deformity 10. To maintain chestwall mobility.
which can further lead to disability and pain.
11. To strengthen respiratory muscles & develop
3. Maintenance of maximal strength to prevent endurance.
disuse atrophy.
12. To To teach principles of pulmonary hygiene
4. Maintenance of maximal functional & assisted coughing.
capabilities by using appropriate adaptive
equipment 13. To help preserve maximal hand function.

5. Emphasizing home programs. 14. To teach caregivers proper handling & transfer
techniques
6. Interventions required to maintain
ambulation. Long term Goals:
1. To prevent deformity & contracture.
Physiotherapy in Middle stage Following loss
of ambulation: 2. To maximize & maintain strength &
endurance within the limits of fatigue.
1. Continuation of early stage program.
3. To maximize & maintain respiratory status.
2. Spinal care and management to prevent
4. To maintain ambulation.
further deformity because of loss of
ambulation. 5. To maintain functional mobility.
6. To maintain highest possible level of
3. Transfers requiring less energy expenditure
functional independence using adaptive
should be taught.
equipment & orthotic devices.
4. Educating about body mechanics to further 7. Provide patient, family & caregivers with
prevent the contractures and deformity. timely information helping in overall
5. Adaptive devices to modified ADLS. management of disability.
As DMD represents with severe muscular
Physiotherapy in Later stage : weakness resulting in contractures of the hip flexors
1. Continuation of above program. and plantar flexors which later interfere with
2. Evaluating the endurance and fatigue level ambulation and as walking mobility is lost,
required for maintaining any posture or contractures of the hips, knees, ankles, shoulders,
activity. wrists, and fingers set in quickly. Loss of
ambulation leads to another major complication of
3. Maximizing Upper limb function. the development of scoliosis which will lead to
severe thoracic distortion comprising with
Short term Goals: respiration. Again, a loss of ambulation results in
1. To increase / maintain range of motion. reduced caloric expenditure resulting to obese.
2. To increase / maintain strength & endurance Obesity along with loss of strength may lead to
pressure sores, as there are unable to do wheelchair
3. To promote optimal body alignment & push-ups or move the body for extended periods.
symmetry. As there is complete loss of mobility, respiratory
4. To minimize compensatory movement, complications sets in resulting to death.
patterns & position used for function. Exercise will be proven beneficial as increase in
5. To prevent the development of scoliosis. strength will improve performance of daily
activities such as stair climbing, chair rising, and
6. To maintain functional ambulation. walking. To stretch the contractures, flexibility
366 Neuro-Rehabilitation : A multi disciplinary approach

program should be included to reduce the thought to be beneficial in preventing disuse


progression of contractures, thus allowing persons atrophy, maintaining strength, functional status
with DMD to ambulate longer. Finally, and flexibility. Studies proving which type of
strengthening of postural muscles may decrease the muscle activity can be beneficial or detrimental is
formation of scoliosis. Increased energy limited.
expenditure due to exercise may reduce the
Eccentric exercise increases muscle damage thus it
prevalence of obesity as there is no ambulation.
should be eliminated, i.e., dynamic constant
external resistance exercise, stepping down off a
Endurance
step as it results in fiber splitting,vacuoles &
In one of the animal studies, results showed that necrosis with proliferation of connective tissue as
mdx mice ran significantly less distance per week well as fiber hypertrophy. Recent report also shows
and ran at a significantly slower daily speed that isokinetic eccentric exercise did not elevate CK
compared to normal mice and when exercise was in healthy individuals. With dynamic constant
administrated it showed improvement in resistance external resistance, (ex. machines or free weights)
to fatigue, increased proportion of oxidative fibers, there may be an increase in muscle damage with
and improvements in force production of the soleus the change in the direction of the velocity at the
and extensor digitorum longus of exercised mdx point where the concentric contraction is followed
when compared to sedentary mdx mice. by an eccentric contraction whereas submaximal
isokinetic exercise if given in early stage of disease
Muscular Strength is beneficial when the strength was at its maximum.
A certain amount of muscle activity has been

General Exercise Guidelines


Type Frequency Intensity Duration
Flexibility Passive/Active Daily Low 3x (10-30 sec)
Endurance Walking, Variable Variable
Exercise Cycling, 1-7 x per Low 1-20 minutes
Swimming week
Muscular Isokinetic, Variable Variable
Strength Concentric, or 1-5 x per Low 1-3 sets
Eccentric only week Low 5-15 repetitions

Specific Exercise Guidelines


Muscle Groups
Flexibility Hip flexors, Shoulder extensors, Elbow flexors, Plantar flexors, Intrinsic hand
flexors, Knee flexors
Endurance Muscles used for ambulation
Exercise
Muscular Hip extensors, Elbow extensors, Shoulder flexors, Dorsiflexors,
Strength Hand and wrist extensors, Back extensors Abdominals.
Muscular Dystrophy 367

Precautions and Contraindications to Exercise


Muscle damaging eccentric exercise (i.e., downhilll
running or walking)
1. Ballistic stretching
2. Sitting or lying for extended periods of
time.

Stretching and Positioning to prevent


deformity:
Effects of chronic positioning, unopposed influence
of gravity and imbalanced muscle activity around
the joints contribute to development of hypo Soleus stretching
extensibility and which can be prevented by
adequate positioning by daily ROM/ stretching and
use of splinting, casting and standing on standing
board.
Mostly severe contractures are seen in 2 joint
muscles and those which are maintaining posture.
In upper limb: 1. Elbow flexors.
2. Forearm pronators.
3. Wrist /Finger flexors.
In Lower limb: 1. Illiotibial band.
2. Tensor fascia lata.
3. Hip fexors.
4. Hamstring
5. Gastrosoleus PNF hamstring
6. Posterior tibialis
In spine: Neck extensors.

Interventions:
1. Passive stretching: should be done daily and
is best achieved by standing.

Tendoachilles Stretching Knee flexor stretching


368 Neuro-Rehabilitation : A multi disciplinary approach

4. Pelvic girdle musculature especially gluteals.


5. Knee extensors.
6. Ankle dorsiflexors.
Strengthening can be achieved by
1. Maintaining proper alignment thereby
maximizing the muscle work and
biomechanical advantage.
2. Positioning and supporting the muscle in their
optimal length so that they are functional.
3. Movement facilitation.
4. Submaximal exercises.
5. PNF active assist.
Psoas_Stretch
6. Functional movements which are used in
ADLS.
Exercises can be categorized according to the
stagewise depending upon:
1. Remaining strength in the available muscles.
2. Ability to perform the functional activity.
3. Endurance.

In Early stage:
muscle power remains functional i.e more than
Hip flexor stretching in Prone Grade 3 but has difficulty in climbling stairs and
getting up from floor showing weakness especially
in gluteal muscles and abdominals muscles.
2. PNF- Contract Relax to improve flexibility
Exercises to strengthen these muscles in early
3. Joint Mobilization -Traction to all joints. stage compromises:
4. Myofascial release techniques. 1. Bed mobility which includes:
5. Modalities: Hot packs to increase plasticity 1. Rolling.
and comfort but should be avoid excessive 2. Leg rotation.
generalized heat which can induce fatigue and
3. Abdominal and back strengthening
reduce strength.
exercises.
Strengthening: 4. Bridging.
5. Quadrapud.
Submaximal levels of isokinetic and aerobic
exercise have been practiced inorder to prevent 6. Cat and camel exercises in Quadrapud.
disuse atrophy and to maintain residual strength 7. Hip extension in Quadrapud.
while avoiding overwork weakness. Exercise in 8. Side sitting in Quadrapud.
early stage of disease proves to be beneficial as long 9. Kneeling
periods of rest and immobility results in functional 10. Kneel walking
loss. 11. From quadrapud coming to kneeling
Loss of muscle strength is seen in: position.
12. Trunk rotation in kneeling.
1. Neck flexors.
13. Hip extension in prone.
2. Abdominals 14. Hip flexion in supine.
3. Shoulder girdle musculature especially 15. Hip abduction in sidelying.
deltoids, latissimus dorsi, stabilizers. 16. Strengthening of scapular and neck
muscles.
Muscular Dystrophy 369

Rolling - 1 Rolling - 2

Rolling - 3 Bridging

Leg Rotation-1 Leg Rotation-2

Grade-II Lower Abdomen-1 Grade-IV Lower Abdomen-2


370 Neuro-Rehabilitation : A multi disciplinary approach

Upper Abdomen Back Extension

Reachouts in Quadrapud Crawling

Cat camel exercises in Quadrapud Cat camel exercises in Quadrapud 1

Hip extension in Quadrapud Hip extension in Quadrapud


Muscular Dystrophy 371

Kneeling - 1 Kneeling - 2

Hip Extension in Prone Hip abduction in sidelying

Active assisted Hip flexion Knee extension Strengthening

Hip extension on Swiss Ball Back Extension on Swiss Ball


372 Neuro-Rehabilitation : A multi disciplinary approach

Resisted knee flexion One leg bridging

Balancing exercises in sitting Balancing exercises in sitting(1)

Going to lying position Going to lying position 1

Neck strengthening exercises Overhead activities


Muscular Dystrophy 373

6. Getting up from sidelying position.


7. Standing with the help of push knee splint and
high boots with posterior steel shank and
walker or wall support for an half an hour to
one hour to stretch the knee flexor contracture
and Tendoachilles contracture.
8. Walking is prolonged either with push knee
splint and high boots with posterior steel
shank and walker or with KAFO.
9. Stretching the hip flexor contracture in
Rhomboids Strengthening with wand and pronelying position for about half an hour to
theraband one hour and constant splinting the lower
limbs at night to prevent the legs going into
2. Getting up from low stool or chair. hip abduction external rotation, knee flexion
3. Quadriceps drill. and foot into talipes equino varus position.

4. Stepping exercises with the low stepper. Late or Non-Ambulatory Stages:


5. Swiss ball exercises to strengthen the trunk and In this stage the muscle is almost replaced by fat
hip musculature. and fibrous tissue thereby muscle power showing
Grade 1+ with only flicker contraction at times to
6. Hydrotherapy.
Grade 0. The child becomes wheelchair bound with
In Middle Stage: spinal deformities developing later. Functionally
the child becomes totally dependent for all his
Patients shows the muscle power around Grade ADLS.
2++ to Grade 2- with frequent falls while walking
as patient develops toe walking because of In this stage, exercises such as:
tendoachilles contracture and lumbar lordosis 1. Breathing exercises are incorporated to
compensating for the weakness of hip extensors improve the strength of the respiratory
and abdominals and difficulty in getting up from muscles and to build up the endurance.
floor and rising from chair showing quadriceps
weakness. Initial weakness starts developing in 2. Suspension therapy exercises and passive -
upper limb with difficulty in doing overhead assisted exercises are included inorder to
activities. maintain ROM and strength in available
muscles.
Exercises in this stage includes strengthening of
muscles and stretching of tight muscles. 3. Stretching to prevent further worsening of the
contractures.
Exercises such as: 4. Standing on a standing board compulsory for
1. Suspension therapy exercises for an hour twice in a day for physiological
strengthening the hip, knee and trunk benefit.
muscles from Grade 2- to Grade 3-.
5. Transfering the child in this stage is difficult
2. Active assisted exercises for abdominal and as holding the axilla and lifting is almost
back musculature. impossible. The only way is to lift him by
holding the child with both the arms around,
3. Quadrapud position achieved with the help
lift him and rotate.
of elbow splint for developing control on the
shoulder, trunk and hip muscles.
Orthosis and casting : Fig 21.
4. Cat and camel exercises and weight shifting 1. At night or during daytime while taking rest
in forward, backward and sideways direction to maintain proper positioning.
exercises are included.
2. Ankle foot orthosis(AFO) for stretching
5. Kneeling position is achieved with the help plantarflexors.
of elbow splint and foot stool infront.
374 Neuro-Rehabilitation : A multi disciplinary approach

Assisted Bridging with bedsheet Wt shifting in Quadrapud

Suspension exercises-Hip abduction adduction Suspension exercises-Resisted Hip adduction

Suspension exercises-Resisted Hip flexion Suspension-Hip extension

Suspension exercises-Knee extension

Kneeling with support of foot stool and elbow splint


Muscular Dystrophy 375

Elbow flexion with wand Upperlimb strengthening with theraband

Neck side flexion exercises Neck rotation exs

Standing on standing board (2) Walking with push knee splint

Lifting 1 Lifting 2 Lifting 3 Lifting 4


376 Neuro-Rehabilitation : A multi disciplinary approach

3. Knee extension splints for stretching knee should be at proper height so that when the elbows
flexor contractures. rest over arm rest and the shoulders are in relaxed
position.
4. Wrist and finger splints for wrist and finger
flexor stretching.
Spinal Management
5. Serial Casting. 1. It is required to prolong ambulation and
Positioning: standing.
1. Prone lying. 2. To promote spinal extension in sitting.

2. Supine -Tieing both the thighs to avoid 3. To maintain symmetry while sitting in
the leg to go to abduction. wheelchair.
4. To optimize upper extremity function in
Standing :
symmetrical pattern.
1. With long leg braces (KAFO) on tilt table
or standing board to minimize Interventions:
osteoporosis and to prevent lower
extremity [Link] A) Sitting posture:
1. Proper sitting posture in wheelchair should be
Emphasis on Standing and Ambulation with attained to avoid any compensation
KAFO : happening at spine level. One should be
Whether to release the contractures and then give constantly leveling his pelvis without rotation.
braces for walking or straightway give braces and If scoliosis develops, a spinal jacket is
make him walk till he could prolong with walker. prescribed inorder to prevent further increased
in curvature. Avoiding kyphotic posture,
Importance of standing with braces: maintaining lower extremity position with no
Standing promotes functional status in walking & hip abduction and having proper foot
ADL. placement.
Lower extremity contractures are stretched 2. Evaluating all functional activities which can
resulting in release of stiffness, and flexibility. produce asymmetrical movement patterns.
Standing results in minimization of severe B) Standing posture:
osteoporosis.
1. To help in controlling Lower extremity
Standing provides weight control. contracture.
It improves cardiovascular and cardiopulmonary 2. To promote spinal extension in standing
functions. posture on standing board or tilt tables.
Standing delays the development of scoliosis. 3. To optimize more physiological benefits.

Wheelchair: When independent or dependent Parents and caregivers should be asked to monitor
walking i.e with or without orthosis becomes symmetry and asymmetry posture attained during
difficult, wheelchair as a mobility orthosis is used sitting or standing and correcting those by visual
as it acts like a functional mobility aid. feedback periodically or by changing position or
support while maintaining those postures.
Usually self propelled wheelchair is given to a child
to used it in indoors as this increases the chest Surgical Management:
expansion and muscle power of upper limb. When
Segmental instrumentation in spine allows
the patient is unable to use the self-propelled
stabilization with immediate postoperative
wheelchair in later stages of disease, then powered
mobilization with no external support required
or attendant propelled wheelchair can be used.
whereas in lower limbs subcutaneous release of
Wheelchair should be light weight, strong and
Achilles tendons and hamstring muscles and
should provide good sitting posture to the patient
fasciotomy of iliotibial bands. At times, rerouting
leaving minimum space on either side of the sitting
of tibialis posterior to the dorsal surface of the scond
area. Feet should be resting at 900 to prevent
or third cuneiform to balance the foot thereby
equines deformity and the armrest of the chair
preventing severe varus position of foot.
Muscular Dystrophy 377

AFO Push Knee Splints Knee-ankle-foot


Orthosis

KAFO KAFO with T-Strap for Body Jacket


preventing inversion

Standing with KAFO independently Walking with KAFO independently outdoors


inside the Parallel Bar without any aid
378 Neuro-Rehabilitation : A multi disciplinary approach

increase chest wall mobility and to


increase the strength of diaphragm
muscles.
2. Swimming is advised to improve
endurance and breathing patterns.
3. To teach efficient coughing.
4. Postural drainage to remove secretions
lobewise should be taught.
5. Inspiratory muscle aids e.g Nocturnal or
daytime IPPV with volume ventilator
BiPAP if required.
6. Expiratory muscle aids e.g mechanical
insufflation - exsufflation.
Scoliosis before correction with spinal brace

Scoliosis corrected with brace Spirometer exercises

It is seen that early surgery for contractures Nutritional management :


followed by intensive physical therapy can prolong Always, steroids becomes main protocol in treating
brace free ambulation. Gait training can begin in Muscular dystrophy. But it produces weight gain
within 48 hours after surgery thereby regaining the by means of fluid retention, increasing appetite and
sense of standing. body fat redistribution and increase in body weight
becomes a burden on the already weak muscles.
Respiratory Management: Treatment induced excess weight gain can be
As there is involvement of respiratory muscles, prevented through a healthy diet regimen having
proper respiratory management [Link] be four meals per day and avoid multiple snacks or
administrated. long fasting periods.
It is required : Foods like all vegetables especially leafy and green
1. To maintain thoracic wall mobility. varieties, raw vegetables salads, Fruits, whole
grams and pulses, sprouted grams, dals, lean meat,
2. To maintain strength and endurance in
Chicken without skin, Fish & egg should be
respiratory muscles.
included.
3. To establish proper breathing pattern.
Foods like Fast foods, Junk foods, Fry foods (Poori,
4. To make use of non-invasive inspiratory
Vadai),Salty foods, Chocolates & milk sweets,
and expiratory aids.
Cookies/cakes, Candies, Cheese, Butter, Ghee,
Interventions: Vanaspathi, Palm oil, Coconut oil, Nuts & oily
1. Inspiratory exercises /Segmental seeds, Bakery products, Organ meats (Liver, Brain)
breathing to improve lung expansion and Shellfish (Prawns, Shrimp) should be avoided.
Muscular Dystrophy 379

OCCUPATIONAL THERAPY FOR • Sports


MUSCULAR DYSTROPHY • Peer Group
Occupational therapists have a unique ongoing role
in supporting and working with patients with III. Work and Productivity Skills
muscular dystrophy as the patient's needs and the • School and Nursery assessments
needs of their caretaker are constantly changing.
They need to assess and evaluate an individual's • Pre Vocational testing
physical, psychological and social needs and focus • Vocational/ Workplace assessment
on maximizing skills, promoting and enabling
• Architectural barriers
independence, as well as improving the quality of
life of the affected individual and his family.
Assessment of Performance Components:
Assessment The following key performance components need
to be assessed.
The OT will be responsible for occupational
performance areas and components. All Dystrophy A. Sensory Processing
areas assessed will be considered in relation to
Proprioception
functional performance and skills in order to
identify realistic and client centered treatment goals. B. Neuromusculoskeletal
The initial step in management of the child with i) Reflex
MD involves taking:
ii) Range of motion
1. Medical History with family concerns
iii) Muscle Tone
2. Aerobic capacity and endurance assessment
iv) Muscle strength
Assessments Of Performance Areas : v) Endurance
I. Activities of Daily Living: Basic ADL vi) Postural control
(BADL) and Instrumental ADL (IADL) vii) Postural alignment
skills
viii) Integumentary (when using orthoses,
BADL:
adaptive equipment, or wheelchair)
• Personal care
• Eating and drinking skills ix) Contracture /deformities
• Dressing x) Atrophy/ Wasting
• Bathing C. Motor
• Toileting
i) Motor control
• Bed Mobility
• Transfer: moving and handling, mobility ii) Gross coordination
aids used ii) Bilateral integration
• Ambulation and stair climbing
iv) Fine coordination or dextertity
IADL:
D. Cognitive Integration
• Domestic chores
• Transportation i) Attention span
• Banking ii) Spatial operations
• Shopping iii) Problem solving
iv) Learning
II. Play and Leisure Skills
• Recreation v) Generalization

• Hobbies E. Psychosocial Skills

• Pets i) Values
ii) Interests
380 Neuro-Rehabilitation : A multi disciplinary approach

iii) Self concept 4. Vignos Functional Rating Scale.


iv) Role performance 5. Brooke's clinical protocol.
v) Social conduct 6. Fall Risk Assessment
vi) Interpersonal skills
PLANNING
vii) Self expression Following the assessment process, short and long-
viii) Coping skills term occupational therapy goals have to be set with
the individual and the family. These goals must be
ix) Time management
based on the person's preferences.
x) Self control
INTERVENTIONS
F. Respiratory Status
STAGE I & II: Early/pre-symptomatic and Early
G. Sleep ambulatory (Walking) Stage
H. Need for Assistive and Adaptive Devices and At this stage, education regarding the condition and
Technologies counseling to the patient and family is of utmost
I. Home Assessment importance. One of the primary considerations in
the early management program is to retard the
J. Wheel Chair Assessment development of contractures. Contractures have not
been shown to be preventable, but the progression
Frequency of Assessment
can be slowed with positioning and an ROM
An occupational therapy assessment or review of program.
the young person should be carried out
systematically and at least annually. More frequent A home ROM program should be emphasized and
reviews may be necessary at times of change, such the family instructed in the stretching exercises.
as following periods of ill health or after surgery Cycling and swimming are excellent activities for
and following loss of ambulation. overall conditioning and are often preferred over
formal exercise programs Standing or walking for
Standardised Assessment Tools a minimum of 2 to 3 hours daily is highly
recommended.
The main goal of assessment in occupational
therapy is to get a clear understanding of the Breathing exercises have been shown to slow the
individual, their social circumstances and their loss of vital capacity and forced expiratory flow rate.
environment, in order to develop a treatment plan Game activities such as inflating balloons or using
which will improve the quality of life of the person blow-bottles to maintain pulmonary function can
and their family. easily be included in a home program and will
decrease the severity of symptoms during episodes
The quality of the assessment carried out will have
of colds or other pulmonary infections. Night
a direct correlation with the quality of the treatment
splints are helpful to slow the progression of ankle
interventions (Turner et al., 2002). Ideally, in the
contractures.
atmosphere of evidence-based practice,
standardised assessments should be used to
measure the effectiveness of occupational therapy
interventions. Occupational therapists and other
professionals have created many standardized tests
that could be used to assess certain functions that
are problematic for people with muscular
dystrophy.
1. ADL Scales : FIM, Barthel Index, WeeFIM,
2. Muscle test (gonimeter, pinchometer,
dynamometer, hand functions)
3. Range of Motion (R.O.M.)
ADLS-Doffing of Upper Body Dressing
Muscular Dystrophy 381

Play Forward planning is also vital to ensure that the


Play is essential in the psychological development young person's and their family's changing needs
of children. Occupational are provided for in a timely manner.

therapists use play activities in treatment to enhance In the first stages of loss of function, small
the developmental and functional skills of a child independence aids may be useful in maintaining
and to increase the child's enjoyment of play and independent self-care skills. As the condition
playfulness. Play can also be a valuable progresses, these aids become more difficult to use
communication tool used by children to and personal-care tasks a more passive experience
communicate their feelings and anxieties. All play for the young person. When considering self-care
activities should be based on the child's interests, tasks, it is essential to discuss upper-limb function,
not their medical condition. Activities requiring as this is crucial for independence in this area.
repetitive muscle building types of exercise should
be avoided, as they are likely to damage muscle Eating
tissue further For the individual with muscular dystrophy, this
basic survival task becomes very demanding as
Sports muscle weakness progresses, grip strength becomes
Active exercises and participation in sports poor and it becomes increasingly difficult to lift the
activities should be encouraged to help delay the hands/arms against gravity.
development of contractures. Swimming can be Possible options include:
good fun at any age and is an enjoyable form of
• lightweight cutlery and cups or mugs with
exercise for people with muscular dystrophy. A
built-up handles
child in the early stages of muscular dystrophy will
enjoy riding and it is a good exercise for helping • rocker knife;
him to maintain his balance reactions.
• cuffs with inserts for cutlery;
Hobbies • Plate with a rim to contain the food when
Collecting specialised items is a hobby that fosters scooping;
social interaction. Shopping, as well as having a • non-slip mats;
functional purpose like buying food or clothes, can
also be a social experience at the large shopping • mechanical eating aids
malls, the use of computers and video games in • long straw for drinks
occupational therapy treatment programmes is
beneficial to people with muscular dystrophy. Other alternatives such as elevating the plate height,
There are a number of interests that can be carried and angled cutlery will
out with limited upperlimb function; these include minimize the amount of active arm, wrist and hand
reading and creative writing, painting, movement required. Mobile arm supports provides
photography, graphic art and some crafts, such as support to the forearm to facilitate eating and
model-making. People can enjoy leisure pursuits drinking.
on their own, but Passmore and French. found that
social leisure activities were important, as they Grooming
fostered feelings of self-worth and gave participants Consideration should be paid to the design features
a sense of belonging.(12) of items of equipment for shaving, combing hair
Stage III and IV: Late Ambulatory and Early non- such as long-handled brush/comb, and cleaning
ambulatory teeth, including weight and the type of grip. For
grooming tasks normally carried out at the basin,
Loss of function in personal-care activities is a
access for a wheelchair to fit underneath (i.e.
constant and stark reminder of the progression of
without a vanity unit, or wall-mounted) together
muscular dystrophy confronting both the
with support for the elbows at each side of the sink
individual and their family in various ways on a
daily basis. It is time consuming and physically is necessary.
demanding for all involved personal care is an area
that needs to be addressed with the utmost
Bathing
sensitivity. Bath board and shower aids such as hand held
382 Neuro-Rehabilitation : A multi disciplinary approach

shower, non skid mats, shower chair may be muscle power to stand up from, and since the legs
sufficient to provide independence in this area. can be lowered to the floor in a straight-leg position,
rather than trying to rise against gravity from a
Dressing flexed-knee position. Bed-height adjustment is also
For postural stability, balance and energy helpful if the young person is able to manage to
conservation, a seated position with feet firmly on side transfers on/ off the bed using a transfer board.
the floor can be helpful for dressing/undressing.
As the amount of assistance required increases, Postural Management
small items of equipment such as a dressing stick Individuals with muscular dystrophy can develop
and reachers may prove useful. Clothing should spinal problems fairly quickly once they stop
be comfortable and easy to get on and off: loose, walking, so they need good postural management
with big head opening and minimal fastenings zips interventions to slow down the rate of spinal
can be made longer to allow easier access for [Link] management is an approach to
toileting and various sorts of fastenings can be the handling, treatment and positioning of children
considered, such as Velcro and hooks. and adults with muscular dystrophy that will
reduce the risk of contractures and the development
Toileting of postural deformities. Passive and active
Many boys suffer from constipation due to movements of limbs will also slow down the
immobility, self-limited diet, reduced fluid food development of contractures.
intake to avoid the need to go to the toilet and Good positioning will allow the person to carry out
slowing of peristaltic movement. everyday activities with more ease and without
A regular toileting routine can help avoid adopting abnormal postures. If postural problems
disruption, discomfort and stress, particularly in are not addressed, it can lead to pain, spinal
relation to the school and work environment. Whilst problems and breathing difficulties. The main
the young person maintains the ability to carry out pieces of equipment that can help with postural
weight-bearing transfers, rails and a raised toilet management are:
seat or a toilet frame may be sufficient. However, • sleep systems;
as postural control deteriorates, increased support • postural seating;
may be necessary in order to allow for a well-
• wheelchairs with postural seating
supported and relaxed position on the toilet. This
systems;
type of support generally falls into two categories:
• splints/orthotics.
• support which wheels over the toilet;
• frames which fix onto the toilet itself Sleeping
The young person's postural needs must be
Transfers managed throughout their daily lives which
Information and training on how to move and includes overnight positioning. Once pelvic
handle an individual can be offered by the instability is apparent, a postural management plan
occupational therapists, along with advice on should be developed to address the sitting, standing
equipment that can help when transferring the and lying positions that the young person will need.
individual from one position to another. Some of This is essential to minimize the risks of deformity,
the common moving and handling equipment such as the limitations of movement and pain
supplied by therapists are listed below: caused by joint contractures or spinal curvatures
that impact upon lung capacity and respiratory
• transfer boards;
function.
• hoists and slings;
The young person's postural needs will require
• sliding sheets; regular review and the postural management
programme will require to be adjusted accordingly.
• handling belts.
Raising the height of beds and chairs from the floor Postural Seating
can be useful to the young person in the early stage The aims of good seating are: to achieve a good
of the condition as a higher surface requires less postural position; to maintain functional ability; and
Muscular Dystrophy 383

to ensure comfort. Seating which promotes a good games and for socializing and other leisure
sitting posture will also promote effective upper- activities.147.
limb function which is essential for a variety of
activities, including feeding, writing and play. It is Support groups
crucial that seating needs are considered from an Many occupational therapists can provide
early age to prevent or delay deformities and information about and links to support groups for
promote optimal function. This should be the individuals with muscular dystrophy, their
monitored and reviewed on a regular basis to parents or their siblings. Friends and family are the
accommodate any changes as the person's condition most important factor to maintaining an active
progresses. social life. Peer-group friends can provide
opportunities for discussion about all topics,
Wheelchairs including sensitive issues that cannot be easily
Wheelchairs are essential forms of transport for discussed within the family. Pets with a loving and
people with muscular dystrophy; they need them protective temperament can also give hours of
to participate in everyday life when they have enjoyment and company to people with muscular
difficulty walking. They will need different types dystrophy.
of wheelchairs at different stages in their illness.
Occupational therapists are involved in the Housing, School And Workplace Adaptations
assessment and provision of wheelchairs. They may There are many housing adaptations that the
also have to train the individual in how to use their therapist can recommend, that will make life easier
wheelchair. The therapist will have to give for the person with muscular dystrophy and their
recommendations regarding the postural support caretakers. A feware listed below:
and pressure relief required for the chair, as well • ramps;
as the type of controls needed to operate the
• bathroom alterations;
wheelchair.
• extensions;
Transport Issues • handrails;
Transport is vital to children and adults with • door alterations;
muscular dystrophy. They need • hoists;
transport to access education, hospitals, and • lifts.
employment and leisure pursuits. The type of
transport needed will change over the course of Equipment
their illness and the methods of transport used will Occupational therapists can advise and provide
vary to meet their travel needs. Occupational many pieces of equipment that can help the person
therapists will often be involved in assessments to maintain their independence in daily living tasks,
relating to the transport requirements of people school tasks or work tasks. Equipment can also help
with muscular dystrophy. They need to teach the the caretaker with their care tasks. The following
individuals and their caretakers on how to assist are a minute selection of the equipment that could
the patient onto different forms of transport. They assist a person with muscular dystrophy:
can also suggest car modifications.
• hoists and slings;
Access To Play Equipment • shower chairs;
It is important that young patients with muscular • bath lifts;
dystrophy have the opportunity to play to develop • eating aids;
their skills. Occupational therapists can suggest toys • toilet equipment;
and activities that will help with their development. • writing aids.
IT equipment: hardware and software: If an
individual cannot use a standard Written Work / Graphic Skills
mouse and keyboard, details of alternative types Handwriting is a major occupation of education.
of keyboards, word-recognition software and Once handwriting is established, it is important to
joysticks can be supplied for accessing the internet assess the following aspects of it:
and playing console
384 Neuro-Rehabilitation : A multi disciplinary approach

• Speed of written work? Does speed • timetabling to allow alternation of passive and
reduce with sustained effort? active tasks throughout the day to limit fatigue,
such as listening activity preceding written
• Effects of gradual postural changes and
work.
deterioration
• Legibility of written work? Does legibility Information and computer technology
deteriorate with sustained effort? Word processing: Use of computers should be
• Effects of writing - does the child introduced at an early stage as complimentary to
experience fatigue and/or cramps in the handwriting.
hands? Keyboard alternatives: As power and active
• Child's preference - how does the child movement are lost from the shoulders and upper
feel about using technology? Would they limbs, it becomes very difficult for the child to
prefer to use a scribe/ writer? extend their arms to the top and edges of the
keyboard. Trunk flexion is used to compensate,
Children with Duchenne muscular dystrophy may
which is tiring and encourages poor postural
encounter problems with pencil skills on account
positioning. Possible solutions include:
of any of the following factors: reduced muscle
strength; reduced range of movement; reduced grip • on-screen keyboard with mouse;
strength; reduced stamina; and postural and • mouse alternatives, such as touch-pad mouse,
coordination difficulties. Learning difficulties may joystick, trackball or finger operated integral
also be present and these can further impact on joystick;
graphic skills. Possible solutions include:
• compact keyboard and or laptops
• pencils grips (various types), angled writing
boards, resistance provided by • Voice-recognition software

both the writing implement and the paper, paper Teaching New Methods
stands/'page-ups' may improve performance in Everyone is used to carrying out activities in their
early stages; own way. An occupational therapist can look at
how the individual carries out a task and suggest
alternative ways to do it. This may allow the person
to complete the task independently. Examples are:
• teaching a person to get dressed on the bed if
they have balance problems;
• using a computer to do homework as opposed
to having to write it all by hand;
• substitute a battery-operated toothbrush for an
ordinary toothbrush.

Pre Vocational Testing


Children need to select subjects that they find
motivating and stimulating that could lead to
careers that they can pursue. But they also need to
Various Pencils Grips be realistic in the courses that they select.
• reduction in the amount of writing required, Developing a vocational identity is an important
such as by using worksheets on which the part of adolescent development, regardless of their
child fills in missing words/phrases; health status. Occupational therapists can have a
role by encouraging them to talk about what they
• word-processing technology, including voice- want to do when they are older. They can also raise
activation programs; the subject of the boy's expectations regarding
• use of a scribe or writer; employment, as well as establishing what their
parents' and their teachers' views are of the boy's
• more oral responses;
Muscular Dystrophy 385

work prospects. This will ensure that everyone has Diet


a realistic view. An immobile individual may gain weight very
quickly. This is obviously
Workplace Assessments
Occupational therapists can also offer practical help detrimental to health and increases the physical
in suggesting adaptations to the workplace and strains on caretakers. Often, the patient tends to
work methods to enable the individual to carry out select dry/finger foods in order to avoid the
their job. An employment assessment helps to find physical difficulties involved in cutting or tearing
out what skills a person with muscular dystrophy and reaching the mouth. It is important that the
can bring to an employer. It may also identify skills occupational therapist helps the patient make
that have to be developed to improve the person's healthy choices.
employment opportunities. The assessment will
also discuss the types of work that the person is Transfers: Moving and Handling
interested in obtaining and how their medical Moving and handling needs and the needs of the
condition may influence their choice of work. individual's and caretakers will change over time;
therefore, regular reviews need to be carried out.
Fatigue Management Before any handling task is carried out, it should
Energy-conservation methods can be used, to be explained and consent taken for the move.
reduce fatigue and pain by planning and pacing Postural issues such as trunk and head control have
activities. Some methods of saving energy are listed to be assessed to ensure that any equipment or
below: movement approaches used have the right level of
• If it is not important to the individual to do support, such as chairs with lateral supports or
the task, can someone else do the work? slings with head supports. The condition of the
• Does the task need to be done every day? young person's skin will also influence moving and
• Spread the tasks over the whole day rather handling methods. If his skin is vulnerable, make
than trying to do everything in one time sure that any equipment used will not cause
period. soreness or rubbing. A profiling bed may be useful
• Can any tools, equipment or adaptations make as part of a postural management positioning
the tasks easier? programme. Profiling beds also allow the height of
• Stress reduction and relaxation techniques can the bed from the floor to be adjusted. Caretakers
also help with fatigue and pain management. will also find the ability to raise the bed to an
optimum-working height invaluable for transfers,
Ongoing Assessments helping with dressing, carrying out stretches or
Once the actions and programmes have been put helping the young person to move. The risk of back
in place, the occupational therapist needs to make strain is then minimised. Occupational therapists
sure that these interventions are fulfilling the can provide advice regarding the number of
original goals set by the individual and their transfers required and can also advise on how to
caretakers following the assessment process. If their eliminate unnecessary moves. Several equipment
goals have not been met, the therapist will have to can be utilized to facilitate transfers under different
re-evaluate their treatment plan and seek conditions and requirements. For eg.
alternative ways for the person with muscular
dystrophy to achieve their goals. • Mobile Shower Chairs, Shower Trolleys And
Lifting Bath Seats can be used for bathroom
Stage V & VI: Late non-ambulatory and and toilet transfers.
Palliative Care Stage
• Hoists and Slings are often used for safe
As the condition progresses, the individual find that
transfer of individuals within their home and
the aids become more difficult to use and personal-
also in different locations outside. Depending
care tasks a more passive experience.
on the hoist design, slings are made with loops,
Call Systems rings or clips to attach to the hoist. Mesh slings
A call system should be put in place which can be are used for bathing, as they dry quickly.
easily operated by the young person and alerts the Padded slings should be used where the
caretaker to their needs. person's skin is vulnerable.
386 Neuro-Rehabilitation : A multi disciplinary approach

• Tilt-in-space facilities in a chair as well as


independently adjustable back rests and
footrests facilitate a change in position for an
individual who may be unable to achieve this
himself.

Sleep Management
As the condition progresses, it may be necessary to
provide an increased level of support to manage
the young person's lying posture effectively. At this
stage, a sleep system is worth considering. The aim
of a sleep system is to combine symmetrical
positioning with a comfortable and supportive
position for sleep. Other sleep systems consist of a
mattress overlay that can be moulded, by the
positioning of padded supports, to provide
contoured all-round body support. For any sleep
system, an assessment is required to create an
individually customized combination of supports.
The following factors would need to be considered:
o the quality of sleep that the person gets and how
many times a night the person's and caretaker's
sleep is disturbed.
• Establish the cause of sleep disturbances. Is it
respiratory, dietary, pain-related or
psychological?
• Check whether the bed used is a standard or
specialist bed.
• Does it meet the needs of the individual and
their caretakers?
• Check whether the mattress has pressure-
relieving qualities or whether they are using a
sleep system to provide positioning support.
Lifters
Pain Management
• Stair-climbers and Lifts Stair-climbers and lifts
There are a number of interventions that
are obviously used to move 151 people and so
occupational therapists can suggest that can help
they can be deemed manual handling
with pain management. This may be the provision
equipment. Stair climbers are often operated
of pressure relief equipment, such as the following:
by carers, who therefore need training in how
to use them. • mattress;
• seating and wheelchair seating;
Seating
• There are several aspects involved in the • pressure cushions for commodes, shower
assessment for specialised seating, including chairs and baths;
seat height, width and depth, arm rests, • padded and sheepskin slings.
footplates and head rest.
• As the individual becomes more immobile, Skin Protection and Management
pressure relief, possibly in the form of a It is vital to ensure that any equipment issued will
pressure cushion, becomes increasingly not damage the individual's skin.
important. If the skin is vulnerable, pressure-relieving
Muscular Dystrophy 387

materials should be used where the skin comes into accompany traumatic loss of aspects of oneself, as
contact with the equipment and measures should in paralysis, injury and relationship breakdown.
be taken to limit moving and handling tasks. It is
Thought needs to be given to the fragility of one's
advisable to review how the person is moved and
confidence, self-esteem and identity when a young
how many times a day he has to be moved, as it
person is still growing and developing with a
may be possible to change the methods of handling
deteriorating condition. It is not always recognised
to reduce skin contact or to reduce the number of
that children grieve, as bereavement is often
times the person is handled throughout the day. If
understood tobelong to adulthood.
the individual wears splints, ensure that these are
not causing marking or chaffing of the skin. Advice Hospices and Palliative Care
on changing the individual's position when seated
A hospice is defined as a programme, or a facility,
in one chair or a bed for long periods of time will
to provide palliative care and attend to emotional,
also help to prevent skin problems. This can be
physical, spiritual and social needs of terminally ill
made easier for the caretaker and the individual
patients and their families, at the hospice or within
by providing adjustable beds and tilt-in-space
the home.153. The emphasis is on the relief of pain
chairs so that the area that pressure is on can be
and promoting quality of life. In this way, it can be
changed easily with the push of a button.
seen that hospice care has developed into a concept
Sexual Health and Well-Being of care, as it is not limited to the hospice building
itself. The term 'palliative rehabilitation' has been
Sexuality is fundamental to an individual's health
developed in recognition that there is an ongoing
and well-being, irrespective of whether a disability
adaptation and a re-adjustment to living with a
is involved. These needs to be addressed in adults
deteriorating condition
with muscular dystrophy, It is not just about the
sexual act. It may be about how medication or Caring for the Caregivers
incontinence issues affect this aspect of their life. It
Occupational therapists also have a duty of care to
is also about how they view themselves as a sexual
ensure the needs of the parents and caretakers are
person.
addressed separately from those of the person with
Bereavement and Anticipatory Grief muscular dystrophy. It is necessary to be both
aware and sensitive to the possibility of the different
Individuals with muscular dystrophy experience
experiences and depths of loss when working with
the loss of muscle strength and associated functions children and young people with muscular
and skills. The loss experienced is ongoing as the dystrophy.
condition progresses. This loss is observed but not
always understood by the health professional. In The focus at all times for the occupational therapist
addition, in Duchenne's muscular dystrophy as the is on living and enabling
young man reaches his late teens and early twenties, independence but the attitude and approach of the
he becomes acutely aware of his own prognosis. therapist are fundamental to a positive working
This is compounded by the deterioration and death relationship with the young man and his family.
of his peers. The impact of these deaths and the Tact, sensitivity and diplomacy are required by the
proximity to the young man himself cannot be occupational therapist together with an insight into
underestimated, although it is not always fully the difficulties which a family may be experiencing.
recognised. When a realisation or anxiety of
impending loss is experienced in advance of the PSYCHOLOGICAL
loss, this is anticipatory loss Anticipatory loss can REHABILITATION
be experienced by people close to the person, too. IN MUSCULAR DYSTROPHY
Bereavement can be understood to be an emotional
and psychological event, which Introduction:
may occur several times in one's life. It affects one's A patient diagnosed with Muscular Dystrophy and
sense of well-being and provokes questions of a his caregivers go through a lot of psychological
spiritual and religious nature, challenging one's changes. This starts with having very little
existence, sense of meaning and purpose. knowledge about the disease which leads to
Bereavement and the associated mourning can also confusion, eventually getting to know about not
388 Neuro-Rehabilitation : A multi disciplinary approach

many ways to cure it, or lack treatment plan, hyperactivity or impulsivity, which would differ
deteriorating condition, family issues, social in their type like predominantly hyperactive-
withdrawal, and embarrassment about the impulsive type, predominantly inattentive type and
condition and eventually coming in terms about the combined type. These patients suffer from muscle
issue of death. During this process the patient and weakness and physical limitations, the symptom
the caregivers undergo many emotional and of hyperactivity may be less likely in patients with
psychological changes like confusion, stress, muscular dystrophy.
anxiety, restlessness, irritability, etc. Hence, as the
patients undergo psychological distress they need Cognitive Deficits:
psychological help which would enhance their Role of dystrophin: While scientists are still figuring
overall well being and would help the patient and out the exact role of dystrophin in the brain, as some
their caregivers to cope with the situation in a research suggests that not having dystrophin seems
healthier manner. to cause an increased risk for specific weakness and
learning difficulties, this does not suggest that all
Comorbid Disorders: patients with muscular dystrophy will have deficits
in these areas.
Autism:388
Research shows an increased risk of Autism in Developmental Delay and Intellectual Ability:
children with Duchenne's muscular dystrophy. The most common delays are in gross motor skills
Problems with, nonverbal communication skills, like sitting, walking, etc. However, patients with
language delay, repetitive language, excessive Duchenne's muscular dystrophy are at an increased
fixation to an object, poor socialization skills and risk for delays in speech development, fine motor
deficits in attention appears to be a common feature skills, etc. Patients suffering from Duchenne's
of autism in children with Duchenne's. Children muscular dystrophy are at an increased risk for
suffering from Duchenne's muscular dystrophy having an intelligence quotient that ranges from
who are suspected of suffering from autism or below average to mental retardation (i.e. IQ below
showing symptoms of autism should be assessed 70).
by a mental health or behavior professional such
as a psychologist, psychiatrist, neurologist, or Attention Span and Memory:
developmental pediatrician. The amount of information that a patient with
muscular dystrophy is able to grasp in one time
It has been seen that as age increases the autistic
(short term memory) may be less as compared to
symptoms in these children reduce in the following
other children. It is usually seen that Duchenne's
areas: In addition, some autistic behaviors in
muscular dystrophy patients with memory
children with Duchenne may improve with age,
cognitive deficits or who have good IQ score would
including: Reciprocal conversation, sharing interest
have difficulty in verbal information and visual
or enjoyment, make a believe play activity, verbal
information. These patients have slow processing
and nonverbal communication.
speed and also have difficulty multi-tasking.
Obsessive-Compulsive Disorder: Executive Functioning:
There may be an increased prevalence of obsessive They have difficulty in planning, organizing, goal
and compulsive like behaviors in patients with oriented behaviours and self-analysis. Mental
Duchenne's muscular dystrophy. In some cases flexibility in particular appears to be more
obsessive-compulsive (OC) behaviors may be due problematic in Duchenne's than in the general
to sensory sensitivities or due to deficits in mental population because of which these patients have
flexibility or adaptability. While many Duchenne difficulty in adapting to expected changes or
children may have these tendencies, most would transitioning from one activity to another.
not be severe enough to receive an OCD diagnosis.
Learning Disabilities:
Attention-Deficit/Hyperactivity Disorder Research suggests that children with Duchenne are
(ADHD): at increased risk for all three types of specific
Patients with muscular dystrophy are at a higher learning disabilities: dyslexia (reading disorder),
risk of suffering from attention deficit hyperactivity dyscalculia (mathematics disorder), and dysgraphia
disorder. Symptoms would signify inattention, (disorder of written communication) (13)
Muscular Dystrophy 389

Emotional Impact in Muscular Dystrophy: opportunity to discuss the impending death in an


The patients go through a lot of emotional changes accepting environment with the psychologist who
and for which they need emotional support, is experienced in handling patients with muscular
understanding, love and patience to ensure their dystrophy (15)
emotional well being. There may be times when it's
difficult to keep up with the progression of the PSYCHOLOGICAL ASSESSMENT:
disease, anger, frustration, embarrassment, sadness The most crucial time to consider assessment
or anxiety. These emotional issues may occur includes the time around diagnosis as this is the
especially during the developmental period or as window period of adjustment after diagnosis for
the disease progresses (14). the patients and the family members.
Behavioural, Emotional Adjustment or Coping:
Social Isolation: Patients with muscular dystrophy should undergo
As the level of mobility decreases, there is a sense a brief screening of emotional status either in every
of loss of independence and this could lead to social 6 months or annually, as many of the patients slip
isolation and depression. Patients with late onset into depression and this may worsen their physical
in muscular dystrophy may prefer living in condition. Standardised tests like Beck's depression
isolation if they don't have enough family support inventory, Hamilton's anxiety rating scale, etc. can
to keep them socially isolated. These patients have be used to evaluate their emotional state.
a low self-esteem and concept which hinders them
from socializing. Neurocognitive:
Comprehensive developmental or
Depression and Anxiety: neuropsychological assessments is recommended
According to studies it has been found that as at near the time of diagnosis as this would help
compared to normal people, people suffering from parents either to go in for a normal school or special
Muscular Dystrophy stand higher chances from school to cater to the special needs of the child.
suffering from depression and anxiety. Standardised performance and verbal based tests
or rating scales are used to evaluate the IQ or the
Behavioural Issues: cognition of the patient like Wechsler's Intelligence
Scale for Children, Wechsler's Adult Memory Scale,
Behaviour problems are quiet common in children
etc. (16)
who are diagnosed as Duchenne's muscular
dystrophy. Young boys with Duchenne's have more PSYCHOPHARMACOLOGICAL
difficulty with impulsivity and emotional control INTERVENTIONS:
than other children of the same age. Most of them
This should be considered for the treatment of
are likely to be inflexible in their thinking which
moderate to severe psychiatric symptoms as a part
may result into non-compliance or oppositional.
of multimodal treatment plan that includes
Aggression: Patients with muscular dystrophy are appropriate psychotherapies and educational
at a higher risk of having significant problems with interventions.
following directions, temper tantrums, problems
with arguing and refusing to do what they are asked Intervention for Co- morbid Disorders:
to do. Behaviour Therapist with the help of Autism: A patient who is suffering from both
Behaviour modification therapy would be helpful Muscular dystrophy and Autism needs to be treated
in developing alternative strategies to modify the for both but keeping in mind which disorder or
likely triggers to negative behaviours. disease is causing maximum dysfunction needs to
be tackled at first. There are several interventions
Family Issues: specifically to reduce autistic features like
When the patient's starts showing signs of muscular pharmacotherapy for under the guidance of a
dystrophy initially the parents overlook the disease psychiatrist, applied behaviour analysis which is
but as the disease progresses and after the diagnosis conducted by a psychologist, cognitive therapy,
is confirmed, the parents go through an emotional floortime, occupational therapy, animal therapy,
turmoil. As, the patients and family members group therapy and TEACCH. Other interventions
undergo, emotional stress, frustration, anxiety, are usually problem-focused, and designed to
depression, etc. they should be given the address specific problem areas.
390 Neuro-Rehabilitation : A multi disciplinary approach

Obsessive Compulsive Disorder: ADHD has on their daily activities. This


After listing the obsessions and compulsions of a includes things like increasing more structure
patient the patient usually undergoes various and oversight during activities, implementing
psychotherapies, of which cognitive-behaviour compensatory strategies at school or home,
therapy has been very effective in treating OCD developing supports for memory and
patients, along with it other forms of therapy like organizational weaknesses, and implementing
systematic desensitization and pharmacotherapy is routines that can be followed consistently.
administered if nonpharmacological treatment is Intervention for Emotional and Behavioural
not successful. Few strategies for the caregivers of Issues:
OCD with comorbid muscular dystrophy patients
are: • Psychotherapy:
1. A calm, supportive atmosphere is important The primary focus of psychotherapy should
to overcoming OC behaviours. be the patient, where the patients emotions,
maladaptive behaviour, poor socialization
2. If your child is struggling with significant OC
should be taken care of with the help of various
behaviours at school, provide the school with
psychotherapies like cognitive therapy,
a written summary of your child's OCD
cognitive behaviour therapy, rational emotive
challenges and needs.
behaviour therapy, reality therapy, play
3. School accommodations need to be therapy, art therapy, etc should be
individualized based on a student's unique administered during the psychological session
pattern of OCD behaviours and coping skills. depending on the patients problems.

