RAJIV GANDHI UNIVERSITY OF HEALTH SCIENCES,

BENGALURU, KARNATAKA

“OROFACIAL PAIN”
By

Dr. BHAGYA BALAKRISHNAN

Library Dissertation Submitted to the Rajiv Gandhi University of Health Sciences

Bengaluru, Karnataka

In partial fulfilment of the requirements for the degree of

M.D.S. (MASTER OF DENTAL SURGERY)

In

ORAL MEDICINE AND RADIOLOGY

Under the guidance of

Dr. VATHSALA NAIK, MDS
Professor and HOD

Department of Oral Medicine and Radiology

Bangalore Institute of Dental Sciences, Hospital and Post- Graduate
Research Center, Bengaluru-560029

2020-2023

i
 RAJIV GANDHI UNIVERSITY OF HEALTH SCIENCES
BANGALORE, KARNATAKA

DECLARATION BY THE CANDIDATE

I hereby declare that this dissertation entitled “OROFACIAL PAIN” is a bona fide and

genuine research work carried out by me under the guidance of Dr. VATHSALA NAIK,

Professor and HOD, Department of Oral Medicine and Radiology, Bangalore Institute of

Dental Sciences, Hospital and Post Graduate Research Centre, Bengaluru.

Date: / / 2022 Signature of the Candidate

Place: Bengaluru Dr. BHAGYA BALAKRISHNAN

ii
 RAJIV GANDHI UNIVERSITY OF HEALTH
SCIENCES,KARNATAKA

CERTIFICATE BY THE GUIDE

This is to certify that the dissertation entitled “OROFACIAL PAIN” is a bona fide

research work done by Dr. BHAGYA BALAKRISHNAN in partial fulfilment of the

requirement for the degree of MASTER OF DENTAL SURGERY (M.D.S) IN ORAL

MEDICINE AND RADIOLOGY.

Date:

Dr. Vathsala Naik, M.D.S

Place: BengaluruProfessor and HOD

Department of Oral Medicine and Radiology
Bangalore Institute of Dental Sciences &
Hospital

iii
 ENDORSEMENT BY THE HEAD OF THE DEPARTMENT,
PRINCIPAL/HEAD OF THE INSTITUTION

This is to certify that the dissertation entitled “OROFACIAL PAIN” is a bonafide

research work done by DR. BHAGYA BALAKRISHNAN under the guidance of Dr.

VATHSALA NAIK, Professor and HOD, Department of Oral Medicine and Radiology,

Bangalore Institute of Dental Sciences, Bengaluru.

Dr. VATHSALA NAIK Dr. VINAYA S.PAI

Professor and Head
Department of Oral Medicine and Radiology
Bangalore Institute of Dental Sciences and Hospital
Bengaluru – 560029 Seal and Signature of the Principal
Date: / / 2022
Place: Bengaluru

Seal and Signature of HOD

Date: / / 2022
Place: Bengaluru
Bangalore Institute of Dental Sciences and Hospital
Bengaluru -560029.

iv
 COPYRIGHT

DECLARATION BY THE CANDIDATE

I hereby declare that the Rajiv Gandhi University of Health Sciences, Karnataka shall

have the rights to preserve, use and disseminate this library dissertation in print or

electronic format for academic / research purpose.

Date: / / 2022 Signature of the candidate

Place: Bengaluru DR. BHAGYA BALAKRISHNAN

© Rajiv Gandhi University of Health Sciences, Karnataka.

5
ACKNOWLEDGEMENT

“Thankfulness is the beginning of gratitude. Gratitude is the completion of thankfulness.

Thankfulness may consist merely of words. Gratitude is shown in acts.‖

I am ever grateful to the ALMIGHTY for the immense shower of blessings in my life.

Though only my name appears on the cover of this library dissertation, a great many people

have contributed to its formation. I owe my gratitude to all those people who have directly

and indirectly contributed towards the completion of this dissertation and because of whom

my post graduate experience has been one that I will cherish forever.

I owe my sincere and deepest gratitude towards my Guide and Head of the Department, DR.

VATHSALA NAIK for her constant support and encouragement throughout my study

period. I have been amazingly fortunate to have her as my teaching faculty and mentor, who

gave me the freedom to explore on my own.

I wish to express my heartfelt gratitude toDr. AMANDEEP SODHI and Dr. SWAROOP R

TELKAR for all the support, guidance and encouragement.

I would also wish to extend my sincere gratitude toDr. SANGEETHA R,Dr.GAURAV and

Dr. GANGA G.K. for their immense guidance. They were instrumental in providing me with

the encouragement and support required to carry out this study.

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This journey would not have been possible without the unconditional love, support &

encouragement of my Parents, Mr. V. BALAKRISHNAN AND Mrs. GEETHANJALI

BALAKRISHNAN, my in laws Dr K.K.BHASKARAN, Mrs. MINI BHASKARANmy

sister Mrs.BHAVYA BALAKRISHNAN, my brother Mr. BINOY BALAKRISHNAN,

my husband Mr. SWAROOP K B and my dearest daughter LAKSHMI K. SWAROOP.

I am very thankful to my dearest senior PGs Dr. SUNITHA H M,

Dr. RASHMI J. KURUP AND Dr AGHINA PRADEEP and my beloved juniors Dr.

KUNTALIKA SARKAR and Dr. YAISANAfor their cooperation and support during my

library dissertation preparation.

I would extend my acknowledgement to Mr. K. SUBBA RAO, Dr. SWAIRA K., and Dr.

SIRI KRISHNA, Directors of Bangalore Institute of Dental Sciences for giving me an

opportunity to be a student in this esteemed institution and providing the facilities required.

I would also like to thank Dr. VINAYA S. PAI, Principal, Bangalore Institute of Dental

Sciences, for her continuous help, support, guidance, encouragement & support.

Date

Place : Dr. Bhagya Balakrishnan

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 CONTENTS

 Introduction 10

 Pain –Definition 11

 Neuroanatomy / Neurophysiology 12

 Neurotransmitters 16

 Pain pathways 18

 Theories of pain 22

 Classification of Oro-Facial Pain 29

 Principles of Oro-facial pain diagnosis 36

 Characteristics and reactions to pain 39

 Types of pain 40

 Effects of pain 43

 Assessment of pain 44

 Methods of pain control 48

 Differential diagnosisof Oro-Facial Pain 50

 Odontogenic pain 52

 Neuralgias 53

 Historic perspectives and Epidemology54

 Pathophysiology and mechanism of neuralgic pain 55

 Classification 57

 Trigeminal neuralgia 59

 Glossopharyngeal neuralgia 89

 Nervus intermedius neuralgia 97

 Post herpetic neuralgia 100

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  Occipital neuralgia 114

 Superior laryngeal neuralgia 123

 Supra orbital neuralgia 123

 Infra orbital neuralgia 124

 Burning mouth syndrome 128

 Primary headache disorders 140

 Migraine 140

 Trigeminal autonomic cephalgias 150

 Cluster headache 152

 Paroxysmal hemicrania 160

 Short lasting neuralgia from headache attacks with conjunctival

injection and tearing (SUNCT) 161

 Short lasting UnilaterallNeuralgic headache with Autonomic

symptoms (SUNA) 162

 Hemicrania continua 163

 Tension type headache 166

 Disorders of masticatory muscles 168

 Myofascial pain 170

 Atypical facial pain 190

 TMJ disorders 193

 Conclusion 209

 References 210

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Introduction

The International Association for the Study of Pain (IASP) defined pain as ―An unpleasant