Attention Deficit Hyperactivity Disorder: • Individual Therapy:


Treatment and intervention often has three
This is suggested for internalizing behaviours
components:
like depression, anxiety, low self- esteem,
• The use of medication is the most effective adjustment and coping difficulties.
component for many boys. Approximately 80
to 90% of children with ADHD obtain some • Group Therapy:
benefit from medication, although this is not This is recommended as the patients are
always complete improvement. Stimulants are mostly socially withdrawn and cuddle into a
the most commonly prescribed medications. nutshell, as this is quiet common in patients
Most boys with Duchenne respond well to with muscular dystrophy as with their
treatment with stimulant medication. deteriorating condition they become
However, stimulant medications should be increasing shy, embarrassed so in order to get
used with extreme care in any child with heart them out its necessary that they undergo group
problems, which are common in older boys therapy which would help them resolve their
with Duchenne. Thus, the doctor should issues about themselves and help them build
closely monitor the cardiac status of boys with confidence. Group therapy for children could
Duchenne who are prescribed stimulant be of various forms i.e. Art therapy, Play
medications. therapy, etc.
• Another component of ADHD intervention
• Family Therapy:
consists of psychosocial strategies.
Behavioural therapy is very effective in This is quiet important as the patient becomes
changing the core features of hyperactivity, dependent on the family members for all his
impulsivity, and inattention. Behavioural needs, which leads to added burden to the
therapies are usually the most effective before family members and a change in their lives.
the child is 10 or 11 years old, so early The family members should be well equipped
intervention is the key. to cope with the situation avoid caregiver's
burnout and at the same time help support the
• The third component of ADHD intervention patient. Early in the child's life, the family
usually involves modifying or adapting the should be guided to encourage the child's
child's environment to reduce the impact that independence and to discourage
Muscular Dystrophy 391

overprotection. It is important to help the child • Try to keep calm when a child is misbehaving.
and the family identify realistic goals for Angry parents and teachers tend to escalate
independence (17). the situation. This would worsen the situation
as the child would feel neglected and that
• Psychoeducation for Caregivers: nobody understands him/ her.
Parental management and training, where the • Focus on the positive. Strategies that only focus
parents and caregivers are guided on the ways on punishment do not promote positive
to help them and the patient cope with the behaviours, increase motivation, or change
situation and to avoid parent-child conflict attitudes. Rewarding/praising/encouraging
which could aggravate and add on to the good behaviour is more effective in the long
existing problems. run. Look for opportunities to say "yes" instead
of "no." ("Yes, you can have a cookie, after
• Applied Behaviour Analysis: you").
This is especially needed if a patient has
comorbid, psychological disorders like autism
or attention deficit hyperactivity disorder.
References:
• Pharmacological Intervention: 1. [Link]: Neurological
Selective Serotonin re-uptake inhibitors are Rehabilitation, Fifth edition.
prescribed for patients with muscular
2. Muscular dystrophy : Hope through research.
dystrophy who also suffer from depression,
anxiety and obsessive compulsive disorders. 3. Laura.E. Case: Physical therapy management
Mood stabilisers are prescribed for aggression, of dystrophinopathies; Parent Project
anger and emotional dysregulation. Muscular Dystrophy Annual Conference, July
Stimulants are prescribed for attention-deficit 2006.
hyperactivity disorder. 4. [Link] : Handbook of Physical
Medicicne and Rehabilitation, 2006.
Strategies for Caregivers:
5. Kenneth W. Lindsay, Ian Bone, Robin
Ways to Mange Aggressive and Difficult
Callander:Neurology and neurosurgery
Behaviour:
illustrated, Fourth edition.
• Develop a routine for the patient for the week
6. The diagnosis and Management of Duchenne
and stick to it. Explain to the patient the time-
Muscular Dystrophy: a guide for families -
table and do incorporate rewards as children
March 2010.
are usually noncompliant after exercising
everyday. 7. Wayne Stuberg: MMI Symposium : Physical
Management ; Challenge for mobility : April
• Recreational activities need to be also
2010.
incorporated in the time table as, it is very
important to have something to look forward 8. Disability / Condition : Duchenne Muscular
to during the day that motivates and does not Dystrophy and Exercises, 2007.
let the patient be depressed about his or her
9. Bushby K, Finkel R, Birnkrant DJ, Case LE etal
condition For example: Painting or playing
: DMD care consideration working group.
board games with siblings or watching
Diagnosis and Management of Duchenne
television.
Muscular Dystrophy, Part 2. Implementation
• Explain to the patient the situation if there are of multidisciplinary care : Lancet Neurol 2010.
any changes made or if he is taken for some
10. Muscular Dystrophy Association India-
therapy that he denies to undergo, for example
Nutrition, 2006-2009.
if the patient does not want to exercise on a
particular day explain to him the situation, 11. [Link] : Goel's Physiotherapy :
how doing so would have repercussions in the Physiotherapy in medical conditions, surgical
long run and set up a reward system if conditions & sports injuries. 2nd edition.
necessary. Vol 2.
392 Neuro-Rehabilitation : A multi disciplinary approach

12. [Link] Sharma : Importance of 15. Nereo NE, Hinton VJ. Three wishes and
Neurorehabilitation in Muscular Dystrophy psychological functioning in boys with
:Stem Cell Therapy & Other Recent Advances Duchenne muscular dystrophy. J Behav Dev
in Muscular dystrophy. Pediatr. 2003;24:96Y103.
13. Hinton, V.J., Fee, R.J., Goldstein, E.M., & De 16. Cotton, S., Crowe, S.F., & Voudouris, N. (1998).
Vivo, D.C. (2007). Verbal and memory skills Neuropsychological profile of Duchenne
in males with Duchenne muscular dystrophy. muscular dystrophy. Child Neuropsychology,
Developmental Medicine and Child 4, 110-117.
Neurology, 49,123-128.
17. Thompson RJ, Zeman JL, Fanurik D, et al. The
14. Gartstein MA, Short AD, Vannatta K, et al. role of parent stress and coping and family
Psychosocial adjustment of children with functioning in parent and child adjustment to
chronic illness: an evaluation of three models. Duchenne Muscular Dystrophy. J Clin
Dev Behav Pediatr. 1999;20:157Y163. Psychol. 1992;48:11Y19.
Motor Neuron Disease 393

Ch.10 Motor Neuron Disease


Dr. Mamta Lohia, [Link], F.N.R,CBE(USA), [Link] Mishra, [Link], F.N.R.,
Ms. Akshata Shetty, M.A.(Clinical Psychologist), Dr. Manasi Jani(Speech Therapist).
MND is also known as Charcot's disease, and Lou sclerosis" refers to hardening of the anterior and
Gehrig's disease or Amyotrophic lateral Sclerosis. lateral corticospinal tracts as motor neurons in these
It is characterized by progressive degeneration of areas degenerate and are replaced by gliosis. Thus
anterior horn cells of the spinal cord causing lower ALS exhibits mixed presentation of UMN and LMN
motor neuron type of weakness ; the corticospinal symptoms which is the diagnostic criteria.
tracts causing upper motor neuron symptoms ; and The syndrome of progressive muscular atrophy
certain motor nuclei of the brain stem leading to (PMA) accounts for 5-10% of patients with MND,
bulbar symptoms. It is one of the most devastating and indicates a pure lower motor neuron syndrome
types of neurological disorder, with no known without accompanying upper motor neuron signs.
cause. There is gross wasting and weakness, along with
fasciculations in small muscles of all four limbs,
Prevalence : leading to foot drop and reduced grip strength.
The prevalence of this disorder is 5 per 1,00,000 and Unlike ALS, this disease progresses very gradually
there is a male to female ratio of 2:1[17] upto 10-15 years. [19]

In about 10% of cases, ALS is caused by a genetic Primary lateral sclerosis (PLS) is a clinically
defect. In the remaining cases, the cause is progressive pure upper motor symptoms that
unknown. cannot be attributed to any other disease process.
Patient may have involvement of both corticospinal
There are no known risk factors, except for having and corticobulbar paths. Pathologically there is loss
a family member who has a hereditary form of the of pyramidal cells in the precentral gyrus and
disease. cerebral cortex, with degeneration of corticobulbar
MND manifests in different forms like: and corticospinal tracts.
1. Amyotrophic lateral sclerosis (ALS) Progressive Bulbar Palsy is a condition with
2. Progressive muscular atrophy (PMA) involvement of motor nuclei in the lower brain
stem. There is primary involvement of the bulbar
3. Progressive bulbar palsy (PBP)
muscles like the jaw muscles, facial muscles, tongue,
4. Primary lateral sclerosis (PLS) larynx and pharynx. Hence patient suffers from
Amyotrophic lateral sclerosis (ALS) can be defined dysphagia and dysarthria. As muscles of
as a neurodegenerative disorder characterized by mastication and deglutition are affected the food
progressive muscular paralysis reflecting particles get stuck to one corner of the mouth
degeneration of motor neurons in the primary Weakness of the pharyngeal muscles causes
motor cortex, brainstem and spinal cord. It usually improper pushing of food particles into oesophagus
begins at the age of 40 years of age. "Amyotrophy" giving rise to choking. Fibrillation of tongue is a
refers to the atrophy of muscle fibers, which are common symptom. It is a fast progressive disorder
dennervated as their corresponding anterior horn leading to death with in a span of 2 years of
cells degenerate, leading to weakness of affected beginning of symptoms and mainly due to
muscles and visible fasciculations. "Lateral respiratory failure.

Subtype Nervous System Pathology


Amyotrophic Lateral Sclerosis Degeneration of the corticospinal tracts, neurons in the motor
cortex & brain stem & anterior horn cells in the spinal cord.
Primary Lateral Sclerosis Degeneration of upper motor neurons
Progressive Bulbar Palsy Degeneration of motor neurons of cranial nerves IX to XII
Progressive muscular atrophy Loss or chromatolysis of motor neurons of the spinal cord &
brainstem.

(Reference: Rowland, LP:Diverse forms of Motor neuron [Link] Neurol 36:1, 1982.)
394 Neuro-Rehabilitation : A multi disciplinary approach

Clinical Features: have noticed fasciculations (noticed as involuntary


muscle twitching) or cramps preceding the onset
The features of ALS were first clearly described as
of weakness or wasting for some months (or years).
a clinico-pathological entity by Jean Martin Charcot
The weakness is usually of insidious onset, and
in 1869
patients may notice that symptoms are exacerbated
Approximately two thirds of patients with typical by cold weather. Although it is usually
ALS have a spinal form of the disease (classical asymmetrical at onset, the other limbs develop
'Charcot ALS'). They present with symptoms weakness and wasting sooner or later, and most
related to focal muscle weakness where the patients go on to develop bulbar symptoms and
symptoms may start either distally or proximally eventually respiratory symptoms.
in the upper limbs and lower limbs. Patients may

Lower motor neuron symptoms showing weakness and


wasting of thenar and hypothenar muscles.
Patients with bulbar onset ALS usually present with develop sialorrhoea (excessive drooling) due to
dysarthria. Rarely, patients may present with difficulty in swallowing saliva and mild UMN type
dysphagia for solid or liquids before noticing bilateral facial weakness which affects the lower
speech disturbances. Limbs symptoms can develop part of the face. 'Pseudobulbar' symptoms such as
almost simultaneously with bulbar symptoms and emotional lability and excessive yawning are seen
in the vast majority of cases will occur within 1-2 in a significant number of cases.
years. Almost all patients with bulbar symptoms

Common Impairments Associated with MND


Type of Impairment / Location Clinical Manifestation of Pathology
Impairments related to LMN pathology Muscle weakness, hyporeflexia, hypotonicity, atrophy,
muscle cramps, fasciculations.
Impairments related to UMN pathology Spasticity, Pathological reflexes, hyperreflexia, muscle
weakness.
Impairments related to bulbar pathology Dysphagia,dysarthria,sialorrhea,peudobulbar affect.
Respiratory Impairments Exertional dyspnea, nocturnal respiratory difficulty,
orthopnea,hypoventi lation.
Other Impairments Dementia, Cognitive Impairments
Rare Impairments Ocular Palsy, bowel & bladder dysfunction.
Indirect & Composite Impairments Fatigue, weight loss,cachexia,decreased ROM,pain,gait
disturbance,depression,anxiety,deconditioning,balance &
postural control impairments
Motor Neuron Disease 395

Summary of Revised El Escorial Research


Diagnostic Criteria for ALS (Brooks et al., 2000)

The diagnosis of ALS requires:


1. Evidence of LMN degeneration by clinical,
electrophysiological or neuropathological
examination;
2. Evidence of UMN degeneration by clinical
examination, and
3. Progressive spread of symptoms or signs
within a region or to other regions, as
determined by history or examination,
Together with the absence of:
Facial Muscles Weakness
1. Electrophysiological and pathological
On examining the cranial nerves, the jaw jerk may evidence of other disease that might explain
be brisk, especially in bulbar-onset disease. An the signs of LMN and/or UMN degeneration,
upper motor neurone type facial weakness affects and
the lower half of the face causing difficulty with lip
seal and blowing cheeks, but often varying degrees 2. Neuroimaging evidence of other disease
of UMN and LMN facial weakness coexist. The gag processes that might explain the observed
reflex is preserved and is often brisk while the soft clinical and electrophysiological signs
palate may be weak. Patients develop fasciculations UMN signs: Clonus, Babinski sign, absent
and wasting of the tongue, and tongue movements abdominal skin reflexes, hypertonia, loss of
are slowed due to spasticity. Sensory examination dexterity.
is almost always normal. Respiratory failure and
other pulmonary complications are the usual cause LMN signs: atrophy, weakness. If only
of death in ALS. [19] fasciculation: search with EMG for active
denervation. Regions reflect neuronal pools: bulbar,
cervical, thoracic and lumbosacral.

Pathogenesis of motor neuron


degeneration in MND
Most ALS cases are sporadic but 5-10% of cases are
familial, and of these 20% have a mutation of the
SOD1 gene and about 2-5% have mutations of the
TARDBP (TDP-43) gene. Two percent of apparently
sporadic patients have SOD1 mutations, and
TARDBP mutations also occur in sporadic cases.
The diagnosis is based on clinical history,
examination, electromyography, and exclusion of
'ALS-mimics' (e.g. cervical spondylotic
myelopathies, multifocal motor neuropathy,
Kennedy's disease) by appropriate investigations.
The pathological hallmarks comprise loss of motor
neurons with intraneuronal ubiquitin-
immunoreactive inclusions in upper motor neurons
and TDP-43 immunoreactive inclusions in
degenerating lower motor neurons. Signs of upper
motor neurone and lower motor neurone damage
Tongue Fasciculation , atrophy of
not explained by any other disease process are
tongue muscles.
suggestive of ALS.
396 Neuro-Rehabilitation : A multi disciplinary approach

The clinical diagnosis is confirmed by also be tested to evaluate aerobic conditioning.


Electromyography (EMG) and muscle biopsy. On
10. Because of being in bed for long time without
EMG, the motor neuron conduction velocity is
mobility there are chances of getting trophic
normal until late in the disease, but the amplitude
ulcers: periodic skin inspection should be
is reduced. On needling, the mechanical stimulation
done.
of the needle causes fibrillation potentials, and
spontaneous fibrillation and fasciculation potential 11. Functional Status: Functional Independence
are seen when the needle is stationary in relaxed Measure (FIM) can be used to document
muscle, with greater duration and amplitude of the functional status.
action potentials. When the muscle contracts there 12. Environment Barriers: should be considered
is marked decrease in the number of spikes leading for easy accessibility and safety.
to incomplete interference pattern.
13. Fatigue: Fatigue Severity Scale to be used.
Examination :
1. Joint Integrity, Range of Motion and Muscle Speech Affection in MND :
length: is examined using standard tools. Motor neuron disorder affects the motor neurons
2. Pain : seen in ALS is assessed by Visual Analog of the brain and the spinal cords. Depending on
Scale (VAS) the type of motor neurons affected, the signs and
symptoms vary. The patients where the upper
3. Muscle Performance: is examined by Manual motor neurons are affected present with brisk
Muscle Testing (MMT), isokinetic muscle reflexes/hypertonicity in the jaw, tongue, lips and
strength testing or hand -held dynamometer. palatal and pharyngeal areas. The patients where
4. Motor Function: Due to Spasticity, and the lower motor neurons are affected present with
weakness of muscles there could be many reduced tonicity, weakness, muscle wasting and
manifestations like Impairments in dexterity, fasciculations. Usually, individuals with motor
incoordination of both gross and fine neuron disorder have both upper as well as lower
movements as well as loss of motor control. motor neuron affected hence present with mixed
Therefore Functional assessment of both symptoms.
upper and lower extremities should be done.
Specific Measures for MND :
5. Tone and Reflexes: Tone can be assessed by
ALS Functional Rating Scale (ALSFRS): The
Modified Ashworth Scale and reflexes by deep
functional status of ALS patients can be rated by
tendon reflexes.
ALS Functional Rating Scale (ALSFRS) and revised
6. Cranial Nerve involvement should be version ALSFRS-R It correlates with muscle
assessed. Pseudo Bulbar and Progressive strength of both upper and lower limbs. ALSFRS-
Bulbar varieties of MND only show R includes respiratory muscles measures also.
involvement of cranial nerves.
7. Postural malalignment and imbalance are seen Conventional therapies:
which can be assessed by Tests like Tinetti The management of ALS/MND has considerably
Performance Oriented Mobility Assessment changed over the past two decades, with an
(POMA), Berg Balance Scale, Timed Up andGo emphasis on coordinated multidisciplinary care
Test and Functional Reach Test between specialist, like physician, speech therapist
8. Gait: Deviations due to muscle imbalance occupational therapist, physical therapist, dietician
should be assessed, so also endurance. and nurses. Advanced directives are on end of life
care, respiratory and nutritional management
9. Respiratory Function: There could be during late stages of life are focused on.
involvement of respiratory muscles resulting
into breathlessness, Low vital capacity and Riluzole is the only approved drug that has been
lack of cough effectiveness. Therefore shown to have a modest effect on prolonging life
Respiratory Function evaluation should be in ALS patients. The mechanism of action of riluzole
done in detail by using a hand-held is not entirely certain but is thought to include
spirometer. Aerobic capacity and interference with N-methyl-D-aspartate (NMDA)
cardiovascular pulmonary endurance should receptor mediated responses, stabilisation of the
Motor Neuron Disease 397

inactivated state of voltage-dependent sodium


channels, inhibition of glutamate release from pre-
synaptic terminals, and increasing of extracellular
glutamate uptake, thereby preventing degeneration
of motor neurons.
Treatments to control symptoms are also helpful:
• Baclofen or diazepam may be used to control
spasticity that interferes with daily activities.
• Trihexyphenidyl or amitriptyline may be
prescribed for patients with problems in
swallowing their own saliva. Stage 1 Patient, exercises to strengthen the knee
• Choking is a common symptom. Patients may flexor muscles with resistive theraband exercises.
decide to have a tube placed into their stomach
for feeding. This is called a gastrostomy or
Percutaneous Endoscopic Gastrostomy (PEG).
A nutritionist's help is very important for them
as they tend to lose weight. The illness itself
increases the need for food and calories. At
the same time, problems with swallowing
make it difficult to eat enough.
Role of Physiotherapist in Motor Neuron Disease:
The efficacy of therapeutic interventions is related
to:
1. Timing of interventions,
2. Motivation and persistence of patient in Stage 1 Patient, exercises like all fours given for
carrying out the program. weight bearing and strengthening of trunk and limb
3. Support from family members. muscles.
Rehabilitation intervention plan depends on the
following:
1. The rate of progress of the disease
2. Presence of spasticity, bulbar involvement,
respiratory involvement causing hypoxia and
fatigue.
3. Phase of Disease. Exercises are to prescribed
according to level of impairment, functional
limitation and level of disability
Phase I : Patient is independent in all ADL, is
ambulatory, except for mild weakness
and clumsiness in movements.
Stage 1: In case of mild weakness advice is to
continue normal activities.
In case of clumsiness, stretching
exercises like Yoga
In case of ambulatory patients, gentle Stage 1 Patient, exercises to strengthen upper limb
resisted exercises with caution not to muscles with weights within fatigue tolerance.
fatigue or over work.
398 Neuro-Rehabilitation : A multi disciplinary approach

Stage 2: Patient presents with moderate selective Stage 5: Patient presents with severe lower
weakness in ankles, wrist and hand extremity weakness,
Advice : Stretching exercises to avoid Advice : Emphasize on teaching family members
contractures. proper techniques of transfer and
positioning of patients limbs.
In case of decreased independence in
ADLs like climbing, overhead activities In case of severe upper extremity
and difficulty in buttoning etc, weakness, consider modifications at
strengthening exercises to be prescribed home like high dinning table to facilitate
avoiding fatigue. eating etc.
In case of difficulty in Ambulation,
Orthotic devices like AFO, hand splints
to be considered.

Stage 5 patient with weakness of antigravity lower


limb muscles and suspension exercises given within
fatigue threshold. Mainly knee musculature
suspension strengthening exs.
Stage 2 Patient, exercises to strengthen hip extensor
muscles (antigravity muscles of lower limb), in all
fours position.
Stage 3: Patient presents with fatigability in long
distance ambulation, needs supervision
in ADL.
Advice : Continue stage 2 and emphasize on
deep breathing exercises to be added.
In case of non-ambulatory patients,
consider wheelchair, (standard or
motorized).
Phase 2: (Partially Independent)
Stage 4: Patient may present with additional
pain and edema in hand and feet,
Advice : Consider modalities like massage,
elevation and active exercises.
In case of severe weakness in Stage 5 Patient, treated with Passive movements
extremities, caution is to be taken to of limbs and made to stand on standing board for
support the joints while doing rotations. physiological benefits of standing.
In case of Fatigability in ADLs, Phase 3 : (Dependent)
encourage isometric upto level of
tolerance and to consider slings or arm Stage 6: In case of totally bedridden patients
support, motorized chairs etc. with dysphagia, consider suction, soft
diet, tube feeding, PEG feeding etc.
Motor Neuron Disease 399

In case of severe breathing difficulty, breathing exercises in the form of incentive


frequent clearing of airways, spirometer.
tracheotomy and respiratory support if
• Proper positioning of patient is necessary
needed.[19]
because once the limbs become weaker and
wasting progresses, it becomes increasingly
difficult to maintain upright position for the
patient. This difficulty is noted while standing,
sitting in wheel chair or on bed, hence proper
positioning is necessary. At times patients also
develop neck drop. In order to minimize the
effect of gravity on the body, it is advised to
incline the patient back from vertical position,
as in this position the line of gravity passes in
front of head and neck through thorax. It also
causes relief of pressure of thorax on abdomen,
allowing diaphragm to work efficiently and
aid in breathing with ease, as their breathing
is restricted due to intercostal muscle
weakness.

Role of occupational Therapy in Motor Neuron


Disease
An occupational therapist (OT) is an integral part
of multidisciplinary team that cares for patients
Neck muscle strengthening exercises.
with motor neuron disease. They help to maintain
The research evidence suggests: mobility, function and independence in long term.
As the patient's condition deteriorates, OTs can
1. Overuse weakness does not occur in muscles advise on different ways of performing tasks and
with MMT grade 3(fair) or greater out of the selection, acquisition and adaptation using
5(normal). disability aids.
2. Moderate resistance exercises can increase Calman describes quality of life (QoL) in terms of a
strength in muscles with a MMT grade3 or match between an individual's hopes and
greater out of 5. expectations and current reality. For illnesses such
3. Strength gains are proportional to initial as MND, where there is no curative treatment, the
muscle strength. role of occupational therapist to emphasize on the
maintenance and improvement of Quality of
4. Heavy eccentric exercise should be avoided.
life.[21].
5. Exercises may produce functional benefits.
Primary objectives and aims
6. Psychological benefits have yet to be
determined. The primary objective of occupational therapy is
to enable individuals to participate in self-care,
Patients with severe respiratory and bulbar work and leisure activities that they want or need
complications may not benefit from active to perform, thereby optimising personal fulfilment,
exercise programs. The goal in end stage is to well-being and quality of life
optimize health and increase quality of life.
The tendency toward rapid progression of the
• Secondary complications in the form of disease necessitates regular review and
pressure sore and contractures should be reassessment, and makes planning for the future
avoided and when the patient goes into essential. Liaison with family and care givers is
respiratory failure, chest physiotherapy important, to ensure they are aware of the
becomes necessary. The therapist should make implications of the condition.
all efforts to maintain the chest expansion of
the patient by teaching and supervising
400 Neuro-Rehabilitation : A multi disciplinary approach

Occupational Therapy Intervention adductors of shoulder. Kneading dough and


Model of Human occupation- it facilitates ongoing forming clay objects give resistance and thus
assessment of the impact of the disease throughout strengthening arms and hands.
human system and the impact it has on Neck mobility and Strengthening exercises like -
occupational performance, patterns and order of Flexion, extension and rotation of neck can be given
behaviour and choices and motivation. for neck support patient can use soft collars and
It provides a good position from which to assess under chin supports.
the individual's social, physical and temporal
context, emphasising the experience of individual Management of Spasticity
and the uniqueness of their life. MOHO describes Active and passive range of motion exercises in the
three subsystems that contribute to our innate drive full range are given.
to act on the environment 1) Stretching: it reduces the spasticity directly in
Volition: the motivations, values, interests and the muscle being stretched by depressing
choices that drive our will to act on and engage with muscle spindle (Kaplan 1962) and it also
the environment reduces possibility of contractures.
Habituation: the automatic patterns, habits and 2) Bed mobility exercises, like rolling, shifting,
roles that shape our occupational behaviour all fours, forward backward shifting in all
fours etc (weight bearing exercises) reduce
Mind-brain-body performance: the
spasticity.
musculoskeletal, neurological, perceptual and
cognitive structures and system required to 3) Casting- casting in inhibitive postures has
produce behavioural output. been shown to be effective in tone reduction.
These three subsystems in this model resonate Joint problems: Muscle weakness can put strain
throughout each other. MOHO provides a reasoned on a joint, causing pain and stiffness. Inactive
approach to framing the presenting problems in muscles in the shoulder area may also result in a
context of the individual's unique experience of "stiff shoulder", which causes painful movement.
them. So gentle stretching and hot water fomentation can
help to relieve pain.
This approach considers occupational therapy
assessment and intervention designed to enhance Postural issues: The loss of normal postural control
and compensate for deteriorating function. may cause pain in the muscles of the neck,
shoulders, hips or knees. Electrically operated beds
Management for Upper extremity strength of and chairs are useful and special cushions and
the disease mattresses providing extra padding and support
In Motor neuron disease, after partial or complete can be helpful. The basic philosophy of seating is
denervation of muscle tissue and during inactivity same for all patients that "the body should be
or disuse, muscle strength decreases. When maintained in balanced, symmetrical and stable
strength is inadequate substitution pattern or trick posture that is both comfortable and maximizes
movements are likely to develop active, active- function (Barnes 1993).
assisted exercises are used to increase strength.
Adaptive devices for Hand functions
A muscle must contract at or near its maximal
capacity and for enough repetitions and time to Operating locks, door handles and light switches
increase strength. may become difficult and may require special
modification. Hands free telephones can alleviate
Many purposeful activities can be given to problems with the weight of a telephone receiver.
maintain/improve strength. For instance, leather Environmental control systems should be
lacing can offer slight resistance to the anterior considered earlier rather than later, as they can
deltoid if lace is pushed in upward direction.. involve a lengthy wait -- additional elements (TV,
Suspension exercises are advised for all the lights, door openers, etc.) can be added in at a later
shoulder muscles. date. Book rests, page turners and talking books
If the patient is able to hold pegs or blocks, reach can prove valuable.[22]
outs in sitting position to strengthen abductors and
Motor Neuron Disease 401

Unilateral weight bearing during peg transfers

Gripper exercise to improve hand muscles Finger flexor exercise to improve fine motor activity

Therapeutic activity to improve introssei function

Suspension exercises to maintain upper extremity muscles


402 Neuro-Rehabilitation : A multi disciplinary approach

Modification for self care management caused by unstable joints, weight loss exposing
bony prominences, muscle cramps and spasticity.
With reduced use of arms and hands, toileting can
be a problem, so according to patient conditions Many patients will begin to have difficulty walking.
Occupational therapist should advise for toilet Loss of balance due to foot-drop, muscle atrophy,
modification like raised toilet seats. Clothing can and spasticity can make walking extremely difficult
also be difficult to manage because of poor hand and dangerous. A typical progression in mobility
functions like difficulty in buttoning, zipping or aids is: ankle-foot orthosis (AFO), then cane, then
doing other fine motor activities. Velcro can walker, then manual wheelchair, then power
overcome problems with zips and buttons and it wheelchair. While most patients will
will facilitate dressing and independence for toilet understandably resist the use of a mobility aid for
purposes. Devices such as dressing sticks and sock as long as possible, it is important that they accept
aids may be difficult due to their weight. Bathing that they are going to get weaker and that mobility
is another likely problem. The solution needs to take equipment can help them maintain independence,
into account the likely progression. If an adaptation conserve energy and most importantly, avoid the
to the bathroom is being considered, grab rails, a perils of a serious fall and related injury. This last
level access shower will allow for longer term needs point cannot be over emphasized. Catfish Hunter,
whilst an over bath shower is likely to serve only the famous baseball player who was stricken with
in the short term. Commodes and shower chairs ALS, died relatively soon after diagnosis as a result
can be useful. of a head injury sustained as a result of a serious
fall. Living with ALS is challenging enough without
Equipment for Dressing, Grooming and the added burden and pain of injury.
Feeding
Ankle Foot Orthosis (AFO)
Dressing
One common mobility symptom resulting from
velcro for assistance with clothing and shoes
MND is the inability to hold the toe of one or both
long handled aids for washing and hair feet up while walking. This is commonly referred
Grooming to as foot-drop and results in the patient having to
toothbrush holders and toothpaste lift the foot more than normal while walking to
avoid tripping. Correcting foot-drop with a
squeezers
lightweight ankle-foot orthosis can be helpful to
wash mitts minimize falls and maintain endurance.
Alternatively, low-heeled cowboy boots may be
Feeding
helpful if the patient does not have hip weakness
modified cups, plates and cutlery and if the extra weight of the boots is not
non-slip mats burdensome.
cup holders AFO's are made of lightweight plastic and are
collars available in a variety of types, styles and cost
splints ranges. The simplest and least expensive is the off
the shelf, catalog variety. These are a one-piece unit,
Management for Transfer and Mobility which come in a variety of sizes. The best and most
expensive are custom made devices, which
With increased difficulty in transferring, moving incorporate a spring-loaded hinge at the ankle.
and handling equipment may be useful. An Unfortunately, as with most things, you get what
adjustable height bed can help reduce risk to carers you pay for. The catalog variety tends to be less
if it is necessary to provide personal care in bed. functional and much less comfortable due its one
Increasing problems with mobility bring increased piece, one size fits all design.[23]
risk of pressure sores, and regular formal
assessment of risk is essential. Special cushions and Example of walking aids
mattresses may be necessary. When providing hoist
slings, seating, shower chairs, mattresses and Canes
pressure relief cushions, consideration should be
There are basically three styles of cane available,
given to pain experienced by the person, often
Motor Neuron Disease 403

the standard cane, tripod cane and the quad cane. • walking frame
The standard cane has a single tip, the tripod has a • rails/ramps
triangular (three tip base) while the quad cane has
• splints
a rectangular four tip base for improved stability.
The quad cane is typically considerably heavier and • wheeled trolley
can actually be more awkward making it less stable • wheelchairs - carer or patient operated,
depending on patient balance and strength. Newer • manual/electric
model quad canes are designed using lightweight
plastic making them lighter and allowing slight flex Management for fatigue
which provides some self-leveling. Which style
works best for an individual patient will vary Fatigue is common in MND. By recognising the
depending on condition and can only be adequately factors that worsen symptoms and by learning how
determined by having the patient try each style. to conserve energy, people with MND can improve
their quality of life. Some strategies are: plan
Walkers activities in advance, take regular rest periods, rest
between activities and before going out, do not
When a cane does not provide enough support and
exercise to the point of excessive fatigue, cramps,
the risk of falling becomes more frequent, it is time
or muscular weakness. (refer to MS chapter for
to upgrade to a walker. There many styles and
some energy conservation techniques)
designs of walkers. The standard walker has wheels
in front, grippers for hard surfaces or glides for Home Modification
carpets in the back and typically folds up for travel.
More elaborate walkers are available with features 1-Use of grab bars to prevent falls and further
such as larger wheels, three or four wheel designs, injury.
hand brakes, baskets for carrying items and fold 2- If the patient is wheelchair bound, there should
down seats. Most tend to be larger and heavier than a ramp will increase independence in indoor
the standard walker but typically fold up for mobility. Home should be modified according to
transport. patient's needs like adjustment of furniture,
increasing door width, removal of architectural
Manual Wheelchairs barriers etc.
If walking becomes more difficult and exhausting, Kitchen tasks are likely to be problematic early in
it is usually a good idea to obtain a manual the course of the disease, with difficulties in lifting,
wheelchair to use for longer outings. This carrying, opening jars and reaching higher shelves.
wheelchair can also be utilized long term as a Equipment such as vegetable draining baskets, jar
backup to a power wheelchair. Manual openers, tin openers and trolleys may help, as may
wheelchairs, like power wheelchairs, are optimally a rearrangement of the kitchen surfaces to facilitate
custom fitted for the individual patient. Important sliding objects from one area to another instead of
features include lightweight folding construction, lifting. Upper limb difficulties may also necessitate
removable wheels, removable flip up footrests, use of modified cutlery, cups and plates, non-slip
removable or flip up armrests and a comfortable mats, cup holders and arm supports.[24]
seat.
Use of Adaptive Devices in MND
Power Wheelchairs Adaptive devices promotes greater independence
As the disease progresses many MND patients will by enabling people to perform tasks that they were
eventually need a power wheelchair. There are a formerly unable to accomplish, or have great
large variety of power wheelchair designs and difficulty accomplishing, by providing
options available with costs varied range. While a enhancements to, or changing methods of
low cost wheelchair will probably suffice early on interacting with, the technology needed to
it will quickly become inadequate as the disease accomplish such tasks.
progresses. Accepting any adaptive equipment can be difficult
for the person, as the rapidity of the progression of
Equipments for Mobility
the disease allows little time for adjustment. The
• walking sticks
404 Neuro-Rehabilitation : A multi disciplinary approach

psychological impact of having to rely on a hoist developmental patterns for specific types of
can be devastating, so the issue needs to be intellectual disabilities.[14] AAC can be used to aid
approached carefully, and with a positive emphasis both spoken and written language, and can
on maintaining independence. supplement or replace speech and writing as
necessary. AAC can be a permanent addition to a
Adaptive devices for Communication person's communication or a temporary aid.[10]
The systems used in AAC include gestures, hand
Communication problems can include writing,
signals, photographs, pictures, line drawings,
using phones and speaking. Some people need to
words and letters,[25] which can be used alone or
use speech synthesisers or other communication
in combination to communicate.[26]
equipment.. Dysphagia is another difficulty to be
aware of, due to damage to some of the cranial Adult AAC users generally have satisfying
nerves (hypoglossal, accessory, vagus, trigeminal, relationships with family and friends and engage
facial, glossopharyngeal). in pleasurable and interesting life activities.

Augmentative and alternative communication Accessible input devices for computers


(AAC) This is a sip-and-puff device which allows a person
with substantial disability to make selections and
navigate computerized interfaces by controlling
inhalations and exhalations.
QWERTY Key board is the most common modern-
day keyboard layout.

An AAC user uses number coding on an eye gaze


communication board
Augmentative and alternative communication
(AAC) is an umbrella term that encompasses
methods of communication for those with
impairments or restrictions on the production or
comprehension of spoken or written language.[10]
AAC systems are extremely diverse and depend
on the capabilities of the user. They may be as basic
as pictures on a board that they are used to request
food, drink, or other care; or they can be advanced
speech generating devices, based on speech
synthesis, that are capable of storing hundreds of Ergonomic accessories like, height-adjustable
phrases and words.[11] furniture, footrests, wrist rests, and arm supports
to ensure correct posture. Key guards fit over the
AAC interventions are highly individualized, keyboard to help prevent unintentional key presses.
taking into account specific abilities of language Alternatively, Assistive Technology may attempt
comprehension, social-relational characteristics, to improve the ergonomics of the devices
learning strengths and weaknesses, and themselves:
Motor Neuron Disease 405

More ambitiously, and quite crucially when


keyboard or mouse prove unusable, AT can also
replace the keyboard and mouse with alternative
devices such as the LOMAK keyboard, trackballs,
joysticks, graphics tablets, touchpads, touch
screens, foot mice, a microphone withspeech
recognition software, sip-and-puff input, switch
access, and vision-based input devices, such as eye
trackers which allow the user to control the mouse
with their eyes.

Accessibility software
• Ergonomic keyboards reduce the discomfort In human-computer interaction, computer
and strain of typing. accessibility (also known as Accessible computing)
refers to the accessibility of a computer system to
• Chorded keyboards have a handful of keys
all people, regardless of disability or severity of
(one per digit per hand) to type by 'chords'
impairment
which produce different letters and keys.
• Expanded keyboards with larger, more widely Human Computer Interactions for Amyotrophic
spaced keys. Lateral Sclerosis Patients
• Compact and miniature keyboards. Human Computer Interactions is a communication
and device control channels, which are helpful for
• Dvorak and other alternative layouts may
Amyotrophic lateral sclerosis (ALS) patients.
offer more ergonomic layouts of the keys.
There are also variants of Dvorak in which the Human computer interactions (HCIs) will be
most common keys are located at either the discussed in three respects; electrical brain
left or right side of the keyboard. activities, eye movements and hemoglobin level
in the blood. With technological advances, fighting
Input devices may be modified to make them or minimization side effects of the diseases is the
easier to see and understand: main purpose of biomedical research. Gradually
• Keyboards with lowercase keys when disease progress patients feel difficulty in
controlling muscles and consequently have
• Keyboards with big keys. problems in moving the entire body. Some of these
• Keyboards with less and big keys, or patients can only move their eyes. In severe
multifunctional keys, such us the special conditions of the progressive motor neuron
keyboard Pi- Tech, with only five big rounded diseases, patients cannot move their eyes nor can
keys, which is used with a special software for they speak. Establishing an efficient communication
writing[5] channel without overt speaking and hand motions
makes the patient's life a bit easier and increases
• Large print keyboard with high contrast colors their quality of life. HCIs are a research field which
(such as white on black, black on white, and includes interactions such as communication and
black on ivory).
device/machine control between a user and a
• Large print adhesive keyboard stickers in high computer. The aim of the HCI is to improve
contrast colors (such as white on black, black performance of the interaction, meaning a
on white, and black on yellow). minimization of the barrier between the human and
the computer. [27]
• Embossed locator dots help find the 'home'
keys, F and J, on the keyboard. Man-machine interface (MMI), brain-machine
interface (BMI) and BCI can be thought of as
• Scroll wheels on mice remove the need to
applications of HCIs. If communication or control
locate the scrolling interface on the computer
is established directly from the brain, it is called
screen.
BCI and it is the only method of interaction for the
• Footmouse - Foot-operated mouse. individuals with complete Amyotrophic Lateral
Sclerosis.
406 Neuro-Rehabilitation : A multi disciplinary approach

Research in this field is typically focused on several Use of Splints and Orthoses
areas of improvement for HCIs in order to increase
its usefulness and effectiveness. These areas are: Certain of the specific disabilities resulting from
selective muscle atrophy may be overcome by the
i. High performance use of splints or other appliances. Atrophy of the
a. Accuracy thenar muscles, which affect fine finger movements
and pinch grip, are assisted by a suitable splint
b. Reliability which holds the thumb in the abducted position
c. Fast and allows opposition to the index finger. Power
of finger flexion is also seriously impaired in the
d. Robustness
presence of wrist drop, and is aided by application
ii. User friendliness (including user training) of a static wrist extension splint. Foot drop due to
iii. Ease of application weakness of the long toe extensor muscles may be
aided by a suitable splint. In mildly affected cases
iv. Cost effectiveness. a lightweight slipper type of appliance, extending
up the calf of the leg and fitting inside the shoe,
Equipments for Communication may be adequate. Lightweight boots are preferable
• hands-free telephone to shoes because of the added ankle stability. Later,
• call bells a more sturdy external appliance with toe-spring
attached t o the shoe or boot proves necessary, often
• personal alarms with an external below-knee caliper. Weakness of
• computerised communication aids including the quadriceps muscles causing sudden instability
of the knees in walking may be aided by a long-leg
• light touch keyboards and voice synthesisers caliper with knee-locking device. A cervical collar,
• eye-gaze boards Sometimes with suitable chest plate support, is
frequently necessary to compensate for
• computer programs
paravertebral muscle paresis and flopping of the
• voice ampli?ers head onto the chest. Lower limb appliances may
be used in conjunction with a walking stick or
Durable medical equipment (DME) crutches, depending on the degree of disability.
• Seating products that assist people to sit Eventually a wheelchair proves necessary,
comfortably and safely (seating systems, especially for outdoor excursions. A suitable folding
cushions, therapeutic seats). chair which is easily stowed in a car is preferable
for outdoor use. When severe weakness affects both
• Standing products to support people with
upper limbs, independence within the home may
disabilities in the standing position while
be maintained by the provision of a battery-
maintaining/improving their health (standing
powered wheelchair with suitable microswitch
frame,standing wheelchair, active stander).
controls. The latter may also advise about the pro-
• Walking products to aid people with vision of Velcro fastenings rather than buttons or
disabilities who are able to walk or stand with buckles. Similarly, alterations to clothing, with
assistance (canes, crutches, walkers, gait application of Velcro fastenings in place of buttons
trainers). or hooks, will facilitate dressing and independence
• Advanced technology walking products to aid for toilet purposes.
people with disabilities, who would not at all Relaxation: Relaxation techniques help to relieve
able to walk or stand (exoskeletons). anxiety and have been of great value to many
• Wheeled mobility products that enable people people with MND, especially those with breathing
with reduced mobility to move freely indoors or swallowing problems.
and outdoors (wheelchairs/scooters)
Positive coping
• Vehicles modified with Height adjustable Despite the physical and emotional suffering of
suspension, to allow wheelchair entry to the MND, there is a large number of patients who cope
vehicle well and find positive meaning in life.
Motor Neuron Disease 407

Role of Psychology in agreement to the quality of life. Although MND is


Motor Neuron Disease: most often associated with persistent physical
deterioration, and providing relief for this is very
Psychological Aspect: essential, the view has been expressed that a solely
physical focus is limited. "Concentration of
Motor Neuron Disease is an incurable illness rehabilitation efforts in this respect leads to a serious
involving the progressive degeneration of upper underestimation of the significance of assessing and
and lower motor neurons [1]. The physical aspects managing the major psychological distress
of motor neuron disease, frequently receives apparent in most patients with ALS."
maximum attention whereas, the psychological
aspects are most often overlooked or are secondary Emotional Liability:
[2]. As MND is a rapidly deteriorating disease, it
Some people with MND experience 'emotional
requires the individual to continually adjust to new
liability'. This can result in uncontrollable laughter
losses and continue making changes. Shorter
or crying in response to something that is only
survival times and higher rates of mortality have
moderately funny or sad, for example a television
been reported for patients with high levels of
programme. This can seem inappropriate at times
helplessness and hopelessness. Depression is
and cause confusion.
relatively common i.e. a prevalence rate of about
50% is noticed, as there are other forms of
Social Support and Social Disability:
psychological distress in the MND population,
which is not associated with illness severity and There is a reciprocal relationship between social
functional status [3]. Depression strongly correlates support and depression. The stress-buffering model
with the quality of life. explains that stress, such as that linked with a
chronic or terminal illness can be appreciably
Mood Disturbances: moderated by good social support. However, the
social interaction of many patients with MND is
It is only natural that adapting to changes caused
quiet restricted. Also, as the family and spouse are
by MND may lead to changes in mood. Also, as
completely involved in taking care of the needs of
the condition in MND is constantly, there is a need
the patient, right from visiting doctors, to therapy
to make constant adjustment or adaptation to new
sessions to day - to - day work, patients might lose
ways of functioning. Many people with MND will
a lover or companion. Patients might experience
experience feelings of frustration, anxiety, stress
increasing isolation from friends, family and
with changing situations, and guilt. For a small
socializing, which is what is known as "social
number of people these feelings can be
disability" [6]. Some patients either withdraw from
overwhelming and as a result, they become
others as they see their own mortality and are very
depressed. If someone finds it difficult to engage
scared, where as others prefer to live in "social
with a task or concentrate on things, it may be due
vacuum". Social support is very helpful and acts as
to feeling low, rather than cognitive change. Some
a protection from depression which is associated
people may choose to take certain medication when
with low social support, withdrawal and feeling of
they feel like this, e.g. anti-depressants or they may
being bounded at home. The loss of or difficulty in
seek counselling.
communication and mobility are examples of the
Depression, Anxiety and Psychological limitations to socialisation that MND imposes.
Distress:
Hopelessness and Suicidal Ideation:
Brown and Mueller first studied the psychological
Hopelessness is often associated with loneliness and
state of individuals with MND, describing them as
irrational beliefs, a perception of loss of control over
'stoic' and cheerful, with a capacity to rule out
life, and loss of 'Purpose in Life' [7]. As, patients
dysphoric affect from consciousness [4]. Studies
with MND deal with deteriorating condition and
which have used the psychological tool to assess
increasing dependence, this leads to losing hope
depression i.e. Beck's Depression Inventory with a
with every loss of function. Physical function is not
sample size of over 100 have reported a prevalence
a significant predictor of hopelessness; however,
of depression between 11% and 15% [5]. Depression
depression is a major predictor. As the patient gets
has been correlated to suffering, hopelessness, fast
to know his/ her diagnosis the dreams and
deteriorating and social withdrawal which is in
aspirations that he or she has for himself or herself
408 Neuro-Rehabilitation : A multi disciplinary approach

no longer are significant. The focus is then shifted which involves severe cognitive changes, executive
to visiting doctors, medication, therapies i.e. functions, language, and behaviour changes.
fighting against deterioration. During these times Approximately 15% of people with motor neuron
hopes are an important aspect, as a diagnosis of disease suffer from frontotemporal dementia
MND threatens hopes, dreams and expectations on [13].Approximately 5 percent of patients with
has from life. Patients usually feel helpless about motor neuron disease develop frontotemporal
their condition and that they can't do anything to dementia. Further, it is reported that about 35%
stop the deterioration. have mild cognitive impairment such as mild
aphasia and/or may have behavioural change.
Goggin et al. [8] emphasise the relevance of
hopelessness to MND, reporting that hopelessness
Consequences of Cognitive Changes:
scores of MND patients exceeded those of HIV/
AIDS patients. A study recently reported that out The few effects of cognitive changes seen in patients
of 136 MND patients 22% were moderately with motor neuron disease are mentioned below
hopeless and 10% severely hopeless. Another study [14]:
reported that higher levels of hopelessness were • Changes in personality (rigidity or
characteristic of MND patients who considered aggressiveness)
hastened death and that those who were more
• Slowing down of psychological processes like
hopeless also experienced more suffering [9].
decision-making, answering questions, etc.
Quality of Life (QoL): • Emotionality (uncontrollable crying, laughter
Calman [10] describes the quality of life in terms of or anger)
the match between individual's hopes, expectation • Difficulties with problem solving and
and the present reality. As, MND is a disease with generating new ideas and strategies when old
no definite cure, the focus of the disease should be ones prove unsuccessful
on preservation and improvement of the quality of
life. Clarke et al. [11] reported that the patients with • Difficulties in divided attention (being unable
greater disability are more likely to be affected due to do two things at once, like walking and
to psychosocial aspects of life rather than the talking)
physical aspect. Simmons et al. [12] also found no • Patients have difficulty in concentration i.e.
correlation between QoL and physical functioning difficulty during reading or dealing with
or strength, but that psychological and existential household bills.
domains of life were important contributors to QoL.
• They find it extremely difficult to learn new
Various studies report that psychosocial aspects of
activities.
care are important, and that there is more to
maintaining QoL than simply attending to a • To finish a task through conclusion.
person's physical state.
• To start a conversation and to sustain it if there
is distraction in the background.
Cognitive Changes in Motor Neuron Disease:
With regard to cognition, patients suffering from • They find it quiet stressful and difficult to have
Motor Neuron Disease fall into one of the four a sequential activity or plan ahead.
groups: • Patients also have difficulty in responding to
1. No cognitive changes experienced social situations.

2. Subtle cognitive and behavioural changes • Patients have difficulty in comprehend


complex sentences.
3. Severe cognitive and/ or behaviour changes
where the patient eventually develops • Patients have difficulty in finishing a task.
frontotemporal dementia (FTD)
Diagnosis of Cognitive Impairment:
4. Patients with motor neuron disease who go
on to develop motor impairments i.e. motor Cognitive and/or behavioural change may occur
neuron disease is diagnosed after dementia. at the onset of the disease. A full
neuropsychological assessment and advice by a
Frontotemporal Dementia is a type of dementia
clinical neuropsychologist is warranted, where
Motor Neuron Disease 409

cognitive impairment has been seen to be very Hope is the "ability to see a path to the future" and
helpful in overcoming the cognitive deteriorations. is important to maintaining psychological and
Paucity of local neuropsychology services may be physical health. Hope has also been described as
an issue but this must note prevent assessment and the 'driving force' of perseverance and most
should not add to delays in this vulnerable group significantly, the key to the maintenance of quality
of patients. It has been proven that ventilatory of life. In the study of Dal Bello-Hass et al. [15] the
failure if develops during the course of the illness majority of patients reported a religious practice
can exaggerate cognitive symptoms. Both and there was a positive correlation between
respiratory muscle weakness and dehydration can religious well-being and QoL.
be a cause of cognitive change, which should
therefore be excluded. Cognitive Rehabilitation:
A neuropsychologist, depending on formal
Behavioural Changes: assessments i.e. neuropsychological tests, and
informal assessment i.e. interviewing the client and
Behavioural changes occur in the patients as a result
observation and a few other medical assessments
of their deteriorating condition, changes in the
would suggest strategies to help the patient regain
family structure, work place and constant changes
the lost cognitive functions or stop from further
in their functioning, which would escalate their
losing those functions. For example: work on the
emotional and behavioural problems like:
orientation of the patient.
• Feeling restless and impatient
• Lacking drive or initiative Talk Therapy:
This would help the patient take professional help
• Acting impulsively without thinking about the
to vent out their anxieties, frustrations, doubts,
consequences
irrational thoughts and issues related to survival
• Having unhealthy eating habits i.e. eating and death. The various therapies used would be
excessive quantity of sweets. cognitive behaviour therapy, cognitive therapy,
rational emotive behaviour therapy, gestalt
• Becoming fixated on one activity or routine
therapy, group therapy, etc.
• Lacking empathy and appearing indifferent,
e.g. when you or someone else close to the Caregiver's Support:
person with MND is distressed. Psychoeducating the caregiver and family member,
as to how to deal or respond to the patient's
Psychological Intervention: emotions, behaviours, mood changes and cognitive
changes is extremely important. The caregivers
Positive Coping Strategies: should distribute the work of the patient amongst
Motor Neuron Disease leads to physical and the family members, which would not burden a
emotional suffering, in spite of which many patients particular individual in the family. The caregivers
cope well and find positive meaning in life. It is should not neglect their health and overlook their
important to find purpose of life by using and needs of leisure time, work and other needs by
developing things around us rather than focusing being there for the patient at all times. As, this
on things which cannot be controlled. This can be would increase the level of frustration and anxiety
done by recreational activities, replacing the within them and which would eventually lead to,
negative self-concept to more positive ones and not being able to give complete support to the
living each day as it comes. There are many studies patient.
which report that patients who have a positive
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11. Clarke S, et al. 2001., Assessing individual Sydney
quality of life in amyotrophic lateral sclerosis. 25. Young RR: spasticity: a review, Neurology
Quality of Life Res;10:149-58.
26. Upper Motor Neurone Syndrome and
12. Simmons Z, et al. 2000., Quality of life in ALS Spasticity: Clinical Management and
depends on factors other than strength and Neurophysiology
physical function. Neurology;55: 388-92.
27. By Michael P. Barnes, Garth R. Johnson
13. Oliver D 2007. 'Palliative Care' in Kiernan MC
28. Roles and Responsibilities of Speech-Language
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Ch.11 Polyneuropathy
[Link] Mishra, [Link], F.N.R., Dr. Nancy Thomas, MPTh (Neuro).

Polyneuropathy describes damage to the peripheral infection and nerve inflammation causing tingling
nervous system that transmits information from the feet.
brain to the spinal cord and to every other part of
the body. Peripheral nerves also send sensory Mononeuritis multiplex
information from the body, back to the brain and Mononeuritis multiplex occurs when multiple
spinal cord
nerves are damaged in the body due to diabetes
The Nervous system can be divided into two parts mellitus (diabetic neuropathy), Churg-Strauss
• Central nervous system (CNS) : The CNS syndrome, HIV, amyloidosis and rheumatoid
consist of the brain and the nerves that make arthritis. It is present with dull pain in legs and back
up the spinal cord, which runs through the mostly at night. Diabetics may have severe pain in
vertebrae. thigh of either side with weakness and knee reflex
• Peripheral nervous system (PNS): The PNS absence.
consists of the sensory-somatic nervous
system which carries information about Polyneuropathy
sensations and transmits instructions to carry Polyneuropathy affects nerve cells anywhere in the
out actions (such as movements). The
body irrespective of the nerve path. It can cause
autonomic nervous system which controls and
regulates bodily function (for example, changes in axon, neurons cell bodies and myelin
breathing). sheath surrounding axons. Distal axonopathy is the
condition affecting only the axons with intact
Polyneuropathy/peripheral neuritis is the
dysfunction of the peripheral nervous system by neurons. In sensory neuronopathy and motor
nerve damage. It can be the result of the underlying neuron disease sensory and motor neurons are
disease and also the diseases affecting the nerves. affected respectively. Polyneuropathy produces
The main symptoms are numbness of feet with symptoms such as numb feet, burning, erectile
tingling sensation, weakness, incoordination, dysfunction and imbalance in bladder function.
pain, burning sensation and invisible 'glove-like' This neuropathy treatment involves three steps. It
sensation, abnormal heart rate, reduced sweating starts with removing the cause, then strengthening
and sexual problems. muscles and their function and in the last pain relief
Infection, injury, nutritional deficiency, excessive by using neuropathy creams containing capsaicin.
alcohol consumption and disorders such as
diabetes can cause peripheral neuropathy. The Autonomic Neuropathy
cause varies according to the specific neuropathy. The fourth pattern in the peripheral type of
Polyneuropathy can be classified into more than neuropathy is the autonomic neuropathy causing
100 forms and produces different set of symptoms alterations in the autonomic nervous system. It
and have different prognosis. Motor, sensory and affects the non-involuntary nerves reaching urinary
autonomic nerves damage can occur. If the motor bladder, digestive system, sexual organs and the
nerves are affected then imbalance occurs in the heart. Chronic diabetic patients are prone to this
coordination of walking, holding things and other neuropathy. Autonomic neuropathy can also be
voluntary muscle movements. Damage in sensory present in combination with other neuropathies. It
nerves results in the loss of sensations like touching produces symptoms such as incontinence of urine,
or pain whereas autonomic nerves affect the
pain in abdomen with vomiting, diarrhea or
involuntary nerves controlling vital organs.
constipation, tachycardia, hypotension and
CLASSIFICATION OF impotency.
PERIPHERAL NEUROPATHY
Etiology
Mononeuropathy
Polyneuropathy may be either acquired or
When only one nerve is affected then it is called as inherited.
mononeuropathy. It can be caused by compression
to the nerve, carpel tunnel syndrome or some Causes of acquired peripheral neuropathy include
412 Neuro-Rehabilitation : A multi disciplinary approach

physical injury (trauma) to a nerve, tumors, toxins, Kidney disorders can lead to abnormally high
autoimmune responses, nutritional deficiencies, amounts of toxic substances in the blood that can
alcoholism, and vascular and metabolic disorders. severely damage nerve tissue. A majority of
patients who require dialysis because of kidney
I. Acquired peripheral neuropathies are grouped
failure develop polyneuropathy. Some liver
into three broad categories:
diseases also lead to neuropathies as a result of
• Those caused by systemic disease, chemical imbalances.
• Those caused by trauma from external agents, Hormonal imbalances can disturb normal
and metabolic processes and cause neuropathies. For
• Those caused by infections or autoimmune example, an underproduction of thyroid hormones
disorders affecting nerve tissue. slows metabolism, leading to fluid retention and
swollen tissues that can exert pressure on
One example of an acquired peripheral peripheral nerves. Overproduction of growth
neuropathy is trigeminal neuralgia (also known hormone can lead to acromegaly, a condition
as tic douloureux), in which damage to the characterized by the abnormal enlargement of
trigeminal nerve causes episodic attacks of many parts of the skeleton, including the joints.
excruciating, lightning-like pain on one side of the Nerves running through these affected joints often
face. In some cases, the cause is an earlier viral become entrapped leading to neuropathy.
infection, pressure on the nerve from a tumor or
swollen blood vessel, or, infrequently, multiple Vitamin deficiencies and alcoholism can cause
sclerosis. In many cases, however, a specific cause widespread damage to nerve tissue. Vitamins E, B1,
cannot be identified. Doctors usually refer to such B6, B12, and niacin are essential to healthy nerve
neuropathies with no known cause as idiopathic function. Thiamine deficiency, in particular, is
neuropathies. common among people with alcoholism because
they often also have poor dietary habits. Thiamine
Physical injury (trauma) is the most common cause deficiency can cause a painful neuropathy of the
of injury to a nerve. Injury or sudden trauma, such extremities. Some researchers believe that excessive
as from automobile accidents, falls, and sports- alcohol consumption may, in itself, contribute
related activities, can cause nerves to be partially directly to nerve damage, a condition referred to
or completely severed, crushed, compressed, or as alcoholic neuropathy.
stretched, sometimes so forcefully that they are
partially or completely detached from the spinal Vascular damage and blood diseases can decrease
cord. Less dramatic traumas also can cause serious oxygen supply to the peripheral nerves and quickly
nerve damage. Broken or dislocated bones can exert lead to serious damage to or death of nerve tissues,
damaging pressure on neighboring nerves, and much as a sudden lack of oxygen to the brain can
slipped discs between vertebrae can compress cause a stroke. Diabetes frequently leads to blood
nerve fibers where they emerge from the spinal vessel constriction. Various forms of vasculitis
cord. (blood vessel inflammation) frequently cause vessel
walls to harden, thicken, and develop scar tissue,
Systemic diseases are disorders that affect the decreasing their diameter and impeding blood flow.
entire body and often cause peripheral neuropathy. This category of nerve damage, in which isolated
These disorders may include metabolic and nerves in different areas are damaged, is called
endocrine disorders. Nerve tissues are highly mononeuropathy multiplex or multifocal
vulnerable to damage from diseases that impair the mononeuropathy.
body's ability to
Connective tissue disorders and chronic
• Transform nutrients into energy, inflammation can cause direct and indirect nerve
• Process waste products, or damage. When the multiple layers of protective
tissue surrounding nerves become inflamed, the
• Manufacture the substances that make up inflammation can spread directly into nerve fibers.
living tissue. Chronic inflammation also leads to the progressive
Eg. Diabetes mellitus, characterized by chronically destruction of connective tissue, making nerve
high blood glucose levels, is a leading cause of fibers more vulnerable to compression injuries and
peripheral neuropathy. infections. Joints can become inflamed and swollen
and entrap nerves, causing pain.
Polyneuropathy 413

Cancers and benign tumors can infiltrate or exert The human immunodeficiency virus (HIV), which
damaging pressure on nerve fibers. Tumors also causes AIDS, also causes extensive damage to the
can arise directly from nerve tissue cells. central and peripheral nervous systems. The virus
Widespread polyneuropathy is often associated can cause several different forms of neuropathy,
with the neurofibromatoses, genetic diseases in each strongly associated with a specific stage of
which multiple benign tumors grow on nerve active immunodeficiency disease. A rapidly
tissue. Neuromas, benign masses of overgrown progressive, painful polyneuropathy affecting the
nerve tissue that can develop after any penetrating feet and hands is often the first clinically apparent
injury that severs nerve fibers, generate very intense sign of HIV infection.
pain signals and sometimes engulf neighboring Lyme disease, diphtheria, and leprosy are bacterial
nerves, leading to further damage and even greater diseases characterized by extensive peripheral
pain. Neuroma formation can be one element of a nerve damage.
more widespread neuropathic pain condition called
complex regional pain syndrome or reflex Viral and bacterial infections can also cause indirect
sympathetic dystrophy syndrome, which can be nerve damage by provoking conditions referred to
caused by traumatic injuries or surgical trauma. as autoimmune disorders, in which specialized cells
Paraneoplastic syndromes, a group of rare and antibodies of the immune system attack the
degenerative disorders that are triggered by a body's own tissues. These attacks typically cause
person's immune system response to a cancerous destruction of the nerve's myelin sheath or axon
tumor, also can indirectly cause widespread nerve (the long fiber that extends out from the main nerve
damage. cell body).

Repetitive stress frequently leads to entrapment Some neuropathies are caused by inflammation
neuropathies, a special category of compression resulting from immune system activities rather than
injury. Cumulative damage can result from from direct damage by infectious organisms. These
repetitive, forceful, awkward activities that require inflammatory neuropathies can exhibit a pattern of
flexing of any group of joints for prolonged periods. alternating remission and relapse. Acute
inflammatory demyelinating neuropathy, better
The resulting irritation may cause ligaments,
known as Guillain-Barré syndrome, can damage
tendons, and muscles to become inflamed and
motor, sensory, and autonomic nerve fibers. Most
swollen, constricting the narrow passageways
people recover from this syndrome although severe
through which some nerves pass. These injuries
cases can be life threatening.
become more frequent during pregnancy, probably
because weight gain and fluid retention also
1-GBS (Guillain-Barre syndrome)
constrict nerve passageways.
Guillain-Barre syndrome (GBS) in developed
Toxins can also cause peripheral nerve damage. countries in the post-poliomyelitis era, is the most
People who are exposed to heavy metals (arsenic, common cause of acute progressive flaccid
lead, mercury, thallium), industrial drugs, or paralysis. Guillain-Barre syndrome typically,
environmental toxins frequently develop manifests as rapidly progressing limb weakness,
neuropathy. Certain anticancer drugs, accompanied by paresthesias and often cranial
anticonvulsants, antiviral agents, and antibiotics nerve dysfunction. The age of onset is 40 years.
have side effects that can include peripheral nerve
damage, thus limiting their long-term use. Pathogenesis and Pathophysiology
Infections and autoimmune disorders can cause All subdivisions of GBS appear to be autoimmune
peripheral neuropathy. Viruses and bacteria that diseases resulting from aberrant immune response
can attack nerve tissues include herpes varicella- against various components of peripheral nerve
zoster (shingles), Epstein-Barr virus, fibers. With AIDP (acute inflammatory
cytomegalovirus, and herpes simplex-members of demyelinating polyradiculoneuropathy), both
the large family of human herpes viruses. These humoral and cell-mediated factors appear to be
viruses severely damage sensory nerves, causing operative, with both antiganglioside antibodies and
attacks of sharp, lightning-like pain. Postherpetic activated T cells functioning in an attack on myelin
neuralgia often occurs after an attack of shingles sheaths and possibly Schwann cell membranes by
and can be particularly painful. sensitized macrophages. Pathophysiologically,
414 Neuro-Rehabilitation : A multi disciplinary approach

these focal areas of demyelination result in Management:


conduction slowing or conduction block, The treatment of GBS has two components:
depending on the severity of the process supportive care and specific therapy.

Epideiology and risk factors: Supportive care remains the corner stone of
therapy, because most patients recover function if
AIDP is a relatively rare disorder, having an
they advance past the acute phase of the illness.
average yearly incidence rate of 1-2 cases per
The major reductions in the deaths from GBS over
100,000 population. It occurs worldwide, affects all
the past few decades have been due to advances in
races, and attacks more males than females. Persons
supportive care, particularly in mechanical
of any age are at risk, although the incidence is
ventilation. Respiratory compromise is the most
higher in the elderly. AIDP is responsible more than
common serious complication and underlines the
90% of the GBS cases that occur in North America,
reason that approximately 30% of GBS patients who
Europe and Australia. It occurs principally in
are hospitalized require treatment in an ICU.
epidemics and during the summer months, has a
Weakness of the respiratory muscles, particularly
very strong association with preceding
the diaphragm, is most often responsible for
Campylobacter jejuni infections, and typically
respiratory failure. Other factors include weakness
affects the children and young adults.
of various bulbar-innervated muscles and
AMAN(Acute motor-axonal neuropathy) also
pulmonary complications, including atelectasis,
occurs in North America, Japan, India and Central
pneumonia, and pulmonary embolism. Vital
America.
capacity measurements provide the most useful
Clinical features and associated disorders information regarding the degree of respiratory
compromise. Respiratory function may be
The cardinal features of GBS are weakness,
monitored closely. Intratracheal intubation is
paresthesias, and diminished or absent DTRs.
complicated in patients with GBS because of
The initial neurological symptoms vary from dysautonomia, which can result in marked
patient to patient. Distal, usually symmetrical, hypotension, arrhythmias, and sudden death due
paresthesias involving the toes, finger, or both to various drugs and to airway manipulation and
herald the onset of the disorder in at least 50% of because of hyperkalemia, induced by
patients. As the disease progress, these paresthesias succinylcholine, which can cause arrhythmias and
typically spread proximally but seldom extended cardiac arrest. Dysautonomia occurs to some extent
beyond ankles and wrists. Facial paresthesias, in majority of GBS patients but more frequently and
which are usually perioral, are less common. more severely in those with respiratory failure and
Gradually weakness in lower extremities especially severe motor deficits. Pulmonary embolism may
in proximal muscles, so that patients may feel occur in about 5% of patients immobilized with
difficulty in climbing stairs and rising from chairs. GBS, typically after the second week of
Weakness soon spreads to the upper extremities. immobilization. Preventive measures include
However, some times the illness progresses, leading subcutaneous heparin-switched to warfarin if
to complete paralysis of arms and legs. About one patients are long term-and intermittent calf
quarter of the time, the paralysis continues up the pneumatic compression devices. Plain support
chest causing respiratorydysfunction stockings can reduce the incidence of thrombosis
significantly.
Cranial nerve (VII, Facial diaplegia), occasionally,
fasciculations are noted. Sometimes deep pain also Two non life threatening complications of GBS that
occur, which is more prominent in shoulder girdle, require attention are pain and psychological
back and posterior thighs and is notoriously more trauma. Simple analgesics and nonsteroidal anti-
severe in night. inflammatory medications frequently prove
inadequate. Gabapentin or carbamazepine often is
Substantial sensory abnormalities are found
beneficial during the acute stage. Narcotic
infrequently and contrast with the high incidence
analgesics are required in many patients. However,
of sensory symptoms. Typically, vibration and
they must be used with caution because they tend
proprioception, the sensory modalities mediated
to exacerbate respiratory failure and to produce
over large myelinated fibers, are the most severely
ileus. The specific treatment for GBS since 1978 has
affected.
been plasmapheresis. With plasmapheresis, less
Polyneuropathy 415

mechanical ventilation is required, and both ICU In many respects, it is similar to the evidence
and hospitalization time is decreased. The advanced for AIDP being an immune- mediated
drawbacks of plasmapheresis include its expense disorder. The onset or relapses of CIDP on occasion
and rare complications such as hypotension and seem to be triggered by a preceding event, such as
sepsis. an infection or vaccination that could initiate an
immune-mediated response. In addition, most
Prognosis: patients with CIDP response to corticosteroids and
Overall, approximately 5% of patients die; mortality other immunosuppressants, plasma exchange, and
rates of 15% to 20% have ocurred in ICUs, most IVIG, therapies designed to treat immune-mediated
often from adult respiratory distress syndrome. disorders.
Approximately 75% of patients recover without Epideiology and risk factors: The age of onset of
serious neurological residuals, and recovery CIDP has been reported to range from 1 year
typically ensues over the 6 to 12 months after onset through the eight decade or later, with mean in the
and is usually maximal by 18 months after onset. fourth decade. With the mean age of onset being in
Some patients may be left with persistent minor the fourth decade, women of childbearing age may
weakness, paresthesias, or sensory loss. develop CIDP. Furthermore, there is evidence that
Approximately 7% to 15% of patients have the disorder may relapse or worsen during
permanent, substantial neurological sequelae (e.g., pregnancy and in the postpartum period.
bilateral footdrop, intrinsic hand muscle weakness
and wasting, sensory ataxia, burning dysesthesias Clinical Features and Associate Disorders
). Only a very few, however, are permanently
CIDP is a chronic disorder that typically evolves
bedridden by the disorder
slowly, almost always requiring a period of 8 weeks
Differential Diagnosis: Transverse myelitits and or longer for symptoms to reach their peak, and
Vasculitic neuropathies may superficially resemble they typically do so over many months. CIDP can
GBS,. Acute intermittent porphyria can produce a follow either a slowly progressive monophasic
PNS disorder that can be mistaken for GBS. course, which occurs in approximately two thirds
of patients, or a cyclical relapsing course seen in
2-Chronic inflammatory demyelinating
the remainder.
polyneuropathy (CIDP), generally less dangerous,
usually damages sensory and motor nerves, leaving The symptoms of CIDP can be variable, but usually
autonomic nerves intact. weakness tends to predominate over sensory
symptoms. The distribution of weakness often
This slowly progressive steroid-dependent
conforms to a typical peripheral neuropathy pattern
polyneuropathy can occur at any age (mean age of
of symmetrical involvement, with the lower
onset in the 5th decade).
extremities being affected more than the upper
Pathogenesis and Pathophysiology : CIDP is an extremities and distal muscles being affected more
apparent immune-mediated disorder of PNS. The than the proximal muscles. However, in many
term chronic inflammatory demyelinating patients, the weakness is asymmetrical, and in
polyradiculopathy was coined to emphasize that others, it is more prominent proximally. The degree
the disorder is a chronic process that results in of weakness varies markedly from patient to
demyelination as well as inflammatory cell patient. In majority of patients, it is mild to
response in peripheral nerves and spinal nerve moderate in degree, but rarely, the weakness can
roots; typically, there is a mononuclear cell be severe and generalized, sometimes associated
infiltration involving the endoneurium and with respiratory failure necessitating ventilatory
epineurium of peripheral nerve fibers. The support. Muscle cramping and fasciculations are
predominant physiological feature is Segmental infrequent symptoms.
demylination, although usually there is some
Sensory symptoms are usually confined to mild
degree of axon loss as well. These pathological
paresthesia or modest sensory loss. However, in
changes have been found to involve roots, plexuses,
those patients with chronic progressive courses,
proximal nerve trunks, as well as some autonomic
sensory symptoms may become more prominent.
nerves.
In addition to numbness and parathesia, pain
The evidence that CIDP is immune-mediated is sometimes is reported, although this occurs in less
compelling, although still somewhat inconclusive. than 20% of patients. Very infrequently, CIDP may
416 Neuro-Rehabilitation : A multi disciplinary approach

present with predominately sensory symptoms, 3-Chronic idiopathic sensory neuropathy (CISN):
including numbness, parathesia, and sensory This is basically a sensory form of CIDP with limb
ataxia. However, in most instances in which the ataxia, numbness and pain, loss of proprioception,
disorder begins as sensory neuropathy, it later normal muscle strength but generalized areflexia.
evolves to a more typical pattern of sensory and
4-Alcoholic Polyneuropathy: Alchoholic
motor involvement. On occasion, the cranial nerves
Neuropathy is a disorder involving decreased nerve
may be affected, causing symptoms of diplopia,
functioning caused by damage that results from
facial weakness or numbness, dysarthria and
excessive drinking of alcohol. The onset is often
dysphagia. Autonomic symptoms are uncommon,
insidious and presents with distal symmetric
although incontinence and erectile impotence have
dominant sensory-dominant polyneuropathy
been reported.
confined to the legs. Painful sensations with or
The physical signs noted in CIDP reflect the clinical without burning represents the initial and major
symptoms. Distal symmetrical weakness, with the symptom. Other manifestations are dysesthesias,
lower extremities more severely affected than the paresthesias or sensory ataxia. Autonomic features
upper extremities. However, asymmetrical may be present (commonly abnormal sweating or
involvement and more prominent proximal diarrhea, less frequently postural hypotension,
involvement can occur, and they serve as important vomiting, micturition difficulties, impotence and
clinical clues to diagnosis of CIDP. Generalized retrograde ejaculation). Electrophysiological
hyporeflexia or areflexia is characteristic. Sensory studies show axonal neuropathy predominantly
loss often conforms to a distal to proximal gradient, affecting the sensory nerves. Progression is gradual
in the so-called stocking-glove pattern, with all continuing over months or years. Abstinence
modalities affected to a nearly equal extent. reveals gradual improvement in the
Occasionally, sensory ataxia may be observed. polyneuropathy. Rapidly progressive
Cranial nerve findings may include signs of facial, polyneuropathy resembling GBS but without raised
bulbar and neck weakness. protein or slowed NCVs can occur in alcoholics.
Some patients with CIDP have abnormalities on 5-Idiopathic peripheral facial palsy (Bell palsy):
MRI scans of the head consistent with multiple The incidence of Bell palsy is 2-3‰. Retroauricular
sclerosis. pain usually precedes the paralysis by 1 or 2 days.
Differential Diagnosis, CIDP may be confused with Almost 50% show maximal paralysis in 2 days.
a wide variety of chronic sensorimotor Recovery takes weeks - 2 month. Ramsay-Hunt
polyneuropathies, HIV infection,Familial syndrome (zoster around the ear, acute peripheral
hypertrophic polyneuropathies facial palsy, and symptoms involving the VIII
cranial nerve) is caused by a reactivation of
On occasion, CIDP may be difficult to differentiate
VZV(Varicella Zoster Virus). The same virus causes
with AIDP. Patients with subacute courses of CIDP
acute peripheral facial palsy without skin lesions
have been described and sometimes CIDP may
(zoster sine herpete). About 15% of patients will
begin with a relatively acute onset. Overtime, CIDP
have permanent sequelae. The facial palsy
usually declares itself by a typical chronic relapsing
associated with Ramsay-Hunt syndrome is more
course or a more slowly progressive monophasic
severe and has a lower recovery rate than that of
course. The distinction between CIDP and AIDP is
Bell palsy. The diagnosis of Ramsay-Hunt
significant because the ultimate course, prognosis,
syndrome is relatively easy in the presence of
and treatment are different.
typical skin rash.. A combination of acyclovir (4000
Prognosis mg daily for 5 days) and prednisone (60 mg/day
for 4 days) provide a 100% cure rate if started within
The prognosis is favorable for most patients with
3 days after the onset.
CIDP who are properly diagnosed and treated. In
one large group of patients whose disease duration 6-Diabetic Neuropathies
averaged 5.7 years and who were followed for a i. Diabetic polyneuropathy Diabetic
minimum of 24 months, nearly 40% were neuropathies are a family of nerve disorders
asymptomatic and nearly 50% had either minor caused by diabetes. People with diabetes can,
symptoms that did not have any impact on their over time, have damage to nerves throughout
level of function or had only very modest restriction the body. Neuropathies lead to numbness and
in their lifestyle. sometimes pain and weakness in the hands,
Polyneuropathy 417

arms, feet, and legs. Problems may also occur NIDDM ( non-insulin dependent diabetes
in every organ system, including the digestive mellitus ) patients in their 6th or 7th decade.
tract, heart, and sex organs. People with Pelvic girdle and thigh muscle weakness and
diabetes can develop nerve problems at any atrophy are evident, but mainly affecting the
time, but the longer a person has diabetes, the quadriceps muscle and developing over the
greater the risk. next few days or weeks. The knee jerks are lost.
Despite the unilateral onset, bilateral weakness
An estimated 50% of those with diabetes have
eventually occurs in 50% of patients. The
some form of neuropathy, but not all with
plantar responses may be extensor! Upper
neuropathy have symptoms. The highest rates
extremities are rarely affected. Deep and
of neuropathy are among people who have
superficial sensation may be intact or mildly
had the disease for atleast 25 years
affected. The neuropathy is associated with
ii. Diabetic small-fiber sensory profound weight loss. Femoral NCV is
polyneuropathy: This is most prevalent in delayed. CSF is slightly elevated.
IDDM ( Insulin- Dependent Diabetes Mellitus) Improvement of the condition is usually seen
of more than 20 years duration, and in those after some months (or years). Only 20% of
with AHT(antihypertensive treatment) or poor patients have complete recovery of muscle
glycemic control. The majority of patients strength. 1/5th of patients experience
present initially with relative symmetric recurrence and often affecting the opposite
sensory symptoms including paresthesia, limb.
burning and lancinating pain in the legs with
v. Diabetic mononeuritis multiplex: This
stocking distribution. Pain and temperature
painful unilateral or asymmetrical multiple
sensation is affected; vibratory sensation and
neuropathy tends to occur in elderly with mild
joint position sense are lost in the toes. Positive
diabetes or unrecognized diabetes. It tend to
Romberg sign with sensory gait ataxia may be
occur during periods of transition, when
found. Ankle jerks are lost, while others are
severe hyperglycemia or hypoglycemia arises,
preserved. Autonomic features may include
following the initiation of insulin therapy or
warm dry foot with hard vulnerable skin with
in association with rapid weight loss.
cracking. Neuropathic joints may develop.
Distal muscle weakness (with atrophy) is vi. Diabetic Mononeuropathy: Painful
unusually except in longstanding cases. oculomotor nerve palsies, often sparing the
pupil are common in older diabetics and
iii. Distal polyneuropathy: Distal, symmetrical,
resolve spontaneously.
primarily sensory neuropathy is the
commonest form of chronic neuropathy in vii. Diabetic Truncal neuropathy: Attacks of
diabetes. The lifetime incidence is estimated truncal pain and sensory loss affecting one or
at 37-45% in type 2 diabetes and 54-59% in type more thoracic roots can occur. It typically
1diabetes. The clinical presentation consists of occurs in the 5th - 7thdecade in NIDDM and
distressing numbness and paresthesias in the is associated with weight loss. Focal paralysis
feet and lower legs, worse at night. The pain of the abdominal wall muscles may be seen.
is often reported as superficial presenting as EMG shows denervation of paraspinal
allodynia, sharp, stabbing, or burning pain in muscles. Recovery is spontaneous but may
the feet. The ankle and knee jerks are absent. take months.
Trophic changes may occur. There is generally
7-Uremic polyneuropathy: 50% of patients with
a family history of diabetic polyneuropathy
end-stage chronic renal failure have clinical or
and obesity is commonly found. Tendon
electrophysiologic evidence of polyneuropathy.
reflexes and distal sensation are reduced.
Uremic polyneuropathy develops gradually and
iv. Proximal diabetic radiculopathies: There are occurs with glomerular filtration rates below 10 ml/
two distinctive forms of radiculopathy in min. In contrast to the underlying causes of renal
diabetic patients; the painful asymmetric failure, which are often resulting in focal or
radiculopathy often referred to as "diabetic demyelinating polyneuropathy, uremic
amyotrophy" and the painless proximal polyneuropathy is axonal in nature. Restless legs
symmetric amyotrophy. The diabetic (burning paresthesias, itching sensation) are often
amyotrophy is most commonly observed in the initial manifestation, followed by muscle
418 Neuro-Rehabilitation : A multi disciplinary approach

cramps and fatigability and, finally, muscle distal subsequently followed by leg muscle
weakness and atrophy. The earliest objective signs weakness. Ankle jerks are invariably lost.
are loss of vibration at the toes and absent ankle SNAPs are absent and motor conduction is
jerks. NCVs indicate axonal degeneration of motor initially mildly slowed. Sural nerve biopsy
and sensory fibers. Renal transplant greatly shows axonal degeneration. Improvement
improves the condition. Occasionally an acute over years occurs but is often incomplete.
uremic polyneuropathy may develop that Other features in acute poisoning are
resembles GBS. NCVs may show demyelinating encephalopathy, pancytopenia, eosinophilia,
features and raised CSF protein. liver and/or kidney failure depending on the
amount. Chronic arsenic exposure in industrial
8-Thyroid/Pituitary Neuropathies
workers may produce an asymptomatic
Mucinous deposits in soft tissue resulting in nerve sensorimotor neuropathy detectable only by
compression and carpal tunnel-like symptoms have NCVs.
been implicated in neuropathy associated with
iii. Thallium polyneuropathy: High doses of
hyperthyroidism. Neuropathy associated with
thallium (rodenticide) cause shock due to
excess growth hormone or acromegaly has been
gastroenteritis and dehydration. When this
associated with subperineurial-tissue proliferation
initial phase is survived sensorimotor
and diminished myelinated and unmyelinated
neuropathy becomes apparent in a few days.
fibers.
Sensory symptoms occur first and consist of
9-AIDS-associated Neuropathy painful paresthesias and allodynia affecting
Polyneuropathy affects as many as one-third of the feet. The reflexes remain preserved. The
individuals with acquired immunodeficiency neuropathy progresses rapidly to involve the
syndrome (AIDS), most commonly manifested as respiratory and bulbar muscles resembling
distal, symmetrical polyneuropathy. GBS. Associated autonomic neuropathy
results in tachycardia and hypotension. CNS
10-Toxic polyneuropathy manifestations are optic neuropathy, ataxia,
i. Acrylamide polyneuropathy: High-dose confusional psychosis and involuntary
intoxication which occurs after drinking of movements. Systemic features include dark
contaminated water causes subacute pigmentation at the hair followed by rapid
encephalopathy followed some days later by complete alopecia, dry skin, Mees lines on the
mild polyneuropathy. Chronic low dose nails helps to differentiate this form of
intoxication (construction workers) results in neuropathy from other forms of acute
polyneuropathy within 4 weeks after polyneuropathy. Sural nerve biopsy shows
exposure. Diffuse areflexia is an early sign. axonal degeneration.
Ataxia may be prominent. The neuropathy iv. Lead polyneuropathy: Workers in metal
involves both sensory and motor. Contact smelting and battery (fumes) manufacturing
dermatitis, blistering and hyperhydrosis of the are often the subject of chronic inorganic
palms and the soles may occur. SNAPs are poisoning. The presentation is very similar to
small or absent. Sural nerve biopsy shows porphyria. Abdominal cramps are often the
degeneration and regeneration of axons. first manifestation. Classically a pure motor
Complete recovery is possible. neuropathy develops affecting most often the
ii. Acute arsenic neuropathy: This type of radial or peroneal nerves. Sensory loss is
poisoning occurs either acute or chronic uncommon. Anemia, basophilic stippling of
(smelting workers). In acute poisoning red cell precursors and free erythrocyte
neurologic symptoms appear 2-3 weeks after protoporphyrin level are the best guide to
the initial gastrointestinal manifestations chronic lead exposure. Motor NCVs may be
(abdominal burning pain, nausea, vomiting slowed. Sural nerve biopsy shows loss of the
and diarrhea within hours-days of ingestion) large myelinated axons.
or shock. Subacute polyneuropathy (7-14 days) v. Mercury polyneuropathy: Chronic exposure
presenting as numbness and often painful to inorganic or elemental mercury produces
paresthesias with loss of vibration and position mild sensorimotor peripheral polyneuropathy.
sense are the initial manifestations, Elemental mercury poisoning resembles
Polyneuropathy 419

MND. Organic mercury poisoning (e.g. weakness, tremor and limb ataxia may
Minamata disease) typically causes the ultimately develop. All modalities of sensation
combination of paresthesias, sensory ataxia, may be impaired distally in the limbs and
and visual field constriction. Paresthesias acrodystrophic changes secondary to sensory
around the mouth and fingers and toes with loss may develop. Scoliosis, pupil
sometimes normal NCVs may point towards abnormalities, or extensor plantar responses
the diagnosis. Measurement of mercury levels occasionally occur. Diaphragmatic weakness
in blood, urine and hair confirm the diagnosis. may cause dyspnoea or respiratory failure.
Palpable nerve thickening (great auricular
II. Inherited forms of peripheral neuropathy nerve) is found in 25% of patients.
Inherited forms of peripheral neuropathy are Progression of disease is slow.
caused by inborn mistakes in the genetic code or
by new genetic mutations. Some genetic errors lead ii. HSMN - II (Charcot-Marie-Tooth disease type
to mild neuropathies with symptoms that begin in 2 or adult onset axonal form): The age of onset
early adulthood and result in little, if any, significant is 2nd to 3rd decade of the life and this is
impairment. More severe hereditary neuropathies mostly autosomal dominant (X-linked and
often appear in infancy or childhood. autosomal recessive forms exist) Males are
more commonly affected than females.
The most common inherited neuropathies are a Common symptoms are distal leg muscle
group of disorders collectively referred to as wasting and weakness, with absent ankle jerks.
Charcot-Marie-Tooth disease. These neuropathies Hand weakness, tremor and ataxia of the arms,
result from flaws in genes responsible for sensory loss, generalized areflexia or pes cavus
manufacturing neurons or the myelin sheath. Main are less common than in HSMN - I. There is
symptoms of Charcot-Marie-Tooth disease include no nerve hypertrophy. Nerve biopsy shows
extreme weakening and wasting of muscles in the axonal loss. Late-onset HSMN should be
lower legs and feet, gait abnormalities, loss of differentiated from chronic idiopathic axonal
tendon reflexes, and numbness in the lower limbs. polyneuropathy in which sensory features and
1-Hereditary Sensorimotor neuropathy (HSMN)/ progression are characteristic.
(Charcot-Marie-Tooth disease) 2-Hereditary neuropathy with liability to pressure
This is the most common cause of distal leg muscle palsies (HNPP): This condition also known as
wasting and weakness ("peroneal muscular tomaculous (sausage-like) neuropathy is autosomal
atrophy" syndrome), usually accompanied by pes dominant with the onset of symptoms is in the 2nd
cavus. The age of onset is variable and or 3rd decade of life and translates in a tendency to
asymptomatic, yet affected elderly relatives may be develop painless focal and recurrent demyelinating
identified. In HSMN males are commonly affected, sensory and motor peripheral mononeuropathies
whereas females are more often asymptomatic. due to unusual vulnerability to pressure or traction.
Positive sensory symptoms (paresthesias) are Exposed nerves such as ulnar nerve, radial nerve
unusual and should rather suspect acquired and superficial peroneal nerve are especially
neuropathy. Associated features (spastic vulnerable. Painless brachial plexus lesions may
paraparesis, optic atrophy, retinitis pigmentosa, result from traction or prolonged abnormal
deafness and mental retardation) can occur. With postures. Recovery occurs over days, weeks or
the availability of genetic testing, noninvasive months, but permanent disability may develop after
accurate diagnosis is now possible and omits the recurrent episodes. Typically, patients experience
need for nerve biopsies. tingling of the fingertips when using scissors.
i. HSMN - I (Charcot-Marie-Tooth disease type NCVs show prolonged distal motor latencies or
1 or adult-onset demyelinating): The age of reduced SNAPs with conduction blocks and minor
onset is 1st to 2nd decade of life and this is slowing of motor conduction velocities both in
mostly autosomal dominant (autosomal affected and asymptomatic gene mutation carriers.
recessive, and X-linked forms exist). Clinically, Almost 80% of patients have a deletion of PMP22
distal leg muscle atrophy ("inverted gene at chromosome 17p11.2.
champagne bottle"), lost ankle jerks and Nerve biopsy shows sausage-like pattern of the
weakness, usually accompanied by pes cavus nerve (also found in familial brachial plexus
characterize the disorder. Some hand
420 Neuro-Rehabilitation : A multi disciplinary approach

neuropathy, Ehler-Danlos and paraproteinemic absent tears, depressed deep tendon reflexes,
neuropathy). absent corneal reflex, postural hypotension
and relative indifference to pain. Scoliosis is
3-Hereditary sensory and autonomic neuropathy:
frequent. Intelligence remains normal. Many
Five clinical different entities have been described patients die in infancy and childhood.
under hereditary sensory and autonomic Histopathology of peripheral nerve shows
neuropathies - all characterized by progressive loss reduced number of myelinated and non-
of function that predominantly affects the myelinated axons. The catecholamine endings
peripheral sensory nerves. Their incidence has been are absent.
estimated to be about 1 in 25,000.
iv. (Congenital Insensitivity to Pain and
i. (Hereditary Sensory Radicular Neuropathy): Anhidrosis): It is an autosomal recessive
It is the most common of the hereditary condition and affected infants present with
sensory and autonomic neuropathies (HSAN). episodes of hyperthermia unrelated to
It is transmitted as autosomal dominant trait environmental temperature, anhidrosis and
and is characterized by a sensory deficit in the insensitivity to pain. Palmar skin is thickened
distal portion of the lower extremities, chronic and charcot joints are commonly present. NCV
perforating ulcerations of the feet and shows motor and sensory nerve action
progressive destruction of underlying bones. potentials to be normal. The histopathology of
Symptoms appear in late childhood on early peripheral nerve biopsy reveals absent small
adolescence with trophic ulcers as pain unmyelinated fibers and mitochondria are
sensation is affected. Many patients have abnormally enlarged.
accompanying nerve deafness and atrophy of
v. (Hereditary Sensory and Autonomic
the peroneal muscles. Histopathologic
Neuropathy): It also manifests with congenital
examination reveals a marked reduction in the
insensitivity to pain & anhidrosis. There is a
number of unmyelinated fibers. Motor nerve
selective absence of small myelinated fibers
conduction velocities are normal, but the
differentiating it from type 4
sensory nerve action potentials are absent.
ii. (Congenital Sensory Neuropathy): It is Diagnosis
characterized by onset of symptoms in early Diagnosing peripheral neuropathy is often difficult
infancy or childhood. Upper & lower because the symptoms are highly variable. A
extremities are affected with chronic thorough neurological examination is usually
ulcerations and multiple injuries to fingers and required and involves;
feet. Pain sensation is affected predominantly
and deep tendon reflexes are reduced. • Taking an extensive patient history (including
Autoamputation of the distal phalanges is the patient's symptoms, work environment,
common and so is neuropathic joint social habits, exposure to any toxins, history
degeneration. The NCV shows reduced or of alcoholism, risk of HIV or other infectious
absent sensory nerve action potentials and disease, and family history of neurological
nerve biopsy shows total loss of myelinated disease),
fibers and reduced numbers of unmyelinated • Performing tests that may identify the cause
fibers. It is inherited as an autosomal recessive of the neuropathic disorder, and
condition.
• Conducting tests to determine the extent and
iii. (Familial dysautonomia, Riley-Day type of nerve damage.
syndrome): It is an autosomal recessive
A general physical examination and related tests
disorder seen predominantly in Jews of eastern
may reveal the presence of a systemic disease
European descent. Patients present with
causing nerve damage. Blood tests can detect
sensory and autonomic disturbances.
diabetes, vitamin deficiencies, liver or kidney
Newborns have absent or weak suck reflex,
dysfunction, other metabolic disorders, and signs
hypotonia and hypothermia. Retarded
of abnormal immune system activity. An
physical development, poor temperature and
examination of cerebrospinal fluid that surrounds
motor in coordination are seen in early
the brain and spinal cord can reveal abnormal
childhood. Other features include reduced or
antibodies associated with neuropathy. More
Polyneuropathy 421

specialized tests may reveal other blood or Nerve biopsy involves removing and examining a
cardiovascular diseases, connective tissue sample of nerve tissue, most often from the lower
disorders, or malignancies. Tests of muscle strength, leg. Although this test can provide valuable
as well as evidence of cramps or fasciculations information about the degree of nerve damage, it
indicate motor fiber involvement. Evaluation of a is an invasive procedure that is difficult to perform
patient's ability to register vibration, light touch, and may itself cause neuropathic side effects. Many
body position, temperature, and pain reveals experts do not believe that a biopsy is always
sensory nerve damage and may indicate whether needed for diagnosis.
small or large sensory nerve fibers are affected.
Skin biopsy is a test in which doctors remove a
Based on the results of the neurological exam, thin skin sample and examine nerve fiber endings.
physical exam, patient history, and any previous This test offers some unique advantages over NCV
screening or testing, additional testing may be tests and nerve biopsy. Unlike NCV, it can reveal
ordered to help determine the nature and extent of damage present in smaller fibers; in contrast to
the neuropathy. conventional nerve biopsy, skin biopsy is less
invasive, has fewer side effects, and is easier to
Computed tomography, or CT scan, is a
perform
noninvasive, painless process used to produce
rapid, clear two-dimensional images of organs,
Management
bones, and tissues. Neurological CT scans can detect
bone and vascular irregularities, certain brain Physical Therapy assessment includes:
tumors and cysts, herniated discs, encephalitis, 1. History: including family and medical history
spinal stenosis (narrowing of the spinal canal), and or any exposure to any other drugs should be
other disorders. assessed.
Magnetic resonance imaging (MRI) can examine Family history: Majority have a family history
muscle quality and size, detect any fatty
of the condition, or having neuropathy in
replacement of muscle tissue, and determine
family history. Presentation of pes cavus or
whether a nerve fiber has sustained compression
abnormal gait represents of having some
damage. The MRI equipment creates a strong
inherited disorder like CMT. Family tree
magnetic field around the body. Radio waves are
then passed through the body to trigger a resonance tracking both sides of the family for 3
signal that can be detected at different angles within generations to rule out who was affected and
the body. A computer processes this resonance into what mode of inheritance is present whether
either a three-dimensional picture or a two- anybody had trouble with walking, balance,
dimensional "slice" of the scanned area. tripping, falling, or with their hands or
sensations should be find out.
Electromyography (EMG) involves inserting a fine
needle into a muscle to compare the amount of An autosomal dominant type will have people
electrical activity present when muscles are at rest affected in all generation and male-to-male
and when they contract. EMG tests can help transmission can be seen thus males are
differentiate between muscle and nerve disorders. severely affected. Whereas autosomal
Nerve conduction velocity (NCV) tests can recessive pedigrees may have only one person
precisely measure the degree of damage in larger or sibling affected with no family history
nerve fibers, revealing whether symptoms are being • At times with no family history does not
caused by degeneration of the myelin sheath or the preclude the diagnosis, as new mutations are
axon. During this test, a probe electrically stimulates relatively caused.
a nerve fiber, which responds by generating its own
electrical impulse. An electrode placed further Medical History: includes difficulty in
along the nerve's pathway measures the speed of balancing and problems in finding well-fitting
impulse transmission along the axon. Slow shoes owing to high foot arches and as history
transmission rates and impulse blockage tend to of surgery been done of tendon transfer like
indicate damage to the myelin sheath, while a tight heel cords, hammer toe straightening and
reduction in the strength of impulses is a sign of arthrodesis of ankle.
axonal degeneration.
422 Neuro-Rehabilitation : A multi disciplinary approach

2. Observation: 8. Deformities: like pes caves are common and


Analysis of movement, observation of the are associated with areflexia; give the
condition and shape of muscle contours likelihood of having CMT. It results from
should be noted. Functional difficulty like muscle imbalance as anterior tibialis and the
clumsiness in gripping a cup or difficulty in intrinsic foot muscles are affected with sparing
manipulating small items such as bottle tops of the gastrocnemius muscle thus the stronger
because of wasting of the intrinsic hand pull of gastrocnemies overcomes the weaker
muscles. Even muscle imbalances will lead to pull of anterior tibialis, leading to structural
shortening of unopposed muscle groups foot deformities. In upper limb, claw hand
resulting in contractures and deformities. deformities can be seen.

3. Cranial Nerve Examination: Generally cranial 9. Gait: Assessment of the gait reveals difficulty
nerves are normal at times abnormalities in in walking, twisting of ankles, slapping of the
papillary constriction and in optic nerve. feet, or loss of a heel-to-toe pattern, and the
patient may walk with a high-steppage gait
4. Sensory Examination: like pinprick, light (lifting legs up excessively to clear the toes)
touch, proprioception, vibration, which is due to tight heel cord, and weakness
graphesthesia and temperature should be in tibialis anterior leading to inadequate
assessed in terms of extent and pattern of strength to pull the foot up during ambulation.
involvement. Both small and large sensory Fractures are common. At times may require
nerve fibres show decreased or absent bilateral aids, such as ankle-foot orthoses, to
sensation. Reduction in pinprick and vibration ambulate. If quadriceps muscle weakens,
sensation is seen and more pronounced at the hyper extension occurs at the knees to produce
toes than in more proximal muscles with a rigid structure when weight bearing whereas
Sensory ataxia (i.e., imbalance and in- when proximal muscles in lower limbs like hip
coordination) due to loss of proprioception abductors weakness is seen it results in
may also be seen. positive Trendelenburgs sign positive.
5. Muscle Strength: should be assessed with 10. Autonomic Dysfunction: Sweating due to
MMT where decreased strength is noted in the poor vasomotor control leading to cold feet
distal muscles of the arms and legs like with blotching or pallor of the skin of the feet
intrinsic hand and foot muscles and tibialis is noticed.
anterior as distal muscles degenerate (axonal
loss) first while maintaining strength in 11. Neuropathic pain (burning, tingling,
proximal muscles and in the gastrocnemius shooting): may also occur as a result of the
muscle. They are also associated with atrophy neuropathy, while bone and joint pain may
and weakened foot eversion. Hand weakness result from pressure on the feet. Muscle
is also seen in terms of poor finger control, poor cramps and restless legs is also been noticed.
handwriting, and difficulty using zippers and Neuropathic pain can be treated with
buttons, and clumsiness in manipulating small gabapentin, pregabalin, duloxetine, and
objects.[4, 5] amitriptyline. Topical lidocaine patches may
help localized pain. Narcotic analgesics need
A hand held myometer has been shown to be carefully considered. Joint, bone, and
sensitive and reliable in assessing muscle pains require different approaches and
neuropathies. Weakness in hand muscles can should be treated appropriately.
be assessed by using a grip dynamometer
which is used in distal neuropathies. 12. Respiratory System: is examined in terms of
rate of respiration, chest expansion and vital
6. Reflex Testing: should be tested as deep capacity as in acute phase of polyneuropathy
tendon reflexes are diffusely absent (areflexia) especially in GBS, respiration is compromised
or reduced (hyporeflexia) and can occur in any and at times the patient may need ventilator
condition involving nerve damage. for respiratory assistance.
7. Girth measurement: is done in order to detect 13. Fatigue testing: Fatigue can seen in any
the degree of wasting or atrophy of the activities such as getting tired while writing
muscles. letters or walking distances. Fatigue severity
Polyneuropathy 423

scale has been demonstrated to be reliable suggested as a mean to maintain range of


measure in neuropathies. The patient is asked movement. Even passive ROM is given 2-3 times
to maintain a diary documentation activities in a day. Tightening of posterior crural muscle
and length of time performing each one and group develops rapidly. Able patients should be
the symptoms of fatigue. Later it is to be taught self -stretches in a weight-bearing position.
analyzed to try and develop a structure to the For bedridden patients, foot drop splints should be
patients activities that prevent further fatigue provided and gentle stretches performed. Thus
and also to check whether this is not leading muscles which crosses two or more joints must be
to disuse by cutting out all activity that cause stretched to its full length.
any degree of fatigue.
2. Positioning:
14. Functional Assessment: is also measured to
any change in performance level. For e.g. ability to Frequent position changes are recommended in bed
rise from a chair and standing on heels in case of ridden patients to prevent selective muscle
diabetic neuropathy. shortening and pressure sores. Truncal weakness
can cause the patient to lie with scapulae retracted,
15. Electrophysiological tests: is done to unless a wedge is positioned under the thoracic
differentiate between a demyelinating and an region as well as the head and shoulders. The pelvis
axonal process. It serves as a prognostic factor in should be supported in a position of posterior tilt
determining whether there are any axonal changes with the hip flexors stretched, to prevent
as seen in GBS where once axonopathy is shortening. Every two hourly turning helps in
permanent, there are no changes left for repairing preventing pressure sores. If the sore is developed,
the axon. ultra-violet radiation is given to enhance the healing
16. Gene testing and counselling: Genes for process. The patient's position is maintained with
inherited neuropathies can be identified by the help of sand bags, pillows etc. If splint is given,
deoxyribonucleic acid (DNA) testing. Once a then frequent checking is maintained to avoid the
patient is shown to have the gene, counseling can skin breakdown around the application area.
be offered and the implications for other family
members are discussed. Prenatal testing can be 3. Maintenance of Circulation:
identify a fetus at risk of developing an inherited Passive exercises help in maintaining the
neuropathy and give the parents the option of circulation.
termination.
4. Pain :
Management: Pain is an early symptom in acute neuropathy such
as GBS due to spontaneous discharge in
Physiotherapy Interventions:
demyelinated sensory neuropathies. Freeman
Acute Neuropathies: suggested that the reason why patient with acute
GBS enjoy large amplitude mid range movements
Physical management in acute neuropathies
is because of their pain relieving properties.
includes prevention of contractures, control of pain
and respiratory care.
5. Respiratory Care:
1. Stretching to prevent contractures. In acute cases, the patient may be ventilated and
the role of physiotherapist is to help prevent
Neuropathy may result in severe disability making
atelectasis when breathing is compromised. Since
the patient totally bed-ridden. When the muscles
the respiratory system is involved, the aim would
are not stretched adequately but are left in a
be to maintain clear airway by removing the lung
shortened position, structural changes, involving
secretions through the following technique;
loss of sarcomeres, occur and compromises
potential recovery. The therapist should ensure that 1. Intermittent positive pressure breathing.
all structures including the nervous system are
2. Postural drainage.
moved through their full range. Adverse neural
tension signs can occur if neural tissues are not 3. Suction catheter.
stretched. 4. Vibration and rib shaking.
Continuous passive motion machine has been
424 Neuro-Rehabilitation : A multi disciplinary approach

Tandem walking Side Sitting in Quadrapud (a)

Side sitting in Quadrapud (b) Weight Shifts in Quadrrapud

One leg standing Balance Board Exercises.


Polyneuropathy 425

Once the patient is weaned from the ventilator, he well as preventing tripping. Abnormally prolonged
is taught effective coughing and good breathing muscle ache following exercise or sudden loss of
exercises. functional ability indicates damage due to overuse
and modification of exercise to be tailored made in
When there is facial muscle weakness, care must
order to allow recovery.
be taken to ensure there is lip seal around the mouth
piece when measuring vital capacity. Where the In one randomized study where CMT patients
autonomic system is affected, disturbed blood underwent home-based strength training were
pressure is seen when attempting suction or making evaluated using outcome measures such as
early sitting. isokinetic knee extension and flexion; maximal
voluntary contraction; endurance at 80% MVC;
Chronic Neuropathies: ability to descend and climb stairs and stand up
Physiotherapy plays a major role in chronic from a chair or from lying supine; and time to walk
neuropathies which help in maintaining 6 m at a comfortable pace or 50 m quickly showed
ambulation and prevention of contractures. It helps a significant reduction in the time taken for a 6-
in facilitating functional recovery in bedridden meter walk at 24 weeks after starting the exercise
patients of neuropathies thereby preventing with no other significant improvements in time-
complications and improving the quality of life of scored functional activities
patients with chronic neuropathies. Both studies observed limited improvement in
Physiotherapy interventions in chronic upper-body and lower body strength, but Kilmer
neuropathies include: and coauthors [88] observed a high number of
injuries in their patients and concluded that
1. Strength Training : increases in training frequency, volume, and
The aim is to maintain the strength of weak especially intensity may put patients with
muscles which can be achieved by the neuromuscular disease at increased risk of training-
following techniques: induced injury.

1. Free active exercises. Recently 24-week cycling program using a


cycloergometer in limited number of patients (4
2. Proprioceptive Neuromuscular with CMT1A and 4 with CMT2) proved to be
Facilitation (PNF). effective in improving exercise tolerance and
3. Progressive resisted exercises. functional ability in the absence of significant
changes in fatigue resistance. Another study tested
4. Suspension therapy exercises.
the effects of a 12-week, home-based resistance
5. Equilibrium and righting reactions. exercise program on strength, body composition,
and activities of daily living (ADLs) in 9 men and
Low-intensity to moderate intensity strength
11 women with CMT1A (n=18) and CMT2 (n=2).
training programs in patients with neuromuscular
Strength and ADLs improved equally in men and
disease, including Charcoat Marie Tooth disease
women, and there were no differences between
(CMT), show varying results.
CMT1A and CMT2. Therefore, similar rehabilitative
Kilmer et al.1994 found that higher resistance approaches may be useful in CMT, but with the
exercise was no better than low resistance exercises safety and efficacy. Also, overloading of the
for strengthening muscle and there was some weakened muscles can cause fatigue and weakness
evidence of damage when tested eccentrically. thus overloads principle need to be considered.
Endurance training can improve cardiopulmonary
fitness seen in established neuropathies. Six week 2. Stretching:
home programmed of strengthening, stretching and Gentle stretches for muscle groups particularly
aerobic conditioning increases strength and quality in HMSN, are done to the muscles that are
of life. liable to shorten thereby preventing muscle
Concentrating on the hip and knee helps in tightness and loss of strength. Tightness and
compensating and producing a more stable gait contracture affects the extensibility of muscle
pattern. The additional AFO would also increase and surrounding connective tissues. Regular
stability by allowing the development of plantar stretching of 15 to 30 seconds trice a day can
flexion movement despite very weak muscles, as prevent or reduce joint deformities that may
426 Neuro-Rehabilitation : A multi disciplinary approach

result from uneven pulling of muscle on bones. 6. Functional and mobility aids used for
Serial night casting for 4 weeks induced a small normalizing the gait:
increase in ankle dorsiflexion range in children
Aids used for function and mobility include a
and young adults with CMT.
range of orthotics and wheelchairs.
A recent case report suggested that when
Gait and balance problems are important risk
triceps surae muscles are weakened, stance
factors for falls in CMT as well as in other peripheral
and gait gets affected however range of motion
neuropathies. Where ever there is an abnormal gait
is affected.
pattern, mainly due to the weakness of the
3. Re-education of sensory awareness: The dorsiflexors and /or the intrinsic muscles of the feet,
sensory system can be stimulated by the the use of orthosis should be advised. It has been
cutaneous stimulation which can be given by seen that foot deformities have become irreversible
the different material, textures, shapes and and insoles are necessary to allow the foot to sit
weights. Equilibrium and righting reactions comfortably in a standard shoe and to redistribute
help in reeducating the proprioception of the weight across the total surface of the sole. When
sensory system. Vision can also be used as an foot drop exists, light polypropylene splint should
alternative system in reeducating the sensory be advised.
awareness.
It is essential to discuss skin care with the patient
4. Postural kinesiotherapy: May be helpful in as most of it has sensory deficit also.
reducing the need to control joints from three
Ankle-foot orthoses (AFOs) may be useful in
joints (hip, knee, and ankle) to one joint (hip),
treating patients with CMT. AFOs compensate for
and proprioceptive kinesitherapy may help to
weakness and correct foot drop, can offer a control
improve coordination.
of the foot, can help control unwanted inward
5. Balance Training : rotation of the foot, and facilitate a more normal
Balance training plays major role as weakness gait pattern.
and deformities at foot reduces balance Prescription of AFO for a patient with CMT can
thereby increasing walking in stability. Thus enhance physiological performance and perceived
it should be emphasize by including the exertion at sub maximal activity levels.
following exercises:
Falls can also be prevented by paying attention to
1. Standing with feet apart in comfortable the floor, avoiding uneven ground, being wary of
posture. rugs and carpets, avoiding dark places, using a
2. Standing with feet apart and keeping the handrail when going up and down the stairs,
arms in different positions for support. avoiding haste, and staying slim. When falls are
3. Standing with close feet. frequent, a walking stick, one or two crutches, or a
walking frame is required; it can also be advisable
4. One leg standing.
to use a wheelchair outside of the house.
5. Repeat all the exercises with eyes closed.
Wheelchairs: In some of cases, the neuropathy
6. Balance boards exercises.
progresses to render the patient dependent on a
7. Quadruped side sitting. wheelchair. For e.g. in the early stages of
8. Hip extension in quadruped. rehabilitation, a patient with GBS, a reclining
9. Bilateral arm and leg raise in quadruped. wheelchair is valuable to coping up possible
10. Weight shifts in forward, backward and fluctuations in blood pressure and to allow gradual
sideways direction in quadruped. accommodation in the upright position.
11. Tandem walking. Walking sideways and 7. Manual dexterity training :
backward. Hand function is also compromised through
12. Walking and stopping alternately. weakness or paralysis of intrinsic muscles of
13. Ask the patient to walk in circles. the thumb and fingers causing difficulty in
pincher gripping and finger movement.
14. Walk on toes.
Normal daily activities such as dressing,
15. Walk on heels. bathing, holding cutlery, or writing becomes
16. Intrinsic strengthening. difficult. Tripod pinch strength and thumb
Polyneuropathy 427

opposition are major determinants of manual small diameter C fibers and acts on
dexterity in CMT and should therefore be the mechanisms of endogenous opiates
focus of intervention strategies that aim to thereby blocking pain gate mechanisms.
preserve or enhance manual dexterity in CMT. 5. Ultrasound: has also been used in
Simple thumb opposition splint may allow a peripheral neuropathy as it reduce
patient to produce legible writing for a longer muscle spasm and pain thereby acting on
time, or to grip a cup or knife. At times when both large diameter A fibers and small
there is thenar eminence weakness and diameter Cfibers.
wasting is being seen, a night splint cast in a
functional position will help prevent severe B) Cognitive Strategies: are used in treating
contracture. Use of putty and rubber bands of together the body, mind and soul.
different strengths can be used to improve C) Behavioural interventions: Distraction -
strength of hand and forearm muscles. listening to music or using imagery techniques
8. Adaptive devices: can be helpful during brief episodes of pain
or painful procedures. Music therapy has been
Special tools modification is given at times in used successfully to reduce disruptive
order to avoid overwork and weakness behaviour or aggression attitudes.
thereby improving performance. Hand or
forearm splinting may be advised later once 10. Aerobic Exercises:
wasting of the hand muscles sets in. Adaptive Patients with chronic neuropathies show
equipment may be prescribed to compensate reduced peak oxygen consumption and
for the hand deformities, sensory loss, and decreased functional aerobic capacity, and
weakness which include a button-hook, a long- studies shows aerobic exercise improve
handled shoehorn, and elastic shoelaces. functional ability and aerobic capacity. Aerobic
9. Neuropathic Pain Management: walking has been used in neuromuscular
disorders and was effective in ameliorating
Malalignment of joints due to muscle peak power output and peak oxygen intake,
imbalance often leads to pain. In chronic walking ability, and metabolic changes.
neuropathies, neuropathic pain is seen which
is caused by damage or dysfunction in the 11. Hydrotherapy: is advised as it increases the
nervous system, which includes the spinal muscle power, improve coordination and
cord. It can generally be described as a sharp, balance of the patient. Care is taken in case of
shooting, or burning pain, flaccid joint. Pain which is also a feature of
polyneuropathy can be relieved by
Management of neuropathic pain becomes very hydrotherapy as water has pain relieving
important as it hinders to the day-to-day activities property.
of patients. Pain management can be divided into
physiotherapy, behavioural and cognitive Occupational Therapy Management
interventions.
Occupational therapy professionals should be
A) Physiotherapy interventions include: involved early in the rehabilitation program to
promote upper body strengthening, ROM, and
1. Thermotherapy - can reduce muscle
activities that aid functional self-care. Both
spasm thereby relaxing the muscle.
restorative and compensatory strategies can be used
2. Cryotherapy - can be used in case if there to promote functional improvements. Energy
is any swelling at some distal joints. conservation techniques and work simplification
also may be helpful, especially if the patient
3. Massage - can reduce muscle tension by
demonstrates poor strength and endurance.
gentle touch thereby increasing local
Participation in recreational therapy assists in the
blood circulation and relaxing muscles
patient's adjustment to disability and improves
and soft tissues.
integration into the community. Recreational
4. Transcutaneous Electrical Nerve activities, either new or adapted, can be used to
Stimulation (TENS) : is proved to be promote the growth, development, and
useful in neuropathic pain. Low rate independence of a long-term hospital patient.
TENS or Acupuncture Like TENS acts on
428 Neuro-Rehabilitation : A multi disciplinary approach

Occupational Therapy assessment includes: a greater surface area.