sensory and emotional experience associated with actual or potential tissue damage, or

described in terms of such damage". It was recommended by the Subcommittee on

Taxonomy and adopted by the IASP Council in 1979. Orofacial pains refer to pains

experienced in the mouth, face, and head regions.They might stem from disorders

anddiseases of the temporomandibular joint and masticatory musculature, head and neck

muscle tension, neurovascular disorders (such as migraine), neuropathic disorders (suchas

trigeminal neuralgia), inflammations in the area (such as sinusitis or dental pulpitis), or dent

maxillofacial treatments. Pain in the teeth, mouth, face or head usually has a local cause,

often the sequelae of dental caries (odontogenic pain). Apart from dental caries and

periodontal diseases, musculoskeletal and neuropathological diseases are the most common

cause of orofacial pain However, psychogenic, neurological and vascular conditions, and

conditions where pain is referred from elsewhere, may be responsible. The real significance

to the patient of orofacial pain apart from the pain itself, can range from the benign to

potentially lethal conditions. Some orofacial pain or headaches have an obvious but relatively

unimportant cause (e.g., a hangover – caused mainly by the acetaldehyde resulting from

metabolism of alcohol); others types of pain have no obvious underlying organic pathology2

(and are thus termed medically unexplained symptoms [MUS], e.g., atypical facial pain);

some can threaten important faculties such as sight (e.g., giant cell arteritis) or even life (e.g.,

brain tumours).

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PAIN

Pain is a complex physical, psychological and social experience. Aristotle described it as a

―quale: a passion of the soul‖. Sternbach defined pain as 1) Personal and private sensation of

hurt; 2) A harmful stimulus that signals current or impending tissue damage; and 3) A pattern

of responses that operates to protect the organism from harm.Dorland‘s Medical dictionary

defines pain as a more or less localized sensation of discomfort, distress, or agony resulting

from the stimulation of specialized nerve endings. The International Association for the

Study of Pain (IASP) defined pain as ―An unpleasant sensory and emotional experience

associated with actual or potential tissue damage, or described in terms of such damage". It

was recommended by the Subcommittee on Taxonomy and adopted by the IASP Council in

19798.

STRUCTURAL ORGANISATION OF NERVOUS SYSTEM8

Fig: Organization of the nervous system

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CENTRAL NERVOUS SYSTEM

That part of nervous system which occupies the central axis of the body. It means brain &

spinal cord taken together.

PERIPHERAL NERVOUS SYSTEM

That part which lies outside the CNS i.e. in the periphery. Various nerve fibers, somatic as

well as sympathetic & parasympathetic are in the PNS.

AUTONOMIC NERVOUS SYSTEM

It maintains visceral activities of the body. Two types are

Sympathetic nervous system- originate in the spinal cord between T1&L2

Para sympathetic nervous system - consists of fibers leaving the CNS through the

cranial nerves 3,7,9,10&2,3, sacral spinal nerves

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 TYPES OF NERVES

 Afferent (Ascending) – transmit impulses from the periphery to the CNS.

 Sensory nerves are afferent nerves.

 Efferent (Descending) – transmit impulses from the CNS to the periphery.

 Motor nerves are efferent nerves & supply the muscle or an exocrine gland

Type A fibres

The fast conducting A-alpha, A-beta and A-gamma fibers carry impulses that induce tactile

&proprioceptive responses but not pain. It seems that pain is conducted by A-delta and

C fibers, but these are not specific for pain

Type of nerve fiber thickness velocity function conduction

A-alpha 13-20um 70-120m/sec Motor n., Fastest

myelinated Proprioception conduction

A-beta 6-13um 40-70m/sec Afferents for touch

myelinated

A-gamma 3-8um 15-40m/sec Intrafusal ms. Of spindle

myelinated

A-delta 1-5um 5-15m/sec Afferents for heat &

myelinated pain(fast)

B 1-3um 2.5-15m/sec Autonomic

myelinated preganglionicfibres

C 0.5-1um 0.7-1.5m/sec Afferent for slow pain Slowest

unmyelinated conduction

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NERVE ENDINGS

 A nerve ending is the termination of a nerve fiber in a peripheral structure.

 Nerve endings may be sensory (receptor) or motor (effector).

TYPES OF RECEPTORS

At the distal terminals of afferent nerves are specialized receptors that respond to physical

or chemical stimuli.

Three main groups:

 Exteroceptors

 Proprioceptors

 Interoceptors.

EXTEROCEPTORS

The receptors are located such that they are exposed to the surrounding external environment.

They mostly respond at the conscious level.

1. Merkel‘s corpuscles – Tactile receptors in the submucosa of the tongue and oral

mucosa.

2. Meissner‘s corpuscles – Tactile receptors in the skin.

3. Ruffini‘s – Sense pressure and warmth.

4. Krause – Sense cold

5. Free nerve endings – Perceive superficial pain and touch

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PROPRIOCEPTORS

 They are involved with the functioning of ANS. Most sensations are below

consciousness level.

1. Muscle spindles – Mechanoreceptors sense stretch (myotactic response)

2. Golgi tendon organs – Mechanoreceptors in tendons signal contraction and stretching

are responsible for nociceptive and inverse stretch reflexes.

3. Pacinian corpuscles – Perception of pressure.

4. Periodontal mechanoreceptors

5. Free nerve endings – Perceive deep somatic pain.

INTEROCEPTORS

 Involved with organs of involuntary functioning. They are sensory receptors

located in and transmit impulses from the viscera. Function mostly below

consciousness level.

1. Pacinian corpuscles – Perception of pressure.

2. Free nerve endings – Perceive visceral pain.

Associated with all vascular tissue, including endocardium is a network of sensory receptors

derived from myelinated nerve fibers called endnet, which provides sensory information from

these vessels.

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NEUROTRANSMITTERS

Nerve signal is transmitted from one neuron to next through interneuronal

junction‘ssynapse.Neurochemicals transmitting impulses across the synaptic cleft are known

as neurotransmitters.

 RAPID ACTING (small molecule): - NT-acetyl choline, norepinephrine, glutamate,

aspartate, serotonin, GABA, glycine, dopamine, histamine

 SLOW ACTING (large molecule): -Substance P, endorphins, bradykinin

Found in synapses

 Bradykinin - Many researchers have suggested that bradykinin might be the agent most

responsible for causing pain following tissue damage

 Substance P–it is thought to be responsible for the transmission of pain-producing

impulses,

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Two types of chemical neurotransmitters that mediate pain are

 Endorphins - morphine-like neurohormone; thought to  pain threshold by binding to

receptor sites

 Serotonin - substance that causes local vasodilation & permeability of capillaries. Both

are generated by noxious stimuli, which activate the inhibition of pain transmission

NEUROANATOMY OF PAIN

Fig: Pain pathway from periphery to the central nervous system.

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The transmission of painful impulses in the nervous system

1.Nociceptive afferent pain receptors

2.Dorsal horn of spinal cord

3.Ascending spinothalmic tract

4.Thalamus

5.Higher brain centres

6.Descending pain modifying pathways

PAIN PATHWAYS – ASCENDING LIMB

FIRST ORDER NEURONS9

 Stimulated by sensory receptors

 End in the dorsal horn of the spinal cord

 There are 3 classes of nociceptive afferent neurons which constitute the 1st order :

1. Mechanothermal afferents – Are usually A delta fibers with a velocity of

12 to 18m/s and respond to intense thermal and mechanical stimuli

2. Polymodal afferents – Are C fibers with a velocity of 0.5m/s and respond

to mechanical, thermal and chemical stimuli.

3. High threshold mechanoreceptive afferents – Are A delta fibers again

which respond to intense mechanical stimuli

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SECOND ORDER NEURONS

 The marginal cells and the cells of substantia gelatinosa form the second

orderneurons.

 Fibers from these cells ascend & terminate in ventral posterolateral nuclei of

thalamus.

 There are three types of second order neurons.

1. Low Threshold Mechanosensitive neurons (LTM)

2. Nociceptive specific neurons (NS)

3. Wide Dynamic Range neurons (WDR)

THIRD ORDER NEURONS

 They are the neurons of thalamic nucleus, reticular formation, tectum and grey matter

around aqueduct of sylvius& terminate on post central gyrus.

Dorsal horn of spinal cord is subdivided into 6 Laminae.Nociceptive Specific neurons(NS)

and Wide Dynamic Specific Neurons(WDR) found in the posterior grey column of the spinal

cord transmit stimulus to Laminae I, II and V.Low Threshold Mechanoreceptors(LTM)

transmits sensations to Laminae III and IV.The region of large number of excitatory

interneurons in Laminae II and III is Substantia Gelatinosa of Rolandi.Once the impulses

have been transferred from the primary afferents most of the second order neurons cross to

the opposite side of the spinal cord and enter the anterolateral spinothalamic tract composed

of small myelinated and unmyelinated fibers that transmit at 40m/s.Some 2nd order neurons

remain on the same side and ascend by leminscal system and cross over at the level of

medulla. This is composed of large myelinated nerve fibers that transmit at 30 to

110m/s.Anterolateral spinothalamic tract conducts at a slower rate and transmits pain,

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warmth, cold, crude tactile sensations.Lemniscal tract gives animmediate response. Pressure,

vibration, touch and proprioception is transmitted through it.

Descending Pain Modulation (Descending Pain Control Mechanism)

 Transmit impulses from the brain (corticospinal tract in the cortex) to the spinal cord

(lamina)

Fig: Descending pain pathway

 Periaquaductal Gray Area (PGA) – release enkephalins

 Nucleus Raphe Magnus (NRM) – release serotonin

The release of these neurotransmitters inhibits ascending neurons

PAINPROCESS

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 NOCICEPTORS

NOXIOUS PERIPHERAL
STIMULUS NEURALGIC
PATHWAY

PAIN

CNS
PERCEPTION
MECHANISMS
OF PAIN

Fig: Basic pain process

 Field- described the complex electrical &chemical events involved in the human pain

experience as

6a. NEOCORTICAL PERCEPTION

6b. PALEOCORTICAL PERCEPTION
(LOCALISATION)
(PROTOPATHIC)

CORTEX
7.SUPRASPINAL
REACTION

THALAMUS

MIDBRAIN

5.HT
SPINO-THALAMIC ACH
4.MODULATION
TRACT
3.CENTRAL 1.TRANSDUCTION
FACILITATION
PERIPHERAL
SPINAL CORD 2.TRANSMISSIO NOCICEPTOR
N

5.SPINAL REACTION
Fig: Field‘s description of pain pathway

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  TRANSDUCTION- noxious stimulus acts upon free nerve endings (pain receptors)

leading to electrical activity-> nerve impulse (action potential)

 TRANSMISSION- conveying impulse to CNS

 MODULATION-central neural activity controls pain signals

 PERCEPTION OF PAIN

THEORIES OF PAIN

1. INTENSITY THEORY

This theory was put forward by the Athenian philosopher Plato (c.428 to 347 B.C). He

defined pain not as a unique experience but as an emotion that occurs when the stimulus

is intense and lasting in his work Timaeus. Chronic pain represents a dynamic experience,

profoundly changeable in a spatial-temporal manner. A series of experiments, conducted

during the nineteenth century establishes the scientific basis of the theory. These

investigations, based on the tactile stimulation and impulses of other nature such as

electrical stimulations, provided important information concerning the threshold for

tactile perceptions and the role of the dorsal horn neurons in the transmission/processing

of pain.

2.CARTESIAN DUALISTIC THEORY

One of the first alternative scientific pain theories was introduced by Rene Descartes in

1644. This theory is given as Cartesian dualism theory of pain in current literature.it

hypothesized that pain was a mutually exclusive phenomenon; pain could be a result of

physical or psychological injury. However the two types of injury didn‘t influence each

other, hence making pain a mutually exclusive entity.Descartes also included in his theory

the idea that pain has a connection to the soul. He claimed that his research uncovered

that the soul of pain was in the pineal gland, consequentially designating the brain as the

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 moderator of painful sensations. It lacks an explanation as to why no two chronic pain

patients have the same experience with pain even if they had similar injuries

3.SPECIFICITY THEORY

This theory initially presented by Charles Bell (1774–1842) is similar to Descartes' dualistic

approach to pain in the way of identification of specific pathways for different sensory inputs,

Bell also postulated that the brain was not the homogenous object that Descartes believed it

was, but instead a complex structure with various components. One of the many contributors

to specificity theory was Johannes Muller. In the mid-1800s, Muller published in the Manual

of Physiology that individual sensations were the result of specific energy experienced at

certain receptors and that there was an infinite number of receptors in the skin, and this

surplus of receptors accounted for the ability of an individual to discriminate between

different sensations.In 1894, Maximillian von Frey made another critical addition to the

specificity theory by discovering that there are four separate somatosensory modalities found

throughout the body. These sensations include cold, pain, heat, and touch. This theory fails to

account for factors other than those of physical nature that result in the sensation of pain. It

also lacks an explanation for why sometimes pain persists long after the healing of the initial

injury. This incomplete nature of the specificity theory regarding pain etiology necessitated

additional theories and continued research.

4.PATTERN THEORY

The American psychologist John Paul Nafe (1888-1970) presented this theory in 1929. The

ideas contained in the pattern theory were directly opposite to the ideas suggested in the

Specificity theory in regards to sensation. Nafe indicated that there are no separate receptors

for each of the four sensory modalities. Instead, he suggested that each sensation relays a

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specific pattern or sequence of signals to the brain. The brain then takes this pattern and

deciphers it. Depending on which pattern the brain reads, correlates with the sensation felt. At

the time of its introduction, the pattern theory gained significant popularity among many

researchers. However, through further research and the discovery of unique receptors for each

type of sensation, it can be stated with certainty, that this theory is an inaccurate explanation

for how we feel pain.

In 1894 Goldscheider was the first to propose that stimulus intensity and centralsummation

are the critical determinants of pain.Theory suggested that particular patterns of nerve

impulses that evoke pain are produced by the summation of sensory input within the dorsal

horn of the spinal column.

 Pain results when the total output of the cells exceeds a critical level.

 For Example; touch+pressure+heat might add up in such a manner that pain was

the modality experienced.