• History • Do not persist an activity for prolonged
• Muscle Strength periods of time. Instead, change the tool used
• Range of Motion and the work task often.
• Physical Endurance • Visually examine the skin for edema, redness,
• Gross Motor coordination warmth, blisters, cuts or other wounds (tissue
heals more slowly when there is nerve injury).
• Fine Motor coordination, manipulation
and dexterity • Before bathing or showering, use a
• Sensory Registration or awareness thermometer to test the temperature of the
water.
• Sensory processing: discrimination of
light touch, stereognosis, proprioception, • Always use pot holders when cooking or
and two-point discrimination handling items on a stove or in an oven, and
• Cranial Nerve Examination use thick gloves when washing dishes or
working with sharp utensils. Wear low- or flat-
• Pain
heeled shoes that are of an appropriate size,
• Respiration and wear thick socks to protect against blisters.
• Swallowing
• ADLs Motor intervention
• IADLs After partial or complete denervation of muscle
tissue and during inactivity or disuse, muscle
• Productivity history, skills, interest and
strength decrease. Active- assisted, active exercises
values
are used to increase strength when strength is
• Leisure inadequate substitution pattern or trick movements
are likely to develop.
Occupational Therapy Intervention:
Occupational therapy is instrumental in helping the Suspension exercises are advised for all the
patient cope with the functional, vocational, and shoulder muscles. Many purposeful activities can
social impact of peripheral neuropathy by: be given to maintain/improve strength.

• Improving sensory-motor skills


• Teaching self-care activities
• Teaching the patients management of fatigue.
• Teaching the patient safety issues, (e.g., paying
more attention to the terrain when walking
since falling or tripping may pose a risk for
patients with PN)

Management to improve Sensory Motor Skills:


People with lack of protective sensation are at risk
Clay pressing
of serious injury since they can not feel pinprick or
hot or cold exposures In the presence of contracture, Passive ROM with
gentle movement of joints should be given and it
Some protective sensory reeducation techniques
should not exceed the point of pain.
include:
Manual dexterity is of utmost importance in
• Protect from being exposed to sharp items or
performing activities of daily living and is described
to cold or heat
as the ability to move the hands easily and skillfully,
• Try to soften amount of force when gripping to work with the hands in turning and placing
an objects motions.
• Use built-up handles on objects whenever Some Standardized Test for Hand Functions: In
possible to distribute gripping pressure over HSMN, distal hand muscles weakness leads to
Polyneuropathy 429

difficulty in gripping object and performing fine


motor activities.
There are some standardized tests to evaluate
dexterity of hands.
Sollerman hand function test (SHT): This test is used
for the evaluation of dexterity in HSMN. This test
was developed to provide an overall measure of
hand and grip function when engaging in ADLs
(Sollermen & Ejeskar 1985). It was designed to
measure grips that are needed for certain ADLs
such as eating, driving, personal hygiene, and
Clay pinhing
writing. The test includes subtests that represent
common handgrips (volar, transverse volar,
box to hide things inside. Encourage tearing with
spherical volar and pinch positions - pulp, lateral,
the thumb, index and middle fingers to perform
tripod, and the five finger) and activities (using a
fine motor coordination.
key; picking up coins from a flat surface; writing
with a pen; using a phone; and pouring water from Vegetable stamping-Cut vegetables such as onions
a jug). and potato in half and dip in paint. Print on paper
taped to wall to strengthen shoulder, wrist and
Functional Dexterity test (FDT): fingers muscles.
The FDT measures the ability to perform a tripod Tongs-Use tongs of different sizes and stiffness to
pinch through the timed manipulation of pegs develop hand strength. Pick up objects such as
(administration time about 5 min). A tripod pinch cotton balls, marbles, small dried peas etc; this can
pattern is frequently used during daily activities be graded to increase resistance and skill.
such as eating, writing and tying. This grip pattern
Elastic band pulls-Use elastic bands of different
in particular, may become problematic when the
thickness resistance and, stretch them over pegs in
intrinsic muscles of the hands are affected.
a board or nails hammered into wood etc to
Activity to Improve Hand Strength and Dexterity strengthen intrinsic muscles of hand.
Play dough/cookie dough-Rolling it out, pulling it Sealing bags-Use Jiffy bags or equivalents that can
apart, forming a ball between the palms or a small be sealed by pressing the edges together. Get your
ball with the fingers, pinching it with thumb and child to use just the thumb and index/middle
index finger. fingers if possible
Tug of war-This increase gross grasp strength and
contraction in the bigger joints up the arms. Use a
towel, sheet or soft rope in sitting or standing
position.
Screwing and unscrewing-Use a variety of bottles
and containers with screw tops to build finger
strength. Screwing and unscrewing of nuts and
bolts increase strengthen the small muscles in the
fingers.
Buttering bread-Good for grasp strength and also
provides an opportunity to develop bilateral skills
Clay rolling and motor planning and sequencing skills.
Posting activities-Pushing pennies or dried pulses Peeling fruit-peeling fruit develops finger strength
into a slot or hole in the top of a Pringles tin box or
a plastic container. Vertical drawing/vertical activities-Drawing on a
vertical surface is an excellent way to improve grasp
Tearing activities-Tearing paper, junk mail etc into strength. Break the chalk or crayon into small pieces
strips then crushing it into a ball and filling up a of around half to one inch long as this helps to
430 Neuro-Rehabilitation : A multi disciplinary approach

develop the tripod grasp and encourages a better • If holding one or both arms up to wash
wrist position. Working on an elevated vertical or style hair is fatiguing, try sitting at a
surface also improves shoulder stability. desk or dressing table. Prop an elbow on
a book (or 2) covered with a soft towel.
Because many neuropathy patients lose sensations
in their hands and feet, it may be necessary to take
D. DRESSING:
several steps to ensure that everyday tasks are safe.
• Sit down to dress
Management for maximizing independence in • Reorganize closet so that the shelves are
ADLs: (Self Care Activities) lower and clothes are hung at a lower
Adaptive and assistive devices may be introduced height
to decrease effort during daily activities • Use long handled shoehorns and sock
aids
BATHROOM: • Wear supportive and proper fitting shoes
A. BATHING: • Wear "pretend" or clip-on neckties
• Sit on a tub bench or bath stool • To make zippers easier to grasp, use a
zipper pull, add a loop chain or large
• Use a bath mitt or long handled brush/
paper clip
sponge
• Replace buttons with velcro if possible,
• Install grab bars around the tub
or use a buttonhook
• Install lever-type faucets or build up the
faucet handles to decrease stress on Management for fatigue
hand/finger joints
An important aspect of treatment is to educate the
• Use a non-skid rubber mat or strips in the client and family members to use energy
tub or shower conservation and work simplification strategies
• Keep towels in easy reach when engaging in ADLs, IADLs and leisure
• Soap on a rope or liquid soap to avoid occupations.
dropping soap
II. KITCHEN:
• Do not use glass containers that could be
dropped and broken A. ORGANIZING SPACE/WORK AREA
• Keep pots and frying pans near the stove
B. TOILETING:
• Keep frequently used appliances (e.g.
• Put grab bars around the toilet
Toaster) on the counter
• Use a raised toilet seat • Set up a specific area to make coffee (i.e.
• If a raised toilet is too high, then push a A coffee station where you keep
low footstool under your feet once you everything you need nearby.
are sitting • Keep all baking equipment in one
cupboard
C. GROOMING:
• Keep items to the front of the shelves and
• Build up or extend the handles on the rows shallow
brushes, combs, toothbrushes etc., using
• Use plastic lid holders on inside of
rulers, foam rubber, or pipe insulation
cupboard doors to keep lids organized
• Use an electric toothbrush
• Eliminate clutter by organizing drawers
• Use pump dispenser type toothpaste. If and dividers
you have to squeeze out toothpaste,
• Keep unused duplicate items in other
squeeze the tube between both palms or
areas if needed or give away or throw out
place the tube over a damp washcloth
(i.e. scissors)
and lean onto it
• Keep frequently used items to the front
• Place a foam curler over eyeliner or
of the shelf and items used less often to
lipstick to build up a handle
the back
Polyneuropathy 431

• Use space savers (e.g. lazy susans) and • Use an easy to grip sponge to clean up
pullout shelves rather than a thin dish cloth
• Keep heavy jars and boxes at waist level
III. LAUNDRY:
• Store only light objects on the higher • Do only what is necessary: buy
shelves only if absolutely necessary and permanent press clothing
use a long handled reacher or tongs to
reach items • Use separate baskets to sort clothes before
bringing them to the laundry room or
• Keep heavy items on top shelf of keep hampers/baskets in the laundry
refrigerator, near the front room to collect dirty laundry

B. COOKING: • Use a rolling cart to move wet clothes to


the clothesline if a dryer is not available
• Plan meals ahead to decrease/prevent
last minute tasks • Sit when sorting, folding and ironing
• Use precut vegetables, chopped nuts clothes (some ironing boards are
(convenience food) adjustable)

• Sit town while preparing vegetables, • Label a basket for each family member
meat, etc., for cooking and have each one put their own laundry
away
• Use a mirror over the stove to monitor
food while sitting • Sort clean clothing and linen into different
baskets and have other family members
• Use electric appliances (e.g. Microwave,
put them away.
electric mixer/can opener/knives/fry
pan) • Use a rolling cart if you put the laundry
• Can use egg slicer for any soft vegetables away

• Place a damp cloth underneath a bowl or HOUSECLEANING:


plate to keep it from moving • Store cleaning supplies everywhere they
• Use lightweight dishes/pans and serve are used
from them • Wear an apron with large pockets to carry
• Use a wheeled cart to move heavy items necessary cleaning supplies
around
• Use a sponge mop with an easy lever-type
• Re-arrange kitchen to make it accessible squeezer, a "janitor's pail". move the
from a seated position bucket/pail of cleaning solution on a
• Prepare large amounts of food that rolling dolly/caster
freezes well, then freeze for later meals • Use a long handled feather duster or dust
• Use built up handled utensils for more with a mitt
comfortable grip • Use automatic toilet bowl cleaner & spray
• Use wall mounted manual or electric jar on mildew remover to eliminate
and can openers. scrubbing
• Put casters on furniture so they can be
C. CLEAN-UP: moved easily
• Use disposable aluminum baking pans or • When making beds, finish one side at a
plastic baking bags time
• Use a nonstick product on pans or line • Alternate heavy and light tasks, and
with foil before baking or frying
remember to take a break between/
• Sit on a stool while washing dishes during tasks
• Use a wheeled cart to collect dishes from • Do only one major cleaning task a day
table (e.g. Laundry, cleaning the bathroom)
432 Neuro-Rehabilitation : A multi disciplinary approach

WORKPLACE: General Precautions:


• Keep frequently used, necessary items in • Watch for redness over bony areas of the body
easy reach and change the person's position every two
• Sit in a swivel chair with proper hours
adjustable back support • Watch the person for signs of fatigue. Do not
• Set the work surface at a comfortable continue activity if signs of fatigue are present
height • Have the person maintain good posture and
• If you are required to make many positioning at all times to protect joints while
errands, use the elevator and organize the muscles are weak.
errands to make as few trips as possible • Avoid irritation of inflamed nerves. Go slowly
LEISURE: on exercise program.
• Use a card rack for holding playing cards • Teaching the patient to pay attention to issues
which involve automatic functions (e.g.,
• Use a bookstand. Lay the newspaper on
learning how to change positions smoothly to
an open table rather than holding it up
avoid a sudden drop in blood pressure and
with your arms
the risk of falling)
• Use felt tip pens which require less
pressure to write. Also, use larger sized References:
pens for easier grip
1. Brown MJ, Asbury AK: Diabetic Neuropathy.
• Use a push-button phone or pen tip to
Ann Neurol 15:2-12, 1984
dial
2. Neuropathy in Diabetes by Richard C.
• Attach a gooseneck to the table then to
Eastman, MD
the telephone receiver to eliminate
holding the receiver. 3. De Jonghe, P., et al., A novel type of hereditary
• Get a headset for the phone to eliminate motor and sensory neuropathy characterized
the need to hold the receiver. by a mild phenotype Arch Neurol,1999

GARDENING OR YARDWORK: 4. Occupational Therapy Willard and


Spackmans-10th edition
• Use lightweight tools with extended or
built up handles. 5. Pedretti's, Occupational Therapy- 6th edition

o Use a gardening stool 6. Imreova H, Pura M. Differential diagnosis


ofperipheral neuropathy. Cas Lek Cesk 2005
o Use raised flower boxes or window boxes [Article in Czech]
FOR TRAVEL: 7. Imreova H, Pura M. Differential diagnosis of
o ask for a wheelchair escort to the gate peripheral neuropathy. Cas Lek Cesk 2005
[Article in Czech]
o Ask a porter to assist with luggage or get
a luggage cart, or get luggage with wheels 8. orlikowski D, Prigent H, Sharshar T,et al:
Respiratory dysfunction in GBS
o use elevators, escalators moving
walkways 9. Diavkara Kedlaya: Physical Medicine and
Rehabilitation for Charcot-Marie -Tooth
CAR: Disease : Clinical presentation
o get power options steering, windows, 10. 2. Shy M, Lupski JR, Chance PF, et al. The
brake, locks, seat controls hereditary motor and sensory neuropathies:
o use a wide-angled mirrors if you have an overview of the clinical, genetic,
decreased movement in your neck electrophysiologic and pathologic features. In:
Dyck PJ, ed. Peripheral Neuropathy. Vol 2. 4th
o build up the key tops or use key holders
ed. Philadelphia, PA: WB Saunders; 2005:1623-
to make turning easier
1658.
Polyneuropathy 433

11. Hoogendijk JE, Hensels GW, Gabreels-Festen 85(8):1217-23.


AA, et al. De-novo mutation in hereditary
19. Vinci P, Esposito C, Perelli SL, Antenor JA,
motor andsensory neuropathy type 1. Lancet.
Thomas FP. Overwork weakness in Charcot
1992;339:1081-1082.
Marie Tooth. Arch Phys Med Rehabil. 2003;
12. Chung KW, Kim SB, Park KD, et al. Early onset 6:825-7.
severe and late-onset mild Charcot-Marie-
20. Rose KJ, Raymond J, Refshauge K, North KN,
Tooth disease with mitofusin 2 (MFN2)
Burns J: Serial night casting increases ankle
mutations. Brain. 2006;129:2103-2118.
dorsiflexion range in children and young
13. Darcy Umphered: Neurological adults with Charcot-Marie-Tooth disease: a
Rehabilitation, 5th edition. randomized trial. J Physiother.2010; 56(2): 113-
19.
14. El Mhandi L, Millet GY, Calmels P, et al.
Benefits of interval-training on fatigue and 21. Kilmer DD. Response to aerobic exercise
functional capacities in Charcot-Marie-Tooth training in humans with neuromuscular
disease. Muscle Nerve. 2008;37:601-610. disease. Am J Phys Med Rehabil. 2002;
81: S148-50.
15. Karas MA, Hoy DJ. Compensatory midfoot
dorsiflexion in the individual with heelcord 22. Bean J, Walsh A, Frontera W. Brace
tightness: implications for orthotic device modification improves aerobic performance in
designs. JPO.2002;14:82-93. Charcot-Marie-Tooth disease: a single subject
design. Am J Phys Med Rehabil.
16. Aitkens SG, McCrory MA, Kilmer DD,
2001;80(8):578-82.
Bernauer EM : Moderate resistance exercise
program: its effect in slowly progressive 23. Vinci P, Perelli SL, Esposito C. Charcot-Marie-
neuromuscular disease. Arch Phys Med Tooth disease: poor balance and rehabilitation
Rehabil. 1993;74(7):[Link] Phys Med [abstract]. J Peripher Nerv Syst. 2001;6(1):58.
Rehabil. 1994;75(5):560-3.
24. Videler AJ, Beelen A, Van Schaik IN,
17. El Mhandi L, Millet GY, Calmels P, Richard Verhamme C, Van Den Berg LH, De Visser M.
A, Oullion R, Gautheron V, et al. Benefits of Nollet F: Tripod pinch strength and thumb
interval-training on fatigue and functional opposition are the major determinants of
capacities in Charcot?Marie?Tooth disease. manual dexterity in Charcot-Marie-Tooth
Muscle Nerve. 2008;37(5):601-10. disease type 1A. J Neurol Neurosurg
Psychiatry. 2010;81(8):828-33.
18. [Link] RD, Gutmann L, Tarnopolsky M,
Ullrich IH, Yeater RA. Resistance training 25. [Link]: Handbook of Physical
effectiveness in patients with Charcot-Marie- Medicine and Rehabilitation, 2006 edition.
Tooth disease: recommendations for exercise
26. J Nicklin etal : Disordres of polyneuropathies;
prescription. Arch Phys Med Rehabil. 2004;
Maria strokes 2006 edition.
434 Neuro-Rehabilitation : A multi disciplinary approach
Polyneuropathy 435

Section 4
Miscellaneous
436 Neuro-Rehabilitation : A multi disciplinary approach
Spasticity 437

Ch.12 Spasticity
Dr. Alok Sharma, M.S, MCh, Dr. Sanjay Kukreja, M.B.B.S, Dr. Naren Naik, M.S, MCh,
Dr. Nancy Thomas, MPTh (Neuro), Dr. Hema Biju, MOTH (Neuro)

Lance in 1980 gave the definition of spasticity as include clonus, the clasp-knife phenomenon,
'Spasticity is a motor disorder characterized by a hyperreflexia, the Babinski sign, flexor reflexes, and
velocity-dependent increase in tonic stretch reflexes flexor spasms.
(muscle tone) with exaggerated tendon jerks, The stretch reflex arc is the most basic neural circuit
resulting from hyperexcitability of the stretch reflex, contributing to spasticity. When a muscle is
as one component of the upper motoneuron stretched, an impulse is generated in the muscle
syndrome 1 spindle and is transmitted via the sensory neuron
Upper motor neurons originate in the brain and to the grey matter of the spinal cord. Here the
brain stem and project to lower motor neurons sensory neuron synapses with the motor neuron,
within the brain stem and spinal cord.2 The lower and the transmitted impulse results in muscle
motor neurons are of two types, both of which contraction. While agonist muscles contract in
originate in the ventral horn of the spinal cord: (1) response to stretching, antagonist muscles must
alpha motor neurons project to extrafusal skeletal relax. Their relaxation is brought about via an
fibers and (2) gamma motor neurons project to inhibitory neuron within the spinal cord.
intrafusal muscle fibers within the muscle spindle.2
The alpha motor neuron and the muscle comprise
With a lesion of the CNS comes interruption of the
the final common pathway in the expression of
signals sent via the upper motor neurons to the
motor functions, including spasticity. There are
lower motor neurons or related interneurons.
numerous excitatory and inhibitory modulatory
Spasticity usually is accompanied by paresis and synaptic influences on this pathway. An imbalance
other signs, such as increased stretch reflexes, in these influences results in hyperexcitability of the
collectively called the upper motor neuron stretch reflex arc, which is thought to be the basis
syndrome. Paresis generally affects distal muscles, for spasticity. Some of the factors that play a role in
with loss of the ability to perform fractionated suppressing hyperactivity of the final common
movements of the digits. The upper motor neuron pathway (Fig. 2) include cerebral inhibitory
syndrome results from damage to descending pathways (from the brain) and spinal mechanisms
motor pathways at cortical, brainstem, or spinal such as nonreciprocal Ib inhibition (from golgi
cord levels and spasticity evolves in the days and tendon organ receptors in tendons), presynaptic
weeks after injury. When the injury that leads to inhibition of the Ia terminal (at the axoaxonic
spasticity is acute, muscle tone is flaccid with synapse between 2 axons), reciprocal Ia inhibition
hyporeflexia before the appearance of spasticity. (inhibition of antagonistic muscles) and recurrent
The interval between injury and the appearance of Renshaw inhibition (inhibitory feedback of the
spasticity varies from days to months according to alpha motor neuron cell body by the inhibitory
the level of the lesion. In addition to weakness and interneuron).3
increased muscle tone, the signs in spasticity

Fig. 1: The stretch reflex arc.


438 Neuro-Rehabilitation : A multi disciplinary approach

Fig. 2: Potential spinal mechanisms of suppression Spastic dystonia is primarily due to abnormal
of hyperactivity in the final common pathway supsraspinal descending drive, which causes a
(alpha motor neuron and muscle). There are failure of muscle relaxation and is sensitive to the
numerous excitatory and inhibitory modulatory degree of tonic stretch imposed on that muscle
synaptic influences on this pathway. An imbalance (Denny-Brown 1966). There is inappropriate
in these influences results in hyperexcitability of recruitment of antagonist muscles in spastic co-
the stretch reflex arc, which is thought to be the contraction upon triggering of the agonist under
basis for spasticity. Factors that play a role in volitional command. This occurs in the absence of
suppressing hyperactivity of the final common phasic stretch and is sensitive to the degree of tonic
pathway at the spinal cord level include stretch of the co-contracting antagonist (Gracies et
nonreciprocal Ib inhibition (from golgi tendon al. 1997). For instance, triceps will be recruited
organ receptors in tendons), presynaptic inhibition during volitional action of biceps and will lead to
of the Ia terminal (at the axoaxonic synapse between elbow stiffness Associated reactions are found in
2 axons), reciprocal Ia inhibition by the inhibitory muscles that are not particularly stretch sensitive.
interneuron (inhibition of antagonistic muscles [see They include, when there is extra-segmental co-
Fig. 1]) and recurrent Renshaw inhibition contraction due to cutaneous or nociceptive stimuli,
(inhibitory feedback of the alpha motor neuron cell or inappropriate muscle recruitment during
body by the inhibitory interneuron). autonomic or reflex activities, such as yawning.
The resultant pattern is determined by the age, size
Pathophysiology and location of the lesion. Supra-bulbar lesions
Spasticity arises from prolonged disinhibition of present predominantly with flexor patterns of
spinal reflexes as a result of UMN lesion. These spasticity, whereas spinal cord lesions produce
spinal reflexes include stretch, flexor and extensor extensor patterns predominately. Patients with
reflexes and are under supraspinal control by partial lesions, where sensation is intact or partially
inhibitory and excitatory descending pathways. intact, are typically bombarded by nociceptive
Stretch reflexes are proprioceptive reflexes, and are inputs and display greatly increased ?-motor
either phasic or tonic. The tonic stretch reflex arises neuron activity. Different patterns emerge early on
from a sustained muscle stretch and is the cause of after the neurological insult and later, when patients
spasticity (Sheean 2002). Stretch reflex is dependent may find themselves in a rehabilitation unit. The
on tendon lengthening and excitatory post synaptic following figure shows the effects of the different
potentials (EPSPs) carried by I-a afferents but scenarios.
Inhibitory post synaptic potentials (IPSPs) arising
from antagonistic muscle spindles, oligosynaptic
and polysynaptic pathways also have an important
role in the maintenance of tone (Lance 1980, Young
1994, Nathan 1973).
Damage to pyramidal tracts alone does not result
in spasticity. It occurs only when the lesion involves
premotor and supplementary motor areas. Selective
damage to area 4 in the cerebral cortex of primates
produces paresis that improves with time, but
increases in muscle tone are not a prominent
feature. Lesions involving area 6 cause impairment
of postural control in the contralateral limbs.
Combined lesions of areas 4 and 6 cause both
An explanation of Figure 1 is available
paresis and spasticity to develop.[3] Physiologic
evidence suggests that interruption of Immediately after injury, a period of neuronal shock
reticulospinal projections is important in the genesis occurs and spinal reflexes are lost, which include
of spasticity.[4] In spinal cord lesions, bilateral stretch reflexes. A flaccid weakness in seen, but
damage to the pyramidal and reticulospinal even during this, the positive features of hypertonia
pathways can produce severe spasticity and flexor can start to be seen. Limbs are not sufficiently
spasms, reflecting increased tone in flexor muscle stretched and may be immobilized in shortened
groups and weakness of extensor muscles.4 positions. Rheological changes occur within
Spasticity 439

muscles in the form of loss of proteins and that is commonly thought to result in increased
sarcomeres and accumulation of connective tissue muscle tone in response to passive stretch following
and fibroblasts (Ward 1999). Unless treated, tendon SCI. This hypertonia is velocity-dependent, with
and soft tissue contracture and limb deformity are faster stretching velocities being associated with
established. Altered sensory inputs such as pain, greater amounts of reflex activity. The development
recurrent infection and poor posture, maintain a of tonic stretch reflex hyperexcitability could be due
further stimulus to lead to yet further shortening, to a lower threshold, an increased gain of the stretch
and this cycle is difficult to break. reflex, or a combination of the two. The resultant
increase in muscle tone is thought to be due to a
Spasticity is set up later on, as plastic rearrangement
combination of increased denervation
occurs within the brain, spinal cord and
hypersensitivity and changed muscle properties.11,
muscles.5,6,7,8,9,10 This attempt at restoration of
Denervation leads to an initial downregulation of
function through new neuronal circuitry creates
neuronal membrane receptors, followed by an
movement patterns based on existing damaged
upregulation, with enhanced sensitivity to
pathways. Neuronal sprouting occurs at many
neurotransmitters.2 Gradual changes in muscle
levels with interneuronal endings moving into
properties also occur following SCI, such as fibrosis,
unconnected circuits from decreased supraspinal
atrophy of muscle fibers, decrease in the elastic
command through the vestibular, rubrospinal and
properties, decrease in the number of sarcomeres,
reticulospinal tracts (Krenz and Weaver 1998). The
accumulation of connective tissue, and alteration
end-effect is muscle over activity and exaggerated
of contractile properties toward tonic muscle
reflex responses to peripheral stimulation (Farmer
characteristics, which likely contribute to the
et al. 1991). This process occurs at anytime, but is
increased passive tension.11,
usually seen between one and six weeks after the
insult. Muscle over activity declines over time and
the following are suggested as possible causes:
Intrinsic phasic spasticity
• Structural and functional changes due to Intrinsic phasic spasticity encapsulates symptoms
plastic rearrangement such as tendon hyper-reflexia and clonus, and is
due to exaggeration of the phasic component of the
• Axonal sprouting
stretch reflex.2 Tendon hyper-reflexia is identified
• Increased receptor density as an exaggerated muscle response to an externally
applied tap of deep tendons.7 Reduced presynaptic
Intrinsic tonic spasticity Ia inhibition is thought to play an important role in
Decq2 has differentiated intrinsic tonic spasticity this hyper-reflexia, as the occurrence of reduced
(increased muscle tone) as that component of presynaptic inhibition of group Ia fibers appears
spasticity resulting from an exaggeration of the to correlate with the excitability of tendon
tonic component of the stretch reflex. Briefly, the reflexes.36
stretch reflex is a monosynaptic reflex pathway that Clonus has been defined as 'involuntary rhythmic
originates in the muscle spindles embedded parallel muscle contraction that can result in distal joint
to the muscle fibers and travels via a Ia afferent to oscillation'37 and most often occurs at the ankle.2,
the spinal cord, where it synapses either first with 7, 9 Clonus is elicited by a sudden rapid stretch of a
interneurons or directly with an alpha motor muscle.38 The prevailing theory explaining the
neuron innervating the muscle from which the underlying mechanism responsible for clonus is
stimulus originated.11 The tonic component of the that of recurrent activation of stretch reflexes.11,
stretch reflex associated with increased muscle tone 37, 38 According to this theory, dorsiflexing the
results from a maintained stretch of the central ankle causes activation of the Ia muscle spindle
region of the muscle fibers and the reflex is afferents and induces a reflex of the triceps surae,
polysynaptic.11 Upon a sustained stretch, both type resulting in plantar flexion of the ankle.11, 37, 38
Ia and type II afferents (from secondary spindle This reflex contraction is brief, essentially phasic,
endings) synapse with interneurons within the and ceases rapidly.2 The muscle then relaxes,
ventral horn of the spinal cord. Synapses of the causing the ankle to be dorsiflexed once again, due
interneurons with alpha motor neurons facilitate either gravity or the stretch being sustained by an
contraction in the muscle being stretched. examiner.2 The result is a new stretch reflex, etc.2,
It is the hyperexcitability of this tonic stretch reflex 37 Ultimately, it is the disinhibition of the stretch
440 Neuro-Rehabilitation : A multi disciplinary approach

reflex due to interruption of descending influences spasm, which can appear as a coordinated flexion
with SCI, that is thought to cause exaggeration of of all joints of the leg.35, 39
the phasic stretch reflex pathway and, hence,
spasticity can have a negative impact on quality of
clonus.3
life through restricting activities of daily living
The second theory is that clonus is the result of (ADL), inhibiting effective walking and self-care,
activity of a central oscillator or generator within causing pain and fatigue, disturbing sleep,
the spinal cord, which rhythmically activates alpha compromising safety, contributing to the
motor neurons in response to peripheral events.37, development of contractures, pressure ulcers,
38 Beres-Jones et al37 outline observations that they infections, negative self-image, complicating the
feel support such a hypothesis: (1) reports of similar role of the caretaker, and impeding rehabilitation
frequencies of clonus among ankle, knee, and wrist efforts.12-20 Spinal Cord (2005) 43, 577-586.
muscles, (2) observations that the clonus frequency doi:10.1038/[Link].3101757; published online 19 April
is not entrained by the input frequency, suggesting 2005
that clonus cannot be solely stretch-mediated, (3)
But it has been suggested it also has beneficial effect
the finding that stimuli other than stretch evoke
as symptoms of spasticity may increase stability in
clonus, and (4) the observation of a refractory
sitting and standing, facilitate the performance of
period following the clonic EMG burst where
some ADL and transfers, increase muscle bulk and
tendon tap, H-reflex stimulation, and vibration fail
strength of spastic muscles (thereby helping
to elicit an efferent response. Therefore, whereas
prevent osteopenia), and increase venous return
reduced presynaptic inhibition of group Ia fibers
(possibly diminishing the incidence of deep vein
appears to be among the contributing factors to
thrombosis). This potential for a beneficial effect of
tendon hyper-reflexia, the underlying mechanism
spasticity on QOL has a large impact upon decisions
of clonus has not been clearly elucidated.
regarding its management.11
Extrinsic spasticity Aetiology
In addition to the various intrinsic factors that
Spasticity typically occurs in patients following:
contribute to symptoms of spasticity, involuntary
muscle spasms can also occur in response to a 1. stroke,
perceived noxious stimulus originating extrinsic to 2. brain injury (trauma and other causes, e.g.
the muscle: extrinsic spasticity.2, 3, 7 Flexion anoxia, post-neurosurgery),
spasms are the most common form of extrinsic
spasticity, triggered by afferent input from skin, 3. spinal cord injury,
muscle, subcutaneous tissues, and joints 4. multiple sclerosis and
(collectively referred to as 'flexor reflex afferents').
5. cerebral palsy.
These flexor reflex afferents mediate the
polysynaptic reflexes involved in the flexion 6. Other disabling neurological diseases.
withdrawal reflex.3, 35, 39 SCI can interrupt the
inhibition of these reflexes by supraspinal Measurement of Spasticity:
pathways, making them hyperexcitable.2, 3, 40 In
Spasticity depends on several factors like presence
other words, whereas flexor withdrawal reflexes
of noxious stimuli, the patient's physical and mental
occur normally in individuals without SCI, upon
status and the position of the body. Therefore it is
disruption of normal descending influences, the
difficult to measure spasticity because of its
threshold for the flexor withdrawal reflex may
multifactorial nature. Different methods are
become lowered, the gain of the system may
available for measurement as measurement is
become raised, or both may occur together.3 A
essential to assess the response to treatment but
recent study has provided evidence to implicate
none of them is precise and reliable enough to
plateau potentials in the spinal interneuronal and
quantify the severity of spasticity clinically.
motoneuronal circuitry in the hyperexcitability of
flexion withdrawal reflexes in individuals with
chronic SCI.41 Intrasegmental polysynaptic
Ashworth Scale
connections cause the flexor reflex initiated by a This scale is based on the assessment of resistance
localized stimulus to generate a widespread flexor to stretch when a limb is passively moved. It was
Spasticity 441

originally validated for patients with multiple increased neurogenic muscle tone and mechanical
sclerosis and was validated by Ashworth (1964). limb stiffness. The major modification (Modified
Its reliability is questioned by the subjectivity Ashworth Scale) was proposed to differentiate
required by the observer to carry out the test and between mild and moderate spasticity, as
by the fact that it measures multiple aspects of limb discrepancies appeared in clinical judgement at the
stretch. However, it is in general use and has good lower end of the original scale. Bohannon validated
inter-and intra-rater reliability (Ashworth 1964). the scale in elbow flexion in post-stroke patients
The original Ashworth scale is only validated for and attempts have been made to widen the validity
measuring spasticity in the lower limb (Lee et al. (Bohannon and Smith 1987). A grade 1+ was added
1989). In addition, it does not distinguish between and the top of the scale was reduced from 5 to 4.

Score Ashworth (Ashworth 1964) Modified Ashworth (Bohannon and Smith 1987)
0 No increase in tone No increase in tone
1 Slight increase in tone giving a Slight increase in tone giving a catch, release and
catch when the limb is moved in minimal resistance at the end of range of motion
flexion /extension (ROM) when the limb is moved in flexion/extension
1+ Slight increase in tone giving a catch, release and
minimal resistance throughout the remainder (less
than half) of ROM
2 More marked increase in tone, More marked increased in tone through most of the
but the limb is easily moved ROM, but limb is easily moved
through its full ROM
3 Considerable increase in tone - Considerable increase in tone - passive movement
passive movement difficult and difficult
ROM decreased
4 Limb rigid in flexion and extension Limb rigid in flexion and extension

Tardieu Scale 2. R2 Slow Velocity: Passive joint range of motion


or muscle length.
The angle at the point of resistance is noted by
stretching a limb passively. This is performed
during as slow a movement as possible (V1), under
Quality of Muscle Reaction
gravitational pull (V2) and at a fast rate (V3). The
Course of Passive Movement
examiner will feel a catch in a muscle under the
influence of an overactive stretch reflex. Five levels 0 No resistance
have been described at the point of this catch to 1. Slight resistance
capture the quality of the muscular reaction. In 2. Clear catch at precise angle, interrupting the
essence the scale assesses dynamic and static muscle passive movement,followed by release
length as well as joint range of motion. The inter
3. Fatiguable clonus at precise angle,interrupting
and intra-rater reliability is generally good (Gracies
the passive movement,followed by release
2001).
4. Unfatiguable clonus at precise angle, (less than
Stretch Velocity 10 sec when maintaining the pressure)
1. V1: Slow as possible occurring at a precise angle, followed by
release.
2. V2:Speed of limb falling under gravity
5. Rigid limb & joint
3. V3 Fast as possible
Wartenberg Pendulum Test
Y Angle (Dynamic Range of Motion)
In this, the leg moves under gravity and the
1. R1 Fast Velocity: Movement through full range
observer measures the pendular activity of a spastic
of motion.
442 Neuro-Rehabilitation : A multi disciplinary approach

limb as it relaxes. It is best carried out on the lower ill-health and pain will have a negative effect and
limb, for it is not so reliable for other limb segments. patients and their carers may find reduced quality
of life. Complications that may result due to
Other methods for evaluating or assessing spasticity are interference with function, nursing
spasticity include: care and hygiene, pain, deformity and
1. Muscle grading, disfigurement, contractures, joint subluxation and
dislocation, peripheral neuropathy and pressure
2. Deep tendon reflexes ulcers. Although associated with complications,
3. Range of Motion measuring, spasticity is beneficial to some patients. It may help
to transfer, stand and ambulate, maintain muscle
4. Bilateral adductor tone score,
bulk, prevent deep vein thrombosis and
5. Visual analogue scale, osteoporosis.
6. Spasm frequency score.
Indications of Antispastic Treatment
7. Torque devices Non-ambulatory patients who have moderate to
8. Electrophysiological studies (including severe weakness, hyperflexia, clonus and painful
dynamic multichannel EMG, tonic vibratory flexor spasms which interferes with their ADLS
reflexes and electrical tests related to the H usually require treatment of spasticity. Patients may
reflex and F wave). fulfill more than one indication, e.g. pain relief and
care management.
Most of these methods are:
1. To improve the functional improvement in
1. Time consuming, terms of mobility by enchancing the speed and
2. Expensive, endurance of person in gait or wheelchair
propulsion thereby improving transfers. It also
3. Require specialised equipment and improves hand functions in dexterity and in
4. Mainly used in research. reachouts. It eases the person while
performing sexual acts.
Treating Spasticity:
2. It helps in relieving pain and muscle spasms
Spasticity can be disabling in itself and, if left thereby keeping the legs in anticontracture
untreated, may lead to consequences, such as: postures enabling the person to wear the
• muscle shortening, splints or orthosis.

• contractures (leading to abnormal body 3. It enhances the body image thereby improving
segment loading and sensory change), posture.

• limb deformity and altered body mechanics, 4. It decreases the burden of caretakers interms
of dressing, positioning the patients for
• altered body image, feeding and in personal hygiene.
• the need for special wheelchairs and seating 5. It helps in carryout the rehabilitative therapies
and pressure-relieving equipment, thereby delaying or preventing surgery.
• loading on pressure points,
Principles of Management
• pressure sores,
The main goal of therapy is to increase functional
• difficulty in the management of pressure sores, capacity, relieve symptoms and decrease carer
• pain from muscle spasms, burden. This should be clear to the physician, the
patient and the care giver. The consequence of
• degenerative joint disease, reduction of spasticity should be assessed. If
• loss of function, and spasticity offers stability to a joint, its reduction may
decrease the patients function. But, if there is
• mood problems and inability to participate in
minimal weakness with significant spasticity,
rehabilitation.
treatment will result in considerable improvement
Complications will prevent patients from achieving in the patient's function.
their optimal functioning and deconditioning from
Spasticity 443

Spasticity requires treatment when it is causing spasticity here would not be helpful and physical
harm and this is the sole indication. Some patients measures to utilise the developing movement
early on after their stroke or brain injury are helped patterns would be the treatment of choice, but
by their spasticity. For example, patients may start where the spasticity gives rise to problems for either
to support their weight by using their spastic lower the patient or the carer, then treatment is required.
limb when the degree of weakness in the leg would
It is sometimes quite difficult to distinguish
not allow it. Physical management (good nursing
between severe spasticity and contracture
care, physiotherapy, occupational therapy) through
formation, but it is important to do so. The clinicians
postural management, exercise, stretching and
and the patient/carer can then know what anti-
strengthening of limbs, splinting and pain relief is
spastic treatment can or cannot achieve and realistic
the basis of spasticity management (British Society
expectations can then be identified. Severe,
of Rehabilitation Medicine 1992). The aim of
inadequately treated spasticity will go on to
treatment is to reduce abnormal sensory inputs, in
develop a limb contracture through shortening the
order to decrease excessive a-motor neuron activity
muscle and tendons. A contracture may be fixed
(Ward 1999). All pharmacological interventions are
and will require serial splinting or surgery to correct
adjunctive to a programme of physical intervention.
it, but before it becomes fixed, the spasticity
Stretching plays an important part in physical
contributes to a dynamic contracture and treating
management, but needs to be applied for several
the underlying spasticity may allow easier
hours per day (Tardieu et al. 1998). Limb casting
treatment of the contracture. One way to do that is
has been developed in this field to provide a
examination under sedation. It is advisable to use
prolonged stretch. Some studies have suggested
a general anaesthetic for children. This relaxes
that task-specific training might be more effective
spastic muscles and allows the range of passive joint
(Socialstyrelsen 2006).
movement to be assessed. One particular use is in
assessing patients, who externally rotate their leg
Patient Assessment
during walking. The adductor muscles can
Spasticity is a movement disorder and patients compensate for weak hip flexors and the patient
cannot be adequately assessed unless they are rotates the leg accordingly. Blocking the obturator
observed during movement and function. nerve reduces the function of the adductors and it
Physiotherapists and occupational therapists is then possible to see the degree of hip flexor
contribute to the observation and examination weakness, so that a programme of muscle
process, but some patients with complex movement strengthening can be started rather than of BTX
patterns need assessing in a gait laboratory. The injections to weaken the adductors.
assessment process highlights the differences in
patterns of limb posture and movement following Medical Management of spasticity:
an upper motor neuron lesion. Where there is no
Spasticity is a symptom and not a disease.
movement, the assessment process is fairly
Management of spasticity begins with the
straightforward, but where there is loss of motor
assessment of the underlying disorder.
control rather than a spastic dystonia, one has to
attempt to identify the different aspects of motor Medical Management:
impairment. Patients with longstanding problems
also develop compensatory movements, which may
Treatment options:
or may not require treatment and the clinician has [Link] Drug Therapy: A number of drugs are
to be clear about the underlying pathophysiological available for the treatment of spasticity. These
processes. drugs reduce muscle tone and painful spasms,
however their use is often limited by their side
One can then identify how function is impaired and effects. The efficacy of oral antispastic drugs is small
whether the problem is generalised, focal, or more and evaluation of the effect on patient's quality of
regional. This will then point to the options for life is lacking from the available studies. 23
treatment. The indication for pharmacological
treatment therefore is when spasticity is causing 1. Baclofen: Baclofen is a GABA (?-aminobutyric
the patient harm. Some patients early on in their acid) agonist and acts by inhibiting both
rehabilitation following a stroke or brain injury use monosynaptic and polysynaptic spinal cord
their spasticity to walk on, when their weakness reflexes.24The oral dose of baclofen used to
would otherwise not allow it. Clearly, treating the treat spasticity ranges from 30-100 mg/d in
444 Neuro-Rehabilitation : A multi disciplinary approach

two to three divided doses. Oral baclofen may 2. Intrathecal Baclofen pump: The poor
cause considerable side effects such as penetrataion of blood-brain barrier and
sedation, respiration problems and muscular significant side effects of oral baclofan can be
weakness in higher doses. 25Baclofen must be minimized by intrathecal administration
tapered slowly to prevent withdrawal effects (directly into CSF) via a programmable pump.
like increase in spasticity, fever, altered mental Considerably lower doses are required in
status, seizures, malignant hyperthermia. intrathecal injection and it is without the
development of tolerance. [11,12,13] It should
2. Benzodiazepines: (Diazepam and
be considered in patients unresponsive to oral
clonazepam) Benzodiazepines act by
pharmacotherapy and a severity of 3 on the
increasing the affinity of GABA receptors for
Ashworth scale for at least 12 months. A test
endogenous GABA.26Diazepam can be
dose should first be given intrathecally before
started at 5 mg at bedtime, and if daytime
the pump is implanted. The dose range is 12-
therapy is indicated, the dosage can be
2000 mcg/d and should be fine tuned
increased slowly to 60 mg/d in divided doses.
according to the severity of symptoms and
Clonazepam can be started at 0.5 mg at night
response to therapy. The complications of
and slowly increased to a maximum of 20 mg/
intrathecal baclofen pump implantation are
d in 3 divided doses. The side effect includes
relatively few and usually are limited to
sedation, confusion, habituation and
mechanical failures of the pump or the
tachyphylaxis. 22
catheter. Adverse drug effects are usually
3. Dantrolene: Dantrolene is more useful for temporary and can be managed by reducing
spasticity of cerebral origin. Dantrolene the rate of infusion.
interferes directly with the excitation-coupling
reaction. It acts at the level of the muscle fiber,
affecting the release of calcium from the
sarcoplasmic reticulum of skeletal muscle and
thus reducing muscle contraction.]27
Dantrolene is given in a dose of 0.5-3.0 mg/
kg/d. Dantrolene may cause side effects like
muscle weakness and hepatotoxicity. 22
4. Tizanidine: Tizanidine decreases the
excitability of α and γ motor neurons in the
spinal cord by reducing the release of
excitatory neurotransmitters in the spinal cord
and decreasing the action of these excitatory
neurotransmitters at their receptors. 28
Tizanidine also acts by inhibiting the release
of substance P from small sensory afferent
nerve fibers as well as slowing the firing of
the locus ceruleus. 29 Tizanidine should be
started at a low dose, 2-4 mg, preferably at
bedtime. The average maintenance dosage of
tizanidine is 18-24 mg/d. The maximum
recommended dosage is 36 mg/d. The side
effects of tizanidine are sedation, dizziness, dry
mouth, and hypotension. Tizanidine does not
cause any muscle weakness. 30 Therefore, it
might be preferable over other antispasticity
medications that do cause weakness, such as
baclofen and dantrolene, especially in patients Figure 4.1.1 and 4.1.2
whose strength is already compromised by 3. Neurolysis with Neurotoxins: Botulinum
neurological disease. toxin: Botulinum toxin is a neurotoxin
produced by the clostridium botulinum
Spasticity 445

bacterium. Botulinum toxin acts by causing of muscles, peripheral nerves and spinal cord
reversible block of neuromuscular has been used in the management of spasticity.
transmission by inhibiting acetylcholine Surface electrical stimulation of spastic
release. 34There are seven different serotypes muscles cause reduction of spasticity by
of botulinum toxins (A,B,C,D,E,F,G). stimulating cutaneous afferents and
Botulinum toxin is injected intramuscularly suppressing motoneuronal excitability. 40
into the spastic muscles using a very fine Electrical stimulation of peripheral nerves
needle. Injections should be targeted to spastic reduces spasticity by inducing complete and
muscles responsible to functional loss using reversible conduction block.41 Electrical
EMG guidance. 35 The effect of botulinum stimulation of the dorsal columns of the spinal
toxin starts within two weeks after injection. cord through epidurally placed electrodes may
The clinical effect appears 4-7 days after reduce spasticity. 42 Direct stimulation of
injection, reaches a maximum after about 2 spinal cord suppressess excitability of spinal
months thereafter the effect tapers off. The motoneurons and cause reduction of spasticty.
effect of botulinum toxin is not permanent and
6. Transcranial magnetic stimulation: Repetitive
lasts for 3-4 months. After 3-4 months the effect
high-frequency (5 Hz) and low-frequency (1
gradually fades away and repeat treatment
Hz) transcranial magnetic stimulation may
may be required depending on the
improve spasticity.
symptoms/ dysfunction caused by spasticity.
[16] Botulinum toxin dosing has to be
Surgical Management:
individualized and is dependent upon muscles
involved, prior response, and functional goals. Surgery can play a very important role in the
Botulinum toxin is used in patients with treatment of chronic spasticity or to allow more
localized or multifocal spasticity. 37,38 normal bone and muscle growth. Surgical
American Academy of Neurology treatment of spasticity involves neurosurgery and
recommends botulinum toxin to reduce orthopaedic surgery. Neurosurgical treatment for
muscle tone and improve passive/active spasticity is reserved for severe cases in which
function. [19] Botulinum toxin therapy is medical management has been ineffective or has
approved for the treatment of cervical lost its effectiveness. Neurosurgical procedures for
dystonia, strabismus, and blepharospasm in spasticity include Selective Posterior (Dorsal)
patients older than 12 years. Rhizotomy (SDR), microsurgical DREZotomy,
Peripheral Neurotomy, Longitudinal Myelotomy
Antibodies may form against botulinum toxins
and Neurectomy.
and are a common cause of absence of any
beneficial effect. Botulinum toxin injections are Selective Posterior (Dorsal) Rhizotomy (SDR)
usually needed at 3- to 6-month intervals to involves cutting of the dorsal nerve roots that lie
maintain therapeutic benefit. Repeated, high- just outside the vertebral column and transmit
dose injections are likely to result in antibody nerve impulses to and from the spinal cord. SDR is
formation. To decrease the possibility of primarily indicated in conditions exhibiting severe
antibody formation, repeat injections should spasticity that interferes with mobility or
not be given in less than 3 months. The smallest positioning. 43 Microsurgical DREZotomy (Dorsal
amount of botulinum toxin necessary to Root Entry Zone-otomy) is a type of selective
achieve therapeutic benefit should be used, rhizotomy. It involves cutting the nerve fibers at
and the interval between treatments should be the entry zone and suppresses afferent discharges
extended as long as possible. to the spinal cord. It is more effective in the
treatment of severe spasticity limited to the upper
4. Chemical neurolysis (Phenol or alcohol
or lower limbs. [24] Peripheral neurotomy involves
Injections): A peripheral nerve innervating
cutting of peripheral nerves at the point at which
the spastic muscles is injected with phenol or
they enter the muscle. It is indicated in the treatment
alcohol solutions, which destroys myelin. It is
of spastic neck, elbow, hand, hip, and foot.
used in patients with focal spasticity. The
45Longitudinal Myelotomy involves longitudinal
effectiveness of the injection diminishes over
division of the spinal cord to sever crossing sensory
time and repeated injections are needed.
fibers and produce localized analgesia. It used to
5. Electrical Stimulation: Electrical Stimulation be performed earlier for severe and painful
446 Neuro-Rehabilitation : A multi disciplinary approach

spasticity. 46 Neurectomy involves the cutting of 1) Body Positioning:


the nerve branches as they enter the targeted
It is advisable to inhibit the unwanted activity of
muscle. It is indicated in patients with focal
the released reflex mechanisms by positioning the
spasticity refractory to botulinum toxin. 44
head and neck of the patient as they have tendency
Orthopaedic surgerical procedures for the to elicit strong postural reflex mechanisms. Proper
management of spasticity include muscle / tendon head and neck positions can inhibit these reflexes
surgery and bone surgery. Muscle / tendon surgery thereby
involves lengthening or release of muscles and
preventing the rest of the body from going into the
tendons. In contracture release surgery,
reflex pattern. Sidelying with pillow support is most
contractured muscle is cut and the joint is then
preferred position as it avoids stimulation of the
positioned at a more normal angle allowing
tonic labyrinthine reflex and the asymmetrical tonic
regrowth of the tendon to this new length. Tendon
neck reflexes. Rhythmical trunk rotations can be
transfer involves moving the attachment point of a
easily given in both passive and active assisted
spastic muscle to allow improved active function
exercises which helps in reduction of spasticity.
or retention of only passive function. Bone surgery
can be osteotomy or arthrodesis. Osteotomy • Equipment that Improves Positioning
involves removal of a small wedge of bone to allow Controlling the position of a child's pelvis, knees,
it to be repositioned or reshaped. Arthrodesis is and ankles can help decrease arching, increased
fusion of bones that normally move independently head control and shoulder stability, and improve
and thereby limiting the spastic muscle to pull the functional hand use. Children may need different
joint into an abnormal position. types of equipment at different times during their
growth and development. Again, the goal is to
Physical Therapy Management: increase control of movement and manipulation of
Spasticity sets in gradually after the initial insult to objects in the environment. Some examples of
the central nervous system. It becomes noticeable equipment that can help improve positioning are:
in the first few months, but the timing varies 1. Adapted strollers
depending on the extent of underlying neurologic
insult. Once recovery from the neurologic deficit 2. Wheelchairs
stabilizes, the spasticity also tends to stabilize. 3. Bath chairs
Spasticity is not always detrimental as a weak
4. Adapted car seats
flaccid limb can interfere with such daily activities
as transferring, dressing, grooming and perineal 5. Adapted seating for general use
care, spasticity provides posture and tone to a limb 6. Standers
that can assist with weight bearing even if the
patient cannot walk. However, excessive tone may 7. Gait Trainers
interfere with these activities. Thus, it is only when 2) Rotatory Movements:
spasticity interferes with function or puts the
Trunk rotation produces lower limb to extend,
individual at risk of hurting himself or herself, it
abduct and externally rotate which is the
needs to be treated.
position opposite to reflex pattern. Limb
According to Lockley 2006, physical interventions rotations are also very effective in normalizing
helps in minimizing the viscoelastic properties of the muscle tone of the patient.
the connective tissue; altering the neural patterns
3) Pressure over undersurface of Foot:
of spasticity or spasms and maintaining levels of
function for the individual. When the pressure is applied to the ball of the
foot it stimulates an extensor reflex which is a
The goal of physiotherapy in the management of pathological pattern of extension, adduction,
spasticity should include : and medial rotation of hip with plantar flexion
1. Reduction of excessive tone. of the foot, which is undesirable event in case
of spasticity. When the pressure is applied
2. Facilitate normal movement patterns. under the heel of the foot, purposeful
3. To give a sense of normal position and normal contraction of muscle occurs giving a normal
movement. supporting pattern. One should facilitate
Spasticity 447

normal Movements Patterns thereby avoiding 3. Ice packs


triggering factors. Due to reflex release, some
4. Ice massage
motoneurone pools are already in an
excitatory state and any volitional effort will 5. Ice application with exercises
act as a trigger to those motoneurone pools 6) Neutral Warmth:
resulting in muscle contraction in the spastic
pattern. In such patients strong volitional Retention of body heat stimulates
effort should be discouraged as this facilitates thermoreceptors, autonomic nervous system
the movement in the spastic pattern. mainly parasympathetics, which produces
generalized inhibition of tone, calming effect,
Other factors such as quick movements, relaxation and decreases pain. It should be
abruptly performed, noisy surroundings, applied for about 10 to 20 minutes.
anxiety, excitement, over exertion should also Overheating should be avoided as it might
be avoided as it may increase spasticity. increase arousal or tone.
4) Slow Sustained Stretching: Techniques used are as:
Stretching forms the basis of spasticity 1. Wrapping body or body parts: ace wraps,
treatment. Stretching helps to maintain the full towel wraps
range of motion of a joint, and helps prevent
contracture, or permanent muscle shortening 2. Application of snug fitting clothing
as it activates muscle spindles (Ia & II endings), (gloves, socks, tights) or air splints
golgi tendon organs (Ib endings) which are 7) Relaxed Passive Movements:
sensitive to length changes, inhibits or
Rhythmical, passive movements performed
dampens muscle contraction and tone due
slowly throughout the range of motion in
largely to peripheral reflex effects. It is effective
normal pattern may help in reducing
in extensor muscles than flexors due to the
spasticity.
added effects of II inhibition. Stretching when
done forcefully against severe spasticity, it 8) Deep Rhythmical Massage (Tendon Rolling):
increases hyperexcitable stretch reflex more
Deep rhythmical massage with pressure over
strongly damaging periosteum of bone when
the muscle insertions can be given to reduce
excessive tension is applied to the tendons of
spasticity.
the stretched muscles.
9) Inhibitory Pressure (Weight-Bearing):
Techniques used to maintain slow sustanied
stretching are as: Prolonged pressure to long tendons inhibits
the hypertonicity of a muscle as it activates
1. Manual contacts
muscle receptors (muscle spindles, golgi
2. Inhibitory casting or splinting tendon organ) and tactile receptors. It can be
applied manually or by body weight. Weight
3. Reflex-inhibiting patterns
bearing postures provides inhibitory pressure,
4. Mechanical low-load weights such as quadruped or kneeling postures can
5) Prolonged Cold Application: be used to promote inhibition of quadriceps
and long finger flexors and sitting, with hands
Application of cold packs to spastic muscles open, elbow extended, and upper extremity
(usually for 10 minutes or longer) may supporting body weight can be used to
improve muscle tone. While the effect is not promote inhibition of long finger flexors.
long lasting, it helps to improve function for a
short period of time, or to ease pain by 10) Biofeedback:
activating thermoreceptors thereby decreasing Biofeedback is the use of an electrical monitor
neural, muscle spindle firing leading to that creates a signal usually a sound as a
inhibition of muscle tone. spastic muscle relaxes. Thus, the person with
Techniques used spasticity may be able to train himself to
reduce muscle tone consciously.
1. Immersion in cold water; ice chips
11) Functional Electrical Stimulation:
2. Ice towel wraps
448 Neuro-Rehabilitation : A multi disciplinary approach