5.GATE CONTROL THEORY

1965, Patrick David Wall (1925–2001) and Ronald Melzack announced the first theory that

viewed pain through a mind-body perspective. This theory became known as the gate control

theory. The gate control theory of pain states that when a stimulus gets sent to the brain, it

must first travel to three locations within the spinal cord.

 Substantia Gelatinosa (SG) in dorsal horn of spinal cord which acts as a ‗gate‘ – only

allowing one type of impulses to connect with the Second order neuron.

 Fibers in the dorsal column

 Transmission Cell (T-cell) – distal end of the SON

The sensation of pain that an individual feels is the result of the complex interaction among

these three components of the spinal cord. When the signal traveling to the spinal cord

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reaches a certain level of intensity, the ―gate‖ opens. Once the gate is open, the signal can

travel to the brain where it is processed, and the individual proceeds to feel pain. If A-beta

neurons are stimulated – SG is activated which closes the gate to A-delta & C neurons. If A-

delta & C neurons are stimulated – SG is blocked which closes the gate to A-beta neurons.

Impulses from faster fibers arriving at gate 1st inhibit pain impulses (acupuncture/pressure,

cold, heat, chem. skin irritation).

The Gate Control Theory was one of the first to acknowledge that psychologicalfactors

contributed to pain as well. Current research has also suggested that a negative state of mind

serves to amplify the intensity of the signals sent to the brain as well. In their original study,

Melzack and Wall suggested that in addition to the control provided by the substantia

gelatinosa, there was an additional control mechanism located in cortical regions of the brain.

6.Neuromatrix Model

Almost thirty years after introducing the gate control theory of pain, Ronald Melzack

introduced another model that contributed to the explanation of how and why people feel

pain. Although Melzack had contributed to these previous theories, it was his exposure to

amputees that were experiencing phantom limb pain in well-healed areas that prompted his

inquiry into this more accurate philosophy of pain. The theory he proposed is known as the

neuromatrix model of pain. This philosophy suggests that it is the central nervous system that

is responsible for eliciting painful sensations rather than the periphery.The neuromatrix

model denotes that there are four components within the central nervous system responsible

for creating pain namely

a. the ―body-self neuromatrix,

b. the cyclic processingand synthesis of signals,

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 c. the sentinel neural hub,

d. and the activation of the neuromatrix.‖

According to Melzack, the neuromatrix consists of multiple areas within the central nervous

system that contribute to the signal, which allows for the feeling of pain. These areas include

the spinal cord, brain stem and thalamus, limbic system, insular cortex, somatosensory

cortex, motor cortex, and prefrontal cortex.The signal that these areas of the central nervous

system work together to create is responsible for allowing an individual to feel pain is

referred to as the ―neurosignature.‖ The input coming in from the periphery can initiate or

influence the neurosignature, but these peripheral signals cannot create a neurosignature of

their own.This idea that peripheral signals can alter the neurosignature is an important

concept when considering the effect that nonphysical factors have on an individual‘s

experience with pain. Melzack‘s theory claimed that not only are there specific

neurosignatures that elicit certain sensations, but when there is an alteration in a certain

signal, this allows for memory formation of these particular experiences. If the same

circumstances occur again in the future, it is this memory that allows for the same sensation

to be felt. In addition to the hypothesis that pain was a product of different patterns of signals

from the central nervous system, the neuromatrix model continued to elaborate on the idea

that was initially brought forward in the gate control theory, that pain can be affected not only

by physical factors but by cognitive and emotional factors. Melzack suggested that

hyperactivity of the stress response has a direct effect on pain. Hyperactivity of the stress

response is when an individual exposed to increased levels of stress experiences a higher

level of pain. Taking all of these claims into consideration, it is evident that pain is a complex

issue that cannot be accounted for by physical factors alone. Even though the neuromatrix

model further established the idea that pain gets influenced by cognitive and emotional

factors as well as physical factors, it still fails to account for social constructs of pain.

26
Therefore, a new theory of pain must be utilized to appropriately explain the mechanism

behind pain and why each individual‘s experience with pain is unique.

7.Biopsychosocial Model

The biopsychosocial model provides the most comprehensive explanation behind the etiology

of pain. This specific theory of pain hypothesizes that pain is the result of complex

interactions between biological, psychological, and sociological factors, and any theory

which fails to include all of these three constructs of pain, fails to provide an accurate

explanation for why an individual is experiencing pain. Although the term biopsychosocial

was not introduced until 1954 by Roy Grinker (1900-1993), a neurologist and psychologist,

there have been many physicians who had considered the utility of using such a model to

approach the management of a patient‘s pain long before this.One of the most prominent

physicians who utilized this more comprehensive approach to pain was John Joseph Bonica

(1917-1994), a Sicilian American anesthesiologist at Madigan Army Hospital, known as the

founding father of the discipline of pain medicine. In the 1940s, Bonica was caring for many

patients who had returned home from World War II and were now experiencing debilitating

pain due to injuries they had suffered in the war. He had recognized that the pain these

wounded soldiers were experiencing was rather complex and not easily managed. This

situation led him to propose that to adequately manage these patients, physicians needed to

create interprofessional pain clinics comprising multiple disciplines. At this moment in

history, there was little support for the idea that pain was more than just the result of an

injury, and Bonica was relatively unsuccessful in establishing these clinics. It wasn‘t until

1977 that the biopsychosocial model was scientifically suggested as an explanation for the

etiology of some medical conditions. George Engle claimed that to treat disease adequately,

one must consider multidimensional concepts and manage the whole patient instead of

focusing on a single issue. This methodology takes into account that the human body cannot

27
be divided into separate categories when considering treatment options. Instead, it is

beneficial to acknowledge the fact that illness and disease are the results of complex

interactions between biological, psychological, and sociological factors, and they all affect an

individual‘s physical and mental well-being. Although Bonica had technically been the first

physician to comprehend the importance of using a biopsychosocial approach to pain, John

D. Loeser, another anesthesiologist, has been credited as the first person to use this model in

association with pain.Loeser suggested that four elements need to be taken into consideration

when evaluating a patient with pain. These elements include nociception, pain, suffering, and

pain behaviours. Nociception is the signal that is sent to the brain from the periphery to alert

the body that there is some degree of injury or tissue damage. Pain, on the other hand, is the

subjective experience that occurs after the brain has processed the nociceptive input. The last

two components of pain that merit consideration is suffering and pain behaviours. The

thinking is that suffering is an individual‘s emotional response to the nociceptive signals and

that pain behaviours are the actions that people carry out in response to the experience of

pain. Both of these can be either conscious or subconscious. Loeser‘s four elements of pain

account for the biological, psychological, and sociological factors that can create or influence

an individual‘s experience with pain. Failing to consider any one of these four elements when

determining the cause or establishing a management plan could be a consideration as

inadequate assessment or care.Loeser‘s findings prove that the Biopsychosocial Model of

pain offers the most comprehensive philosophy and provides the framework that is needed to

start appropriate therapy to manage patients with chronic pain adequately.

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CLASSIFICATION OF ORO-FACIAL PAIN

(A)International Headache Society‘s classification of headache disorders, cranial neuralgias,

and facial pain

 The primary headaches

1. Migraine

2. Tension-type headache

3. Cluster headache and other trigeminal autonomic cephalalgias

4. Other primary headaches,

 The secondary headaches

1. Headache attributed to head and neck trauma

2. Headache attributed to cranial or cervical vascular disorder

3. Headache attributed to non-vascular intracranial disorder

4. Headache attributed to a substance or its withdrawal

5. Headache attributed to infection

6. Headache attributed to disturbance of homoeostasis

7. Headache or facial pain attributed to disorder of cranium, neck, eyes, ears,

nose, sinuses, teeth, mouth or other facial or cranial structures

8. Headache attributed to psychiatric disorder cranial neuralgias, central and

primary facial pain and other headaches

9. Cranial neuralgias, central causes of facial pain

10. Other headache, cranial neuralgias, central and primary facial pain

29
(B)The IASP classification, originally published in 1986 and revised in 1994, is composed of

five axis.

Axis Definition

1 Regions (eg: head, face and mouth)

2 Systems (eg: nervous system)

3 Temporal characteristics of pain (eg: continuous,recurring irregularity,

paroxysmal)

4 Patients statement of intensity: time since onset of pain

(eg. mild, medium, severe;, month or less, more than 6 months)

5 Etiology (genetic, infective, psychological)

(C)According to American Academy of Orofacial Pain Classification

 Intracranial pain disorders – Neoplasm, Aneurysm, abscess, Hemorrhage,

Hematoma, and Edema.

 Primary headache disorders: Migraine, Migraine variants, Cluster Headache,

Paroxysmal Hemicrania, Cranial Arteritis, Carotodynia, Tension type Headache.

 Neurogenic pain disorders:Paroxysmal Neuralgias (trigeminal,

Glossopharyngeal, Nerves Intermedius, and Superior Laryngeal).

 Continuous pain disorders: Deafferentiation, Neuritis, Post Herpetic Neuralgia,

Post Traumatic and Post Surgical Neuralgia, Sympathetically maintained pain.

 Intra oral pain disorders: Dental pulp, Periodontium, Mucogingival tissues,

Tongue.

30
  Temporomandibular disorders: Masticatory muscle, Temporomandibular joint,

Associated structures.

 Associated structures: Ears, Eyes, Nose, Paranasal sinuses, Throat,

Lymphnodes, Salivary glands, Neck.

Classification of Localized Syndromes of Head and Neck (IASP)

 Neuralgias of the head and face.

 Craniofacial pain of musculoskeletal origin.

 Lesions of the ear, nose and oral cavity.

 Primary headache syndromes, vascular disorders, and cerebrospinal fluid

syndromes.

 Pains of psychological origin in the head, face and neck.

 Sub occipital and cervical musculoskeletal disorders.

 Vascular pain in the neck.

31
Orofacial pain classification as proposed by Okeson JP

32
Classification of Chronic orofacial pain by Burket

 NEURALGIAS

1. Primary trigeminal neuralgia (tic douloureux)

2. Secondary trigeminal neuralgia (CNS lesion, facial trauma)

3. Herpes zoster

4. Post-herpetic neuralgia

5. Geniculate neuralgia

6. Glossopharyngeal neuralgia

7. Superior laryngeal neuralgia

8. Occipital neuralgia

 PAIN OF MUSCULOSKELETAL ORIGIN

1. Cervical osteoartheritis

2. TMJ disorders

3. myofascial pain dysfunction

4. Fibromyalgia

5. Cervical sprain or hyperextension

6. Stylohyoid ( Eagle‘s) syndrome

 PRIMARY VASCULAR DISORDERS

1. migraine with aura

2. migraine without aura

3. cluster headache

4. tension type headache

5. mixed headcahe

6. cranial arterits

7. carotodynia

33
 8. hypertensive vascular changes (embolism, aneurysm)

 PSYCHOGENIC PAINS

1. Delusion / hallucinatory

2. Hysterical / hypochondriac

 GENERALIZED PAIN SYNDROMES

1. Post traumatic sprain

2. Causalgia

3. Phantom pain

4. Central pain

 LESIONS OF EAR NOSE & ORAL CAVITY

 maxillary sinusitis

 Otitis media

 Odontalgia – dentin defect, pulpitis, periapical pathology etc.

 Atypical odontologia

 Periodontal pathology

 Occlusal trauma

 Dental impaction

 Cysts and tumors

 Osteitis

 Mucocutaneous disease

 Salivary gland disease

 Atypical facial pain

 Glossodynia

34
 Classification of facial pain according to International Classification Of
Orofacial Pain ,1st edition ,2020 ( ICOP) .

1. Orofacial pain attributed to disorders of dentoalveolar and anatomically related

structures
1.1 Dental pain
1.2 Oral mucosal, salivary gland and jaw bone pains
2. Myofascial orofacial pain
2.1 Primary myofascial orofacial pain
2.2 Secondary myofascial orofacial pain
3. Temporomandibular joint (TMJ) pain
3.1 Primary temporomandibular joint pain
3.2 Secondary temporomandibular joint pain
4. Orofacial pain attributed to lesion or disease of the cranial nerves
4.1 Pain attributed to lesion or disease of the trigeminal nerve
4.2 Pain attributed to lesion or disease of the glossopharyngeal nerve
5. Orofacial pains resembling presentations of primary headaches
5.1 Orofacial migraine
5.2 Tension-type orofacial pain
5.3 Trigeminal autonomic orofacial pain
5.4 Neurovascular orofacial pain
6. Idiopathic orofacial pain
6.1 Burning mouth syndrome (BMS)
6.2 Persistent idiopathic facial pain (PIFP)
6.3 Persistent idiopathic dentoalveolar pain
6.4 Constant unilateral facial pain with additional attacks (CUFPA)

The first three categories are diseases concerning adjacent structures and are usually
treated by dentists; groups 4–6 comprise facial pain syndromes in which no

35
 morphological correlate of the teeth, periodontium, or temporomandibular joint
explains the pain.

PRINCIPLES OF PAIN DIAGNOSIS

The examination and assessment of patients with chronic orofacial pain is challenging for all

the clinicians.In most disorders, no specific biologic marker validated diagnostic criteria

orgold standard exists. A systematic approach for collecting diagnostic information is needed

to minimize the risk of missing critical information.

Identifying the true source of pain

History, physical examination, and behavioral assessment usually serve as the basis for

diagnosis. Frequent re-evaluation including assessment of the effects of treatment is an

important part of this.A description of the pain complaint should take into account

detailsconcerning the following:

 Anatomic location of Pain

 Intensity of pain

 Mode of onset

 Manner of flow of pain

 Quality of pain

 temporal behavior

 Duration of individual pains.

 localization behavior

 Effect of functional activities

 Concomitant symptoms.

36
Location of pain

The patient‘s ability to locate the pain with accuracy has diagnostic value, but the examiner

should guard against assuming that the site of pain necessarily identifies the true source of

pain – the structure from which the pain actually emanates.

Intensity of pain

The intensity of pain should be established by distinguishing between mild and severe pain.

This can be based on how the patient appears to react to the suffering – mild pain being what

the patient describes but to which he displays no visible physical reactions and severe pain

being what causes muscular and autonomic effects that are objectively evident to the

examiner

Mode of onset

The mode of onset of individual pains is important diagnostically. The onset may be wholly

spontaneous. It may be induced by certain activities such as yawning, chewing, drinking hot

or cold liquids, or bending over. It may be triggered by major superficial stimulation such as

touch or movement of the skin, lips, face, tongue or throat.