Electrical stimulation may be used to stimulate 14) Inhibitive Casts to Improve Function
a weak muscle to oppose the activity of a
Inhibitive casts are used to increase function
stronger, spastic one thereby improving motor
more than to improve range of motion.
activity in agonistic muscles and reducing the
Sometimes, inhibitive casts will be used to
tone in antagonistic muscles. Once stimulation
"give more information" (proprioception) to
has been stopped, the effect last for less than 1
the ankle joint or the foot in order to prevent a
hour probably because of neurotransmitter
child from using "reflex patterns" of
modulation within reflex arc. It improves
movement. They are often utilized when a
standing, walking, and exercise training as
child with muscle imbalance is learning to
well as decreases upper extremity
walk. They can give better proprioceptive
contractures.
input to the foot when compared to the AFO
12) Tone Reducing Orthosis: since they are heavier and can have special
Orthotics are designed to help provide support features built in to them. For example,
to weak muscles and minimize the risk of joint inhibitive foot plates can be built into the base
deformity. There are a variety of orthotics of each cast. The inhibitive foot plates apply
made from a number of different materials. pressure to different areas of the foot to give
The goal is to use an orthotic which can give better proprioceptive input to the joint which
support depending on a child's pattern of inhibits reflex patterns of movement. Often
movement, avoid skin breakdown, and be times, we will use inhibitive casts prior to
comfortable. If areas of the skin become red, prescribing orthotics, depending on a child's
this indicates that the orthotic may not be function.
fitting appropriately, especially if the redness 15) Slow Maintained Vestibular Stimulation:
lasts more than half an hour after removing
Low-intensity vestibular stimulation such as
the orthotic.
slow rocking produces generalized inhibition
1. Ankle foot orthoses (AFO's): These are of tone as it facilitates primarily otolith organs
plastic AFO's in which foot plate and (tonic receptors); less effects on semicircular
broad upright are designed to modify canals (phasic receptors). Slow rolling
reflex hypertonicity by applying constant movements, assisted rocking in a weight-
pressure to the plantarflexors and bearing position or rocking with equipments
invertors. Foot plate may be modified like rocking chair, Swiss ball, equilibrium
which maintains the toes in an extended board, Hammock can help in reducing the
or hyperextended position, thus assisting tone.
individual to walk with better foot and
16) Proprioceptive Neuromuscular Facilitation
knee control thereby preventing the foot
Techniques :
to go into equinovarus position.
Proprioceptive neuromuscular facilitation is a
2. Leg braces or casts
neuromuscular treatment that uses repetitive
3. Hand splints stretches where one muscle is contracted while
4. Soft body jackets another muscle is relaxed at the same time.
There are several techniques performed in
13) Serial Casts to Gain Range of Motion
proprioceptive neuromuscular facilitation
Serial casts can either be used for the arms or which increases range of motion, improves
the legs. The goal is to maintain or increase range of stretch, increase strength and develop
range of motion of a muscle, tendon, or joint. healthy muscle tissue.
The casts provide a sustained stretch across
Techniques used in reducing spasticity are as:
the joint. Prolonged stretch can help muscles
relax. The cast may also help "soften" tendons. 1. Rhythmic Initiation - Voluntary
Serial casts are normally changed one time per relaxation followed by passive
week. The number of total weeks of casting movements through increments in range,
varies depending on the need of each child. followed by active movements
The goal is to slowly gain more joint range progressing to resisted movements using
without causing significant discomfort to the tracking resistance to isotonic
child. contractions.
Spasticity 449

2. Rhythmic Rotation - Voluntary relaxation OCCUPATIONAL THERAPY


combined with slow, passive, rhythmic
INTERVENTIONS IN SPASTICITY
rotation of the body or body part around
a longitudinal axis, followed by passive Intervention for hypertonicity and spasticity is
movement into the antagonist range. necessary when it interferes with ADL's, gait, sleep
or wheelchair positioning or when it causes severe
3. Contract Relax Active Contraction -
pain and limits hygiene (for [Link] client is unable
Isotonic movement in rotation is
to wash hand or axilla) or when spasticity leads to
performed followed by isometric hold of
contractures or pressure sores50.
the range limiting muscles in the
antagonist pattern against slowly Spasticity, if not managed can lead to secondary
increasing resistance followed by problems such as 51:
voluntary relaxation and active • Deformity of the limbs, specially the distal
movement into the new range of the upper limb (elbow to digits)
agonist pattern.
• Maceration of the palm tissue. Possible
17) Manipulating Key Points: masking of underlying motor control.
Spasticity can be reduced by manipulating the • pain syndromes resulting from loss of normal
key points in the upper limb and lower limb kinematics
like the movements with thumb in abduction
helps in reducing the spasticity in upper limb • impaired ability to manage BADL tasks (
and in lower limb in sitting position, placing especially upper extremity dressing and
one hand over the lower back and other near bathing of the affected hand and axillawhen
the xiphoid process and moving the patient in flexor positioning is present in a patient with
the figure of 8 pattern forwards and stroke).
backwards thus manipulating the pelvis helps • loss of reciprocal arm swing during gait
in reducing spasticity. activities.
18) Examples of different therapy models include: However before treating hypertonicity, the
1. Neurodevelopmental therapy (NDT), therapist should closely evaluate the function of the
tone. Hypertonicity can have beneficial effects such
2. Conductive education. as aiding him in standing and transfers,
3. Manual therapy (myofascial release and maintaining muscle bulk and prevent deep vein
craniosacral therapy). thrombosis, osteoporosis and oedema. 50
4. Hippotherapy, or horseback riding, can A uniformly acceptable, reliable and practical
help a child increase balance and improve measure of spasticity continues to elude the
trunk control. therapists 52. Objective assessment of muscle tone
in clients with hypertonia and spasticity is difficult.
5. Aquatic therapy can help promote more
The postural reflex mechanism, the position of body
typical patterns of movement that are
and head in space, the position of head in relation
often affected by gravity.
to body and stereotypical reflexes and associated
19) Alternative Treatment reactions all influence the degree and distribution
Alternative and complementary therapies of abnormal muscle tone. 53
include approaches that are considered to be
Manual rating scale for hypertonicity and
outside the mainstream of traditional health
spasticity:
care. Techniques that reduce stress, such as
yoga, Tai Chi, meditation, deep breathing The Ashworth Scale 54 and the Modified Ashworth
exercises, guided imagery, and relaxation Scale 55 are 2 most widely used scales to manually
training, may be helpful to induce relaxation rate spasticity 56, 57. There is a controversy in
and manage spasticity. Acupuncture and literature about the validity and reliability of these
biofeedback training also may help induce scales.
relaxation. Brashear et al concluded that in upper limb
spasticity, the Ashworth Scale had good intra and
450 Neuro-Rehabilitation : A multi disciplinary approach

inter - rater reliability when used by trained medical myotonometer could provide objective data about
professionals 58. the tone reducing efficacy of various tone reducing
procedures 63.
Tardieu Scale: Modified Tardieu Scale 59 and the
Tardieu Scale58 both measure spasticity. Modified In the past, sensorimotor approaches were used to
Tardieu Scale has an interrater reliability coefficient treat patient's with abnormal skeletal muscle
of 7 and was shown to be more reliable than activity. These approaches were developed by
Modified Ashworth Scale 61. Rood, Bobath, Knorr and Voss (PNF) and
Brunnstorm and are based on an understanding of
Mild-Moderate-Severe Spasticity Scale and
CNS dysfunction. Although these interventions are
Preston's Hypertonicity Scale: Some therapists find
commonly used, their effectiveness is being
it easier to use these scales.
challenged as occupational therapist move towards
models of evidence-based practice64.
MILD-MODERATE-SEVERE
SPASTICITY SCALE: For years, occupational therapists have been using
weight bearing activities to reduce hyper-tonicity
Mild: The stretch reflex (palpable catch) occurs at and to remediate paresis in patients with upper
the muscles end range (i.e. the muscle is in a motor neuron lesions. At present there is a limited
lengthened position). amount of research to support these
Moderate: The stretch reflex (palpable catch) occurs neurofacilitation approaches. Brouwer and
in mid range. Ambury concluded that cortico-spinal facilitation
of motor units occurred during weight bearing.
Severe: The stretch reflex (palpable catch) occurs They believed that afferent input from weight
when the muscle is in a shortened range. bearing increased motor cortical excitability65.

PRESTON'S HYPERTONICITY Chakerain and Larson studied the effects of upper


extremity weight bearing on hand opening and
SCALE:
prehension in children with spastic cerebral palsy.
0: No abnormal tone detected during slow Computer calculation of the patients hand surface
passive movement. area were measured and it was found that there
was an increase in surface area after weight bearing
1. Mild: First tone or resistance is felt when the
and an increase in the maturity of movement
muscle is in a lengthened position during slow
components needed for prehension.66
passive movement.
McIllroy and Maki demonstrated that if the affected
2. Moderate: First tone or resistance is felt in the
arm is used when weight bearing, postural
mid range of the muscle during slow passive
responses occur throughout the weight bearing
movement.
extremity and occur during other perturbations of
3. Severe: First tone or resistance occurs when Posture.67Although, not many studies have
the muscle is in the shortened range during documented how and why weight bearing works
slow and passive movement. physiologically, it certainly is a requirement for
improving performance 50
MECHANICAL AND COMPUTER
An important occupational therapy objective is to
RATING SYSTEMS FOR have client manage muscle tone to engage in and
HYPERTONIA AND SPASTICITY complete basic and instrumental activities of daily
living. Positioning and movement in patterns
Mechanically determined parameters are more
opposite to hypertonic or synergistic patterns are
reliable than the manual methods. McCrea et al,
important to expand the motor repertoire and
concluded that using a linear spring damper model
develop movement that is as close to normal as
to assess the hypertonic elbow was reliable and
possible.
valid 62. However it is time consuming and certain
muscle groups are difficult to assess with it. The client should also be taught how to incorporate
the affected extremity as much as possible into all
Leonard et al investigated the construct validity of
ADLs. ADLs, crafts, games, and work activities can
myotonometer, a newly developed computerized,
be used to teach incorporation of the extremities
electronic device. The authors concluded that the
for a total approach to treatment 68
Spasticity 451

Casting in inhibitive postures has been shown to and movement: Robert Wartenberg Lecture.
be effective in tone reduction 69,70. The beneficial Neurology 1980; 30: 1303?1313.
effect of casting on hypertonia is well documented
2. Lundy-Ekman L. Neuroscience Fundamentals
in literature71,72,73. Serial casting is effective in
for Rehabilitation W.B. Saunders Company:
presence of a contracture and its use should cease
Toronto 2002;.
when desired position is achieved and tone is
manageable using a splint. 3. Lalith E. Satkunam: Rehabilitation medicine
:[Link] of Adult Spasticity.
Multiple commercially available spasticity reducing
splints are used to keep hand and wrists in 4. Zeba F Vanek: Introduction and
inhibitive postures. The client and family need to Pathophysiology of Spasticity.
be educated in continuing to incorporate the 5. Ashworth B. 1964. Preliminary trial of
extremities in occupations and to bear weight on carisprodal in multiple sclerosis. Practitioner
extremities as much as possible; to retain the ROM 192:540-542.
gain achieved during casting 74
6. Bes A, Eyssette M, Pierrot-Deseilligny E, et al.
Physical agent modality such as cold, superficial 1988. A multi-centre, double-blind trial of
heat, ultrasound and Neuromuscular Electric tizanidine, a new antispastic agent, in
Stimulation can be used as a preparation for or in spasticity associated with hemiplegia. Current
conjunction with purposeful activity and muscle Medical Research and Opinion 10: 709-718.
reeducation, provided the therapist has the
appropriate training62. 7. Bodine-Fowler SC, Allsing S, Botte MJ. 1996.
Time course of muscle atrophy and recovery
The Functional Tone management (FTM) Arm following a phenol-induced nerve block.
Training Program is based on distal activation Muscle and Nerve 19(4):497-504.
model focusing on the key points of early initiation
of upper extremeity movements that incorporate 8. Bohannon RW, Smith MB. 1987. Inter-rater
grasp and release. In order to incorporate the hand reliability of a modified Ashworth scale of
into FTM arm training, a dynamic orthosis for the muscle spasticity. Physical Therapy 67: 206-
hand called the SaeboFlex is used. This orthosis 227.
assists an individual who exhibits hypertonia in the 9. Krenz NR, Weaver LC. 1998. Sprouting of
hand to place the hand in an open functional primary afferent fibres after spinal cord
position and also to produce a graded muscle transection in the rat. Neuroscience 85: 443-
contraction of the finger flexors in order to grasp 458.
an object. The FTM program combines high
10. Mayer NH. 2002. Clinicophysiologic concepts
repetition grasp and release with task specific arm
of spasticity and motor dysfunction in adults
training drills to progress the client towards a
with an upper motor neuron lesion. In: Mayer
functional goal. A significant body of research
NH, Simpson DM, editors. Spasticity:
support the FTM arm training program 26, 27, 28.
Etiology, evaluation, management and the role
Clinically observed improvements with FTM arm
of botulinum toxin. New York. We Move. p 1-
training Program include increased AROM at the
10.
shoulder, elbow and wrist, improved upper
extremity Fugal- Meyers Scores and decreased 11. M M Adams and A L Hicks: Spasticity after
modified Ashworth Scores78. spinal cord injury
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Ch.13 Orthosis
Dr. V. C. Jacob, [Link], D.P.T, Dr. Hema Biju, MOTH(Neuro).

LOWER LIMB ORTHOSIS 6. Thermoplastics (polypropylene) requires


high temperature (150 degrees Celsius) for
A lower limb orthosis is an external device applied molding and it is used mainly for high stress
or attached to a lower extremity to improve activities.
function by controlling motion, providing support,
reducing pain through transferring of the load to 7. Rubber is used for padding
another area, correcting flexible deformities, 8. Metals are used for joint components and
preventing the deformities getting worsened. uprights.
Orthosis is the medical terminology for brace or
splint. External Shoe Modifications:
1. Cushion heel: to minimize impact on heel,
• AFO: Ankle Foot Orthosis.
encourage plantarflexion and minimize knee
• KAFO: Knee Ankle Foot Orthosis. flexion.
• HKAFO: Hip Knee Ankle Foot Orthosis. 2. Heel wedge: a) Medial wedge to promote
inversion (for flat feet).
• THKAFO: Trunk Hip Knee Foot Orthosis.
b) Lateral wedge to promote eversion (for
Orthosis are of two types : varus foot)
1. Static: Support the weakened joint. 3. Crooked and elongated heels: Medially (for
2. Dynamic: Allows joints movements flat feet) Reverse crooked and elongated heels
during locomotion. (C/E Heels) i.e. elongated lateral part of heel
(for varus foot)
Materials used are of:
4. Heel elevation: to compensate fixed equinus
1. Metal deformity or length discrepancy.
2. Plastic
5. Compensation platform: for length
3. Leather discrepancy
4. Synthetic fabrics.
Sole Modifications
Plastic materials such as thermosetting materials/
thermoplastic are commonly used. 1. Metatarsal bar at posterior metatarsal head to
shift the weight bearing on metatarsal heads,
They can be easily moulded after heating and commonly used in Diabetes Mellitus and
hardened after cooling. anesthetic foot.
Low temperature thermoplastics can be fabricated 2. Sole wedge : Medial wedge to promote
easily. supination of the foot

Principles: Lateral wedge to promote pronation.


In all orthosis :
Internal Shoe Modifications:
1. 3 point pressure is essential. 1. Cushion Heel: Soft pad with scooped out
2. Orthotic joints should be at the anatomical central part under the painful points.
joint level 2. Heel wedge: When given medially, can
3. Should be light weight, strong, durable and promote inversion of hind foot.
cosmetically acceptable. When given laterally, can promote eversion
4. Static or dynamic stabilization. of hind foot.
5. Flexible material. Tissue tolerance to 3. Metatarsal pad: shifts stress from metatarsal
compressive and shear force. head to meta tarsal shaft.
456 Neuro-Rehabilitation : A multi disciplinary approach

4. Innersole excavation: at one or more T-strape (for valgus foot) or lateral T-strape
Metatarsal heads. (for varus foot)
5. Valgus pad or medial arch support. (for flat
feet)

Foot Orthosis:
1. Plastic insert: This is a rigid plastic insole for
manual correction. It has rigid medial, lateral
or posterior walls.
2. Heel Cup: Plastic cup under the heel with rigid
wall posterior and medial and lateral aspect
of the heel (for pronated heel)

Custom made orthosis are always better than High boots with Plastic AFO
ready made devices. steel shank

Ankle Foot Orthosis (AFO):


• Prescribed for weakness of 1) ankle
dorsiflexors 2) plantarflexors 3) inverters
4) everters.
• They are used for maintaining the feet in the
neutral position while walking.
• They are also used to prevent deformities.
• In a case with an UMN weakness of the foot
with spasticity in the inverters and
plantarflexiors, which would give an equino-
varus tendency of the foot, it is prescribed to
maintain the foot in corrected position and also Plastic AFO
prevent foot drop. This would help in the gait
training KAFO (Knee Ankle Foot Orthosis)
• The position of foot affects the stability of knee KAFO is prescribed whenever there is an inability
indirectly. to lock the knees due to weak muscles around the
1) Equinus foot with spasticity could lead knees (Quadriceps and Hamstrings). There is a
to hyper extension of knee. tendency for buckling of the knee.

2) Calcaneal foot may lead to knee flexion. Types of KAFO


3) varus foot may cause Genu varum 1. With Knee Joint
4) valgus foot could lead to Genu valgum 2. Without Knee Joint
• An AFO can reduce energy consumption in 3. Ischial Weight Bearing
Diplegics, Hemiplegics and in LMN foot drop. 4. With Free Knee Joint
• Knee Joint is provided for the patient to
TYPES OF AFO wear a brace and fold the knee.
1. Fixed Ankle: a) Plastic insert • Ischial Weight Bearing KAFO is
b) High boots with steel shank. prescribed when the weight bearing has
to be on the ischium (in cases of fractures
2. Hinged AFO: Adjustable ankle hinges with
of the weight bearing bones like femur
desired ankle plantarflexion or dorsiflexion.
or tibia)
3. Conventional below knee braces with ankle or • KAFO with free knee joint is prescribed
without ankle joints, along with medial whenever there is strong quadriceps but
Orthosis 457

the knee joint has tendency for 4. Spinal Dysraphysm e.g. Meningomyocoele,
hyperextension. Spina Bifida
Many a times, trial splints like Push Knee Splints 5. Post surgical conditions like Laminectomy
are prescribed to see whether the patient can
manage locking the knee and walking. (Provided Types of spinal orthosis
the foot has got proper control) 1. Lumbosacral belt or corset
2. Thoracolumbar Corset
3. Close Contact Spinal Orthosis
4. Milwaukee Brace
5. Taylor's Brace
6. Minerva Jacket
7. Anterior Hyperextension Brace
By far, a close contact spinal jackets can give the
best support for scoliosis and other deformed spine.

Knee Ankle Foot Push Knee Splints


Orthosis

Hip Knee Ankle Foot Orthosis (HKAFO)


HKAFO is prescribed whenever there is a lack of
control of hip and knee. A pelvic bandis provided
in order to give Hip stability. Hip is controlled
mainly by hip extensors and abductors,in the
absence of which, the hip becomes wobbly and with
bilateral involvement walking is very unstable. Close Contact Spinal Lumbosacral belt or
Thus patient may require use of a pair of crutches Orthosis corset
or a walker also.

Anterior Hyperextension Brace

Hip Knee Ankle Foot Orthosis

SPINAL ORTHOSIS
Spinal Orthosis are prescribed for
1. Unstable Spine due to weak musculature
2. Unhealed Fractures of the spine
3. Correction of deformities like Scoliosis,
Kyphosis, etc. Taylor's Brace
458 Neuro-Rehabilitation : A multi disciplinary approach

CONCLUSION: • To reduce weight bearing forces for a


Various types of orthotic devices are useful for particular purpose
preventing deformities getting worse, maintaining • To otherwise correct the shape and/or
corrected position of the lower extremities and also function of the body, to provide easier
improving the gait pattern as much as possible. movement capability or reduce pain
They compensate for the weak muscles for stability.
Prescribing the correct orthotic devices is one of the Team Approach
most essential part of Rehabilitation, especially in Creating and employing upper limb orthoses often
Neurological conditions involves a team approach, especially in
rehabilitation settings. Custom-made upper
UPPER LIMB ORTHOSIS extremity orthoses may be fabricated by physical,
occupational, and hand therapists. The therapist
Definition may help the patient learn to use the orthosis but
An orthosis is a device that is applied to the body can also recommend ready-made devices or refer
in order to protect and stabilize body parts, to the patient to an orthotist.
prevent or correct scarring and deformities, or to
Health care professionals who create and fit upper
aid in performance of certain functions. Upper limb
limb orthoses must have a good understanding of
orthoses are applied to the shoulder, elbow, arm,
the anatomy and physiology of the upper limbs.
wrist, or hand. These devices may be called
They must also understand the mechanics and
orthoses, orthotic devices, or splints.
forces involved in making various body
Upper extremity orthoses are used frequently on movements, and they must be familiar with the
patients who have materials and tools involved in constructing
• musculoskeletal problems like those resulting orthoses. Certified orthotists are specialists who
from trauma, sports, and work-related injuries. focus exclusively on fitting and building orthoses.
• neurologic problems, such as stroke, traumatic The professional relationship between the
brain injury(TBI), multiple sclerosis (MS), physical/ occupational/ hand therapist and
cerebral palsy (CP), spinal cord injury (SCI), orthotist is very important in identifying patient
and peripheral nerve injury. functional goals and a variety of predictable
outcome.
• arthritic conditions
Basic Principles in Splinting:
Purpose of Splinting
I. Mechanical Principles: Use of mechanical
Upper limb orthoses can be used for a wide variety
principles helps make splints comfortable,
of purposes. Some of the more common uses
durable, effective and diminishes of additional
include:
injury secondary to splint application.
• stabilizing fractures or unstable joints
Mechanically, splints may be grouped into two
• immobilizing joints to promote healing
categories:
• preventing or correcting joint contractures
1. Those that apply three point pressure: Three
• Protect weak muscles from overstretch point pressure splints function through a
series of reciprocal forces with the middle force
• correcting subluxation of joints or improper
directed opposite to the two end forces.
alignment of tendons
2. Those that pull adjacent articulated bony
• Helping to provide enhanced function
segments together through circumferential
• Serving as an attachment for assistive devices pressure. In the circumferential pressure
• Blocking unwanted movement of a joint splint, the middle reciprocal force is absent.

• maintaining correct joint alignment Pressure and shear can be reduced by increasing
the surface area of the splint. Short narrow splints
• assisting movement of joints or components are often problematic since they
• reducing muscle tone in spastic muscles apply pressure to a small area creating pressure
necrosis of underlying soft tissues. Longer splint
Orthosis 459

designs increase mechanical advantage and make • Avoid dependence on others for
splints less susceptible to causing pressure assistance
problems. (Willard)
8. Design for several functions into one splint to
Other Mechanical principles that need to be taken avoid confusion and simplify the wearing and
into consideration are exercise routines.

1. Use Optimal Rotational Force III. Specific Principles of Design


• Rotational element- To produce joint 1. Identify the key joints to be splinted by careful
rotation evaluation.
• Translational element- To produce joint 2. Review purpose to immobilize, to increase
distraction or compression passive motion or to substitute for active
motion by re-evaluating for functional
2. Consider the Torque Effect objective of a given splint.
• Increase torque by increasing the distance 3. Decide whether to employ Static or Dynamic
between the joint axis and the point of forces.
attachment of the dynamic assist
4. The surface of the extremity on which the
3. Consider the Relative Degree of Passive splint will be based is determined and
Mobility of Successive Joints secondary joints that need to be controlled or
4. Consider the Effects of the Reciprocal Parallel positioned are noted.
Forces 5. Identify area of diminished sensibility to avoid
• Use first -class lever system pressure necrosis.
• If Mechanical Advantage is increased, the 6. Consider kinetic effects: Since application of
middle opposing force is decreased the splint often alters internal and external
forces to proximal or distal joints, the kinetic
5. Provide Contours effects of the splint must be considered
• Eliminate Frictions by proper padding, including what will be the forces on un-
forces, stockinet, etc splinted joints and what will be the
ramifications to extrinsic and intrinsic
II. General Principles of Design musculo-tendinous structures.
1. Consider individual patient factors such as 7. Choose the most appropriate materials and
age, intelligence, motivation, body size, adapt to general properties of the selected
activity level, socio economic status etc. splint materials.
2. Consider the length of time the splint in to be
IV. Principles of Construction
used
1. Use fabricating equipment appropriate to
• Shorter the anticipated need of splint, material.
simpler its design, material type and
construction should be. 2. Use type of heat and temperature appropriate
to the material
3. Strive for simplicity and pleasing appearance
• Over heating or under heating can
4. Allow for optimum function of the extremity disrupt the material.
• Avoid needless immobility of normal 3. Strive for good cosmetic effect.
joints
4. Round corners and smoothen edges
5. Allow for optimum sensation
• For increased strength, durability,
• Palmar tactile surface areas should be free cosmesis and comfort
6. Allow for quick, low cost and efficient 5. Stabilize Joined Surfaces
construction and fit.
• By using one or two rivets as per the
7. Provide for ease of application and removal requirement
460 Neuro-Rehabilitation : A multi disciplinary approach

6. Rivets should be flush with the material - Preserve the ligament structure in correct
surface or apply tape/moleskin over internal position and tension to prevent
metal rivet to prevent rusting. inflammation, attenuation and disruption
of ligament tissue
7. Provide small ventilation holes to improve air
circulation 3. Arches
8. Perforation should be made from inside out • Maintain the skeletal arches (proximal
to give a smoother inner surface transverse, distal transverse and
longitudinal arches)
9. Secure Padding
4. Joints
• Allow ease of splint application and
removal • Align splint axis with anatomic axis
• Apply extra padding to the edges, to • Use optimum rotational force in 90
allow curl around splint edges degree angle of pull
10. Secure straps to provide splint stability by 5. Skin
gluing or riveting and the strap ends should
• Use skin creases as boundaries
be rounded and smoothed to prevent fraying.
Kinesiologic Considerations
V. Principles of Fit
1. Allow for Kinematic changes when fingers and
a. Mechanical Considerations thumb are in flexion or in extension
1. Use Principles of mechanics
2. Employ kinetic concepts
• The mobilizing force should always be
directed perpendicular to both t2he Technical Considerations
segment being moved and to the 1. Develop patient rapport and trust
rotational axis of the joint
• Give instructions and explanations
• The leverage system applied (to the regarding the importance and function of
placement of straps, finger cuffs and the splint
fingernail hooks) should help in
dissemination of the applied force and 2. Work efficiently
reduce pressure and elimination of 3. Change method according to properties of
friction. material used
b. Anatomic Considerations
Prefabricated or Custom fabricated Orthoses
Contiguous fit of the splint to the extremity
Prefabricated orthoses are easily available in a cost
reduces pressure on bony prominences as well
effective and timely manner. As a general rule,
as soft tissue.
prefabricated orthoses should be considered for
1. Bone patients having a normal anatomy, and or who will
• Accommodate bony prominences require the orthosis for a short period of time.
Though these prefabricated orthoses are easy to
- To avoid tissue necrosis apply, the therapists need to understand the
• Incorporate Dual Obliquity concepts indications, contra indications and limitations of
these devices.
- The progressive decrease of length of the
metacarpals from the radial to ulnar Custom made orthoses are generally used when
aspects of the hand orthotic device is required for extremities or when
the spine have deformities or in case of unusual
- The immobility of the second and third
sizes and/ or when they have to be used indefinitely
metacarpals as compared to the mobile
first, fourth, and fifth metacarpals The advantages of custom fabricated orthotics are:
2. Ligaments • Designed to patient specification
• Consider ligament stress • easily adjusted if uncomfortable
Orthosis 461

• Any position requested can be achieved with which the orthosis will receive relatively little stress
the low-temperature plastic or is intended for temporary use. These orthoses
are especially important when a device is needed
• Able to clean as needed
quickly, such as in postsurgical or trauma
• Can get wet treatment.
• Adjustable as swelling decreases or as able to Both high- and low-temperature orthoses must be
get into a better position attached to the body. Most modern orthoses use
straps made of hook-and-loop tape for this purpose.
Materials and construction This material is lightweight, durable, and readily
Although ready-made orthoses are available for adjustable, and comes in a variety of widths and
some applications, many are custom made to fit the colors. Orthoses can also include padding to
specific needs of each patient. The material used in cushion sensitive areas, as well as specialized
orthotic devices typically includes low-temperature linings. Patients often use a separate interface that
thermoplastics that are readily shaped for fit and absorbs perspiration and protects the skin, and
function. Other materials used include casting, which can be washed or replaced as needed.(7)
metal, straps, and hook-and-loop. Most orthoses
have employed lightweight thermoplastic Types of orthoses
materials, which are plastics that become pliable The upper limbs comprise a complex system of
when they are heated and retain their shape once muscles, joints, ligaments, and tendons, which are
they cool. The thermoplastic sheets can be molded capable of a number of distinct movements. For this
to fit body parts exactly, and some can be reshaped reason, a wide variety of upper limb orthoses have
repeatedly as the treated body part changes shape. come into existence. These devices often go by
The resulting orthotic device is lightweight and multiple names, reflecting the name of the
relatively easy to use and maintain. manufacturer, the name of the person who
Thermoplastic materials are usually classified into developed the device, or the anatomy and function
high- and low-temperature types, based on the it serves.
temperature at which they become pliable. High- In the 1970s, a group of American professionals
temperature thermoplastic materials must be involved in the field of orthotics-put forth an effort
molded at a temperature that is too high to come to classify orthoses by their function and acronyms
in contact with human skin. These materials must describing the joints that are encumbered by the
be molded over a plaster model of the body part, orthoses. From this effort sprung the current
but have the advantage of being stronger and more nomenclature : AFO- ankle foot orthosis, TLSO-
durable than low-temperature thermoplastics. They thoracolumbosacral orthosis, WHO- wrist hand
are used in situations where the orthosis will under- orthosis, etc. However, the nomenclature based on
go a lot of stress or will be used for a long time. the function of the orthosis; such as assist
High-temperature thermoplastics require special dorsiflexion at ankle, limit wrist flexion to 10
tools for cutting and shaping, and orthoses made degrees, resist thoracolumbar rotation has not
from these materials are usually constructed by an found wide spread acceptance.
orthotist, a technician who specializes in making
these devices. Orthoses are usually classified under the
Many upper limb orthoses are constructed of low- following three categories:
temperature thermoplastics. These material - static,
becomes pliable below 180°F (80°C), and can be - serial progressive
molded directly against the body. It is relatively
easy to cut and shape, and many therapists - dynamic or functional
construct orthoses using these materials. Precut Static orthoses hold a body part in a fixed position
shells made from low-temperature thermoplastics and do not allow joint movement. Some static
are also available. The therapist can use a precut orthoses do not contain joints, as with fracture
thermoplastic shell as the base for a device and then orthoses that stabilize the long bones of the arm
modify it to fit by trimming and adding pads and after a fracture. Most others simply maintain the
straps. Orthoses made from low-temperature joint at a particular angle, providing support and
thermoplastics are commonly used in situations in proper positioning. For example, a static wrist
462 Neuro-Rehabilitation : A multi disciplinary approach

orthosis can be used to hold the wrist in a neutral compensating arm orthosis that helps to loosen the
position to promote healing and prevent injury grip of the affected hand and help stroke patients
during activities. Sometimes static orthoses include gain greater functional independence.
attachments that help patients perform functional
However, a systematic review that examined 4 trials
activities. For example, a hand-wrist orthosis may
including 126 patients with stroke or other
include an attachment for pens or eating utensils.
nonprogressive brain lesions found that upper limb
Static orthoses sometimes serve the function of
orthoses showed no effect on upper limb function;
promoting eventual joint movement.
ROM at the wrist, fingers, or thumb; or pain.
Complications associated with use of static
Types of upper-limb orthoses
orthoses
• Upper-arm orthoses
1. Skin breakdown
• Clavicular and shoulder orthoses
2. Contractures
• Arm orthoses
3. Infection
• Functional arm orthoses
Serial or progressive orthoses loosen joints that
have become frozen due to contractures or arthritis. • Elbow orthoses
Serial orthoses involve several similar devices used • Forearm-wrist orthoses
in a series, with each successive device gradually
increasing the range of motion of the affected joint • Forearm-wrist-thumb orthoses
by providing a gentle stretching action. • Forearm-wrist-hand orthoses
Progressive orthoses accomplish similar goals, but • Hand orthoses
do so by allowing adjustments in the device so that
• Upper-extremity orthoses (with special
it gradually increases the amount of stretch created
functions)
in the joint.
Serial and progressive orthoses must be designed Clavicular and shoulder orthoses include the
and used carefully to provide the correct amount following:
of stretching in the joint. Excessive stretching can • Figure-8 harness/clavicular brace - This is
damage the tissues, and inadequate stretching will used to restrict motion in patients with
be ineffective. clavicular fractures so as to allow tissue
Dynamic/functional orthoses allow or create joint healing and bone remodeling
movement. These devices hold the joint in the • Shoulder sling - This is used to restrict motion
proper position while assisting movement using in subluxated shoulders by providing humeral
springs, rubber bands, or other mechanical features. cuff and chest straps to keep the humeral head
Dynamic orthoses are useful for patients who have in the glenoid cavity
weakened muscles or limited neuromuscular
control, because they allow the patient to perform • Overhead sling suspension - This is used for
actions that would be difficult or impossible patients with proximal arm weakness or
without assistance. These devices promote paralysis to allow hand or arm use when the
independence in patients who have handicapping muscles are at least antigravity in strength
conditions, and they are common in rehabilitation • Hemiplegic arm sling - This is used for
settings. Since no single device can perform all the immobilization of the hemiplegic shoulder,
movements that the human hand can perform, the which helps to decrease pain and subluxation
patient may need to use several different dynamic (see the image below)
devices in order to carry out activities of daily
Functional arm orthosis are used primarily in
living. Some dynamic splints have a dual or bilateral
patients with proximal arm weakness involving the
tension-providing mechanism that can safely
shoulder and arm,[7] such as that resulting from
accommodate moments of spasm and thus
spinal cord injury (SCI) or peripheral nerve lesions.
potentially limit or prevent soft-tissue injuries.
They include a shoulder saddle from which a
One study indicated that patients who have proximal forearm cuff is suspended by means of
sustained a stroke can be aided by a gravity- straps or a Bowden cable.
Orthosis 463

Elbow orthoses include the following: Forearm mobilization (corrective) orthosis-


• Posterior elbow splint - This is used dynamic supination/pronation splints used to
particularly for elbow immobilization in increase supination or pronation in forearm
patients rotational contracture, or to increase passive or
active-assisted ROM in spinal cord injury
• Serial cast - This is used for preventing or
correcting contractures through promotion of Shoulder-elbow-wrist-hand orthosis (SEWHO)
soft-tissue stretch and passive range of motion consists of a forearm trough (attached by a hinge
(ROM) joint to a ball-bearing swivel mechanism) and a
mount (which can be mounted on the WC, on a
• Air splint - This is a form of circumferential table or working surface, or onto the body jacket).
inflatable sleeve, used to maintain or increase It helps support the forearm and arm against
elbow extension and also used for contractures gravity and allows patients with weak shoulder and
and elbow immobilization elbow muscles to move the arm horizontally and
• Dynamic elbow splints, static progressive flex the elbow to bring the hand to the mouth (e.g.,
elbow splints, turnbuckle elbow splints-gently patients with spinal cord injury, Guillain-Barré
elongate the soft tissues over a long period to Syndrome, polio, muscular dystrophy, and brachial
attempt to reverse joint malalignment plexus injury).
(contractures, burns, and late phase of Forearm-Wrist Orthoses: Forearm-wrist orthoses
fracture). They are not used in spastic muscles may be either volar or dorsal and either gutter-
as they may further increase tone. based or circumferential. Ideally, the wrist should
• Dorsal elbow-extension mobilization be positioned in 15-30° of dorsiflexion (wrist
orthosis - extend the elbow as well as provide extension), except in carpal tunnel syndrome. The
mediolateral elbow stability and rotational volar type should allow for metacarpophalangeal
forearm stability (MP) flexion by ending before the distal palmar
crease.
• Dorsal elbow - flexion mobilization orthosis
- flex the elbow and provide mediolateral Examples of forearm-wrist orthoses include the
stability and rotational forearm stability following:
Forearm orthoses • Wrist cock-up splint
Balanced forearm orthosis (BFO) - This supports • Wrist extension splint
the weight of the forearm and arm against gravity • Ulnar gutter splint
and is used primarily in patients with high-level
tetraplegia or severe proximal arm weakness or Volar wrist-hand stabilizers/wrist cock-up
paralysis[7] ; it may be attached to a wheelchair or splint
table; patients may be able to perform tabletop
• Wrist-hand orthosis (WHO) extending from
activities; prerequisites for its use include a power
distal two-thirds inch of forearm to about one-
source, such as neck or trunk muscles (to shift the
quarter proximal to the distal palmar crease
trunk center of gravity) or adequate scapular
to allow full metacarpophalangeal flexion
movement
while maintaining the functional position of
Requirements the wrist and hand
• Some residual muscle strength (MMT at least • Uses include resting wrist and hand in acute
poor or grade 2) and coordination of elbow arthritis (RA), wrist sprain/contusion, flexor/
flexion (can be used for C5 quad) extensor tendinitis, carpal tunnel syndrome,
• Adequate trunk stability and balance postsurgical wrist extensor tendon repair,
wrist fusion, and skin grafting, contractures
• Adequate endurance in a sitting position prevention, reduce pain, reduce spasticity,
• Preserved ROM of the shoulder and elbow prevent ulnar/radial deviation wrist/hand
joints (e.g., RA)
Other uses: they also may be used in spastic patients
to allow them to self-feed by dampening muscle Dorsal wrist-hand stabilizers
tone through a friction device • WHO are used to provide the same functions
464 Neuro-Rehabilitation : A multi disciplinary approach

Hemiplegic arm cuff /sling Resting hand splint

Basic Opponens Splint Long Opponens Splint

'C' bar splint Ulnar deviation correction splint

(A) Antispasticity "Ball" splint (B) Hand Cone splint

Universal Cuff Patient Using U-cuff for brushing teeth


Orthosis 465

of the volar WHO as well as greater • Prefabricated from spring steel wire and
stabilization because of rigid dorsal hand padded steel bands to assist wrist extension
section by tensing the steel wire, thus aiding finger
flexion through tenodesis effect
• More difficult to fabricate and fit than the volar
WHO
Wrist-driven prehension orthosis (tenodesis
Forearm-Wrist-Hand Orthoses: The basic types of orthosis, flexor hinge splint)
forearm-wrist-hand orthoses are the resting hand Used in C6 complete tetraplegia (in which no
splint (see the image below), the functional resting muscles to flex or extend fingers remain innervated
splint, and the static hand splint. Other types but wrist extension, through the extensor carpi
include the burn splint and the weight-bearing radialis muscle, is intact) to provide prehension
splint. trough tenodesis action and maintain flexibility of
Fig 2 the hand, wrist, and elbow.
Placement may be dorsal, volar, or circumferential • Wrist extensors should be 3+ or better to use
and extends from the tips of the fingers to cover body-powered tenodesis
two thirds of the distal forearm. The dorsal type of
• May interfere with manual WC propulsion.
splint is particularly useful in patients who
demonstrate palmar hypersensitivity and grasp • Rarely accepted by C7 and C8 tetraplegics who
reflex. The position of the wrist is neutral or in slight prefer to use their residual motor power or
dorsiflexion. Immobilization in this position utensil holders.
preferred because metacarpophalangeal joint and
Interphalangeal collateral ligaments are kept RIC tenodesis splint
stretched, minimizing future joint capsule • Orthosis made of low-temperature
contractures. In addition, it provides functional thermoplastics in three separate pieces
thumb position for opposition and three-jaw chuck (wristlet, short opponens, and dorsal plate
pinch. over index and middle finger)
• Easily and quickly fabricated; made as a
Functions of forearm-wrist-hand orthoses
training and evaluation splint for patients;
include the following:
light weight.
• Immobilization in patients who have hand
flexor and extensor tendinitis or who are • Uses a cord/string running from the wrist
undergoing tendon, nerve, or fracture repair. piece, across the palm and up between the
index and ring fingers. The string is lax when
• Maintenance of passive range of motion the wrist is flexed and tightens with wrist
(ROM) in patients with upper motor neuron extension, bringing the fingers close to the
lesions and contractures immobilized thumb, accomplishing three-jaw
chuck prehension.
Wrist Orthoses
1. Wrist control orthosis Wrist driven prehension orthosis.
Promotes slight extension of the wrist or I. Wrist extension: three jaw chuck
prevent wrist flexion, thus assisting weak II. Wrist flexion: release
grasp (via tenodesis effect)
a. Volar wrist-flexion control orthosis (cock- Hand Orthoses
up splints) Wrist, Hand, and Finger Static Orthoses
• Wrist-hand orthoses (WHOs) in which the Positional orthoses
palmar section is extended (usually 20°. They
are used to tighten finger flexors (via tenodesis Opponens orthoses : primarily used to immobilize
effect) and prevent wrist flexion contracture the thumb to promote tissue healing and/or
in patients with radial neuropathy. protection or for positioning of the weak thumb in
opposition to other fingers to facilitate three-jaw-
b. Wire wrist-extension assist orthosis chuck pinch. Examples: short opponens splints, C-
(Oppenheimer splint) bar splints, cone splints, static thumb splints.
466 Neuro-Rehabilitation : A multi disciplinary approach

Opponens orthoses with wrist control 2. Finger mobilization orthosis


attachments a. Interphalangeal extension-mobilization
• Ex: long opponens splints and thumb spica orthoses-passively extend the PIP joints
splints • Uses: Finger IP flexion contracture,
• In addition to the benefits already mentioned Boutonnière deformity, and postsurgical
for opponens orthoses (stabilizes first MCP), release of Dupuytren's contracture
forearm bar maintains wrist in extension and • Examples: Dynamic IP extension splints,
prevent radial and ulnar deviation deformities reverse finger knuckle benders, Capener
splints, safety-pin splints, spring coil
Opponens orthoses with lumbrical bar
assist, eggshell finger extension casts,
• Finger orthosis that prevents metacarpal buddy splints
phalangeal joint hyperextension but allows full
MCP flexion b. Interphalangeal flexion mobilization
orthoses-passively flex PIP joints
• Prevents claw hand deformity (in addition to
the benefits already mentioned for opponens • Use: Finger IP extension contracture
orthoses) • Examples: Dynamic IP flexion splints,
finger-knuckle benders, fingernail book
Opponens orthoses with finger extension assist orthoses, buddy splints
assembly
• Similar to basic opponens orthoses plus finger Ring Stabilizers:
orthosis that assist proximal interphalangeal • Swan neck ring-FO that prevents
and distal interphalangeal extension hyperextension of the PIP joint (via three point
• Used for interphalangeal flex contracture, pressure system) but allows full IP flexion
boutonnière deformity, or postsurgical release • Boutonnière ring-FO that immobilizes the PIP
of Dupuytren's contracture. in extension (prevents flexion) through a three
point pressure system.
Utensil holders/universal cuff (splints)/ADL
splints Metacarpophalangeal ulnar-deviation
• Consist of a handcuff with palmar pocket onto restriction orthosis:
which a utensil can be inserted. • FO used to limit ulnar deviation of the MCP
Protective orthoses: used to protect wrist, hand, with unrestricted (if possible) MCP flex/
and/or fingers from potential deformity or damage extension in arthritic patients with ulnar
by restricting active function/limiting motion deviation at the MCPs.