Manner of flow of pain

The manner of flow yields important information by determining whether the individual

pains are steady or paroxysmal.

Quality of pain

The quality of pain should be classified according to how it makes the patient feel. This

classification is usually termed bright or dull. When the pain has a stimulating or exciting

37
effect on the patient it is classified as bright when the pain has a depressing affect that causes

the patient to withdraw to some extent, it is classified as dull.

Further evaluation of the quality of pain that constitutes the patients complaint should be

made to classify it as pricking, itching, burning, aching or pulsating. Bright, tingling pain is

classified as a pricking sensation. Superficial discomfort that does not reach pain threshold

intensity may be described as itching. As intensity increases, it may take on a pricking

aching or burning quality.

Deep discomfort that does not reach pain threshold intensity may be described as a vague,

diffuse sensation of pressure, warmth or tenderness. As intensity increases it may take on

sore, aching throbbing or burning quality. When the discomfort has an irritating, hot, raw

caustic quality it is usually described as burning. Most pains have an aching quality. Some

noticeably increase with each heart beat and are described as pulsating.

Temporal behavior

Reflects the frequency of pain as well as the periods between episodes of pain. If the

suffering comes and goes leaving pain free intervals of noticeable duration, it is classified as

intermittent. If such pain free intervals do not occur, it is classified as continuous.

Duration of individual pains

A pain is said to be momentary if its duration can be expressed in seconds. Longer lasting

pains are classified in minutes, hours or a day. A pain that continuous from one day to the

next is said to be protracted.

Localization behavior

If the patient is able to define the pain to an exact anatomic location, it is classified as

localized pain. If such description is less well defined and somewhat vague and variable

38
anatomically, it is termed diffuse pain. Rapidly changing pain is classified as radiating. A

momentary cutting exacerbation is usually described as lanciating. More gradually changing

pain is usually described as spreading and if it progressively involves adjacent anatomic

areas, the pain is called as enlarging.

 Activities that increase or decrease pain.

 Associated symptomssuch as hyperesthesia, anesthesia, hypoesthesia,

parasthesia, and swelling should be noted.

Any concomitant change in special senses affecting vision, hearing, smell or taste should

be noted.

CHARACTERISTICS OF PAIN

THRESHOLD AND INTENSITY.

If the intensity of the stimulus is below the threshold, pain is not felt.As the intensity

increases more and more pain is felt more and more and the pain sensation spreads.However,

if the mind is distracted the threshold of pain increases.

ADAPTATION

Pain receptors show no adaptation and so the pain continues as long as the receptors continue

to be stimulated.

LOCALIZATION OF PAIN

Pain sensation is somewhat poorly localized. Superficial pain is comparatively better

localized than the deep pain.

EMOTIONAL ACCOMPANIMENT.

Pain sensations are commonly accompanied by emotions. These emotions as a rule are

unpleasant.

INFLUENCE OF THE RATE OF DAMAGE ON THE INTENSITY OF PAIN

39
If the rate of tissue injury is high, intensity of pain is also high and vice versa.

Eg: cancers in the beginning are mostly painless.

REACTIONS TO PAIN

 BEHAVIOURAL

This includes crying, moaning, whining. In long standing pain frustration, mental irritation or

depression can develop.

 MUSCULAR

Spasm of the skeletal muscles in the affected region develops.

It causes immobilization or ischemia.

 CHANGES IN THE ANS.

Somatic pain is usually accompanied by signs of sympathetic over activity eg: rise of BP,

tachycardia and pupillary dilation

Conversely in visceral pain there is often fall of BP and vomiting.

 REFLEX RESPONSE.

A painful stimulus is usually associated with somatic reflexes eg: pin prick and withdrawal of

hand

TYPES OF PAIN

1. Clinical vs. experimental pain: Pain as presented by patients i.e. the pathologic or clinical

pain is different from the experimental pain that is induced and is studied in a

laboratory.They are explained by different mechanisms, it is likely that the emotional

significance embodied in clinical pain is the real determinant.

40
2. Acute vs. chronic pain:

Acute pain

It is caused by an injury to the body.It can develop slowly or quickly. It can last for a few

minutes to six months and goes away when the injury heals.

Chronic pain

It persists long after the trauma has healed (and in some cases, it occurs in the absence of any

trauma). It usually lasts longer than six months.There is a definite relationship between

duration and intensity of pain. The higher the intensity, the shorter the period the pain can be

tolerated by the sufferer. Low-intensity pain can be sustained for about 7 hours, whereas pain

of maximum intensity can hardly be sustained for a few seconds.

3. Primary vs. secondary pain:

The site where pain is felt may or may not identify the location of the source of the pain. If

the pain does emanate from the structures that hurt then it constitutes a primary nociceptive

input. If however the true source of pain is located elsewhere, the area of discomfort

represents secondary pain, secondary pains are called heterotopic pain.

4. Stimulus evoked vs. spontaneous pain: Most primary somatic pains result form

stimulation of neural structures at the site. The clinical characteristics displayed by stimulus

evoked pain relates to the location, timing, and intensity of the stimulus.

Neuropathic pain maybe felt spontaneously along the distribution of the affected nerve.

Heterotopic pain occurs spontaneously as far as the site of pain is concerned.

41
5. Somatic pain

Noxious stimulation of somatic structures, sensory nervous system.

It can be Superficial or Deep- musculoskeletal.

6. Neuropathic pain

Chronic state due to a series of changes in the nervous system. CNS has been sensitized

by repetitive direct or indirect injury.

7. Psychogenic pain – individual feels pain but cause is emotional rather than physical

8. Referred pain

 Visceral pain is often referred, that is pain is not felt over the area where the viscus is

situated but is felt somewhere else.

Fig: Referred pain

42
EFFECTS OF PAIN

Physiological effects of Pain

 Increased catabolic demands: poor wound healing, weakness, muscle breakdown

 Decreased limb movement: increased risk of DVT

 Respiratory effects: shallow breathing, tachypnea, cough suppression increasing risk

of pneumonia , atelectasis, tachycardia and elevated BP

Psychological effects of Pain

 Negative emotions: anxiety, depression

 Sleep deprivation

 Existential suffering: may lead to patients seeking active end of life.

Immunological effects of Pain

 Decrease natural killer cell counts

 Effects on other lymphocytes not yet defined

43
ASSESSMENT OF PAIN

In the assessment of pain intensity, rating scale techniques are often used. The most

commonly used forms are

1.TheVisualAnalogueScales(VAS)

(e.g. 10 cm line with anchor points at each end). The VAS has been shown to be more

sensitive to change and is therefore more widely used.

2. Wong-Baker FACES Pain Rating Scale

3. MC GILL PAIN QUESTIONNAIRE

Verbal pain scale.

44
 Uses vast array of words commonly used to describe pain empirically.

Items

 1-10 ,18 &19-character or sub-modality of pain

 11-16 and 20- indicate affective (emotional ) pain components

 17-concerns localization

 PPI- patients pain intensity rating

 accompanying symptoms

4. Turk and Rudy have developed the Multiaxial Assessment of Pain (MAP)

classification. Their assessment included a 61-item questionnaire West Haven-Yale

45
 Multidimensional Pain Inventory (WHYMPI), which measures adjustment to pain from a

cognitive behavioral perspective.