Digital stabilizers: Metacarpophalangeal (MCP) mobilization


1. Finger stabilizers/static finger orthoses (FOs): orthoses
a. MCP- extension mobilization orthoses-
Interphalangeal stabilizers (DIP, PIP, and
DIP+PIP gutter splints, static finger splints, • Uses: extend MCP joints in MCP-flexion
stax orstack splints, egg-shell finger casts, etc.) contractures, burns, and post ORIF of
• FOs used to restrict motions at the PIP metacarpal fracture, patients with weak
and DIP finger extension (e.g., radial nerve lesion
and brachial plexus lesion).
• Generally, IPs maintained in full
extension to keep the collateral ligaments • Examples: Reverse MCP knuckle
stretched and to prevent IP flex benders, dynamic MCP extension splints
contracture (unless condition dictates with dorsal outrigger, MCP extension
otherwise) assists; radial nerve splints
• Used to promote healing (e.g., phalanx
b. MCP-flexion mobilization orthoses
fx, PIP/DIP dislocation, etc.) and to
provide prolonged finger stretch (e.g., • Uses: used to flex MCP joints in MCP collateral
burns and contractures) ligament contractures, extensor tendon
Orthosis 467

shortening, median/ulnar lesion, claw hand, integrated once more complicated movements
postcapsulotomy, post ORIF of metacarpal emerge
fracture.
• When the CNS is damaged, primitive reflexes
• Examples: MCP knuckle benders, dynamic reemerge and again dominate motor activity
MCP flexion splints with volar outrigger and
fingernail hooks, MCP flexion assists Pressure over muscle insertion
• Farber reported in 1974 that continuous firm
Thumb stabilizers pressure at point of insertion reduces tone
Thumb carpometacarpal stabilizers/thumb posts
Active and static prolonged stretch
• Thumb orthosis that stabilizes the first CMC
and MCP joints in neutral position to protect • Decrease reflex tone by providing mechanical
the thumb form inadvertent motion. Thumb- stabilization of the joint and altering properties
web space stabilizers/thenar web spacers/c- of the muscle spindle
bar splint.
Orthokinetics
• FO that consists of a rigid C-shaped splint held
• Originally developed in 1927 by Julius Fuchs,
firmly in the thumb and index finger web
an orthopedic surgeon
space.
• Focuses on physical effects to materials placed
• Function: increases or maintains the thenar
over muscle bellies
space and prevents web-space contractures
• Passive field materials (those that are cool,
• Uses: Burns, postsurgical revision of scar, web-
rigid, and smooth) produce inhibitory effect
space contractures
• Active field materials (those, warm, expansive,
Thumb mobilization orthoses and textured) produce facilitatory effect
a. Thumb extension-mobilization orthosis-
dynamic thumb IP extension splints. Use: Examples of Upper Extremity Tone-Reduction
thumb IP flexion contracture. Orthoses are
b. Thumb flexion-mobilization orthosis- A. Anti-spasticity "Ball" Splint.
dynamic IP flexion splints B. Hand Cone Splint.
Use: Thumb IP flexion contractures. • They can be either hand-based wrist-hand
c. Thumb abduction-mobilization orthosis- orthoses (e.g., hand-cone splints) or forearm-
dynamic thumb abduction splint. based wrist-hand-finger orthoses (e.g.,
Use: Thumb-adduction contracture. antispasticity ball splints and Snook splints).
• They can be volar based, dorsal based, or
Upper Extremity Orthoses With Special circumferential
Functions
• Typically worn two hours on and two hours
Tone-Reducing Orthosis off throughout the day

Theoretical basis for tone-reducing orthosis • Forearm based splints usually are more
effective because of the extension positioning
• Inhibition of reflexes
of the extrinsic finger flexors
• Pressure over muscle insertions
Rationales of efficacy of tone-reduction orthoses
• Active and static prolonged stretch include:
• Orthokinetics • Reflex-inhibiting positioning-NDT
technique approach (Bobath)
Inhibition of reflexes
• Firm pressure into volar surface (PALM)-
• A reflex consists of a motor act that is elicited
Rood (sensorimotor) approach
by some specific sensory input
• Dorsal-based splints (eg, Snook): facilitation
• Primitive reflexes appear at birth and become
of muscle contraction by direct contact-it is
468 Neuro-Rehabilitation : A multi disciplinary approach

theorized that stimulation of extensor surface of orthopedic and neurologically impaired patients.
might produce extensor muscle contraction However, the neurologically impaired patients
and balance muscle tone and/or avoid additional factors that challenge prescription
increase flexor tone. criteria for the rehabilitation team. Lack of
propriception, sensation, (hypher or
• Functions: flexor tone reduction, prevent skin
hyposensitivity) and spasticity represent some of
breakdown/maceration of palm by
these special considerations and possible probems
fingernails, increase passive range of motion
with communication add to these patient
via low-load, prolonged stretch (serial static
management complications.
splinting)
Evaluation of the neurologically impaired patient
• Indications: spasticity-upper motor neuron
must be comprehensive. The therapist must assess
lesions (cerebral vascular accident, HI,
the client or family motivation, client ability to
multiple sclerosis, cerebral palsy)
tolerate and or function with the orthosis and risks
Evaluation associated with orthotic intervention. Further a total
evaluation of the patient environment is important
The therapist must understand the complex and
in developing the patient plan.
intricate interrelations of normal anatomic
structures, their kinesio- logic functions and their The medical diagnosis should alert the evaluator
biomenchanical and physiologic ramifications to the patterns associated with identified
before attempting to interpret, define and treat the impairments aand should be used to confirm
abnormalities that accompany upper extremity potential findings. In addition to ROM evaluations,
problems. muscle power testing, assessment of sensation, skin
sensitivity or lack, integrity of the affected limb or
Prior to evaluation, gathering patient history is
spine the therapist must assess what limitations
important and can be used to establish a rapport
initiating the orthotic care may have on other
with the patient and the family members.
important functions.
Information that are required from the patient,
caretakers and health care professionals include Goals of Orthotic Intervention
details/ cause of initial injury, previous medical
The clinical experience of the therapist and patient
care, reason for seeking additional care and desired
evaluation must be used to create a plan of
outcomes of new treatments based on the wide
treatment. Only a well thought out plan that is
range of specific functions a patient performs daily.
thoroughly communicated to all the participants
The evaluation of upper extremities requires inputs can ensure success of the intervention. Several other
regarding strength, ROM, condition of soft tissues key factors that have an impact on the success of
and sensation. In addition, ambulatory status, intervention are:
bilateral or unilateral condition, status of vision, and • Need to address the major complaint of the
condition of the spine and head need to be patient.
considered before deciding on the appropriate
• Need to increase function without
orthotic device for the patient.
complication or patient risk.
Since minor changes in strength dramatically • Need to establish a baseline of function so that
change the orthotic need, many critical muscle tests results of intervention are measureable.
must be made in patients with the upper extremity
• Ability of the patient to independently donn
dysfunction. A patient with unilateral involvement
and doff the orthotic device.
can be provided with a typical prefabricated
positional wrist hand orthosis (WHO) to prevent In some cases, concentrated instructions, orthotic
contracture and injury and a supporting shoulder modifications and time are required before
orthosis for shoulder subluxation. On the other improved function can be observed. It is essential
hand for a patient with bilateral involvement, that the orthotic intervention be as simple as
conservation for grooming, feeding mobility, etc possible and to ensure that the least amount will
must be considered and the patient may be needed help achieve the goals of intervention. The
to be fitted with a more complex and customized treatment goals need to be realistic, manageable and
orthoses. well discussed with the patient and care takers to
be successful.
There are similarities in the orthotic management
Orthosis 469

Operation device. Health care professionals must work closely


Although there is a wide variety of upper limb with the patient to ensure that the patient will
orthoses, most of these devices operate on similar accept the orthosis and use it properly.
principles. The general goal of most orthoses is to Exercise: Splints and exercises must be carefully
provide stability and support while allowing as integrated to allow the patients to achieve their full
much motion as possible. Immobilizing joints for rehabilitation potential. Splints are used to improve
long periods has proven deleterious for most passive motion, substitute for weakened or lack of
patients since muscles atrophy, joints stiffen, skin active motion and are infrequently fabricated to
tightens, and the healing process is ultimately provide resistive exercise. Evaluation measures
slowed. By allowing movement while restricting provide the guidelines from which splinting and
motion that would create stress on joints, muscles, exercise programs are coordinated. Because
or tendons, orthotic devices allow healing and patients with identical diagnosis may respond
preserve range of motion and function. differently to therapeutic interventions, it is
Exact fit is a key element for many upper limb insufficient and often detrimental to adhere to rigid
orthoses. In order to work properly, the orthosis pre determined protocols and the exercise and
must hold the body part in an exact position since splinting programs will require constant alterations
if the orthosis does not fit exactly, it may not work to keep pace with the changing requirements.
and may actually cause harm. This can become a Patients are generally instructed to repeat a splint
problem in situations where the patient has wearing and exercise routine every two hourly
experienced swelling and may require a new fitting through out the day. This involves wearing the
for the orthosis once the swelling has resolved. Poor splint for one hour and forty five minutes and then
fit can also lead to discomfort and the development removing the splint and exercising for 15 minutes.
of pressure sores. Conversely if stiffness is a problem, exercise may
be prescribed more [Link] e.g. every 45 minutes.
Dynamic orthoses usually operate with the aid of Written instructions for splint wearing and exercise
attached outriggers. These provide a place to attach routine should be used to augment verbal
rubber bands, springs, or other materials that assist instructions.
motion. They also provide leverage and help to
ensure that the joint stays in proper position during The patients and the family should also be
movement. These devices require exact fit, as well instructed regarding
as adjustment to ensure that the device works
• Donning and doffing
properly.
• Wearability
Many upper limb orthoses require a period of
training for the patient to learn how to use the • Precautions
device properly. This is especially true with devices Patients must be taught to monitor the status of
that assist motion, because the patient must initiate their splinted extremities. The presence of pain,
the motion properly in order for the orthosis to reddened areas, blisters, swelling, rashes, or other
work. Patients with a long history of paralysis or problems associated with wearing a splint should
immobilization may require considerable time in be noted immediately and the use of splint
order to learn how to use the device. Orthotic discontinued until the appropriate corrective
treatment must maximize what little muscle measures have been taken to rectify the problem.
strength and ROM the patients have. Further,
orthoses used during the day for functional Patients should be cautioned about exposing their
purposes might have to be replaced with positional splints to warm or hot temperatures such as hot
orthoses to preserve gain and to prevent decline. water heaters or stoves or leaving the splints in hot
car interiors since thermoplastic splints may lose
It is very important to consider the patient's or alter their configurations in these conditions.
motivation and attitude toward the orthosis as part
of the treatment plan. Since most upper limb Maintenance
orthoses are removable, patients can choose
Many upper limb orthoses require little or no
whether or not to use these devices. Patients may
maintenance. This is especially true for static
object to orthoses because of discomfort,
orthoses and for those intended for temporary use.
unattractive appearance, or restrictiveness of the
The plastic shell can be wiped clean, and materials
470 Neuro-Rehabilitation : A multi disciplinary approach

worn underneath the orthosis can be washed or lose the curvatures due to prolonged resting in a
replaced. The patient may need to be checked flat position. A resting splint may be considered to
periodically to ensure that the orthosis fits. maintain the hand's natural curves and prevent
Dynamic orthoses may require adjustments and secondary complications developing.
replacement of worn springs, rubber bands, and the
like Functional aims of splinting in stroke patients
• To improve grasp in functional activities.
Splinting in stroke patients.
• To increase range of movement to open hand
Within the literature there is conflicting evidence
easily to enable daily hygiene.
and opinions on whether to use splinting as a form
of intervention for stroke patients. There is a • To use the upper limb pain free within
lengthy debate on the theoretical basis for splinting activities.
within neurology with two conflicting theories of Orthoses for SCI patients: Though the evaluation
biomechanical and neurophysiological approaches and rationale for determining the appropriate splint
(Copley and Kuipers, 1999). Biomechanical per injury level are agreed upon, the time of
rationale argues that splinting is used to prevent providing the splint and whether permanent
and manage length-associated changes in muscles orthoses enhance long term functions is debated.
and connective tissues. The neurophysiological However early splinting for positioning to prevent
rationale recommends that splinting is used to deformity has proven to be effective at all injury
inhibit reflexive contracture of the muscle. The levels.
decision on whether to splint must be made on
sound clinical reasoning. There are a wide variety Clients with C1 to C4 tetraplegia require resting
of materials and types of splints and all have hand splints to assist with proper positioning and
different qualities. Splints should not be considered maintain the support of the wrist and web space
when there is active movement that would be Clients with Tetraplegia at C5 level can be
restricted if a splint should be provided. independent with communication, feeding and
Lannin and Herbert (2003) found a lack of evidence hygiene only with the assistance of an orthosis.
for splinting following stroke. In 2007, Lannin et They must have joint stability and support at the
al. conducted a randomised control trial and found wrist the hand to perform these skills. Static splints
no evidence to support the provision of a splint to such as wrist cockup, long opponence and resting
prevent contracture in the acute phase for spasticity hand splints can be given. A universal cuff can be
as a method of prevention of contracture. Regular provided which include a slot for a spoon, fork,
passive range of movement and stretching is comb, tooth brush or hair brush so that a patient
recommended and it is important to provide can effectively feed or groom himself.
teaching to the patient and carer to perform this Clients with injury at the C6 level can use their wrist
programme. The type of splint chosen will vary for a tenodesis grasp. Clients who are not strong
depending on the ideal position, but may include enough to use their wrist for tenodesis may require
cones or resting splints in volar, dorsal or mid splinting to support their wrist until they can use
prone. their wrist against gravity. Long opponens splint
Reduction of pain can be used to position the thumb for function but
support the weak wrist. Once the wrist strengthens,
Following a stroke, pain can occur in various joints
the long opponens splint can be cut down to a hand
although wrist pain is a common complaint among
based short opponens splint to maintain proper
patients. This can be due to the wrist being in a
web space and thumb positioning and maximize
prolonged flexed position due to spasticity or
tenodesis.
flaccidity, leading to overstretching and/or
shortening of muscles. Provision of a volar resting Clients with C8 to T1 injuries or clients who have
splint may be beneficial to prevent further harm incomplete injuries may experience "clawing". A
and provide support. This must be alongside metacarpo-phalangeal block splint to block the
regular range of movement. MCP joints and promote weak muscle function can
be given. Finally educating the client on the splint
Maintaining joint alignment wearing schedules, skin checks and splint care are
Within the early stages of low tone, the hand may very important to prevent skin breakdown.
Orthosis 471

Orthoses for patients with cerebral palsy: In finger flexor activity following stroke. Conf
children with cerebral palsy, orthotic intervention Proc IEEE Eng Med Biol Soc. 2008;2008:4170
is used in combination with an active task oriented
3. de Boer IG, Peeters AJ, Ronday HK, et al. The
therapy program. The new and wide variety of
usage of functional wrist orthoses in patients
thermoplastic materials available to fabricate
with rheumatoid arthritis. Disabil Rehabil.
customized orthotics provides many options for
2008;30(4):286-95.
upper and lower extremities as well as for trunk.
The least restrictive devices, soft splints can be 4. Yonclas PP, Nadler RR, Moran ME, et al.
constructed from webbing, neoprene, hook and Orthotics and assistive devices in the treatment
loop fastener material and other substances. Soft of upper and lower limb osteoarthritis: an
splints do not limit dynamic mobility and sensory update. Am J Phys Med Rehabil. Nov
feedback as much as do thermoplastics. For 2006;85(11 Suppl):S82-97.
children with moderate degree of spasticity 5. Ramon S Lansang Jr, MD; Upper Limb
stronger moulded thermoplastic materials are Support Devices Chief Editor: Robert H Meier
necessary to provide stability. They are lighter in III, MD
weight than the original metal and leather braces
and are easily cleaned. Some of the low temperature 6. Encyclopedia of Nursing & Allied Health,
plastics can be altered as growth or change occurs. ©2002 References
OT have found that upper extremity casting which 7. Bosmans J, Geertzen J, Dijkstra PU. Consumer
provides prolonged and gentle stretch to spastic or satisfaction with the services of prosthetics and
contracted muscles is an effective adjunct to orthotics facilities. Prosthet Orthot Int. Mar
therapeutic techniques. Results of upper extremity 2009;33(1):69-77.
casting have been significant with increased
8. Tyson SF, Kent RM. The effect of upper limb
strength, control, and spontaneous use of impaired
orthotics after stroke: a systematic review.
arm as well as bilateral hand use during play and
NeuroRehabilitation. 2011;28(1):29-36.
transitional movements. The casting program is
always integrated into the therapy program with 9. Atkins MS, Baumgarten JM, Yasuda YL, et al.
the same functional goals. Mobile arm supports: evidence-based benefits
and criteria for use. J Spinal Cord Med.
Summary: 2008;31(4):388-93.
Therapist's clinical expertise alongwith a sound 10. Meijer JW, Voerman GE, Santegoets KM, et al.
understanding of orthotic principles, knowledge of Short-term effects and long-term use of a
multiple orthotic options available are vital to reach hybrid orthosis for neuromuscular electrical
the desired outcome of the orthotic intervention. stimulation of the upper extremity in patients
Since every patients' need for an orthotic after chronic stroke. J Rehabil Med. Feb
intervention is unique, it is essential to develop an 2009;41(3):157-61.
individualized splint wearing and exercise schedule
with periodic reassessments. Coordination between 11. Yasukawa A and Hill J. (1988) Casting to
individual team members such as Orthotist, improve upper extremity function. In R.
therapist (occupational physical or hand therapist) Boehme (ed.), Improving upper body control
patient and family (especially in case of children) (PP 165 -188)
is essential for successful orthotic intervention. 12. Erhardt. R. "Developmental Disabilities:
Cerebral Palsy" In Willard and Spackman's
References Occupational Therapy (VIII ed)
1. Fess, E. and Kiel J. "Therapeutic Adaptations: 13. Pendleton H. & Schultz-Krohn W. "Pedretti's
Upper Extremity Splinting." In Willard and Occupational Therapy: Practice Skills for
Spackman's Occupational Therapy (VIII ed) physical dysfunction" VI Ed.
2. Seo NJ, Kamper DG. Effect of grip location,
arm support, and muscle stretch on sustained
472 Neuro-Rehabilitation : A multi disciplinary approach

Ch.14 Psychological Rehabilitation


Ms. Akshata Shetty, M.A.(Clinical Psychologist)

DEFINITION OF PSYCHOLOGY: • To conduct various psychotherapies


depending on the issues of the patient
The word psychology is derived from the Greek
words Psyche (which means soul) and logos (which • Help the patient to maintain the condition and
means study). Hence, psychology could be defined • Educate the patient for further maintenance
as a "study of the soul". However, today it is defined
as the scientific study of the behaviour of EMOTIONAL, BEHAVIOURAL AND
individuals and their mental processes (American COGNITIVE PROBLEMS THAT PATIENT
Psychological Association). WITH DETERIORATING INCURABLE
DISEASE OR DISORDERS AND LIFE
IMPORTANCE OF PSYCHOLOGY: CHANGING TRAUMA:
Psychology is important in our daily living as it is
Cognitive Changes or Deficits:
concerned with the study of behaviour and mental
processes and at the same time, it is also applied to Cognitive changes or deficits in patients could be
many different things in human life. It is primarily depending on the whether it is due to traumatic
a study of who and what we are, why we are like brain injury, or due to a deteriorating disease like
that, why we act and think like that and what we dementia, or cognitive deficit since birth or taking
could be as a person. Psychology helps us in place due to general medical condition. Patients
understanding about human perceptions, could have a wide variety of cognitive changes or
cognition, emotion, personality, behaviour and deficits like problems in perception, orientation,
interpersonal relationships. episodic memory, shot-term memory, sequential
memory, long-term memory, working memory,
Psychology also attempts to understand the role poor judgment, poor problem solving, difficulty in
these functions play in social behaviour and in concept formation, difficulty in decision making,
social dynamics, while incorporating the etc.
underlying physiological and neurological
processes into its conceptions of mental functioning. Personality Changes:
It helps the patients who have emotional, cognitive Changes in the personality of patients are especially
and behavioural deficits or issues to understand the
disturbing for their families. Pre-existing
problem, the severity of it, to accept the situation personality traits may be accentuated during the
and ways to either reduce the discomfort caused
course of the illness. Patients may become
or eliminate it with the help of various introverted and seem to be less concerned than they
psychotherapies. previously were about the effects of their behaviour
on others. Patients could also have paranoid
Process of a Psychological Intervention:
delusions are generally hostile to family members
• Build rapport with the patient. and caretakers. The personality changes could take
• To take down the case history and document place depending on the disease for example patients
it with frontal and temporal involvements are likely
to have marked personality changes and may be
• To assess the patient with the help of formal
irritable and explosive. Also, patients with
and informal assessments and make reports
traumatic brain injury could have overt sexual
• To diagnose the patient behaviour, which could be completely different as
compared to their premorbid personality.
• To educate the patient and family members,
about the diagnosis and explain the
Mood:
intervention plan
In addition, to psychosis and personality changes,
• To make referrals to other professionals id depression and anxiety major symptoms in patients,
needed although the full syndrome of depressive disorder
Psychological Rehabilitation 473

may be present in only 10 to 20 per cent. Patients Anger and Aggression:


may also may exhibit pathological laughter or Anger is a common and natural reaction to the
crying that it is, extremes of emotions with no frustrations one encounters while dealing with a
apparent provocation. Some could even quality for disease. While having some anger about living with
a diagnosis of mood disorders. the frustrations, symptoms, and anxieties
associated with having a chronic illness is
Depression, Anxiety and Psychological appropriate and healthy, sometimes anger is
Distress: expressed indirectly as sarcasm, cynicism, or
Depression has been correlated to suffering, irritability. Other people may feel consumed by
hopelessness, fast deteriorating or inability to cope anger. Either way, anger about the directed toward
with the disorder and social withdrawal which is others or bitterness can have damaging effects on
in agreement to the quality of life. It is often seen a person's relationships with others. An important
that adults suffering from an incurable disease have task for many people suffering from a deteriorating
a sense of lack of emotional support, lack of coping disease or a disorder is recognizing the anger at
skills and a very negative view of the future. the disease, learning to direct it properly at the
Patients may suffer from pain which may lead to disease rather than at loved ones, and managing
depression. However, it is seen that it may not be anger and aggression effectively.
so much due to the severity of the disability but it
may depend but would depend on how well they Isolation:
cope with the disability. Children and adults may Many people with a disease or a disorder, as well
develop depression as being unable to control their as families and caregivers, can become socially
body, embarrassment about their body in social isolated. Early in the disease, people may avoid
situations and lack of information about their talking to friends or family about their disease.
situation. Also guilt could be a reason of depression Sometimes they avoid doing so because they fear
as the patients may see their caregivers suffering others will treat them differently, or sometimes they
due to them. avoid these discussions because they don't want to
burden others or make them worry. However, this
Loss of Control: avoidance can lead to increased withdrawal,
Patients often undergo a feeling that they have lost resulting in isolation, loneliness, and depression,
control of their body, thoughts or feelings. This both for the person living with the disease and his
could eventually lead to impetuous decisions like or her loved ones. People who are more severely
resigning from job, or thinking of living alone as affected physically by a disease sometimes find it
they feel their caregivers have to go through a lot physically harder to have contact with others.
because of them, etc. The patients should often learn Sometimes, people with disease (particularly those
to identify the control issues, to manage effectively who don't know anyone else with a similar disorder
and to cope up with situations which are difficult or disease) feel that no one could understand what
to change. it is like living with this disease, so there is no point
in trying to talk with others about it. However, there
Poor or Low Self-Image and Self Doubt: is a strong relationship between isolation,
Am I really sick? Am I imagining this? Am I weak? depression, and increased health problems.
Did I cause this illness? If I had eaten better or While maintaining relationships is not always easy,
exercised more, would I be well today? Patients find it is very important to ones mental and physical
it very difficult to accept themselves. They often health. Maintaining social contact often requires
compare the things that they were able to do before new methods of approaching relationships. Often
and how their condition has deteriorated now. This it requires becoming more open with people about
often leads to a lot of frustration and eventually how the disease affects you and how it might
being unable to accept their self -image. It would impact your relationship. Support groups,
be difficult to accept ones self-image as conditions volunteering, involvement in religious or spiritual
which are deteriorating or incurable need constant practices and organizations, and taking classes at
adjustment to new changes. It is very important to junior colleges are but a few ways people find
examine why the lost abilities were important and meaningful contact with others. Many people have
to find an alternative method of meeting those begun using Internet chat rooms. While the Internet
needs.
474 Neuro-Rehabilitation : A multi disciplinary approach

can be helpful, we caution against spending so Major milestones, such as changing jobs, taking on
much time that it interferes with actual contact with a mortgage, or having a child, all weighty decisions
other people. New research suggests that too much for anybody, can seem impossible to resolve when
time on the Internet, at the expense of time face to the uncertainty of MS is involved. Sometimes,
face with other people, may increase depression people will spend so much of their time and
and social isolation. resources preparing for things they fear in the
future they miss out on pleasures, goals, and desires
Loss of Independence: in the present.
Loss of independence can be a major issue for the Of course, there are also numerous other issues
patients and the family members. These issues can which can be experienced by the patient, including
be emotional, physical, medical and financial. Some feelings of hopelessness, anxiety, issues around
people rush headlong into a dependent role, only work and disability, and many others. These issues
to find themselves, then feeling empty, hopeless, can be overwhelming at times. However, they are
or useless. Others may fight their fears of not insurmountable. Very few people with a
dependency to the point where it is physically particular disorder or disease have difficulties in
dangerous. For example, someone who is all areas all the time. Most people experience
uncomfortable with dependence on walking aids periods of regular day-to-day life interspersed with
may avoid using a cane or crutches, even though periods when one or more of these issues become
he or she is falling and getting injured. Often, people prominent. Most people will also find that they are
have uncomfortable feelings about starting new able to cope with many of these issues much of the
medications, and may reject treatments which can time; yet from time to time, one or more of these
be beneficial. Recognizing ones own issues around issues might becomes overwhelming.
dependency is the first step towards preventing
them from putting you at risk. Emotional Instability:
The emotional instability is manifested by
Abandonment:
irritability, anxiety and depression, as well as
Fear of abandonment can be one of the most euphoria, even to the extent of a manic or
frightening issues a person faces. Many people, depressive psychosis. Denial is manifested by
particularly in the earlier stages of the illness, fear projection of frustration and hostility onto other
their spouse or partner will want to leave them. people, or by unrealistic optimism. Threat, to
These fears are often so anxiety-provoking that personality functioning, produces the
couples avoid talking about this issue. For people compensatory psychological defence mechanisms
with very severe disease late in the illness, people of denial and projection.
may fear being moved to nursing facilities to be
abandoned. For those who are not married or in a Self-dislike:
relationship, there are often questions about
Why should I try to look attractive when I'm so
whether they will ever find a life partner, or if they
dependent? Who wants to employ someone who
will lead a life abandoned by others. These thoughts
might be ill all the time? Why can't I overcome this
can be so frightening that people avoid discussing
illness?
them with anyone. Such fears are sometimes self-
fulfilling, as people fearing abandonment may push Family Problems:
others away through angry, withdrawing, or other
Patients who are suffering from a deteriorating or
behaviors. Avoiding this topic has never been
incurable disorder can cause serious problems
known to save a relationship. Discussing these fears
within the family dynamics. Patients usually
openly often results in feelings of relief for all
undergo feelings of fear, that if they demand
concerned.
anything from their partner, his demands may be
Uncertainty: rejected. Often the relatives become overprotective
with underlying feelings of hostility. This may be a
Because the course of certain diseases like Multiple
serious when a parent becomes overprotective,
Sclerosis, Motor Neuron Disease, Muscular
especially if he sufferer is married. Other patients
Dystrophy, etc is so unpredictable, many people
would deny the disease so much that they refuse
experience considerable uncertainty and anxiety.
to acknowledge any limitations. Life then becomes
This uncertainty often makes life planning difficult.
Psychological Rehabilitation 475

difficult for all members of the family in a different between true stigma, and difficulties one has in ones
way. own self-image which are projected onto others. For
example, a person who grew up feeling
Quality of Family Support: uncomfortable around people in wheelchairs, and
The most important question to be answered is who now finds him/herself in a wheelchair, may
"What kind of relationship the patient has with his imagine that others are uncomfortable even when
close relatives (children, spouse, and extended they are not. In this way, people sometimes imagine
family)?" It is important to know because the there is stigma even when there is none. How
psychological suffering of a patient may result from people choose to deal with discrimination and
tensions within his family (rejection, stigma, stigma varies from situation to situation, and from
exclusion, indifference). Often a psychological person to person. Some people find that confronting
maltreatment develops between the patient and his discrimination directly and advocating socially
relatives; they feel unable to bear the daily progress and/or politically for the rights of people with
of the disease. chronic illnesses and disabilities helps them feel
empowered. Other people find taking an
Support for the Caregivers: educational approach to discriminatory situations
Many patients require long-term physical, financial, is best for them, and still other people find that
and psychological support from family and friends. viewing discrimination as their problem/
Both the physical and mental health of the caregiver weakness/loss, not mine, and instead focusing on
is critical. The burden may be considerable and their own health, needs, and well-being is best.
threaten both. In fact, the more hours dedicated to
the patient, the more depressed the caregiver is PSYCHOLOGICAL TESTING:
likely to be. This leads to a cycle of poor caregiving Intelligence Testing:
followed by reduced functioning in the patient.
Intelligence is defined as the ability to assimilate
According to Boot et al. (2008) psychological factual knowledge to recall either recent memory
input can have benefits in the following areas: or remote events, to reason logically to manipulate
concepts (either numbers or words), to translate the
• Managing their mood better abstract to the literal to the abstract, to analyze and
• Coping better synthesize forms and to deal meaningfully and
accurately with problems and priorities deemed
• Improved levels of daily activity
important in a particular setting. Intelligence varies
• Better understanding of their difficulties for individual to individual.
• Improved relationships
Wechsler Adult Intelligence Scale ® - Third
• Less prone to feelings of suicide Edition (WAIS®-III):
• More confident about managing their future Description: The WAIS- III is the best standardized
with MS. and most widely used intelligence test in the clinical
practice today. It was constructed by David
Stigma: Wechsler at New York University Medical Centre
While self-image refers to how you think about and Bellevue Psychiatric Hospital. WAIS - III has
yourself, stigma refers to how others view you. 11 subtests which are made up of six verbal sub-
Unfortunately, in some environments it is common tests and five performance sub-tests, which yield a
for people to stare, be patronizing or overly verbal IQ, a performance IQ and a combined or full
solicitous, or to be avoidant of a patient whose scale IQ. The sub-tests are as follows:
symptoms that are visible to others. If your • The Verbal subtests are: Vocabulary,
symptoms are not immediately visible to others, Similarities, Arithmetic, Digit Span,
such as cognitive problems or fatigue, some people Information and Comprehension.
may say you're just ignoring me or you're just lazy,
rather than attribute the symptoms to the disease. • The Performance subtests are: Picture
It is difficult for many people to adjust to the Completion, Digit Symbol - Coding, Block
changes in how they are treated by others. Design, Matrix Reasoning and Picture
However, it is also important to distinguish Arrangement
476 Neuro-Rehabilitation : A multi disciplinary approach

Age group: Between 16 to 89 years of age Wechsler Memory Scale Fourth Edition
Target Group: Adults with cognitive deficits due (WMS - IV):
to age related or trauma to the brain, or due to other Description: This is a neuropsychological test
diseases, etc. designed to measure different memory functions
in a person. The current version is the fourth edition
Wechsler Intelligence Scale for Children (WMS-IV) which was published in 2009 and which
(WISC): was designed to be used with the WAIS-IV. WMS-
Description: This test was developed by Dr. David IV is made up of seven subtests: Spatial Addition,
Wechsler, is an individually administered Symbol Span, Design Memory, General Cognitive
intelligence test for children that can be completed Screener, Logical Memory, Verbal Paired
without reading or writing. It is also used to Associates, and Visual Reproduction. A person's
diagnose attention-deficit hyperactivity disorder performance is reported as five Index Scores:
(ADHD) and learning disabilities. The WISC can Auditory Memory, Visual Memory, Visual
be used as part of an assessment battery to identify Working Memory, Immediate Memory, and
intellectual giftedness, learning difficulties, and Delayed Memory.
cognitive strengths and weaknesses. When Age Group: From 16 to 90 years.
combined with other measures such as the
Adaptive Behaviour Assessment System-II and the Time taken for Administration: 75 minutes
Children's Memory Scale its clinical utility can be Target Group: Individuals with Memory deficits.
enhanced. Combinations such as these provide
information on cognitive and adaptive functioning, Neuropsychological Tests:
both of which are required for the proper diagnosis Neuropsychological Tests are specifically designed
of learning difficulties and learning and memory tasks used to measure a psychological function
functioning resulting in a richer picture of a child's known to be linked to a particular brain structure
cognitive functioning. or pathway. Tests are used for research into brain
function and in a clinical setting for the diagnosis
Age Group: Between the ages of 6 and 16 of deficits. They usually involve the systematic
Time taken for Administration: 65-80 minutes administration of clearly defined procedures in a
Target Group: Attention Deficit Hyperactivity formal environment. Neuropsychological tests are
Disorder, Learning Disability, Mentally Retarded typically administered to a single person working
Children, Cerebral Palsy, Autism, Children with with an examiner in a quiet office environment, free
Duchenne's Muscular Dystrophy with comorbid from distractions.
Mental Retardation, etc.
Mini Mental Status Examination:
Description: The mini-mental state examination
(MMSE) or Folstein test is a brief 30-point
questionnaire test that is used to screen for cognitive
impairment. It is commonly used in medicine to
screen for dementia. It is also used to estimate the
severity of cognitive impairment at a specific time
and to follow the course of cognitive changes in an
individual over time, thus making it an effective
way to document an individual's response to
treatment. The MMSE test includes simple
questions and problems in a number of areas: the
time and place of the test, repeating lists of words,
Wechsler's Intelligence Scale for Children
arithmetic such as the serial sevens, language use
and comprehension, and basic motor skills.
Memory Test: Time Taken for Administration: 10 minutes
Memory is defined as "the ability of an organism Target Group: Patients with dementia
to store, retain, and recall information and
experiences".
Psychological Rehabilitation 477

Scoring: and working memory (executive functions). First


25 - 30 : Normal proposed by Swiss psychologist André Rey in 1941
and further standardized by Paul-Alexandre
21 - 24 : Mild Cognitive Impairment
Osterrieth in 1944, it is frequently used to further
19 - 20 : Moderate Cognitive Impairment elucidate any secondary effect of brain injury in
10 - 20 : Mild Cognitive Impairment neurological patients, to test for the presence of
dementia, or to study the degree of cognitive
00 - 10 : Severe Cognitive Impairment
development in children.
A low to very low scores correlate closely with the
presence of dementia, although other mental Halstead-Reitan Neuropsychological Battery:
disorders can also lead to abnormal findings on Description: This is a combination of
MMSE testing. The presence of purely physical neuropsychological tests used to assessment the
problems can also interfere with interpretation if possible physical aspects and localization of
not properly noted; for example, a patient may be neurological damage. The Halstead-Reitan is
physically unable to hear or read instructions typically used to evaluate individuals with
properly, or may have a motor deficit that affects
suspected brain damage. The battery also provides
writing and drawing skills.
useful information regarding the cause of damage
Luria-Nebraska neuropsychological battery: (for example, closed head injury, alcohol abuse,
Alzheimer's disorder, stroke ), which part of the
Description: This is a standardized test based on
brain was damaged, whether the damage occurred
the theories of Alexander Luria regarding
during childhood development, and whether the
neuropsychological functioning. There are 14
damage is getting worse, staying the same, or
scales: motor functions, rhythm, tactile functions,
getting better.
visual functions, receptive speech, expressive
speech, writing, reading, arithmetic, memory,
The Battery includes:
intellectual processes, pathognomonic, left
hemisphere and right hemisphere. It is used with • Trails A and B (which see how quickly a
people who are 15 years or older; however, it may patient can connect a sequence of numbers
be used with adolescents down to 12 years old. Part (trail A) or numbers and letters (trail B).
of A.R. Luria's legacy was the premium that he • Controlled Oral Word Association Test
placed on the observation of a patient completing
(COWAT, or Verbal Fluency) - a measure of
a task; intra individual differences. The modern
a person's ability to make verbal associations
practice of standardized testing tends to neglect this
to specified letters.
aspect of psychology. The Luria-Nebraska
Neuropsychological Battery (now in its third • Halstead Category Test (including seven
iteration) attempts to create an alloy of standardized subtests which form three factors: a Counting
testing and idiosyncratic observation by allowing factor (subtests I and II), a Spatial Positional
comparison to the normative sample, and at the Reasoning factor (subtests III, IV, and VII), and
same time giving the test administrator flexibility a Proportional Reasoning factor (subtests V,
in the administration. VI, and VII).)
Age Group: 15 years and up • Tactual Performance Test
Time taken for Administration: 1 ½ hours and 2 ½ • Rhythm Test
hours.
• Speech Sounds Perception Test
Rey-Osterrieth Complex Figure Test (ROCF): • Finger Oscillation Test
Description: This is a neuropsychological Age Group: 15 years and older
assessment in which examinees are asked to
reproduce a complicated line drawing, first by Personality Tests:
copying and then from memory. Many different
Personality is defined as the particular combination
cognitive abilities are needed for a correct
of emotional, attitudinal, and behavioral response
performance, and the test therefore permits the
patterns of an individual. A personality test is a
evaluation of different functions, such as
questionnaire or other standardized instrument
visuospatial abilities, memory, attention, planning,
designed to reveal aspects of an individual's
478 Neuro-Rehabilitation : A multi disciplinary approach

character or psychological makeup. The first • Support college and career counselling
personality tests were developed in the early 20th recommendations.
century and were intended to ease the process of
• Provide valuable insight for marriage and
personnel selection, particularly in the armed
family counselling.
forces.
Age Group: 18 years to 80 years
Minnesota Multiphasic Personality Inventory- Time taken for Administration: Individual - 35 to
2 - Restructured Form: 50 minutes.
Description: The original authors of the MMPI
were Starke R. Hathaway, PhD, and J. C. McKinley,
MD. This is one of the most frequently used
personality tests in mental health. The test is used
by trained professionals to assist in identifying
personality structure and psychopathology. The
MMPI-2-RF aids clinicians in the assessment of
mental disorders, identification of specific problem
areas, and treatment planning in a variety of
settings. The test can be used to help:
• Assess major symptoms of psychopathology,
personality characteristics and behavioural
proclivities.
• Evaluate participants in substance abuse
programmes and select appropriate treatment
approaches. MMPI Test Answer Sheet

• Assess medical patients and design effective


treatment strategies, including chronic pain Sixteen Personality Factor Questionnaire Revised
management. - 5th Edition (or 16PF- R 5th Ed.): This is a multiple-
choice personality questionnaire which was
• Support classification, treatment, and developed over several decades of research by
management decisions in criminal justice and Raymond B. Cattell and his colleagues.
correctional settings.
Each factor can be measured on a scale, determined
• Identify high-risk candidates in public safety by completing a questionnaire, and the word pairs
screening and selection settings. below indicate the extremes of each scale. The letter
• Give strong empirical foundation for expert codes were ascribed to each scale as a shorthand
testimony in forensic evaluations. notation.

Factors Descriptors
A Warmth Reserved Outgoing
B Reasoning Less Intelligent More Intelligent
C Emotional Stability Affected by feelings Emotionally stable
E Dominance Humble Assertive
F Liveliness Sober Happy- go - lucky
G Rule Consciousness Expedient Conscientious
H Social Boldness Shy Venturesome
I Sensitivity Tough-minded Tender-minded
L Vigilance Trusting Suspicious
M Abstractedness Practical Imaginative
Psychological Rehabilitation 479

N Privateness Straightforward Shrewd


O Apprehension Self-assured Apprehensive
Q1 Openness to Change Conservative Experimenting
Q2 Self- Reliance Group - dependent Self -sufficient
Q3 Perfectionism Self - conflict Self - control
Q4 Tension Relaxed Tense

Age Group: Individuals 16 years and older in the response with reference to shading
• Shading-diffuse - when shading is used in the
Projective Tests:
response
In psychology, a projective test is a personality test
designed to let a person respond to ambiguous • Form dimension - when dimension is used in
stimuli, presumably revealing hidden emotions and the response without reference to shading
internal conflicts. This is different from an "objective • Pairs and reflections - when a pair or reflection
test" in which responses are analyzed according to is used in the response.
a universal standard (for example, a multiple choice
exam). The responses to projective tests are content
analyzed for meaning rather than being based on
presuppositions about meaning, as is the case with
objective tests.

Rorschach inkblot test:


Description: The test is named after its creator,
Swiss psychologist Hermann Rorschach. This is a
psychological test in which subjects' perceptions of
inkblots are recorded and then analyzed using
psychological interpretation, complex algorithms,
or both. Some psychologists use this test to examine Rorschach Inkblot Card No. 2
a person's personality characteristics and emotional
functioning. It has been employed to detect
underlying thought disorder, especially in cases
where patients are reluctant to describe their
thinking processes openly.
The core of scoring revolves around coding the
response according to all of the blot features that
have contributed to the formation of the response.
The following characteristics are coded:
• Form
• Movement - when any movement occurred in
the response
• Chromatic Color - when color is used in the Rorschach Inkblot Card No. 10
response
• Achromatic Color - when black, white or grays
Thematic Apperception Test (TAT):
are used in the response Description: The TAT is often administered to
individuals as part of a battery, or group, of tests
• Shading-texture - when texture is used in the
intended to evaluate personality. It is considered
response
to be effective in eliciting information about a
• Shading-dimension - when dimension is used person's view of the world and his or her attitudes
480 Neuro-Rehabilitation : A multi disciplinary approach

toward the self and others. As people taking the was developed by Florence Goodenough in 1926.
TAT proceed through the various story cards and This is a psychological projective personality or
tell stories about the pictures, they reveal their cognitive test used to evaluate children and
expectations of relationships with peers, parents or adolescents for a variety of purposes. The tester is
other authority figures, subordinates, and possible asked to draw a man or a woman whatever they
romantic partners. In addition to assessing the prefer, after the first figure is drawn i.e. if a boy is
content of the stories that the subject is telling, the drawn then the client is asked to draw the other
examiner evaluates the subject's manner, vocal tone, sex i.e. a girl or a woman. After which the client is
posture, hesitations, and other signs of an emotional inquired as to what he drew and a few related
response to a particular story picture. questions are asked for interpretation and scoring
purpose.
The TAT is usually administered to individuals in
a quiet room free from interruptions or distractions. Aspects such as the size of the head, placement of
The subject sits at the edge of a table or desk next the arms, and even things such as if teeth were
to the examiner. The examiner shows the subject a drawn or not are thought to reveal a range of
series of story cards taken from the full set of 31 personality traits (Murstein, 1965). The personality
TAT cards. traits can be anything from aggressiveness, to
homosexual tendencies, to relationships with their
Children's Apperception Test (CAT): parents, to introversion and extroversion
Description: The Children's Apperception Test was (Machover, 1949).
developed in 1949 by Leopold Bellak and Sonya Time taken for administration: 20 to 30 minutes
Sorel Bellak. It was an offshoot of the widely used
Thematic Apperception Test (TAT), which was Advantages:
based on Henry Murray's need-based theory of
1. Easy to administer
personality. Bellak and Bellak developed the CAT
because they saw a need for an apperception test 2. Helps people who have anxieties taking tests
specifically designed for children. The CAT is (no strict format)
intended to measure the personality traits, 3. Can assess people with communication
attitudes, and psychodynamic processes evident in problems
prepubertal children. By presenting a series of
pictures and asking a child to describe the situations 4. Relatively culture free
and make up stories about the people or animals
in the pictures, an examiner can elicit this Disadvantages:
information about the child. 1. Restricted amount of hypotheses can be
developed
Age Group: 3 to 10 years
2. Relatively non-verbal, but may have some
Time taken for administration: 20 to 45 minutes
problems during inquiry
3. Little research backing

Childhood Apperception Test

Draw - A - Person Test:


Description: Draw-A-Person Test (DAP): This test Draw- A - Person Test (Male figure)
Psychological Rehabilitation 481

Draw - A - Person Test (Female figure)

House-Tree-Person Test (HTP):


Description: This test was created by Buck in 1948, House- Tree - Person Test (Person Drawing)
provides a measure of a self-perception and
attitudes by requiring the test taker to draw a house,
a tree, and a person. The picture of the house is
supposed to conjure the child's feelings toward his
or her family. The picture of the tree is supposed to
elicit feelings of strength or weakness. The picture
of the person, as with other figure drawing tests,
elicits information regarding the child's self-
concept. The HTP, though mostly given to children
and adolescents, is appropriate for anyone over the
age of three.

House- Tree - Person Test Complete House -


Tree - Person Test

Kinetic Family Drawing Test:


Description: The Kinetic Family Drawing,
developed in 1970 by Burns and Kaufman, requires
the test taker to draw a picture of his or her entire
House- Tree - Person Test (House Drawing) family. Figure drawings are projective diagnostic
techniques in which an individual is instructed to
draw a person, an object, or a situation so that
cognitive, interpersonal, or psychological
functioning can be assessed. Children are asked to
draw a picture of their family, including
themselves, "doing something." This picture is
meant to elicit the child's attitudes toward his or
her family and the overall family dynamics. The
KFD is sometimes interpreted as part of an
House- Tree - Person Test (Tree Drawing) evaluation of child abuse.
482 Neuro-Rehabilitation : A multi disciplinary approach

Age Group: 17 through 80 years


Time taken for administration: 5 to 10 minutes self-
administered.

Beck Scale for Suicide Ideation® (BSS®):


Description: This was developed by Aron. T. Beck,
where it assess the severity of suicidal ideation in
an individual.
Age Group: 17 years and older
Time taken for administration: 5 to 10 minutes self-
Kinetic Family Drawing Test
administered.
Behaviour Tests:
Bender Visual-Motor Gestalt Test, Second
Beck's Depression Inventory - Second Edition Edition (Bender-Gestalt II):
(BDI - II): Description: Originally published in 1938 by
Description: The Beck Depression Inventory (BDI, Lauretta Bender, M.D., the Bender Visual-Motor
BDI-II), is created by Dr. Aaron T. Beck, is a 21- Gestalt Test is one of the most widely used
question multiple-choice self-report inventory, one psychological tests. The Bender Gestalt provides
of the most widely used instruments for measuring helpful information in preschool screening as well
the severity of depression. as geriatric assessment. And it can offer insight into
many conditions, including ADHD, mental
Time taken for administration: 10 minutes retardation, giftedness, learning disabilities, autism,
Age Group: From 13 to 80 years and Alzheimer's disease.
Time taken for Administration: No time Limit
Scoring:
0 - 13: Minimal Depression Age Group: 4 to 85 years old

14 - 19: Mild Depression Condition Specific tests:


20 - 28: Moderate Depression Autism: The psychological tests that are conducted
on autistic children and adults are as follows:
29 - 63: Severe Depression
Autism Diagnostic Interview - Revised,
Prelinguistic Autism Diagnostic Observation
Higher total score indicates severe depression
Schedule, Childhood Autism Rating Scale Second
The tests consists of 21 questions, which consists of Edition (CARS- 2), Checklist for Autism in Toddlers
sadness, pessimism, past failures, loss of pleasure, (CHAT), Gilliam Autism Rating Scale - Second
guilt feelings, punishment feelings, self- dislike, self- Edition (GARS - 2), etc. Detailed description
criticalness, suicidal thoughts or wishes, crying, including other tests are mentioned in the autism
agitation, loss of interests, indecisiveness, chapter i.e. Chapter 4.
worthlessness, loss of energy, changes in sleeping
patterns, irritability, changes in appetite, Dementia: The psychological tests that are
concentration difficulty, tiredness or fatigue and conducted on patients suffering from dementia are
loss of interest in sex. as follows: ADAS - Cog, Quality of life - Alzheimer's
disease scale (QOL - AD), DEMQOL, Holden
Beck Hopelessness Scale® (BHS®): Communication Scale, Neuropsychiatric Inventory,
Description: This is a 20 item test developed by Rating Anxiety in Dementia Scale, etc. Detailed
Aron Beck. This is a powerful predictor of eventual description including other tests are mentioned in
suicide to help you measure three major aspects of the dementia chapter i.e. Chapter 5.
hopelessness: feelings about the future, loss of
motivation, and expectations. Responding to the 20 PSYCHOTHERAPIES:
true or false items on the Beck Hopelessness Scale® Psychotherapy is a general term referred to a
(BHS®), patients can either endorse a pessimistic therapeutic interaction or intervention plan
statement or deny an optimistic statement. between a trained psychologist and a client, patient,
Psychological Rehabilitation 483

caregiver or a group. The psychological problems fear of suffocation. Beck during such trying times
are addressed depending on the kind of problem used reasoning to alleviate these anxieties and was
or deficit or the severity of the problem. The main very successful in overcoming his fears and
goal of psychotherapy is to help the client face the anxieties. [1]
situation, deal with it and come up with solution
to the problem during a therapeutic session. Principles of Cognitive Therapy:
Most forms of psychotherapy use spoken The practice of cognitive therapy is based on the
conversation. Some also use various other forms of following principles:
communication such as the written word, artwork, • Changes in thinking, lead to changes in
drama, narrative story or music. Psychotherapy feelings and acting.
with children and their parents often involves play,
• The treatment focuses on the present although
dramatization (i.e. role-play), and drawing, with a
attention is paid to the past when required.
co-constructed narrative from these non-verbal and
displaced modes of interacting. Psychotherapy • Treatment needs to be short-term, problem
occurs within a structured encounter between a focused and goal oriented.
trained therapist and client(s). Purposeful,
• This therapy has a structured and active
theoretically based psychotherapy began in the 19th
approach to treatment.
century with psychoanalysis; since then, scores of
other approaches have been developed and • Treatment requires a sound and collaborative
continue to be created. therapeutic alliance.
Therapy is generally used in response to a variety • Careful assessment, diagnosis and treatment
of specific or non-specific manifestations of planning are integral.
clinically diagnosable and/or existential crises. • This therapy believes in teaching the client to
Treatment of everyday problems is more often identify, evaluate and modify their own
referred to as counseling (a distinction originally cognitions, this promotes emotional health
adopted by Carl Rogers). However, the term and prevents relapse.
counseling is sometimes used interchangeably with
"psychotherapy". • To help the client accurately assess his/ her
assess her cognitions, inductive reasoning and
While some psychotherapeutic interventions are Socratic questioning is extremely important.
designed to treat the patient using the medical
model, many psychotherapeutic approaches do not The Process of Cognitive Treatment:
adhere to the symptom-based model of "illness/
Cognitive treatment is usually time limited and it
cure". Some practitioners, such as humanistic
usually takes 4 to 14 sessions for solving the issue
therapists, see themselves more in a facilitative/
that the client has. The session usually has 10 steps:
helper role. As sensitive and deeply personal topics
are often discussed during psychotherapy, 1. To establish the agenda that is meaningful to
therapists are expected, and usually legally bound, the client.
to respect client or patient confidentiality. The 2. Determine the intensity of the person's mood.
critical importance of confidentiality is enshrined
3. To identify and review the presenting
in the regulatory psychotherapeutic organizations'
problems.
codes of ethical practice.
4. To elicit the person's expectations from the
The various forms of psychotherapies are listed treatment.
below: 5. To educate the client about the therapy and
his contribution in the therapeutic alliance.
Cognitive Therapy:
6. Explain the client about his problems and
History: diagnosis.
Aaron. T. Beck developed cognitive behaviour 7. Establish clear goals.
therapy and he himself had many difficulties
during childhood himself. He used to be ill very 8. Recommend homework.
often, had many anxieties and phobias, including 9. Summarize the session.
a blood injury phobia, a fear of public speaking and 10. Obtain the feedback of the session.
484 Neuro-Rehabilitation : A multi disciplinary approach

Goal: • Mental Filters: The individual focuses more on


The goal of cognitive therapy is to help the client the negative details and does not look at the
identify the errors that take place in his information- broad picture.
processing system and correct them. To help the • Mind Reading: Attributing negative thoughts
client to work through this process the therapist and reactions to others without checking
helps the client identify both the client's immediate whether they are present.
thoughts (automatic) and global thoughts (core)
• Magnifying or Minimization: The individual
and beliefs as well as the associated emotions and
draws negative or positive information in
behaviours.
excess.

Strategies to Alter the Dysfunctional thoughts:


• Mental and emotional Imagery: This is to help
the client adapt to new ways of thinking and
feeling. Also to help them envision new ways
of coping successful.
• Activity Scheduling: Here the therapist helps
the client to try out new behaviours and ways
of thinking and encourages them to remain
active despite feelings of sadness or anxiety
experienced by the person.
• Thought Stopping: This is done by saying
"STOP" to oneself either loudly or subvocally.
Cognitive Therapy This is to help the client stop the persisting
thoughts that are harmful to the patient.
Distorted Thinking Patterns: A few of the
• Self - Talk: Give positive messages and use
distorted thought processes are listed below: encouraging phrases to oneself.
• Overgeneralization: The individual here
draws conclusions that do not have any • Maintaing Diaries: The patient should be
explained the idea of maintain a diary, where
justifications or evidences of truth in them. For
he/ she should write down distorted
example: 'I am not a good parent as my son is
suffering from muscular dystrophy and might cognitions, emotions and the efforts to make
have inherited the disease from me". positive changes should also be jotted down.

Cognitive Thought Record Sheet used by psychologists to help the client a clear view of his
thoughts:

Where were Emotions or Negative Evidence that Evidence that Alternative Emotion or
you? Feelings automatic supports the does not Thoughts feelings
thought thoughts support the
thoughts

Where were Emotions can What What facts What Write down a How do you
you? be described thoughts support the experiences new thought feel about the
with one were going truthfulness indicate that which takes situation
What were
word. For through your of this this thought into account now? Rate 0
you doing?
example: mind? thoughts or is not the evidence to 100%
Who were Angry, sad, image? completely for and
What
you with? or scared. true all the against the
memories or
Rate the time? original
images were
emotions: thoughts.
in your mind?
o - 100%
Psychological Rehabilitation 485

Importance of cognitive Therapy for Patients


with Disability:
Patients with disability have many emotional and
behavioural problems which arise due to negative
and distorted thinking of the world and people
around them. With incurable disease or
deteriorating condition they may have negative
view of themselves, they may have feelings of
helplessness, hopelessness, may bring on death by
not working on improving their condition or giving
up easily. For all these reasons cognitive therapy is
important for patients with disability. Again it is ABCDE Model of Rational Emotive
important to keep in mind that some disabled Behaviour Therapy
people function quiet well.
Goals:
Rational Emotive Behaviour Therapy (REBT):
Rational Emotive Behaviour Therapy is a goal
History: Albert Ellis was the man who developed oriented system which focuses on outcomes such
Rational Emotive Behaviour Therapy; he was born as reduction in symptoms and changes in beliefs.
in1913 in Pittusburgh. He like Beck used to be very It helps people to learn to accept themselves and
ill i.e. nephiritis for which he used to be frequently experience greater happiness in life
hospitalized. To overcome these difficulties he
decided not to be miserable about his condition and REBT Strategies to Dispute Irrational Belief: A
maintained strong positive thoughts about his few strategies are mentioned below: [3]
competence and worth. Eventually he was very
• Empirical Disputes: This focuses on the
successful, published over 700 papers and 55 books.
accumulation of evidence.
Principal of Rational Emotive Behaviour • Rational Alternative belief: Taking up an
Therapy: alternative rational belief.
Rational Emotive Behaviour Therapies core belief • Humorous disputed: Disputing the irrational
is that although thoughts are emphasized emotions, beliefs in a lighthearted way.
thoughts and behaviours are interrelated and
• Didactic style: This is an educational,
inseparable. [2]
explanatory and efficient way in giving
appropriate information for developing
Thoughts
rational beliefs.

Importance of Rational Emotive Behaviour


Emotions Behaviours Therapy for people with Disability:
This therapy believed that an individual's thoughts,
emotions and behaviours are inter-related and if
The widely used model of Rational Emotive they have an irrational belief then it would affect
Behaviour Therapy is the ABCDEF Model: the person in his overall development. It is
A: Activating Event important for the patient to view his condition
positively and should be motivated to work upon
B: Belief which may be rational or irrational
and improve his condition; this would be only
C: Consequence of the belief possible when the negative irrational beliefs are
changed to positive rational belief.
D: Dispute i.e. questioning the belief whether it is
rational or irrational
Behaviour Therapy:
E: Effective i.e. effectively describing the outcomes History: Behaviour Therapy was first developed
during the 1950s and 1960s. Many psychologists
and physiologists have contributed to the
development of this therapy: B.F. Skinner (Operant
486 Neuro-Rehabilitation : A multi disciplinary approach

Conditioning), Ivan Pavlov (Classical • Behaviour therapy seeks to understand and


Conditioning), John Watson, Joseph Wolpe, Albert change behaviour.
Bandura (Modelling), John Dollard and Neal Miller. • Education, promoting a new learning and
[4] transfer of learning to the natural
environment.
Five Models of Behaviour Therapy:
• Focus of the therapy should be the present.
1. Applied behaviour Analysis: This was
derived by B.F. Skinner. ABA whether they Strategies of Behaviour Therapy are
are observed or taught they function on 3 incorporated in Cognitive Behaviour
aspects: Modification below:
• Antecedent (A) - what triggered a
Cognitive Behaviour Modification:
behaviour or what happens before the
behaviour, Miechenbaum in 1993 developed cognitive
behaviour modification in an effort to integrate
• Behaviour (B) - the behaviour itself, and psychodynamic and cognitive treatment system
• Consequence (C) - what happens after with the technology of behaviour therapists.
the behaviour
Three assumptions of Cognitive Behaviour
Therapy are:
1. Constructive Narrative: This suggests that
people construct their own reality and reality
is a product of personal meaning.
ABC Model of Behaviour Therapy 2. Information Processing: People experience
There is 47 % of success rate of applied behaviour negative emotions because they distort the
analysis working in Autistic Patients. reality as a result of cognitive errors and
misperception.
2. Neo - behaviorism: This takes up Ivan
Pavlov's classical conditioning theory and 3. Conditioning: Cognitions are viewed as covert
focuses on the process of conditioning and behaviours that have been conditioned
learning responses.
Strategies of Cognitive Behaviour Modification:
3. Social Learning Theory: This theory is based A few strategies are mentioned below:
on Bandura's research and this approach seeks
1. Behaviour Rehearsal: This strategy gives
to understand to interaction of cognitive,
people an opportunity to practice a
behavioural and environmental factors in
challenging task. The rehearsal might involve
shaping behaviour.
role-play, tape-recording or rehearsing in front
4. Cognitive Behaviour Therapy: This approach of the mirror. This strategy is extremely useful
looks at how cognitions shape behaviours and for people who have fear speaking in public
emotions. This treatment makes use of both or with children who lack social interaction
cognitive and behavioural strategies to effect like that seen in autistic children, etc.
change.
2. Reinforcements: The use of a reward or
5. Multimodal Therapy: This theory was reinforcement can encourage behavior change,
developed by Arnold Lazarus, which is based enhance learning and solidify gains. There are
on principles of behaviour therapy but different forms of rewards, like praises, a toy,
systematically integrates strategies from a candy, T.V time, play time, etc. For example:
wide range of approaches A child when shows desired behaviour is
praised or a star is given on his hand, or a food
Principles of Behaviour Therapy: item that he enjoys.
• Behaviour is learned and acquired largely 3. Activity Scheduling: Planning activities that
through modelling, conditioning and are rewarding and provide a sense of
reinforcement. accomplishment can help people in many
• Behaviour has a purpose. ways. Having a schedule or a time -table can
help give patients focus and direction. For
Psychological Rehabilitation 487

example: A child suffering from muscular 2. The unconscious mind is a reservoir of


dystrophy and who is not cooperative during feelings, thoughts, urges, and memories that
physical therapy is rewarded if he sticks to the outside of our conscious awareness. Most of
time-table. the contents of the unconscious are
4. Visual Imagery: This seen to be very effective unacceptable or unpleasant, such as feelings
in patients, where they are asked to visualize of pain, anxiety, or conflict. According to
the activity that is their chief goal and they Freud, the unconscious continues to influence
want to achieve. For example: A spinal cord our behavior and experience, even though we
injury patient who is unable to walk is asked are unaware of these underlying influences.[5]
to visualize and see himself walking, this
Basic system of belief:
motivates the patient and help him set goals
to achieve what he had visualized. • Id, Ego and Superego: The three parts of the
personality.
5. Aversion therapy: This therapy is based on
the principle that undesirable behaviours with • Stages of Development: Oral, anal, phallic,
a negative experience can be powerful latency period and genital.
motivator for a change in the behaviour. For • Defence mechanisms: Repression, projection,
example: Time-outs given to a child who reaction formation, fixation, regression.
throws temper tantrums.
• Dreams: Dreams are viewed as the gateway
6. Diaphragmatic Breathing: This is based on
to the unconscious.
taking slow, deep breaths and focusing on the
breathing process, this can be very calming • Catharsis: The release of pent-up emotion.
and also sleep induced. This can be helpful for • Corrective Emotional Experience: The
people with sleep disturbances especially seen therapist helps clients alter their self-
in patients with stroke and dementia. perceptions and behaviour.
Psychoanalytic Therapy: Freud formulated this
theory. Treatment Procedures:
• Therapy is long-term and focuses on exploring
According to Freud, the mind can be divided unconscious issues through interpretation,
into two main parts: dream analysis, free association, transference
and other methods. [6]
• Detachment: The therapist is detached,
objective and neutral so that the client can
project onto the therapist things from the
client's unconscious.
• Transference: Traditionally, transference is a
projection of unconscious desires onto the
therapist.
• Counter transference: These are projections
the therapist makes onto the client.

Existential Therapy:
Psychoanalytic Theory of Personality
There are many contributors to this therapy: Victor
Frankl, Rollo May, Irvin Yalom, et al,. The theory
1. The conscious mind includes everything that underlying existential therapy focuses not on the
we are aware of. This is the aspect of our treatment processes but on the universal issues that
mental processing that we can think and talk people go through. Through their understanding
about rationally. A part of this includes our of these issues, clinicians can connect with people
memory, which is not always part of at a very deep and personal level and help them
consciousness but can be retrieved easily at change their lives so that they offer more
any time and brought into our awareness. meaningful life. [7]
Freud called this ordinary memory the Existentialists believe that human condition is
preconscious.
488 Neuro-Rehabilitation : A multi disciplinary approach

difficult one. Life has no inherent meaning and is 1. Therapist-Client Psychological Contact: a
repellent with sadness and loss. They believe that relationship between client and therapist must
the following aspects of the human conditions are exist, and it must be a relationship in which
typically at the root of emotional difficulties: each person's perception of the other is
inevitability of death, existential alienation, important.
meaninglessness of life, anxiety and guilt. These are
some aspects that the existentialists have spoken 2. Client incongruence or Vulnerability: that
about and they believe in. [8] incongruence exists between the client's
experience and awareness. Furthermore, the
Goal: client is vulnerable to anxiety which motivates
The fundamental goal of existential therapy is them to stay in the relationship.
helping people find value, meaning and purpose 3. Therapist Congruence or Genuineness : the
to their lives. The purpose of psychotherapy is not therapist is congruent within the therapeutic
to cure the client but to help increase the level of relationship. The therapist is deeply involved
awareness of what they are doing and to get them him or herself - they are not "acting" - and they
out of the victim role. Existential therapists help can draw on their own experiences (self-
people confront their deepest fears and anxieties disclosure) to facilitate the relationship.
about the inevitable dimensions of life.
4. Therapist Unconditional Positive Regard
Importance of Existential Therapy for people (UPR): the therapist accepts the client
with Disability: unconditionally, without judgment,
As people who are suffering from incurable disapproval or approval. This facilitates
diseases or disorders that do not have any treatment increased self-regard in the client, as they can
option for it and after visiting many doctors and begin to become aware of experiences in which
not receiving proper amount of information or their view of self-worth was distorted by
treatment, patients usually stop making goals for others.
themselves and do not find meaning to their life. 5. Therapist Empathic understanding: the
Hence existential therapy is quiet important as it
therapist experiences an empathic
helps the patient find purpose and meaning to lives.
understanding of the client's internal frame of
reference. Accurate empathy on the part of the
Person-Centered Therapy (PCT):
therapist helps the client believe the therapist's
Person Centered Therapy is also known as person- unconditional love for them.
centered psychotherapy, person-centered
counseling, client-centered therapy and Rogerian 6. Client Perception: that the client perceives, to
psychotherapy. PCT is a form of talk- at least a minimal degree, the therapist's UPR
psychotherapy developed by psychologist Carl and empathic understanding.
Rogers in the 1940s and 1950s. The goal of PCT is
to provide patients with an opportunity to develop Transpersonal Psychology:
a sense of self wherein they can realize how their The word "transpersonal" comes from the Latin
attitudes, feelings and behaviour are being "trans," meaning beyond and through, and
negatively affected and make an effort to find their "persona," meaning mask or personality. This is a
true positive potential. [9] In this technique, school of psychology that studies the transpersonal,
therapists create a comfortable, non-judgmental self-transcendent or spiritual aspects of the human
environment by demonstrating congruence experience. A short definition from the Journal of
(genuineness), empathy, and unconditional positive Transpersonal Psychology suggests that
regard toward their patients while using a non- transpersonal psychology "is concerned with the
directive approach. This aids patients in finding study of humanity's highest potential, and with the
their own solutions to their problems. recognition, understanding, and realization of
unitive, spiritual, and transcendent states of
The core concepts of Person Centered Therapy: consciousness" [10] Issues considered in
Rogers (1957; 1959) stated that there are six transpersonal psychology include spiritual self-
necessary and sufficient conditions required for development, self beyond the ego, peak
therapeutic change: experiences, mystical experiences, systemic trance
and other sublime and/or unusually expanded
Psychological Rehabilitation 489

experiences of living. This therapy is commonly Group Therapy:


used to help patients with depression. The term group therapy, however, was first used
around 1920 by Jacob L. Moreno, whose main
Family Therapy: contribution was the development of psychodrama,
Family therapy, also referred to as couple and in which groups were used as both cast and
family therapy, family systems therapy, and family audience for the exploration of individual problems
counseling, is a branch of psychotherapy that works by re-enactment under the direction of the leader.
with families and couples in intimate relationships Group psychotherapy, like individual
to nurture change and development. It tends to psychotherapy, is intended to help people who
view change in terms of the systems of interaction would like to improve their ability to cope with
between family members. It emphasizes family difficulties and problems in their lives. But, while
relationships as an important factor in in individual therapy the patient meets with only
psychological health. one person (the therapist), in group therapy the
meeting is with a whole group and one or two
Family therapy uses a range of counseling and therapists. Group therapy focuses on interpersonal
other techniques including: interactions, so relationship problems are addressed
• Communication theory well in groups.
• Media and communications psychology
Goal of Group Psychotherapy:
• Psychoeducation The aim of group psychotherapy is to help with
• Psychotherapy solving the emotional difficulties and to encourage
the personal development of the participants in the
• Relationship education group. The therapist (called conductor, leader or
• Systemic coaching facilitator) chooses as candidates for the group
people who can benefit from this kind of therapy
• Systems theory
and those who may have a useful influence on other
• Reality therapy members in the group.
• Attachment-focused family therapy

Family Therapy session


The number of sessions depends on the situation, Group Therapy Session
but the average is 5-20 sessions. A family therapist
usually meets several members of the family at the Different types of Groups:
same time. This has the advantage of making There are many kinds of groups in the group-
differences between the ways family members psychotherapy field. The techniques used in group
perceive mutual relations as well as interaction therapy can be verbal, expressive, psychodramatic
patterns in the session apparent both for the etc. The approaches can vary from psychoanalytic
therapist and the family. These patterns frequently to behavioral, Gestalt or encounter groups. Groups
mirror habitual interaction patterns at home, even vary from classic psychotherapy groups, where
though the therapist is now incorporated into the process is emphasized, to psychoeducational,
family system. which are closer to a class. Psychoeducational
490 Neuro-Rehabilitation : A multi disciplinary approach

groups usually focus on the most common areas of 3. Explore various aspects of self in relation to
concern, notably relationships, anger, stress- others
management etc. They are frequently more time-
4. Develop the capacity for interpersonal
limited (10 to 15 sessions) and thus very appealing
intimacy
in a managed care environment. Each approach has
its advantages and drawbacks, and the participant 5. Develop group skills
should consult the expert which technique matches 6. Develop creativity, expressive freedom, and
her/his unique personality. playfulness with various degrees of structure
Benefits: 7. Stimulate and develop the senses
Group psychotherapy is suitable for a large variety 8. Play, on the spot, with a decisiveness that
of problems and difficulties, beginning with people invites clarity of intention
who would like to develop their interpersonal skills
and ending with people with emotional problems 9. Develop perceptual and cognitive skills
like anxiety, depression, etc. There are support
groups for people in the same situation or crisis
(e.g. groups for bereaved parents, groups for
patients with various disability, etc). Groups are
ideally suited to people who are struggling with
relationship issues, disability, caregiving, etc. The
groups interactions help the participants to identify,
get feedback, and change the patterns that are
sabotaging the relations. The great advantage of
group psychotherapy is working on these patterns
in the "here and now" - in a group situation more
similar to reality and close to the interpersonal
events.

Clinical Music Therapy:


Music Therapy
In music therapy improvisation is defined as a
process where the client and therapist relate to each Therapeutic Techniques:
other. The client makes up music, musical
• Imitating is a basic technique of empathy in
improvisation, while singing or playing,
which the music therapist copies or repeats a
extemporaneously creating a melody, rhythm,
client's response, after the response has been
song, or instrumental piece. In clinical
displayed. The music therapist focuses on any
improvisation, client and therapist (or client and
sound, rhythm, interval or even facial
other clients) relate to one another through the
expression.
music. Improvisation may occur individually, in a
duet, or in a group. The client may use any musical • Reflecting is a technique in which the music
or nonmusical medium within his or her therapist expresses the same moods or feelings
capabilities. Musical media includes voice, body which have been presented by the client.
sound, percussion, and string, wind, and keyboard • Rhythmic grounding is implemented by
instruments. Nonmusical media can consist of establishing a steady beat or rhythm,
images, titles, and stories. supporting the client's improvisation. The use
of a rhythmic ostinato is an example of
Goals of Music Therapy :
rhythmic grounding.
According to Bruscia (1998), clinical goals that can
• Dialoguing is a process in which the music
be achieved through improvisation are as follows:
therapist and the client communicate through
1. Establish a nonverbal channel of their improvisations. Lastly, accompanying is
communication, and a bridge to verbal a technique in which the music therapist
communication supports the client's improvisation by giving
2. Provide a fulfilling means of self-expression an accompaniment that consists of rhythm,
and identity formation melody, and chord progressions.
Psychological Rehabilitation 491

Clinical Music Therapy is extremely useful for Attributes of Child and Counsellor
people with various diseases and disorders like Relationship:
autism, learning difficulties, dementia, emotional • Exclusive: The counsellor should build a good
and psychological problems, etc. rapport with the client and the child should
experience a unique relationship with the
COUNSELING CHILDREN:
counsellor which is not comprised by
The goals of the session are very important to be unwanted intrusion of others such as parents
listed when working with children. or siblings.
• Safe: The counsellor should create a permissive
There are 4 levels of goals:
environment in which the child feels free to
Level 1: Fundamental goals: These goals are act out and to gain mastery over the feelings
applicable to all children in therapy. in safety. The child should feel safe to make
• To enable the child to feel good about himself disclosures with confidence that doing so will
or herself. not have repercussions or consequences which
• To enable the child to deal with painful may be emotionally harmful or damaging.
emotional issues. • Connecting Link: The child and counsellor
• To enable the child to achieve some level of relationship should be a connecting link
congruence in regards to thoughts, emotions between the child's world and the counsellor.
and behaviours. The focus of the relationship is primarily about
connecting with the child and staying with the
• To enable the child to change behaviours, that
child's perceptions. The child may see the
have negative consequences.
environment in which he lives quiet differently
• To enable the child to accept his or her from the way in which his parents see his
limitations and strengths and to feel OK with environment.
the. • Authentic: The child and counsellor
Level 2: Parents goals: These goals are set by the relationship should be authentic i.e. it should
parents who bring their children for therapy be genuine and honest. The authentic
session. They are usually related to the parent's relationship allows the child to give up the
agendas and based on the child's current behaviour. pretence of being someone she is not and to
For example if the child throws temper tantrums allow raw inner self to be exposed.
and is very aggressive then the goal of the parent • Confidential: When working with the children
would be either to reduce or extinguish this the counsellor tries to create an environment
negative behaviour. where the child feels safe enough to share very
private thoughts and emotional feelings. In
Level 3: Goals formulated by the psychologist:
order for the child to feel safe there should be
These goals are formulated by the counselor as a
some amount of confidentiality that is
consequence of hypothesis which the counselor
required. There would be times when the child
may have about why the child is behaving in a
would share information with the counsellor
particular way. The counselor may have goals of
with the counsellor believes needs to be shared
addressing and resolving the child's emotional
with others, for example in case of a child
issues.
abuse or sexual abuse.
Level 4: The child's goals: These goals emerge • Non - Intrusive: When working with children
during the therapy session and are effectively the counsellor needs to join with the child in a
formulated as the child's own goals although the way which is comfortable for the child. Some
child wouldn't be able to verbalize them. If a counsellors believe in questioning the child
counsellor goes into a session with a specific and inquiring about the child's family and
agenda, there are many times when sticking to the background, during the joining processes
agenda will be effective and appropriate. But there which is a useful way of getting to know the
is generally a danger of holding rigidly to a pre- child's world. However asking to many
determined agenda because the child's own needs questions could scare the child as he or she
might be overlooked rather than addressed. may feel intrusion into his or her personal
space.
492 Neuro-Rehabilitation : A multi disciplinary approach

• Purposeful: Children enter into a therapeutic as a means for developing problem-solving skills,
process more willingly and confidently if they ways to relate to others, expressing their feelings,
know exactly why they are coming to see the and working on their behaviours, all at a safe
counsellor. They need time to prepare psychological distance from reality.
themselves for counseling and will usually do
so if given prior suitable notice and if told of Furniture and Associated Items:
the reason as to why they are being brought
for the counseling session.

OTHER FORMS OF THERAPIES FOR


CHILDREN:

Play Therapy:
If play is a child's language, then toys can be thought
of as the words. Through play therapy the child
can work through their challenges and issues using
the toys that they choose, revealing their inner
dialogue. Through play the child is able to test out Play Therapy Room
various situations and behaviours in a supportive Toys: Dolls, doll house, doll clothes, doll family,
environment. Unconditional positive regard and telephones, mirrors, play money, kitchen set, child
acceptance encourages the child to feel safe enough chairs and bean bags, etc.
to be able to explore their inner selves without
censorship. In this environment children are able Equipments and Materials: Sand tray, clay, play
to try out different roles, work through conflicting dough, papers, pencils, paints, crayons, glue,
emotions and thoughts, and try to figure out what scissors, wool, coloured papers, wooden blocks,
the world is like. The child is able to form a farm animals, zoo animals, etc.
relationship with the provider, and through this Dress up Materials: Variety of clothes, jewellery,
relationship they are able to develop trust, wigs, swords, doctor's set, masks, etc.
improved self-esteem, and self efficacy.
Games: Card games, board games, etc.
Books: Colouring books, drawing books, story
books and worksheets, etc.

Art Therapy:
Art therapy for children can provide kids with an
easier way to express themselves since children are
more naturally artistic and creative. A young child
is likely to be more comfortable initially expressing
him/herself with some crayons and markers, for
example, than he/she is going to be at expressing
emotions and feelings through words. A question
and answer type of format can be daunting and
Play Therapy Session intimidating for a child, especially when they have
In non-directive play therapy, the child is in control, to try and explain themselves with their already
within some gently but firmly set limits. Children limited vocabulary. Because of this, art therapy for
often feel that they do not have control over children can be a much more viable solution for
situations in their lives. Through play therapy they communication than simply having a conversation
are able to work through these experiences in an and talking about things. This can be especially true
environment that they are able to control. They can when it comes to children and traumatic events.
make the story be how they want it to be, they are If a child experiences something tragic, that event
in charge of the outcome. This feeling of control is usually gets buried in their subconscious where it
vital to their emotional development as well as affects them in the future. These types of things are
positive mental health. Children are able to use play not easy for kids to talk about, especially when there
Psychological Rehabilitation 493

are deep-rooted emotional issues in play. Through • I sometimes get angry when I think about the
art therapy children can help bring these death. This is my picture of anger.
suppressed emotions to the surface so the art
• These are the things I liked the most about
therapist can then focus on healing the child's
(deceased).
issue(s). Art therapy can also aid a child in achieving
better self-awareness, relief from stress or anxiety, • I get scared when I think of ____________. This
learning disorders, autism, and other traumatic picture shows my fear.
experiences.

Art Therapy Children Supplies:


1. Crayons
2. Pastels
REFERENCES:
3. Colored pencils
1. Robertson, D (2010). The Philosophy of
4. Set of brightly colored markers Cognitive-Behavioural Therapy: Stoicism as
5. Tempera paints and/or watercolor set Rational and Cognitive Psychotherapy.
London: Karnac.
6. Spiral bound pads of art paper/ sketch pads
2. Ellis, A. (2004) Rational Emotive Behavior
7. Scissors and glue
Therapy: It Works for Me--It Can Work for
8. Old magazines You. Amherst, NY: Prometheus Books.
3. Beck, A. (1970). Cognitive therapy: Nature and
And for Younger Children:
relation to behavior therapy. Behavior
Finger paint set Therapy, 1(2), 184-200
Play-Dough set 4. Wolpe, J. & Lazarus, A. (1966) Behavior
Therapy Techniques: A Guide to the
Treatment of Neuroses, pp. 1-2.
5. Brenner, Charles (1954). An Elementary
Textbook of Psychoanalysis.
6. Elliott, Anthony (2002). Psychoanalytic
Theory: An Introduction, Second Edition,
Duke University Press.
7. Yalom, Irvin D.1980.,; Existential
Psychotherapy; Basic Books.
8. Deurzen, E. van, and Kenward, R. (2005)
Dictionary of Existential Psychotherapy and
Counseling, London: Sage Publications.
9. Cepeda, Lisa M.; Davenport, Donna S. (2006).
"Person-Centered Therapy and Solution-
Focused Brief Therapy: An Integration of
Present and Future Awareness".
Psychotherapy: Theory, Research, Practice,
Art Therapy Training (Educational Publishing Foundation)
43 (1): 1-12.
Strategies of Art Therapy: 10. Aziz, Robert (2008). "Foreword". In Storm,
• I am the most sad when I remember_________. Lance. Synchronicity: Multiple Perspectives on
This is my picture of feeling sad. Meaningful Coincidence. Pari Publishing.
494 Neuro-Rehabilitation : A multi disciplinary approach

Ch.15 Speech Rehabilitation


Dr. Manasi Jani (Speech Therapist).

One of the features that put a human being apart • Impaired pragmatics - this includes affected
from other animals is the ability to communicate. social interaction, irrelevant answers to
Human beings communicate through various questions, lack of humour and inability to
modes or channels called modalities. The most understand metaphorical elements.
commonly used modality to communicate is verbal • Impaired swallowing functions - this
modality or spoken modality. Speech is a result of category of symptoms have a variety of
co-ordination between different systems in the body presentations depending on the stage of
namely respiration, phonation, resonance and swallowing affected. The stages of swallowing
articulation. The organs which actively participate are the oral, pharyngeal and esophageal phase.
in speech production are the lungs, the larynx (voice Problems like affected lip seal, poor tongue
box), the vocal tract, the lips, the tongue, the soft control, inability to chew properly affect the
palate and all the sinuses. Also, hearing is an oral phase of swallowing. Problems like
important sensory organ which has direct delayed or absent swallow reflex, multiple
connection to speech production and perception. swallows needed to swallow a single bolus of
An audiologist and speech language pathologist is food are included in the pharyngeal phase.
a professional who deals with diagnosis and Regurgitation of food after swallowing is
treatment of disorders related to speech and included in the esophageal phase of
hearing. swallowing.
Hearing, speech and language defects can arise due • Affected speech clarity due to imprecise
to various neurological problems. Neurological sound production - this includes substitution
disorders that lead to speech and language or inability to produce a certain phoneme or
disorders can be classified into congenital and sound appropriately.
acquired causes. Acquired causes can be classified • Hypernasality.
in to static and progressive disorder. The main
• Affected loudness, pitch and quality of voice
causes of congenital neurological disorders are
- every person has a certain speaking pitch.
cerebral palsy, muscular dystrophy, autism, viral
Neurological disorders have a drastic effect on
encephalitis and mental retardation. Acquired static
the vocal system too. They can result in
causes can be due to stroke, post encephalitis, head
reduction in the loudness of voice, change in
injury while the progressive causes are ALS,
the pitch, harshness or hoarseness of voice.
multiple sclerosis, muscular dystrophy, parkinson's
disease to name a few. • Monotonous speech.
• Affected oromotor structures and functions-
The commonest problems seen in children and affected lip seal, drooling, affected tongue
adults with a neurological defect are: control, and affected palatal movements.
• Impaired comprehension - this includes
problems like inablility to understand concepts Role of A Speech Therapist as
which normal children or adults of that age A Swallowing Therapist:
can. It also includes problems like inability to Swallowing being in a vegetative function is
follow simple commands like 'close your eyes' extremely crucial and requires immediate attention.
or can you show me your nose?'. The long term effects of swallowing problems can
• Impaired expression - this includes inability pose detrimental consequences like aspiration
to verbally express. The patient may speak a pneumonia and malnourishment. What may look
few words to ask for needs or may just use like a mild coughing and choking could have
pointing to express themselves. Sometimes, it serious implications. Swallowing problems can
is observed that the patients use words which occur in any age group. Hence, it is of vital
do not exist in the language and are importance that these problems be tackled under
meaningless. These are called jargon speech. strict supervision of a qualified speech therapist.
Speech Rehabilitation 495

Role of A Speech Therapist as complete attention to the task and hearing the
A Voice Therapist: therapist correctly. It is always advised to
initiate with simple words and few distracters
Larynx or the voice box is an important system in and then include complex and indirect
speech production. Hence, speech therapists play comprehension tasks with multiple distracters.
an important role in optimizing the vocal
4) Complete loss of speech or restricted speech
parameters. Neurological disorders affect the
is seen in children with delayed speech and
nerves supplying the larynx thereby affecting the
language, autism, cerebral palsy and
function of the vocal cords. These can be corrected
individuals with stroke, or head injury. While
by certain breathing exercises, vocal function
dealing with children with speech and
techniques or surgery in case speech therapy
language problems, it is always advisable to
doesn't work. Also, voice therapy should be
work on language in the most naturalistic way
considered before considering any patient for a
possible as that facilitates better generalization
phonosurgery or surgery to improve voice because
of abstract concepts. Using flash cards and real
the efficacy of voice therapy in neurological voice
objects to elicit responses is the widely used
problems like vocal cord palsy is excellent.
method to teach language.
Speech Therapy: The child can be shown a card or an object and
asked "what is this"?. If the child responds
1) Oromotor and PNF exercises are exercises
appropriately, the clinician target to achieve
involving stimulation of jaws, lips, cheeks,
elaborate expression for eg response from a
tongue and palate. The speech therapist aims
ball to this is a red round ball. If the child is
to improve the oral musculature and attain
unable to respond at all, the clinician acts as a
optimal function. The adequate function of
model and the child repeats after the clinician.
oral functions is important for swallowing and
This technique is called modelling. Also,
speech functions.
expansion and extension of responses can be
2) Hearing defects are seen in many children used to achieve more specific and detailed
with cerebral palsy especially athetoid cerebral responses. More opportunities should be
palsy children and in adults with peripheral provided to the children to use the learnt
neuropathy. These disorders have to be target.
diagnosed for the degree and type of hearing
In adults with expressive deficits, various
loss and differentiate a temporary hearing loss
techniques like word retrieval tasks, melodic
from a permanent one. The diagnosis is made
intonation technique etc can be used
using a routine test like pure tone audiometry
depending on the baseline evaluation. Many
(PTA) or brain stem evoked responses (BERA)
a times when a patient presents with severe
in children who cannot respond to the heard
expressive deficits, the functional
sounds. A sensorineural hearing loss or a
communication should be considered.
hearing loss affecting the inner ear is treated
by fitting proper hearing aid and check for 5) Many of the children with cerebral palsy,
auditory perception and discrimination. autism, head injury and adults with stroke,
parkinson's disease, ALS, multiple sclerosis,
3) Comprehension deficits are seen in children
muscular dystrophy present with affected
with mental retardation, autism, cerebral palsy
speech intelligibility called dysarthria.
and adults after a stroke or head injury. In
these patients the main aim is to achieve near Typical signs seen are imprecise or weak
normal comprehensive skills. By pointing consonants, slurring, reduced loudness of
tasks like pointing to flash cards, or answering voice and inability to speak many words in
riddles, the patient can be taught to one breath to name a few.
understand the skills which the patient has not The speech therapist aims to achieve near
achieved or has lost due to the disorder normal speech intelligibility within patient's
causing it. impairment limits. Also, working on the
While working on comprehension, it is monotony in speech and stress patterns,
necessary to ensure that the patient is giving naturalness in speech can be achieved.
496 Neuro-Rehabilitation : A multi disciplinary approach

Augmentive and Alternative lesion and the stage affected is crucial as the
treatment plan changes according to the stage
Communication in Neurological
affected.
Patients:
A patient having an oral stage of swallowing
In our daily clinical practise, we come across very affected has to focus more on strengthening his
severely affected patients or patients with poor oromotor areas and achieve optimal functioning in
prognosis in speech production. Also, when we order to manipulate the bolus in the mouth and
encounter patients with progressive neurological achieve proper chewing. Certain manoeuvres like
conditions like motor neuron disorders, multiple supraglottic swallow; effortful swallow, Shaker's
sclerosis, dementia etc, a long term solution should exercise and Mendelson's manoeuvre are helpful.
be kept in mind. Therefore, a regular follow up with
the speech therapist and evaluation of the Few bodily postural changes like chin back, chin
deterioration is necessary. tuck and head tilts as recommended by the speech
therapist is very helpful. Also, many a times
In patients with a chronic brain damage or severe patients find it easier to swallow a certain
level of deterioration, verbal communication cannot consistency like liquids or solids better than the
be solely depended upon. In such cases, an other. Hence, bolus modification which suits the
alternated mode of communication or another patients and which also fulfils the nutritional needs
mode which will augment the current verbal of the patient can be made by the speech therapist.
production should be considered. According to the
strengths of the patient i.e patient's level of At every point of time in the treatment, airway
understanding, patient's level of performance and safety has to be monitored closely and chances of
motivation, the appropriate mode of silent aspiration and aspiration pneumonia should
communication can be selected. Using drawing be minimized. In patients having a chronic or long
board, using pantomime or using computer assisted standing dysphagia, an enteral feeding like
technologies are the widely used systems today. nasogastric tube or peritoneal esophageal
Also, for severely affected individuals like patients gastrostomy can be considered along with oral trial
in vegetative state, head pointers or eye blinks to feeds.
indicate needs are used. In patients with severe Any neurological condition having bulbar
dysarthria, a specialized system which converts symptoms reveals speech, language and cognitive
typed words in to spoken signals is advised. Hence, deficits as one of the first indications. Therefore,
along with technological advancement, the function the role of a speech language pathologist diversifies
of AAC has gained momentum. in to a that of a voice therapists, hearing specialist
and swallowing therapist apart from a traditional
Swallowing Therapy: speech therapist.
This is one of the most critical areas a speech
therapist works into. Diagnosis in terms of site of
An Overview on Stem Cells and Stem Cell Therapy 497

Section 5
An Overview on Stem Cells and
Stem Cell Therapy
Ch.16 An Overview on Stem Cells and
Stem Cell Therapy
Dr. Prerna Badhe, M.D., Dr. Nandini Gokulchandran, M.D, Dr. Guneet Chopra, M.B.B.S, (PGDM),
Ms. Pooja Kulkarni, M.S. (Biotechnology), Dr. Alok Sharma, M.S., [Link].

An Overview on Stem Cells and endometrium, neural stem cells, etc., which have
Stem Cell Therapy varying potencies for differentiating into different
cell types. A body of work has been ongoing on the
Regenerative medicine is a newly evolving branch use of these cells, in various specialties and
of modern medicine that deals with cell based disorders.
therapies which use healthy cells cultured in the
The nervous system is like the central processing
laboratory to replace damaged cells in adult
unit of the animal body. In humans, it is more
organisms to treat disease. This could therefore
evolved and specialized. Since, disorders and
potentially hold the key for addressing ailments
injuries affecting the nervous system lead to
which currently have no proven treatments or
irreparable damage and disability, this area has
cures, such as, neurological disorders (spinal cord
become a major focus point in the arena of
injury, cerebral palsy, brain stroke, muscular
regenerative medicine. The hope is that by using
dystrophy, Alzheimer's disease, multiple sclerosis,
the plasticity of the nervous system and combining
etc.), diabetes, cardiovascular disorders, bone
it with the regenerative potential of the stem cells
disorders, hematopoietic disorders, cancers,
it would be possible to evolve definitive treatments
hepatic, renal and dermatological disorders.
for degenerative and traumatic disorders of the
One of the building blocks of this therapy is stem nervous system.
cells. Regenerative medicine aims to repair or re-
grow parts or tissues which are lost as a Basics of Stem Cells
consequence of disease or injury. Stem cells have
Every cell in the human body can be traced back to
the capability to multiply manifolds and convert
a fertilized egg that came into existence from the
or differentiate into any specialized cell types of
union of the egg and the sperm. The body is made
the body. Hence, the potential of these invaluable
up of over 200 different types of cells. All of these
assets could even be projected as far as, sometime
come from a pool of stem cells in the early embryo.
in the near future, to replace organ transplantation.
During early development as well as later in life,
Depending on the source, the potency or plasticity the stem cells give rise to the specialized or
of stem cells varies. Stem cells procured from the differentiated cells that make up our body. Over
5-6 day embryo (usually from wasted or excess the past 2 decades scientists have been gradually
fertilized embryos from IVF clinic), referred to as deciphering the processes by which unspecialized
embryonic stem cells, have theoretically the stem cells become the different types of specialized
capacity to give rise to the whole embryo and cells stem cells. Stem cells can regenerate themselves or
of all the germ layers (pluripotent).However, they produce specialized cell types. This is the property
are surrounded by hordes of ethical issues that makes them appealing as a method for creating
regarding the source of these cells. Also, formation medical treatment that can replace lost or damaged
of "teratomas" is a serious possibility in the long- cells. In this chapter we will look at some of the
term with these cells. fundamental basic properties of Stem cells.
In order to bypass the ethical and medical issues
What Are Stem Cells?
associated with embryonic and fetal stem cells,
researchers and clinicians have researched and A stem cell is defined by two properties. First, it is
developed other sources of stem cells, such as a cell that can divide indefinitely, producing a
haematopoietic and mesenchymal stem cells from population of identical offspring. Second, stem cells
the bone marrow and umbilical cord, stem cells can, on cue, undergo an asymmetric division to
from the adipose tissue, olfactory ensheathing, produce two dissimilar daughter cells. One is
500 Neuro-Rehabilitation : A multi disciplinary approach

identical to the parent and continues to contribute Classification of Stem Cells


to the original stem cell line. The other varies in
some way. This cell contains a different set of Stem cells are classified as embryonic stem cells,
genetic instructions (resulting in an alternative umbilical cord stem cells and adult stem cells on
pattern of gene expression) and is characterized by the basis of their origin.
a reduced proliferative capacity and more restricted Embryonic Stem cells:
developmental potential than its parent. Eventually
Embryonic stem cells are pluripotent in nature
a stem cell becomes known as a "progenitor" or
which are derived from the inner cell mass (ICM)
"precursor" cell, committed to producing one or a
of 5 to 7 day blastocyst, obtained from IVF clinics.
few terminally differentiated cells such as neurons
(3)
or muscle cells. (1)
Developmental studies in mouse revealed that the
Developmental Hierarchy in Stem Cell (SC) fertilized oocyte, the zygote, has the capacity to
Compartment: form the whole embryo. It further divides
There exists a hierarchy in the stem cell progressively to give rise to an 8 cell staged, 16
compartment, depending on their 'potency' or fate celled, 32 celled blastomere and then finally the
restriction.1) Totipotent stem cells give rise to blastocyst.
embryonic as well as the extra embryonic tissue. The blastocyst is demarcated into the outer
This means, it has the capacity to form the whole transparent trophoblast (which forms the extra
of the embryo, including the placenta. The embryonic tissue/the placenta) and the Inner cell
physiological totipotent stem cell is a fertilized mass (ICM) which is a 30-34 celled clump.
oocyte (zygote) or first blastomere which comprises The ICM ultimately gives rise to the three germ
of the 8 cell stage. The artificial counterpart is a layers and subsequently the whole embryo. Hence,
clonote obtained by somatic cell nuclear transfer the inner cell mass is the source for the derivation
(SCNT) to an enucleated oocyte.2) Pluripotent stem of the embryonic stem cells, which has lost the
cells in turn have the capacity to give rise to cells of "totipotency" of the zygote, but is now
all the three germ layers of the embryo, i.e., "pluripotent".
endoderm, mesoderm and the ectoderm.
The potential of the embryonic stem cell to form
Pluripotent stem cells are cells from the inner cell
the "germ layers" & its capacity to self renew
mass of the blastocyst (ICM), epiblast (EPSC) and
indefinitely as well as its ability to form any cell
SC obtained as immortalized cell lines - blastocyst type of the body, has led to opening up of this field
derived embryonic stem cells (ES) and Primordial widely, not only with respects to its use in
Germ Cell-derived embryonic germ cells (EG). 3) regeneration, but has thrown up debates regarding
Multipotent stem cells give rise to cells of one of ethics and legalities.
the germ cell layers only, either ecto-, meso- or
However, even before the first embryonic stem cell
endoderm. Sources range from 8 day old embryo
line was derived in 1981, embryonal carcinoma cells
to adult bone marrow. 4) Monopotent stem cells
derived from germline tumors called
are tissue-committed stem cells that give rise to cells
"teratocarcinomas" were widely studied. After
of one lineage, e.g., hematopoietic stem cells,
transplantation to extra-uterine sites of appropriate
epidermal stem cells, intestinal epithelium stem mouse strains, these "funny little tumors" produced
cells, neural stem cells, liver stem cells or skeletal benign teratomas or malignant teratocarcinomas.
muscle stem cells. (2) (4)
Though the above classification has evolved over
decades, understanding of the potency of these cells Uses of Embryonic Stem Cells:
are everchanging. Many of these cells, which were 1. Embryonic stem cells as cellular models
earlier considered to be multipotent, have shown
Experiments designed to understand gene function
limited pluripotent properties. Also, transdifferen-
in the context of an organism require genetic
tation of monopotent/unipotent cells by external
strategies. Enhancer and promoter traps, gene
stimulation or manipulation have shown that these traps, random activation of gene expression (RAGE)
classifications, based on fate restriction or potency, and genome-wide cell-based knockout (GECKO)
are fast becoming redundant. represent genome-wide strategies to identify,
An Overview on Stem Cells and Stem Cell Therapy 501

isolate, or determine gene function. Because of physiological way. Finally, assurances against the
gene-targeting techniques, transgenic mice have formation of ES cell-derived tumors and donor/
also proven critical to the creation and evaluation recipient immunocompatibility are additional
of some models of human disease. Embryonic stem requirements of stem cell-based therapies. As
cell lines have proven to be useful mediums for pointed out, significant progress has been made in
genetic manipulation, for understanding the isolation of defined cell lineages in mouse, and
developmental processes and correction of genetic important advances have already been seen with
defects. (5) hES cells. Before therapeutically applicable, any ES-
based treatment must, however, show limited
2. Embryonic stem cells in pharmacology and potentials for toxicity, immunological rejection, or
embryotoxicology tumor formation, and at present, human ES cell
Stem cells also represent a dynamic system suitable research has not reached this threshold.
to the identification of new molecular targets and The availability of human ES cells, however,
the development of novel drugs, which can be represents an extraordinary opportunity for cell
tested in vitro for safety or to predict or anticipate transplantation that may be applicable to a wide
potential toxicity in humans. (6) range of human ailments. Three properties make
Human ES cell lines may, therefore, prove clinically ES cells relative to adult stem cells very attractive
relevant to the development of safer and more for replacement therapies.1) Human ES cells can
effective drugs for human diseases. Three aspects be grown indefinitely in culture.2) ES cells can be
are relevant to this issue. 1) At present, insufficient genetically manipulated, and loss of function genes
methods exist in some areas of in vitro toxicology (e.g., CTFR) can theoretically be repaired by the
to predict target organ toxicity. 2) In introduction of transgenes into ES cells either by
embryotoxicology, interspecies variation random transgenesis or through gene targeting. 3)
complicates data analysis, and human cell systems Numerous differentiation protocols have already
may enhance the identification of hazardous been established that permit the generation of
chemicals. 3) Human ES-derived cells cultured in almost any cell type, either through the use of
vitro may reduce the need for animal testing in established culture conditions or when coupled
pharmacotoxicology. with genetic manipulations. In theory, hES cells
could be applied to a wide range of human
The application of hES cells in pharmacology and ailments, but the proof of principle has largely come
embryotoxicology could have a direct impact on from the use of mouse ES cells.(7-8)
medical research, but to date, such an approach has
primarily been used with mouse ES cells.
Adult Stem Cells
3. In stem cell based therapies: Adult stem cells are pluripotent, clonogenic, self
The in vitro developmental potential and the renewing, having ability to differentiate into the
success of ES cells in animal models demonstrate mature cell of it resident environment and also, may
the principle of using hES-derived cells as a have transdifferentiating abilities.
regenerative source for transplantation therapies Adult stem cell niches have been found in most
of human diseases. Before transfer of ES-derived organs of the human body,
cells to humans can proceed, a number of
experimental obstacles must be overcome. These eg. liver, brain, bone marrow, adipose tissue, heart,
include efficient derivation of human ES cells in the etc. The primary role of these adult stem cells is
absence of mouse feeder cells, and an initiation of repair process in the organ following
understanding of genetic and epigenetic changes an injury. These cells have been, in practicality,
that occur with in vitro cultivation. It will be difficult to obtain due to the following reasons:
necessary to purify defined cell lineages, perhaps 1) Inaccessibility and small numbers (e.g. neural
following genetic manipulation, that are suitable stem cells)
for cell-based therapies. If manipulated, then it will
2) Lack to markers for characterization and
be important to guard against karyotypic changes
isolation of the "stem cell population" from
during passaging and preparation of genetically
various organs.(9)
modified ES-derived cells. Once introduced into the
tissue, the cells must function in a normal The field of Regenerative medicine, which got
502 Neuro-Rehabilitation : A multi disciplinary approach

opened up widely following the discovery of the mesenchymal stromal cells instead. (10)
embryonic stem cells, is now in search of the 2) Multipotent Adult Progenitor Cells (MAPC):
"almighty" pluripotent stem cell, following ethical,
legal and medical questions raised against the ES MAPC are isolated from BM as well from various
cell research and therapeutic use. adult organs as a population of CD45 GPA-A-
adherent cells and they display a similar fibroblastic
The search has now been directed towards adult morphology to MSC. Interestingly MAPC are the
stem cell niches, which pose a non controversial only population of BM derived stem cells that have
and safe option for use in human subjects. been reported to contribute to all three germ layers
However, the debate over its pluripotency is after injection into a developing blastocyst,
ongoing and the fields as well as the concept of indicating their pluripotency. (11) The contribution
adult stem cell plasticity have been extremely of MAPC to blastocyst development, however,
dynamic. requires confirmation by other, independent
laboratories
Bone Marrow Derived Cells
3) Marrow-isolated adult multilineage
Bone marrow is the most accessible and most inducible (MIAMI) cells:
studied source of adult stem cells. Different types
This population of cells was isolated from human
of stem cells have been found to be present in the
adult BM by culturing BM MNC in low oxygen
bone marrow, which differ in their potential to
tension conditions on fibronectin . MIAMI cells
differentiate and form cells from one or more germ
were isolated from the BM of people ranging from
layers.
3- to 72-years old. Colonies derived from MIAMI
Initially, the bone marrow was thought to contain cells expressed several markers for cells from all
only haematopoietic stem cells. The excitement three germ layers, suggesting that, at least as
regarding HSCs diminished after it was found to determined by in vitro assays, they are endowed
have limited potency. However, increasingly, with pluripotency. However, these cells have not
evidence is pouring in regarding the heterogenous been tested so far for their ability to complete
population of cells having varying plasticity. blastocyst development. The potential relationship
Potential Pluripotent Stem Cells candidates of these cells to MSC and MAPC is not clear,
identified in adult tissues (especially, bone marrow) although it is possible that these are overlapping
populations of cells identified by slightly different
1) Mesenchymal Stem Cells (Multipotent isolation/expansion strategies
Mesenchymal Stromal Cells):
Human mesenchymal stem cells (MSCs) are 4) Multipotent Adult Stem Cells (MACS):
thought to be multipotent cells that have the These cells express pluripotent-state-specific
potential to differentiate into multiple lineages transcription factors (Oct-4, Nanog and Rex1) and
including bone, cartilage, muscle, tendon, ligament were cloned from human liver, heart and BM-
fat and a variety of other connective tissues. Indeed, isolated mononuclear cells. MACS display a high
marrow-derived cells seem to retain a remarkable telomerase activity and exhibit a wide range of
plasticity, since they have much wider differentiation potential. Again the potential
differentiation potential than previously thought. relationship of these cells to MSC,MAPC and
Marrow cells have been reported to contribute to MIAMI described above is not clear, although it is
angiogenesis, somatic muscle development, liver possible that these are overlapping populations of
regeneration, and the formation of central nervous
cells identified by slightly different isolation/
system cell types. It is likely that MSC may be
expansion strategies.
contaminated by other populations of primitive
non-hematopoietic stem cells. This possibility
5) Very Small Embryonic Like (VSEL)
should be considered whenever a
Stem Cells:
"transdedifferentiation" of MSC into cells from
other germ layers is demonstrated. Because various Recently, a homogenous population of rare (~0.01%
inconsistencies have come to light in the field of of BM MNC) Sca-1+ lin- CD45- cells was identified
MSC research, the International Society for Cellular in murine BM. They express (as determined by RQ-
Therapy recently recommended avoiding the name PCR and immunhistochemistry) markers of
of MSC stem cells and changing it to multipotent pluripotent stem cells such as SSEA-1, Oct-4, Nanog
An Overview on Stem Cells and Stem Cell Therapy 503

and Rex-1 and Rif-1 telomerase protein (12) Direct 2) Available in vast abundance; thousands of
electron microscopical analysis revealed that VSEL babies are born each day and the umbilical
(2-4 µm in diameter) display several features typical cord and placenta are discarded as waste.
for embryonic stem cells such as i) a large nucleus 3) Despite its high content of immune cells, it
surrounded by a narrow rim of cytoplasm, and ii) does not produce strong graft-versus-host
open-type chromatin (euchromatin). Interestingly, disease
these cells despite their small size possess diploid
DNA and contain numerous mitochondria. VSEL, 4) Therefore, cord blood grafts do not need to be
however, do not express MHC-1 and HLA-DR as rigorously matched to a recipient as bone
antigens and are CD90- CD105- CD29. marrow grafts. A 4 out of 6 match is sufficient
for clinical use.
Umbilical Cord Stem Cells Hence, cord blood has recently emerged as an
Umbilical cord blood stem cells can be obtained alternative source of hematopoietic stem cells for
from the umbilical cord immediately after birth. treatment of leukemia and other blood disorders.
Like bone marrow, umbilical cord blood is another All over the world, innumerable cord blood banks
rich source of hematopoietic stem cells, since 1988. have cropped up for storage of umbilical cord stem
The blood remaining in the umbilical vein following cells. These are generally either pure public banks
birth contains a rich source of hematopoietic stem or private banks. There are certain banks which
and progenitor cells, has been used successfully as offer both types of banking (mixed type). Umbilical
an alternative allogeneic donor source to treat a cord stem cells banks also differ in the type of
variety of pediatric genetic, hematologic, biological material that they store. Some banks only
immunologic, and oncologic disorders. Fresh cord store the cord blood (from the umbilical vein) which
blood is also a promising source of non- predominantly carries the haematopoietic stem
hematopoietic stem cells. Among others, it contains cells. Increasingly, banks have started storing pieces
endothelial cells, MSCs and unrestricted somatic of the placenta and cord, which are a rich source of
stem cells (USSC). These hematopoietic stem cells mesenchymal stem cells.
are less mature than those stem cells found in the
bone marrow of adults or children. MECHANISM OF ACTION
Umbilical cord blood contains circulating stem cells Stem cells are instrumental in the formation of new
and the cellular contents of umbilical cord blood tissues and thereby promoting repair and
appear to be quite distinct from those of bone regeneration. Their role, in the normal wear and
marrow and adult peripheral blood. The tear of the body, appears to be assistance of repair
characteristics of hematopoietic stem cells in and maintenance of normal tissue structure and
umbilical cord blood have recently been clarified. function. Recreation of this ability in vitro as well
The frequency of umbilical cord blood in animal models of various diseases is the basis of
hematopoietic stem cells equals or exceeds that of devising therapeutic modalities for degenerative
bone marrow and they are known to produce large disorders through remodeling of the injured tissues.
colonies in vitro, have different growth factor Cell-based therapy could therefore potentially be
requirements, have long telomeres and can be used to treat a wide array of clinical conditions
expanded in long term culture. Cord blood shows where cellular damage is the underlying pathology.
decreased graft versus host reaction compared with
bone marrow, possibly due to high interleukin-10 More importantly, the use of adult stem cells as
levels produced by the cells and/or decreased opposed to human embryonic stem cells for therapy
expression of the beta-2-microglobulin. Cord blood avoids ethical problems and has two additional
stem cells have been shown to be multipotent by advantages:1) Adult stem cells can be isolated from
being able to differentiate into neurons and liver patients, and this overcomes the problem of
cells. immunological rejection and 2) The risk of tumor
formation is greatly reduced as compared to the
The advantages of using cord blood as a source use of embryonic stem cells.(13)
of stem cells are:
1) It is a non-invasive source and can be obtained Plasticity, Pluripotency and Production
from the umbilical cord immediately after While pluripotency and plasticity are considered
birth. properties of early ESC, adult stem cells are
504 Neuro-Rehabilitation : A multi disciplinary approach

traditionally thought to be restricted in their may improve perfusion and enhance angiogenesis
differentiation potential to the progeny of the tissue to chronically ischemic tissue. Although the
in which they reside. However, a remarkable particular growth factors contributing to this
plasticity in differentiation potential of stem cells neovascular effect remain to be defined, the list
derived from adult tissues has been seen. (14) includes vascular endothelial growth factor (VEGF),
hepatocyte growth factor (HGF), and basic
The events underlying stem cell plasticity could
fibroblast growth factor (FGF2). (19,20)
relate to a variety of mechanisms such as
dedifferentiation, trans-differentiation, epigenetic
Decreased Inflammation
changes, and/or cell fusion. Rerouting of cell fate
may result from the multistep process known as Stem cells appear to attenuate infarct size and injury
dedifferentiation where cells revert to an earlier, by modulating local inflammation. When
more primitive phenotype characterized by transplanted into injured tissue, the stem cell faces
alterations in gene expression pattern which confer a hostile, nutrient-deficient, inflammatory
an extended differentiation potential. environment and may release substances which
limit local inflammation in order to enhance its
Another mechanism put forward to explain stem survival. Modulation of local tissue levels of pro-
cell switch to a novel phenotype is a process known inflammatory cytokines by anti-inflammatory
as trans-differentiation. Cells may differentiate paracrine factors released by stem cells (such as IL-
from one cell type into another within the same 10 and TGF-?) is important in conferring improved
tissue or develop into a completely different tissue outcome after stem cell therapy. (21)
without acquiring an intermediate recognizable,
undifferentiated progenitor state. (15) or may Anti-Apoptotic and Chemotactic Signaling
undergo cell fusion resulting in nuclear
Stem cells in a third pathway promote salvage of
reprogramming and changes in cell fate. (16,17) It tenuous or malfunctioning cell types at the infarct
is now recognized that adult stem cells from bone border zone. Injection of MSC into a cryo-induced
marrow may fuse with cells of the target organ. So infarct reduces myocardial scar width 10 weeks
far, bone-marrow-derived cells were shown to form later. MSCs appear to activate an anti-apoptosis
fusion heterokaryons with liver, skeletal muscle, signaling system at the infarct border zone which
cardiac muscle, and neurons. There is evidence that
effectively protects ischemia-threatened cell types
such fused cells become mono-nucleated again, from apoptosis.
either by nuclear fusion or by elimination of
supernumerary nuclei.(18) Beneficial Remodeling of the Extracellular
Matrix
The Paracrine Effect
Stem cell transplantation alters the extracellular
Exploration of the various cellular processes
matrix, resulting in more favorable post-infarct
occurring (both during normal physiology as well
remodeling, strengthening of the infarct scar, and
as after tissue injury) in the process of stem cell
prevention of deterioration in organ function. MSCs
renewal and differentiation, suggests that stem cell
appear to achieve this improved function by
treatment or transplantation of stem cells remodels
increasing acutely the cellularity and decreasing
and regenerates injured tissue, improves function,
production of extracellular matrix proteins such as
and protects tissue from further insult. Stem cells
collagen type I, collagen type III, and TIMP-1 which
transplanted into injured tissue express paracrine
result in positive remodeling and function.
signaling factors including cytokines and other
growth factors, which are involved in orchestrating Activation of Neighboring Resident Stem Cells
the stem cell-driven repair process through
Finally, exogenous stem cell transplantation may
increasing angiogenesis, decreasing inflammation,
activate neighboring resident tissue stem cells.
preventing apoptosis, releasing chemotactic factors,
Recent work demonstrates the existence of
assisting in extracellular matrix tissue remodeling
endogenous, stem cell-like populations in adult
and activation of resident/satellite cells which is
hearts, liver, brain, and kidney. These resident stem
discussed further in details.
cells may possess growth factor receptors that can
Increased Angiogenesis be activated to induce their migration and
proliferation and promote both the restoration of
Stem cells produce local signaling molecules that
dead tissue and the improved function in damaged
An Overview on Stem Cells and Stem Cell Therapy 505