5. Dworkin, LeResche and collegues have developed a method for assessing dysfunctional

chronic pain as a part of classification system, the Research Diagnostic Criteria.

 They used:

a) The Graded Chronic Pain severity scale,

b) Depression and vegetative symptom scale,

c) Jaw disability checklist.

6. One of the other scales that can be used to measure pain is Pictorial representation of

illness and self measure or (PRISM).

Prism recording form: where the circle represents self

46
OROFACIAL PAIN SYMPTOMS THAT INDICATE SERIOUS DISEASE.

Pain at the angle Cardiac ischemia

New onset, localized, headache, superficial temporal a. Temporal arteritis.

swelling, transient visual abnormalities, systemic symptoms

New onset of headache in adult life, nausea, vomiting, Intracranial tumor

nocturnal occurrence, neurologic signs

Earache, trismus, altered sensation in the mandibular branch Carcinoma of the

distribution infratemporal fossa

Trigeminal neuralgia <50yrs Multiple sclerosis

Pain associated with altered sensation Neurogenic disorder,

tumor invasion of nerve

OBSERVABLE PAIN BEHAIVOURS

47
Guarding Abnormally slow, still or interrupted movement.

Bracing Stiff, pain avoidant posturing when in a static

position.

Rubbing Touching, rubbing or holding of the painful area.

Sighing Pronounced exhalation of air.

Grimacing Obvious facial expressions of pain.

TREATMENTOFPAIN

Goals Of Therapy

 Decrease the frequency and / or severity of the pain

 General sense of feeling better

48
  Increased level of activity

 Return to work

 Elimination or reduction in medication usage

METHODS OF PAIN CONTROL

1. Removing the cause

It is the most desirable method.it affects pain perception.

Elimination of cause

No more excitation of free nerve endings

No more impulses

2. Blocking the pathway of painful impulses

it is the most widely used method in dentistry.it affects pain perception

LA injected into tissues in proximity to nerve

Prevents depolarization of nerve fibers

Conduction blockade

3.Raising the pain threshold

Pain perception is reduced at first synapse.Pain threshold is increased to limited degree only,

depending upon the type of drug.E.g. NSAIDs &Opioids .Opioids are able to raise pain

threshold to a greater degree than NSAIDs.Certain drugs also act on cortical & subcortical

areas & reduces fear, anxiety & apprehension.

49
4. Preventing the pain reaction by cortical depression

Cortical depression causes

a. Loss of all sensations, especially pain

b. Sleep (unconsciousness) & amnesia

c. Immobility & muscle relaxation

d. Abolition of reflexes

E.g. general anesthetic agents

5.Using psychosomatic methods

It affects both pain perception & pain reaction. Dentists should have honesty & sincerity

towards the patient.Keep the patients well informed about the procedure.Avoid surprises &

rapid movements.Patient‘s central control mechanism aids in pain control.Better than

pharmacologic method with no side effects.

Differential diagnosis of Orofacial pain(American Academy of Orofacial Pain

Classification)

Intracranial pain disorders  Neoplasm, aneurysm, hemorrhage, Abscess,

edema, hematoma

Primary headache  migraine, migraine variants, cluster headache,

disorders(neurovascular paroxysmal hemicrania, cranial arteritis

disorders)  Carotodynia,tension type headache

Neurogenic pain  paroxysmal-TGN, GPN;

 continuous-post herpetic neuralgia, neuritis

Intraoral pain disorder  pulp, periodontium, mucogingiva, tongue

50
Temporomandibular  TMJ joint, masticatory muscle

Associated structures  Ears, eyes, nose, paranasal sinuses, throat, lymph

nodes, salivary glands, neck

VASCULAR PAIN

MIGRAINE CLUSTER ARTERITIS

Throbbing, unilateral, Throbbing intense ache, Throbbing with

frontal, temporal, periorbital,20-50yrs, burning ache, area of

young with history smoking habit artery,>50 yrs

1-2days, Episodic Min-hrs, cluster of days to Hrs-days, tender

Visual, GI, cold weeks, lacrimation, swollen artery, fever,

extremities, sensitive perspiration no prodrome malaise

Diet, tyramine, alcohol, vasodilators Continuous

hypoglycaemia, stress,

HT, alcohol

MAO inhibitors ,stress, Stress, tension, vasodilators Lying position,

estrogen imbalance mastication

Vasoconstrictors, Vasoconstrictors, Pressure in artery,

holding head still distractors upright position

Family history, relief Brought on by sublingual Inflammation-

with ergot nitroglycerin, family biopsy,>ESR

history

MUSCULAR/ JOINT (TMJ PAIN)

51
MUSCULARTMJ

Ache, steady, head, neck, shoulders Steady ache, in ear, periauricular

pain can move.

Constant, nonprogressive, all ages, Fluctuates, staging, all ages limited

limited movement, tenderness, motion, crepitus clicking

Stress, bruxism, trauma, occlusion, jaw Aging, trauma

opening

Exercise, cold weather, systemic Movement, chewing, yawning, occlusal

disorders, stress disharmony

Massage ,heat, exercise Rest, heat, cold

Palpation of muscle trigger point, Examine, radiograph, associated with

referral, injection history myofascial pain

CANCER PAIN

Pain may occur because of progression of the disease, treatment procedures, recurrence of

uncontrolled disease following treatment.Stimulation of mucosal & sub mucosal nerve

endings, tumor infiltration of peripheral nerves, ulceration & infection.Pain may be felt at

the primary site, referred to another site or both

ODONTOGENIC PAIN

Generally, the pathology of orofacial pain is most commonly caused by the disease of the

teeth (odontogenic pain), which is a domain of dental medicine and it should not be a

diagnostic-therapeutic challenge in itself.

Caries:

52
Caries occurs through bacterial invasion of the tooth structures, resulting in decay caused by

formation of acid metabolites.it may occur on enamel, dentinand cementum on exposed root

surfaces. Patients may complain of thermal sensitivity or sensitivity when exposed to sweet

or acidic foods. Pain is sharp, localized and dissipates immediately after removal of stimuli.

Sensitivity derives from lost enamel and increased exposure of dentin and cementum.

Caries is detected and diagnosed both clinically and radiographically. Management of dental

caries varies by the size of the lesion as well as the state of the caries.

53
NEUROPATHIC PAIN

According to the definition proposed by the International Association for the Study of Pain

(IASP), the term neuropathic pain refers to all pains initiated or caused by a primary lesion

ordysfunction of the nervous system8.

Neuralgia is defined as an intense burning or stabbing pain caused by irritation of or

damage to a nerve. The pain is usually brief but may be severe. It often feels as if it is

shooting along the course of the affected nerve. Neuralgia is a neuropathic pain felt in the

distribution of nerve or nerves5.

Historic perspectives:

John Hunter, over 200 years ago, was the first to describe neuropathic orofacial pain.

There is one disease of the jaw which seems in reality to have no connection to the teeth, but

of which the teeth are generally suspected to be cause. This deserves to be taken notice of in

this place, because operators have been deceived byit,& even sound teeth have sometimes

been extracted through an unfortunate mistake9,10.

This pain is seated in some part of the jaws. As simple pain demonstrates noting a tooth

is often suspected & is perhaps drawn out, but still the pain continues, with this difference,

however, that it now seems to be the root of next tooth, it is then supposed by either patient or

the operator that the wrong tooth was extracted10.