tissue. Mesenchymal stem cells have also released factors such as age, weight, marrow distribution,
HGF and IGF-1 in response to injury which when physical status of the patient, physicians experience
transplanted into ischemic myocardial tissue may etc. However the most common site is the pelvis.
activate subsequently the resident cardiac stem The aspiration is easily done from either of the iliac
cells. (22) crests (posterior or anterior). The posterior superior
iliac spine is easily accessible and identifiable,
To sum up, although the definitive mechanisms for
however to access this, the patient has to be turned
protection via stem cells remains unclear, stem cells
in the lateral or prone position which can be
mediate enhanced angiogenesis, suppression of
troublesome and cumbersome. The anterior
inflammation, and improved function via paracrine
superior iliac spine can be accessed with the patient
actions on injured cells, neighboring resident stem
lying comfortably in the supine position. In obese
cells, the extracellular matrix, and the infarct zone.
patient, the landmarks may be obliterated due to
Improved understanding of these paracrine
fat distribution. Sampling is not normally
mechanisms may allow earlier and more effective
discordant between the anterior or posterior iliac
clinical therapies
spines.
Remyelination The site of the aspiration is palpated. For the
Remyelination involves reinvesting demyelinated posterior superior iliac spine, in thin individuals, it
axons with new myelin sheaths. Previous attempts is usually palpated as the bony prominence
aimed at regenerating myelin-forming cells have superior and three finger breadth laterals to the
been successful but limited by the multifocal nature intergluteal cleft. The anterior superior iliac spine
of the lesions and the inability to produce large can be palpated as an anterior prominence on the
numbers of myelin- producing cells in culture. Stem iliac crest. The overlying skin is prepared in a
cell-based therapy can overcome these limitations manner similar to preparation of any site for
to some extent and may prove useful in the future surgery. The area is anaesthetized by intradermally
treatment of demyelinating diseases. administering a local anesthetic such as lignocaine
using a 25G or 26G needle. A 1 cm area is
Contrary to the general expectations that stem cells
anesthetized.
would primarily contribute to formation of tissue
cells for repair, other mechanisms such as paracrine A standard bone marrow aspiration needle is
effects and remyelinations appear to be important inserted through the skin till the bone is felt. Before
ways via which stem cells seem to exert their effect. using the needle it is flushed with heparin. Some
More Basic research to understand these surgeons make a small incision with a surgical blade
mechanisms is underway throughout the world. and expose the bone before putting in the needle,
however in our experience this is rarely required.
SURGICAL ASPECTS OF The needle which is firmly fixed to the obturator is
STEM CELLS THERAPY firmly inserted inside, clockwise and anticlockwise,
in a screwing motion with exertion of downward
The stem cell therapy process using autologous pressure, until the periosteum is reached. With
bone marrow derived stem cells consists broadly similar motion, the needle is inserted till it
of 3 stages. (1) Procurement of the stem cells from penetrates the cortex. At this point initially a sudden
the bone marrow via a bone marrow aspiration in giving way of the resistance is felt as the needle
the operating theatre (2) separation, harvesting, enters the soft trabecular bone and then the needle
enriching &/or expansion and differentiation in feels firmly fixed in the bone. The angle of insertion
the laboratory and finally (3) transplantation or of the needle is important as it has to be in alignment
delivery of the cells to the desired location. The with the curve of the bone. If this is not done
laboratory aspects have already been dealt with in properly the needle will make a through and
the previous chapter therefore in this chapter the through penetration across both the cortical
procurement and transplantation aspects will be surfaces with the tip now being outside the marrow.
discussed. A study of the anatomy of the pelvis with a model
and personal experience over time make this a very
Procurement of Stem cells - Bone marrow simple procedure.
aspiration
The stylet is now removed and a 10 ml or 20 ml
The choice of site may be dependent on various syringe, with some heparin in it, is attached and
506 Neuro-Rehabilitation : A multi disciplinary approach

the aspiration is done. A total of 100-120 ml is Callera et al (2007) demonstrated for the first time
aspirated in adults and 80-100 ml in children. This that autologous bone marrow CD 34+ cells labelled
is collected in heparinized tubes which need to be with magnetic nanoparticles delivered into the
appropriately labeled. The bone marrow collected spinal cord via lumbar puncture (LP) technique
is transported to the laboratory in a special migrates into the injured site in patients with spinal
transporter under sterile conditions.(23) cord injury. They conducted the trial on 16 patients
with chronic SCI. 10 of them were injected
Transplantation of Stem Cells intrathecally with labelled autologous CD 34+ cells
The other surgical aspect in the process of stem cell and the others received an injection containing
therapy is the delivery of the cells which may either magnetic beads without stem cells. Magnetic
be done systemically (through intravenous or resonance images were obtained before and 20 and
intraarterial routes) or locally (intrathecal or direct 35 days after the transplantation. Magnetically
implantation into the spinal cord or brain). Different labelled CD 34+ cells were visible at the lesion site
centers are following different routes to transplant as hypointense signals in five patients, which were
the cells and as of now there are no comparative not visible in the control group.(24)
studies that could tell us which is the preferred
method. However keeping in mind the existence Intraspinal transplantation
of the blood brain barrier, local delivery would seem Direct implantation into the spinal cord may be
to be a more logical option. done in one of many ways:-
a) Through a complete laminectomy from one
Intrathecal delivery
level above to one level below the injury site
The patient is positioned in the lateral decubitus so that there is sufficient access to the
position, in the curled up "foetal ball" position. transplantation site. The dura is incised,
Occasionally, the patient is made to sit, leaning over sparing the arachnoid, which is subsequently
a table- top. Both these maneuvers help open up opened separately with microscissors. The
the spinous processes. The back is painted and dorsal surface of the contusion site is located
draped and local anaesthetic is injected into the L4- under high-power microscopic magnification.
5 or L3-4 space. An 18G Touhy needle is inserted After exposure of sufficient surface in the
into the sub-arachnoid space. After ascertaining contusion site, 300µL aliquots of cell paste
free flow of CSF, an epidural catheter is inserted (total volume, 1.8 mL) are injected into six
into the space, far enough to keep 8-10 of the separate points surrounding the margin of the
catheter in the space. The stem cells are then contusion site. To avoid direct cord injury, 2 ×
injected slowly through the catheter, keeping a close 108 cells are delivered at a rate of 30 µL/min,
watch on the hemodynamics of the patient. The using a 27-gauge needle attached to a 1-mL
cells are flushed in with CSF. The catheter is syringe. The depth of the injection site is 5 mm
removed and a benzoin seal followed by a tight from the dorsal surface. To prevent cell leakage
compressive dressing is given. This procedure is through the injection track, the injection needle
usually done under local anesthesia. General is left in position for 5 min after completing
anesthesia is given to children. the injection, after which the dura and
A spinal needle instead of a catheter is preferred in arachnoid are closed. The muscle and skin are
patients with cardiac problems, where excessive closed in layers. (25)
intravenous infusion is to be avoided, in patients b) Though a minilaminectomy and exposure of
on anti-coagulant or anti-platelet drugs so as to the spinal cord. The dura is opened and a 27
avoid bleeding into the sub-arachnoid space, in case gauge scalp vein is used by cutting one of the
where the spine is scoliotic which happens often in wings. The other wing is held by a hemostat
patients with muscular dystrophy and in some and inserted at a 45 degree angle into the
previously operated cases of lumbar spine surgery. dorsal root entry zone. It is inserted 3mm deep
Sometimes in patients with severe spinal into the spinal cord. Two injections are made
deformities such as scoliosis it is very difficult to on either side above the injury site and two
get the needle intrathecally and at times assistance injections are made below the injury site. In
has to be taken of the C arm to exactly locate the China, surgeons are injecting 35 µL of stem
point and direction of needle placement. cells. In his planned trials, Wise Young is
An Overview on Stem Cells and Stem Cell Therapy 507

Bone marrow aspiration

Bone marrow samples

Lumbar Puncture

Intrathecal injection of stem cells

Intramuscular injection of bone marrow


derived stem cells
508 Neuro-Rehabilitation : A multi disciplinary approach

intending to inject an escalating dose of 4 µL, Intramuscular injection


8 µL and 16 µL. In certain disorders, especially Muscular dystrophy,
c) In their ongoing trials, Geron and Neuralstem cells are also transplanted into the muscle. The
are using stereotactic systems specifically points at which these have to be injected are termed
designed for intraspinal injections. They have as the "motor points. At these motor points, the area
the advantage of precision as well as being less is cleaned with povidone iodine. The cells diluted
invasive. Geron is using a stereotactic frame in CSF are injected with the 26G needle going into
with a straight needle and injecting 25 µL. the muscle at an angle (approx. 45 degrees).The
piston/plunger of the syringe is slightly withdrawn
Intra-arterial injection to verify the needle is not inside a blood vessel. The
Following revascularization surgery such as cells are then injected, the needle removed and the
Carotid endartrectomy or Superficial Temporal site immediately sealed with a benzoin seal.
artery to Middle Cerebral artery bypass, stem cells Stem cell transplantation, in its various forms, has
could be injected directly intra-arterially been practically attempted for various degenerative
immediately after the completion of the disorders, including diabetes, cardiac disorders and
revascularization procedure. The advantage of this neurodegenerative disorders. World wide reports
approach is that the stem cells would go directly to reveal the use of bone marrow derived
the ischemic brain and also that since the artery is mononuclear cells and mesenchymal cells,
already exposed no separate procedure needs to umbilical cord blood stem cells, mesangioblasts,
be done for the stem cell injection. The other method myoblasts, neural stem cells for various incurable
of direct intra-arterial injection would be via the and intractable neurological disorders.
endovascular interventional route. This is done by
making a puncture in the femoral artery and REFERENCES
negotiating a catheter to the arteries supplying the
brain. The advantage of this is that it is a relatively 1. Gerald D. Fischbach and Ruth L. Fischbach.
non invasive procedure and the limitations of Stem cells: science, policy, and ethics. J Clin
intravenous injection are avoided. Invest.2004; 114(10): 1364-1370.
2. Mariusz Z. Ratajczak, Ewa K. Zuba-Surma,
Stereotactic implantation into the brain Marcin Wysoczynski, Wu Wan, Janina,
Cell transplantation for neurological conditions Ratajczak, and Magda Kucia . Hunt for
started with stereotactic implantation of fetal cells Pluripotent Stem Cell - Regenerative Medicine
for Parkinson's disease. (26) However, after a Search for Almighty Cell. J Autoimmun. 2008
randomized trial done by Freed et al showed that ; 30(3): 151-162
the clinical outcomes were not significantly 3. Thomson JA, Itskovitz-Eldor J, Shapiro SS et
different from non transplanted patients this has al. Embryonic stem cell line from human
now been given up. (27) There are many stereo blastocysts. Science 1998; 282: 1145-1147.
tactic systems available all over the world however 4. Evans MJ. The isolation and properties of a
the two most popular ones are the Leksell clonal tissue culture strain of pluripotent
Stereotactic system and the CRW Stereotactic mouse teratoma cells. J Embryol Exp Morphol
system. The Leksell system involves fixing the 1972;28: 163-176.
frame on the patients head and then getting a MRI
5. Durick K, Mendlein J, and Xanthopoulos KG.
done with the frame on. The area where the tissue
Hunting with traps: genome-wide strategies
is to be transplanted is identified on the MRI scan
for gene discovery and functional analysis.
and then using the MRI software the X, Y and Z
Genome Res 1999; 9: 1019-1025.
coordinates are obtained. The patient is now shifted
to the operating room where a small burr hole is 6. Davila JC, Cezar GG, Thiede M, Strom S, Miki
drilled into the skull and then through this the cells T, and Trosko J. Use and application of stem
to be transplanted and inserted at the desired cells in toxicology. Toxicol Sci 2004; 79: 214-
location using the X, Y and Z coordinates. The entire 223.
procedure is done under local anesthesia. 7. Hochedlinger K and Jaenisch R. Nuclear
transplantation, embryonic stem cells, and the
potential for cell therapy. N Engl J Med 2003;
349: 275-286.
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8. Brustle O, Spiro AC, Karram K, Choudhary cardiomyocytes and hepatocytes. Nature


K, Okabe S, and McKay RD. In vitro-generated 2003;425:968-973.
neural precursors participate in mammalian 19. Crisostomo PR, Wang M, Herring CM, Markel
brain development. Proc Natl Acad Sci USA TA, Meldrum KK, Lillemoe KD, et al. Gender
1997; 94: 14809-14814. differences in injury induced mesenchymal
9. Sabine Hombach-Klonisch, Soumya Panigrahi, stem cell apoptosis and VEGF, TNF, IL-6
Iran Rashedi et al. Adult stem cells and their expression: Role of the 55 kDa TNF receptor
trans-differentiation potential-perspectives (TNFR1) J Mol Cell Cardiol.2007;42(1):142-149.
and therapeutic applications. J Mol Med. 2008 20. Vandervelde S, van Luyn MJ, Tio RA,
; 86(12): 1301-1314 Harmsen MC. Signaling factors in stem cell
10. Cosimo De Bari, Francesco Dell'Accio, mediated repair of infarcted myocardium. J
Przemyslaw Tylzanowski, and Frank P. Mol Cell Cardiol. 2005;39(2):363
Luyten. Multipotent Mesenchymal Stem Cells 21. Markel TA, Crisostomo PR, Wang M, Herring
From Adult Human Synovial Membrane. CM, Meldrum DR. Activation of Individual
Arthritis & rheumatism. 2001 : 44( 8), 2001, Tumor Necrosis Factor Receptors
1928-1942. Differentially Affects Stem Cell Growth Factor
11. Jiang Y, Jahagirdar BN, Reinhardt RL, and Cytokine Production. Am J Physiol
Schwartz RE, Keene CD, Ortiz-Gonzalez XR, Gastrointest Liver Physiol. 2007; 293(4):657-62.
et al. Pluripotency of mesenchymal stem cells 22. Wang M, Crisostomo PR, Herring C, Meldrum
derived from adult marrow. Nature KK, Meldrum DR. Human progenitor cells
2002;418:41-9. from bone marrow or adipose tissue produce
12. Kucia M, Reca R, Campbell FR, Zuba-Surma VEGF, HGF, and IGF-I in response to TNF by
E, Majka M, Ratajczak J, et al. A population of a p38 MAPK-dependent mechanism. Am J
very small embryonic-like (VSEL) Physiol Regul Integr Comp Physiol.
CXCR4(+)SSEA-1(+)Oct-4+ stem cells 2006;291(4):880-884.
identified in adult bone marrow. Leukemia 23. Bernadette F. Rodak, George A. Fritsma,
2006;20:857-69. Kathryn Doig. Hematology: clinical principles
13. Smith AG. Embryo-derived stem cells: of mice and applications.
and men. Annu Rev Cell Dev Biol 2001;17:435- 24. Callera et al. Magnetic resonance tracking of
462 magnetically labelled autologous bone
14. Amy J Wagers and Irving L Weissman. marrow CD 34+ cells transplanted into the
Plasticity of Adult Stem Cells. Cell. 2004, spinal cord via lumbar puncture technique in
116(5): 639-648 patients with chronic spinal cord injury: CD
15. Sabine Hombach-Klonisch, Soumya Panigrahi, 34+ cells' migration into the injured site. Stem
Iran Rashedi. Adult stem cells and their trans- Cells Dev. 2007; 16(3): 461-6.
differentiation potential- perspectives and 25. Hyung Chun Park, Yoo Shik Shim, Yoon Ha
therapeutic applications. J Mol Med. 2008; Seung Hwanoon, So Ra Park, Byung Hyune
86(12): 1301-1314 Choi, Hyun Seon Park. Treatment of Complete
16. Vassilopoulos G, Russell DW. Cell fusion: an Spinal Cord Injury Patients by Autologous
alternative to stem cell plasticity and its Bone Marrow Cell Transplantation and
therapeutic implications. Curr Opin Genet Administration of Granulocyte-Macrophage
Dev 2003;13:480-485. Colony Stimulating Factor. Tissue
Engineering.2005;11(5-6):913-922
17. Wang X, Willenbring H, Akkari Y, Torimaru
Y, Foster M, Al-Dhalimy M, Lagasse E, 26. Bjorklund A, Dunnett SB, Brundin P et al.
Finegold M, Olson S, Grompe M. Cell fusion Neural transplantation for the treatment of
is the principal source of bone-marrow- Parkinson's disease. Lancet Neurology 2003;
derived hepatocytes. Nature 2003;422:897-901. 2: 437-45.
18. Alvarez-Dolado M, Pardal R, Garcia-Verdugo 27. Freed CR, Greene PE, Breeze RE et al.
JM, Fike JR, Lee HO, Pfeffer K, Lois C, Embryonic dopamine cell transplantation for
Morrison SJ, Alvarez-Buylla A. Fusion of bone- severe Parkinson's disease. New England
marrow-derived cells with Purkinje neurons, Journal of Medicine 2001; 344(10):710-719.
510 Neuro-Rehabilitation : A multi disciplinary approach

Ch.17 Role of Rehabilitation Therapist


in Stem Cell Therapy
Dr. Mamta Lohia, [Link], F.N.R,CBE (USA), Dr. Guneet Chopra, M.B.B.S, (PGDM),
Dr. Prerna Badhe, M.D., Dr. Nandini Gokulchandran, M.D, Dr. Alok Sharma, M.S., [Link].
STRUCTURE OF A MUSCLE
In human body, muscles are composed of many
muscle fibres, which are separated from each other
by connective tissues called endomysium and are
arranged in bundles called fascicules, where
individual fibres are arranged parallel to each other.
Each fasciculus has an outer connective tissue
membrane called perimysium and muscle as a
whole consists of all these fascicules together with
outer layer called epimysium.

Types of Muscles in human body


There are three major types of muscles skeletal,
smooth and cardiac. The characteristics of each type
are summarized below.

Type of Muscles in Characteristics Location in the


Human Body human body
Skeletal Muscle striped, striated, somatic, attached to skeleton
or voluntary muscles,
most abundant
Smooth Muscles plain, unstriped, often encircle or surround
non-striated, visceral, or the viscera
involuntary muscles
Cardiac Muscle intermediate in structure, form myocardium of the heart
being striated and at the
same time involuntary.
Myoepithelial Cells Function: assist in expulsion present at the bases of
of secretion from the acini. secretary acini of sweat gland

Out of the four kinds of muscles, the skeletal in 0.01 sec or less after stimulation. Fast fibers
muscles are most abundant in the body and have are large in diameter; they contain densely
three major types. packed mofibrils, large glycogen reserves, and
relatively few mitochondria. The tension
TYPES OF SKELETAL MUSCLE FIBERS produced by a muscle fiber is directly
The human body has three major types of skeletal proportional to the number of sarcomeres, so
muscle fibers: fast fibers, slow fibers, and muscles dominated by fast fibers produce
intermediate fibers. powerful contractions. However, fast fibers
fatigue rapidly because their contractions use
i. Fast Fibers: ATP in massive amounts, so prolonged
Most of the skeletal muscle fibers in the body activity is supported primarily by anaerobic
are called fast fibers, because they can contract metabolism. Several other names are used to
Role of Rehabilitation Therapist in Stem Cell Therapy 511

refer to these muscle fibers, including white MUSCLE PHYSIOLOGY


muscle fibers, fast-twitch glycolytic fibers, and
Type II-A fibers. Sarcomere is the contractile unit of a myofibril,
which are repeating units and delimited by the Z
ii. Slow Fibers: bands along the length of the myofibril.
Slow Fibres are only about half the diameter Muscle units are separated from other muscle
of fast fibers and take three times as long to groups by plasma membranes called the
contract after stimulation. Slow fibers are sarcolemma and the cytoplasm within is called the
specialized to enable them to continue sarcoplasm. Within the sarcoplasm are multiple
contracting for extended periods, long after a long protein bundles called myofibrils, and many
fast muscle would have become fatigued. The ATP producing mitochondria, as well as glycogen
most important specializations in them is (a form of stored glucose for energy) and myoglobin
improved mitochondrial performance. Slow (oxygen stored in blood for the breakdown of
muscle tissue contains more extensive glycogen). Bundles of parallel myofilaments make
network of capillaries than in a typical fast up the myofibrils which is where most of the action
muscle tissue and so has a dramatically higher takes place. In the myofilaments are contractile
oxygen supply. In addition, slow fibers contain proteins called myosin (thick filaments), and actin
the red pigment myoglobin . This globular (thin filaments).When signaled, the actin and
protein is structurally related to hemoglobin, myosin interlock and slide over each other to stretch
the oxygen-carrying pigment in blood. Both or slide into one another to contraction. They are
myoglobin and hemoglobin are red pigments signaled from the nervous system followed by a
that reversibly bind oxygen molecules. series of chemical reactions involving ATP, calcium,
Although other muscle fiber types contain sodium and potassium ions.
small amounts of myoglobin, it is most
abundant in slow fibers. As a result, resting
slow fibers contain substantial oxygen reserves
that can be mobilized during a contraction.
Because slow fibers have both an extensive
capillary supply and a high concentration of
myoglobin, skeletal muscles dominated by
slow fibers are dark red. They are also known
as red muscle fibers, slow-twitch oxidative
fibers, and Type I fibers.

There are many other proteins involved in the


process. Aside from the contractile proteins, there
are regulatory proteins called tropomyosin and
iii. Intermediate Fibers: troponin which act like a switch to determine when
The properties of intermediate fibers are to contract and when to relax. On the muscle fiber
the 'I band' is the space between the myosin (thick)
intermediate between those of fast fibers and
filaments, where lies only the thin filaments. In the
slow fibers. In appearance, intermediate fibers
middle of each 'I band' is a dark disc called the 'Z
most closely resemble fast fibers, for they
disc' made of titan, (elastic filament), which is
contain little myoglobin and are relatively
connected to the sarcolemma by the cytoskeleton.
pale. They have a more extensive capillary The space between each Z disc, where these
network around them, however, and are more filaments interact, is called the sarcomere. As the
resistant to fatigue than are fast fibers. muscle contracts the 'I band" shrinks and the
Intermediate fibers are also known as fast- sarcomere shortens and as the Z disc's come closer
twitch oxidative fibers and Type II-B fibers.
512 Neuro-Rehabilitation : A multi disciplinary approach

together pulling on the sarcolemma shortening the thought that its primary function is to provide
cell. This is how the muscle contracts. One of the mechanical reinforcement to the structure of the
most clinically important accessory proteins here sarcolemma and thereby protecting the membrane
is dystrophin which is located just under the from the stress or tearing during contraction.
sarcolemma in the cytoplasm in the area of the 'I
In Muscular Dystrophy patients, as dystrophin is
band'. It is produced by specific genes and links
defective or absent, the membrane breaks down and
the actin filaments to the protein extracellular
molecules like proteins and enzymes leak out of
matrix in the membrane known as the dystrophin-
the fiber into circulation. These enzymes and
associated protein complex. Elements of the
chemicals that leak out are responsible for certain
dystrophin gene and the protein structure have
chemical reactions and disruption of the process of
been identified, yet the exact functional role is still
muscle contraction which thus causes irreparable
a bit unclear. However, as research continues it is
damage.

Pathophysiology of Protein deficient Muscle: upper & middle one thirds of the fleshy belly of the
To summarize, important abnormalities of muscles, although there are exceptions e.g.: the
dystrophin-deficient muscle cells have been motor point of vastus medialis, whose nerve enters
demonstrated in three areas: the lower part of the muscle, is situated a short
distance above the knee joint. Deeply placed
1. Calcium homeostasis,
muscles may be stimulated most satisfactorily
2. An increased susceptibility to oxidative toxins, where they emerge from beneath the more
and superficial ones, e.g.: extensor hallucis longus in the
3. Increased (and stress enhanced) membrane lower one third of the lower leg. This is the point
permeability. on the skin region where an innervated muscle is
most accessible to percutaneous electrical excitation
MOTOR POINT at the lowest intensity. This point on the skin
Motor point is the point at which the main nerve generally lies over the neuro vascular hilus of the
enters the muscle or, in case of deeply placed muscle & the muscles band or zone of innervations.
muscle, the point where the muscle emerges from Muscle fibres do not always extend the whole
under covers of the more superficial ones. length of a muscle & myoneural junctions are not
uniformly spread out all over the muscle but are
Facts about Motor points: concentrated in a confined area-the zone or band
Motor points are frequently at the junction of the of innervations where the greatest concentration of
Role of Rehabilitation Therapist in Stem Cell Therapy 513

A Neuromuscular Junction

The Motor Unit


514 Neuro-Rehabilitation : A multi disciplinary approach

motor endplates & the other large diameter nerve The skin has high electrical resistance as the
fibres may be reached with less concurrent painful superficial layers being dry, contain few ions. The
stimulation of the smaller diameter cutaneous resistance is reduced by washing with soap & water
fibres. to remove the natural oils & moistening with saline
immediately before the electrodes are applied.
The exact location of motor point varies slightly
Breaks in the skin cause a marked reduction in
from patient to patient but the relative position
resistance which naturally results in concentration
follows a fairly fixed pattern. Some motor points
of the current & consequent discomfort to the
are superficial & are easily found, while others
patient. To avoid this broken skin is protected by a
belonging to deep muscles are more difficult to
petroleum jelly covered with a small piece of non
locate.
absorbent cotton wool to protect the pad. The
indifferent electrode should be large to reduce the
CONCEPT OF MOTOR POINT
current density under it to a minimum. This
STIMULATION prevents excessive skin stimulation & also reduces
When a nerve is stimulated at a nerve cell or an the likelihood of unwanted muscle contractions, as
end organ, there is only one direction in which it it may not be possible to avoid covering the motor
can travel along the axon, but if it is initiated at points of some muscles.
some point on the nerve fibre it is transmitted
simultaneously in both directions from the point Preparation of apparatus
of stimulation. Faradic type of current
When a sensory nerve is stimulated the downward A low frequency electronic stimulator with
travelling impulse has no effect, but the upward automatic surger is commonly used. A faradic
travelling impulse is appreciated when it reaches current is a short -duration interrupted direct
conscious levels of the brain. If impulses of different current with a pulse duration of 0.1 - 1 ms & a
durations are applied, using the same current for frequency of 50 - 100 Hz. Strength of contraction
each, it is found that the sensory stimulation depends on the number of motor units activated
experienced varies with the duration of the impulse. which in turn depends on the intensity of the
Impulses of long duration produce an current applied & the rate of change of current. To
uncomfortable stabbing sensation, but this becomes delay fatigue of muscle due to repeated
less as the duration of the impulse is reduced until contractions, current is commonly surged to allow
with impulses of 1 ms & less only a mild prickling for muscle relaxation.
sensation is experienced.
When a motor nerve is stimulated, the upward - Stimulation of Motor points
travelling impulse is unable to pass the first This method has the advantage that each muscle
synapse, as it is travelling in the wrong direction, performs its own individual action & that the
but the downward travelling impulse passes to the optimum contraction of each can be obtained, by
muscles supplied by the nerve, causing them to
contract.
When a stimulus is applied to a motor nerve trunk,
impulses pass to all the muscles that the nerve
supplies below the point at which it is stimulated,
causing them to contract.
When a current is applied directly over an
innervated muscle, the nerve fibres in the muscle
are stimulated in the same way. The maximum
response is thus obtained from stimulation at the
motor point.

Preparation of the patient


Clothing is removed from the area to be plotted &
Electrical stimulator used for stimulation and
the patient is supported comfortably in good light.
plotting of motor points.
Role of Rehabilitation Therapist in Stem Cell Therapy 515

stimulating the motor point. The indifferent


electrode is applied & secured in a suitable area.
The indifferent electrode is placed over the motor
point of the muscle to be stimulated. Firm contact
ensures a minimum of discomfort & where possible
the whole of operators hand should be in contact
with the patient's tissues so that she /he can feel
the contractions produced.
Selection of the Individual muscles for
intramuscular injection of stem cells in Muscular
Dystrophy Patients:
Patients with Muscular Dystrophy have primarily
weak antigravity muscles like hip knee extensors ,
back extensors and ankle Dorsiflexors in lower Figure 6 : Marking of sternomastoid muscle motor
limbs. In upper limb the proximal shoulder girdle point.
muscles like deltoids, biceps triceps and scapular
stabilizers like rhomboids and serratus are the most Although MD can affect several body tissues and
commonly affected. All of these muscles are needed organs, it most prominently affects the integrity of
for mobility and activities of daily living , but as muscle [Link] causes muscle degeneration,
they progressively get weaker , patients begin to progressive weakness, fiber death, fiber branching
get dependent for functional activities. and splitting, phagocytosis (in which muscle fiber
material is broken down and destroyed by
scavenger cells), and in some cases, chronic or
permanent shortening of tendons and muscles.
Also, overall muscle strength and tendon reflexes
are usually lessened or lost due to replacement of
muscle by connective tissue and fat.
So selection of muscles (motor points) for
intramuscular injection depends on manual muscle
testing & patient's complain of weakness &
difficulty in activities of daily living. So
rehabilitation team (Physiotherapists and
Occupational therapists) decides motor points of
Figure 4 : Preparation of the patient for motor point
plotting

Figure 5 : Plotting of motor point


(strenomastoid muscle)
516 Neuro-Rehabilitation : A multi disciplinary approach

which muscles need to be injected with stem cells. muscles. An injection of stem cell in the motor end
Also the Electromyography and Musculoskeletal plate potential, can be identified in the
MRI , aid in locating muscles with severe affection neuromuscular system within few hours, although
in the form of fatty infiltration or reduced the onset of clinical effects is noticed as early as 72
interference pattern on voluntary contraction. hours post transplant, which varies from patient
to patient.
In few selective types of Muscular Dystrophies like
Oculopharyngeal MD , facial muscles are weak and
are therefore considered for intramuscular injection.
MECHANISM OF ACTION OF
INTRAMUSCULAR STEM CELL
Commonly considered muscles for injection are as
follows INJECTION AT MOTOR POINTS
As motor point is the point at which the main nerve
A) Major muscles of upper limbs that are enters the muscle. Delivery of stem cells at this point
generally considered: facilitates further specific implantation of the stem
a) Deltoid: Anterior, middle & posterior cells in isolated individual muscles and aids in
fibres. enhancing the healing of the degenerated muscle.
Also the stem cells promote regeneration by
b) Biceps brachialis.
enhancing angiogenesis, suppression of
c) Triceps: long, lateral & medial heads. inflammation and improved function via paracrine
d) Thenar muscles: Opponens pollicis & actions on injured cells ,neighboring resident stem
abductor pollicis brevis & flexor pollicis cells , extracellular matrix , and the infarcted zone.
brevis. (Refer chapter 12)

e) Hypothenar muscles: abductor, flexor & Post stem cell injection these muscles need
opponens digiti minimi. specific training & individual muscle strengthening
program so that results are seen by following
B) Major muscles of lower limbs that are mechanisms:
generally considered:
a) Quadriceps: vastus medialis, vastus
lateralis, rectus femoris.
b) Hamstrings: Biceps femoris,
Semimembranosus & semitendinosus.
c) Glutei.
d) Dorsilflexors: Tibialis anterior, Peronei
longus & brevis, EHL.

C) In trunk: Plotted motor points of tibialis anterior and


peronei muscle
Abdomen & back extensors are considered, &
in neck muscles sternocleidomastoid.

D) Facial Muscles:
In case of facial muscle weakness : orbicularis
oris, orbicularis oculi, Buccinator, rhizorius,
frontalis, mentalis, etc.
Intramuscular stem cells injection in motor points
within the muscle, ie the area with high
concentration of motor end plates is very specific
transplantation. Also multiple motor points in
choosen muscle group allows for a graded
response, thus allowing increment in muscle
strength clinically depending on, further specific Injection of stem cells in
training & strengthening of individual injected tibialis anterior muscle motor point.
Role of Rehabilitation Therapist in Stem Cell Therapy 517

Injection of stem cells in the glutei muscle motor point.

Injection of stem cell injection in the adductor Injection of stem cells in the lumbrical
pollicis muscle motor point. muscle motor points
518 Neuro-Rehabilitation : A multi disciplinary approach
Role of Rehabilitation Therapist in Stem Cell Therapy 519
520 Neuro-Rehabilitation : A multi disciplinary approach

1. In muscles that contain a mixture of fast and gains efficiency & independency in activities of
intermediate fibers, the proportion can change daily living (ADL).
with physical conditioning. For example, if a
muscle is used repeatedly for endurance REFERENCE
events, some of the fast fibers will develop the
1. Clayton'S Electrotherapy, Theory & Practice,
appearance and functional capabilities of
Ninth edition [Link] Forstet & Nigel
intermediate fibers. The muscle as a whole will
Palatanga.
thus become more resistant to fatigue.
2. R.W Reid,M.D, Prof of Anatomy, University
2. Exercise leads to stimulation of Satellite cells (
of Abeerdeen, Journal Of Anatomy, Vol LIV,
special stem cells which lie adjacent to skeletal
part 4.
muscle fibre and play a role in muscle
regeneration and repair) 3. [Link]
anatomy/types-of-muscles-and-there-
3. As dystrophy patients muscles lack enzyme , functions/
which produces nitric oxide , which in turn
leads to vasodilatation , in order to stimulate 4. Martini -Anatomy Physiology Chapter 10 ,
satellite cells .Natural stimulation of satellite Muscle Tissue.
cells in them is very slow , thus leading to rapid 5. Muscle Physiology and the Pathology of
degeneration and braek down to [Link] Muscular Dystrophy Angela Tompkins
direct stem cell intramuscular transplantation February 23, 2010 Everglades University
and exercise leads to angiogenesis and Biology
vasodilatation , leading to stimulation of 6. [Link]
satellite cells and thus repair and regeneration detail_md.htm
of muscles.
7. Saunders Comprehensive Veterinary
Gradually as the muscle strength increases patient Dictionary, 3 ed. © 2007 Elsevier, Inc.
Ch.18 Clinical Improvements in Neurological
disorders after Stem Cell Therapy
Dr. Nandini Gokulchandran, M.D, Dr. Guneet Chopra, M.B.B.S,(PGDM), Dr. Prerna Badhe, M.D.,
Ms. Pooja Kulkarni, M.S.(Biotechnology), Dr. Mamta Lohia, [Link], F.N.R,CBE(USA),
Dr. Alok Sharma, M.S., [Link].

At NeuroGen Brain and Spine Institute, stem cell Their mean follow up of 18 months showed that
therapy was carried out on disorders like muscular out of 72 patients , 67 (93%) showed positive results
dystrophy, cerebral palsy, multiple sclerosis, stroke, while non responders were only 5 (7%).The major
spinal cord injury, motor neuron disease and other area showing improvements were increase in
rare incurable neurological , neuromuscular trunk strength (32), lower extremity strength (30) ,
diseases including genetic disorders based on the upper extremity strength(20) and improved gait
Helsinki Declaration. (1-8) pattern (11).Many of these patients showed
improved muscle strength on manual muscle
Till date over 700 patients of various disorders (as
testing. Muscle tightness was reduced in 40
mentioned above) have been administered stem cell
patients. (Graph 3, 4 , 5)
therapy. A detailed analysis of their outcome, with
an average followup of 18 months for 300 patients Not only that, these changes also lead to functional
has been presented in this chapter. improvements, as reflected by the shift in the
Functional Independence Measure scores (FIM
Out of these 300 patients,73% of the patients were
scores) in 38 patients. Biochemical response in terms
observed to have improvements, ranging from mild
of reduction in serum creatine phosphokinase was
to significant category (graph 1).The other 25%
seen in 47 patients. Objective evidence of response
were mainly status quo, with no changes, while 2%
to treatment was obtained by:
of the patients deteriorated in their condition ,
despite the therapy , all were suffering from a) increase in amplitudes of compound motor
progressive neurological disorder ( which were units action potential as well as increased
revealed to be patients of motor neuron disease). interference pattern while muscle contraction
(Graph 1) on electromyography (EMG changes seen in
9 patients)
Neurological Disorder Number of patients
b) Imaging (MRI) of the musculoskeletal
Muscular Dystrophy 72
system(both upper and lower limbs) showed
Cerebral palsy 16 reduction in fatty infiltration, some
Spinal Cord Injury 74 regeneration of muscle fibres and molecular
changes on MR Spectroscopy of the muscles-
Autism 22 indicating molecular flux in the intramyo-
Stroke 11 cellular and extramyocellualr lipids. (8)
Multiple sclerosis 21
Motor Neuron Disease 47
The majority of this above cohort was spinal cord
injury and muscular dystrophy.

Muscular Dystrophy
Seventy two muscular dystrophy patients who
underwent intrathecal autologous bone marrow
derived mononuclear cell transplantation, could
broadly be categorized as Duchene Muscular
Dystrophy type,(41) Limb Girdle Muscular
Dystrophy (17), Congenital Muscular Dystrophy
(11), Becker's Muscular Dystrophy(2) and Fascio
Scapulohumeral Dystrophy(1). (Graph 2) Fig.1: Axial T1W images at the level of upper thigh
522 Neuro-Rehabilitation : A multi disciplinary approach

(A)Pre-stem cell therapy show marked fatty (A) Pre-stem cell therapy show marked fatty
infiltration of the right vastus medials (thick arrow) infiltration of the bilateral tibialis anterior muscle
and lateralis muscle (thin arrow), seen as high signal (thin arrows) seen as high signal intensity. (B) Post-
intensity. (B) Post-stem cell therapy shows reduced stem cell therapy shows reduced high signal in
high signal in both the vastus medialis (thick arrow) bilateral tibialis anterior muscle (thin arrows)
and lateralis (thin arrow) suggestive of less fatty suggestive of less fatty infiltration and regeneration
infiltration and regeneration of muscle fibres of muscle fibres.

Fig. 2: Axial T1W images at the level of upper thigh


(A)Pre-stem cell therapy show marked fatty
infiltration of the left semitendinosus (thin arrow) Figure 4: Axial T1W images at the level of the calf
seen as high signal intensity. (B) Post-stem cell (A)Pre-stem cell therapy show marked fatty
therapy shows reduced high signal in the left infiltration of the left medial (thick arrow) and
semitendinosus (thin arrow) suggestive of less fatty lateral gastrocnemius muscles (thin arrow)) seen
infiltration and regeneration of muscle fibres. as high signal intensity. (B) Post-stem cell therapy
shows reduced high signal in left medial (thick
arrow) and lateral gastrocnemius muscles (thin
arrow) suggestive of less fatty infiltration and
regeneration of muscle fibres.

Cerebral Palsy
Cerebral palsy patients who underwent stem cell
therapy (16), showed symptomatic improvement
in oromotor functions (37.5%) like speech &
swallowing , improved neck holding (37.5%), sitting
balance (62.5%),range of limb movements (50%),
cognition (50%) and normalization of overall
muscle tone (62%).Overall improvement was seen
in 87.5% of the treated patients. Apart from
functional changes, objectives changes in
neuroimaging corroborating with actual
improvements is now emerging. This is in the form
of improvement in neurometabolism as seen in PET
Figure 3: Axial T1W images at the level of the calf CT Scan of the brain over 6 months. (graph 7-9)
Clinical Improvements in Neurological disorders after Stem Cell Therapy 523

Spinal Cord Injury improvement in hand function and gait(47% and


67% respectively), speech improvement in 75%
Out of the 74 patients treated, 50 were paraplegics, patients while cognition improved in 60% of those
while 24 were quadriplegics, with road traffic affected. (graph 22-24)
accident (83.7%) being the most important cause
.Majority of these were males (52), while 22 were Multiple Sclerosis
females. (Graph 9- 11)
Twenty two multiple sclerosis patients underwent
Following stem cell therapy, 81% (60) patients intrathecal autologous bone marrow derived
improved, while 14 (19%) patients maintained mononuclear cell transplantation. The mean follow
status quo (neurologically the same). On assessing up of 6 months showed that 11 patients shifted on
them symptom-wise 48 patients showed EDSS Scale showing objective Neurological
improvement in sitting balance (static and Improvement. (Graph 25, 29 ,30)
dynamic),13 showed improvement in muscle
On assessing them symptom-wise 17 patients
strength, 4 showed complete recovery in bladder showed reduction in spasticity,9 showed improved
and bowel functions, 9 showed sensory recovery upper extremity and trunk coordination,, 6
and 8 of them showed complete recovery from improved in speech clarity and 8 showed increased
postural hypotension. In addition 50% (42) patients in muscle strength.(graph 26 , 27 , 28,31)
had a reduction in spasticity and 15 were able to
ambulate with assistance of walker. These changes Motor Neuron Disorder
reflected on a shift on ASIA scale by 2 grades in 12
of these who showed improvements in muscle Forty seven motor neuron disease patients
power and gait with assisted devices. Changes on underwent intrathecal autologous bone marrow
FIM score were appreciated in 38 out of 74 patients, derived mononuclear cell transplantation. In
contrast to other neurological disorders, results seen
score shifts ranging from 2 to 45.(graph 12-16 )
in this group of rapidly deteriorating condition are
Autism not very encouraging, with either no improvement
or deterioration in seen in over 70% of the patients.
Out of 22 cases who underwent intrathecal stem Patients with bulbar symptoms were seen to have
cell therapy,18 (82%) of the children showed continued progression. 14 showed some minor
improvements to varied extents. These changes improvements whereas 33 patients kept on
seen were mainly in social interaction(72%), deteriorating with symptoms of early fatigue,
increased attention span (54%), improved memory weakness, muscle wasting and bulbar symptoms,
(45%), eye contact(81%) and behavior(77%) which which progressed with the natural course of the
includes reduction in hyperactivity, temper disease. Out of the 14 patients who improved,
tantrums and emotional lability. A positive shift symptoms which showed results were improved
in these areas has been beneficial in overall neck holding, speech, swallowing, and reduction
management and training of the children, thereby in fasciculations and a halt in the progression of
helping towards the goal of integrating them into muscle weakness. (graph 32-35)
the mainstream. In addition, PET CT Scan of the
brain done before the therapy helps to pinpoint References
areas of brain which are either hypo or 1. Mamta Lohia, [Link], Prerna Badhe,
hypermetabolic. A followup analysis over a period Nandini Gokulchandran, Guneet Chopra,
of 6-8 months with a repeat PET CT Scan has shown Pooja Kulkarni, Alok Sharma.
improvement in neurometabolism which Neuroregenerative rehabilitation Therapy
corroborates with the clinical findings. (graph 17,18) including stem cells for incurable Neurological
Disorders. Physiotimes 2010;2(3): 20-22
Stroke
2. A. Sharma, P. Badhe, N. Gokulchandran, P.
Out of 11 patients, 55% were suffering from non Kulkarni, V.C Jacob, M. Lohia, J. George
haemorrhagic while 45% from haemorrhagic Joseph, H. Biju, G. Chopra. Administration of
stroke, with a female versus male ratio of 7:4. Autologous bone marrow stem cells
(Graph 19-21) intrathecally in Multiple Sclerosis patients is
Reduction in spasticity and improvement in safe and improves their quality of life. Indian
cognition (60%),were noticed, followed by Journal of clinical Practice. 2011:21(11):622-625
524 Neuro-Rehabilitation : A multi disciplinary approach

3. Alok Sharma, Prerna Badhe, Pooja Kulkarni, 6. Mamta Lohia, [Link], Hema Biju, Omshree
Nandini Gokulchandran, Guneet Chopra, Shetty, Pooja Vijaygopal, Prerna Badhe,
Mamta Lohia, [Link]. Autologous Bone Nandini Gokulchandran, Alok Sharma.
marrow Derived mononuclear cells for the Neuroregenerative neurorehabilitation
treatment of Spinal Cord Injury. The Journal therapy using autologous bone marrow
of Orthopaedics. 2011; 1(1): 33-36 derived stem cells Inconcert with intensive
physiotherapy in a case of spinal cord injury.
4. Alok sharma, Guneet Chopra, Nandini
Physiotimes. 2009: 34-36
Gokulchandran, Mamta Lohia, Pooja
Kulkarni. Autologous Bone Derived 7. Sharma A, Gokulchandran N, Kulkarni P,
Mononuclear Transplantation in Rett Chopra G. Application of autologous bone
Syndrome. Asian Journal of Paediatric marrow stem cells in giant axonal neuropathy.
Practice. 2011;15(1):22-24 Indian J Med Sci 2010;64:41-4
5. Alok Sharma, Prerna Badhe, Omshree Shetty, 8. A. Sharma, P. Kulkarni,G. Chopra, N.
Pooja Vijaygopal, Nandini Gokulchandran, Gokulchandran, M. Lohia P. Badhe.
V.C. Jacob, Mamta Lohia, Hema Biju, Guneet Autologous Bone Marrow Derived
Chopra. Autologous bone marrow derived Mononuclear Cell Transplantation In
stem cells for motor neuron disease with Duchenne Muscular Dystrophy-A Case
anterior horn cell involvement. Bombay Report. In Press.
hospital journal. 2011; 53(1):71- 75
Index 525

Index

constraint-induced movement therapy 197


A Checklist for Autism in Toddlers 232
Ambulation 18 Congenital Muscular Dystrophy 354
Anterior Cord Syndrome 4 Central nervous system 411
ASIA IMPAIRMENT SCALE 9 Charcot-Marie-Tooth disease 419
Ataxia 144 Chronic inflammatory demyelinating
Attention Deficit Disorder 169 polyneuropathy 415
Asperger's Disorder 222 Compensation platform 455
Autism 218 Crooked and elongated heels 455
Aversives 234 Cushion heel 455
agnosia 257 Children's Apperception Test 480
Alzheimer 255 Cognitive Changes 472
Amyloid Precursor Protein 258 Chemotactic 506
Anxiety management 279
Aphasia 257 D
apraxia 257
Amyotrophic lateral sclerosis 393 Decompression 6
Air splint 463 Domestic retraining 26
Ankle Foot Orthosis. 455 Dynamic Balance 32
Abandonment 474 Developmental sequence 184
Aggression 473 Depression 236
Anger 473 Developmental Individual Difference Relationship 243
Adult Stem Cells 503 Discrete Trial Teaching 240
Anti-Apoptotic 506 DSM-IV 218
Delirium 270
Delusions 267
B Dementia 255
Bed Mobility 22 Depression 270
Bowel and bladder training 24 Distal MD 356
Brown –Sequard Syndrome 4 Duchenne Muscular Dystrophy 354
Bobath Approach 101 Dorsal elbow - flexion mobilization orthosis 463
Behaviour Therapy 234 Dorsal elbow-extension mobilization orthosis 463
Binswanger's disease 262 Dynamic elbow splints 463
Benign MS 330
Becker Muscular Dystrophy 354 E
Bell palsy 416
Babinski sign 437 Endurance Training 22
Beck's Depression Inventory - Second Edition 482 Emboli 93
Bender Visual-Motor Gestalt Test, Second Edition 482 Extradural Haematoma 174
Bone Marrow Derived Cells 504 Emotional Instability 474
Embryonic stem cells 502

C
F
Cauda equina lesion 4
Functional Independence Measure 98
complete spinal cord injury 9
Factious Disorder 270
Conus medullaries 4
Functional Dexterity test 429
Corticosteroid Therapy 6
Figure-8 harness/clavicular brace 462
Cerebral Ischemia 93
Forearm mobilization (corrective) orthosis 463
cerebrovascular accident 93
Forearm-Wrist Orthoses 463
Cerebral palsy 142
Faradic 516
526 Neuro-Rehabilitation : A multi disciplinary approach

fascicules 512
Fast Fibers 512
M
MODIFIED ASHWORTH SCALE 9
G Monosialotetrahexosyl ganglioside 6
Middle Cerebral Artery 94
Gilliam Autism Rating Scale 232 Music Therapy 251
Gowers' manoeuvre 359 Marital therapy 285
group psychotherapy 489 Malignant MS 330
Multiple Sclerosis 329
H Muscular Dystrophy 351
Myotonic MD 356
Hemiplegia 4 Motor Neuron Disease 393
Hypothermia 6 Mononeuritis multiplex 411
Hemiplegic Gait 96 microsurgical DREZotomy 445
Hemorrhage 93 mini-mental state examination 476
Hyperbaric Oxygen Therapy 154 Marrow-isolated adult multilineage
Head injury 172 inducible (MI 504
Hallucinations 267 Mesenchymal Stem Cells
Huntington's disease 265 (Multipotent Mesenchymal St 504
Hereditary neuropathy with liability to pressure p 419 Multipotent Adult Progenitor Cells (MAPC) 504
Hereditary Sensorimotor neuropathy 419 Multipotent Adult Stem Cells (MACS) 504
Hydrotherapy 427 Motor point 514
Heel elevation 455 muscles 512
Heel wedge 455
Hemiplegic arm sling 462 N
Hip Knee Ankle Foot Orthosis. 455
Hypernasality 494 Neuroplasticity 109
Neurovascular Syndromes 94
NIH Stroke Scale Work sheet 98
I Neuro Developmental Technique 150
incomplete spinal cord injury 9 Neuroprotection 174
Inotropic 6 Nutritional management 378
intugementary 31 Neurectomy 445
ischemia 14
Idiopathic peripheral facial palsy 416 O
Intrinsic tonic spasticity 439
Isolation 473 Occupational therapy 20
Impaired pragmatics 494 Obesity 363
Intraspinal 508 Osteoporosis 364
Intrathecal 508 orthosis 455
Intermediate Fibers 513 Overhead sling suspension 462

K P
Knee Ankle Foot Orthosis. 455 Paraplegia 16,20
Pressure Relief 14
Pressure Relieving Maneuvers 14
L Prenatal 148
Lewy Body Disease 264 Post Traumatic Amnesia 179
Leisure skills 27l Proprioceptive Neuromuscular Facilitation 184
Lhermitte's sign 333 Pervasive Developmental Disorder 218
Limb-girdle MD 356 Pica 223
Longitudinal Myelotomy 445 Play Therapy 252
Lower limb orthosis 455 praxis 250
Index 527

Parkinson's disease 265 Secondary-Progressive MS 330


Pick's disease 263 Scoliosis 362
positron emission tomography 257 Selective Posterior (Dorsal) Rhizotomy (SDR) 445
Primary Progressive MS 330 Spasticity 437
Primary lateral sclerosis 393 Serial cast 463
Progressive bulbar palsy 393 Shoulder sling 462
Progressive muscular atrophy 393 Shoulder-elbow-wrist-hand orthosis 463
Peripheral nervous system 411 Self-dislike 474
peripheral neuritis 411 Sixteen Personality Factor Questionnaire Revised - 478
Polyneuropathy 411 Swallowing 496
Posterior elbow splint 463 Stem Cell Therapy 501
Peripheral Neurotomy 445 Sarcomere 513
Personality Changes 472 Slow Fibers 513
Psychology 472
Paracrine Effect 506
Plasticity 505
T
Pluripotency 505 Tethering 6
Tetraplegics 28
R Transfers 16
The Brathel Index. 98
Rehabilitation 1 The Glasgow Coma Scale 172
Rolling 31 traumatic brain injury 177
Rett's Disorder 222 Thumb stabilizers 467
Resolution therapy 281 Trunk Hip Knee Foot Orthosis. 455
Relapsing/Remitting 330 Thematic Apperception Test 479
Respiratory Affection 364
Rorschach inkblot test 479
Regenerative medicine 501
U
Remyelination 507 Upper Extremity Strengthening 22
Upper Extremity Orthoses 467
S Umbilical cord blood stem cells 505

Self care retraining 22


Somatosensory evoked potential 4
V
spinal cord 1 Vascular complications. 106
Spinal cord injury 2 Visual Problems 198
spinal cord injury 1 Video Modeling 241
Spinal traction 6 Vascular dementia 256,260
Stabilization 6 Very Small Embryonic Like (VSEL)
Seizure. 107 Stem Cells: 504
STREAM Scoring 98
Stroke 93
Sensory integrative therapy 152
W
Self-Management Training 241 Wheelchair 16
single photon emission computed tomography 220 Wechsler Adult Intelligence Scale 475
Speech Therapy 251 Wechsler Intelligence Scale for Children 476
Stereotype Behaviour 224 Wechsler Memory Scale Fourth Edition 476
Sundrowners Syndrome 269

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