In recent years there has been great progress toward understanding the etiology of

neuropathic pain, however, much is still unknown, it is apparent that some neuropathic pain

has a strong peripheral component, while others seem to have a significant central

component. Even within one specific neuropathic pain disorder there are often both

peripheral & central mechanisms3,6,10.

Epidemology:

54
 Neuropathic pain, including neuropathic orofacial pain (NOP), is believed to be prevalent.

However, there is a lack of accurate data to support this, partly due to inconsistent definitions

& clinical criteria. In primary medical care settings the prevalence of neuropathic pain is

between 2 & 11%(Clark 2002; Hasselstrom et al 2002; Koleva et al 2005) .Out of 300

consecutive patients visiting orofacial pain clinic 21% were diagnosed as NOP, most of

whom suffered from painful traumatic neuropathy (10%) or classical trigeminal neuralgia

(9%). In one recent study the prevalence of pain of predominantly neuropathic origin

questionnaire & revealed that about 8% of a British population suffered from neuropathic like

pain. The epidemiology of four common conditions, postherpetic neuralgia, trigeminal

neuralgia, phantom pain & painful diabetic neuropathy, is around 83.8 per 100000 person

(Hall et al 2006). Increased life expectancy & disease survival rates will increase the

prevalence of age associated neuropathic pain syndromes such as trigeminal neuralgia or

AIDS related or diabetic neuropathies8.

Neuropathic pain and its treatment lead to impaired quality of life, reduced employment

and productivity and extensive usage of healthcare facilities (Meyer-Rosberg et al 2001;

McDermott et al 2006). Chronic neuropathies are characterized by pain of moderate to severe

intensity. Treatment often requires long term prescription medications that have significant

side effects (McDermott et al 2006)8,9,10.

Pathophysiology of neuralgic pain:

Kerr & Miller have shown that significant pathologic changes occur in the myelin sheath

of fibers in the ganglion, dorsal root, or both. These changes are recognizable by both light &

electron microscopy, consists of disintegration of myelin sheath -i.e, demyelination. There is

some evidence that demyelination results from a progressive degenerative process.

55
Demyelination affects the fibers in such a way as to permit ephatic exchange of neural signals

at the site of neuropathy8,9,10.

There are several reasons for demyelination of nerve fiber. It may be associated with a

structural abnormality, where an adjacent structure applies constant force to nerve root. In

case of trigeminal neuralgia, the nerve root can be affected in the posterior fossa as it exists

the pons before reaching the gasserianganglion . One source of such compression is an

aberrant loop of the superior cerebrellar artery that lies on the nerve root & produces a

significant microvascular compression. This compression is thought to cause the

demyelination of the nerve9.

Other source of compression that need to be considered are space occupying lesions in

this region of the brain, the most frequent of which is a cerebellopontine angle tumor.

Posterior fossa tumors or basilar artery aneurysms are also possible sources, along with a

meningioma of meckel‘s cavity or epidermoid cyst. It is established that malignant tumors

that initiating pain are extramedullary. Destructive lesions of the nerve located within the

brain tissue from the entrance of the trigeminal nerve into the pons throughout the numerous

sensory circuits to the cortex donot cause neuralgias9.

Demyelination of the trigeminal nerve may result from systemic condition, such as

multiple sclerosis. Approximately 1 out of every 100 multiple sclerosis patients suffer from

trigeminal neuralgia. Multiple sclerosis may present with pain initially, once established, may

continue throughout the course of disease, occasionally the pain may be paroxysmal

neuralgia8,9.

Mechanism of neuralgic pain:

It is noticeable that the conflict always involves the proximal portion of the trigeminal

root, the so-called root entry zones (REZ). Myelin at this level is still of central type and this

56
could impact the resistance of the nerve to mechanical stimuli. The compression probably

causes a mechanical twist of the fibres and their secondary demyelination, probably mediated

by microvascular ischemic damages. These changes can significantly lower the excitability

threshold and promote an inappropriate ephaptic propagation towards adjacent fibres .

Because of the cross-talk, the tactile signals coming from the fast myelinic fibres (A-beta)

can directly activate the slow nociceptive fibres (A-delta) and produce repetitive high-

frequency discharges responsible for the typical ―lightening‖ pain triggered by tactile stimuli.

After a few seconds or minutes the repetitive discharges spontaneously run out and are

followed by a brief period of inactivity that explains the ―refractory period‖ clinically evident

in many cases. The aetiological role of the focal demyelination of the REZ also explains the

increased prevalence of TN in subjects with MS, in which demyelinated foci in the mid-pons

and the REZ are usually demonstrated11.

It has been suggested that a hypersensitivity of the tactile A-beta nerve fibres, initiated by

the same nerve injury, leads to a sensitization of the wide dynamic range neurons (WDRn) .

These peculiar nociceptive neurons are placed both in the V lamina of the dorsal horns and in

the trigeminal nerve nuclei and are characterized by a progressive facilitation of excitability

following repeated stimulation. Since these peculiar nociceptive II order neurons receive

convergent information from both tactile A-beta and nociceptive A-delta and C fibres, their

sensitization can ultimately facilitate nociceptive input at the central level while promoting

the perception of pain in response to tactile stimuli, a phenomenon called allodynia.

Allodynic sensitisation of thalamic nociceptive neurons has been recently documented in TN

patients presenting with a continuous pain between attacks11.

Classification of cranial neuralgias:

57
 I. IHS classification of headaches for cranial neuralgias & central causes of

facial pain7,8,9,11

1. Trigeminal neuralgia

i. Classical trigeminal neuralgia

ii. Symptomatic trigeminal neuralgia

2. Glossopharyngeal neuralgia

i. Classical glossopharyngeal neuralgia

ii. Symptomatic glossopharyngeal neuralgia

3. Nervus intermedius neuralgia

4. Superior laryngeal neuralgia

5. Nasociliary neuralgia

6. Supraorbital neuralgia

7. Other terminal branch neuralgia

8. Occipital neuralgia

II. Neuropathic pain20

Paroxysmal Continuous

1. Trigeminal neuralgia 1. Postherpetic neuralgia

58
 2. Glossopharyngeal neuralgia 2. Post – traumatic neuralgia

3. Nervus intermedius neuralgia 3. Atypical odontalgia

4. Occipital neuralgia

5. Neuroma

TRIGEMINAL NEURALGIA:

Synonyms – Tic douloureux, Fothergill‘s disease, Trifacial Neuralgia.

Definition: Trigeminal neuralgia is defined by IASP as ―a sudden, usually unilateral, severe,

brief, stabbing, recurrent pain in the distribution of one or more branches of the fifth cranial

nerve‖

According to International Headache Society (IHS) it is a painful unilateral affliction of

the face, characterized by brief electric shock like pain limited to the distribution of one or

more divisions of the trigeminal nerve8,9,12,15.

Historical perspective:

Hundreds of years ago Trigeminal neuralgia was thought to be caused by evil spirits and/or

mental disorders. More recently, has it been recognized as a true neuropathy. Trigeminal

neuralgia has long been recognized in the medical literature; infact, it was described as early

as the first century AD in the writings of Aretaeus of Cappodocia& those of Arab physician

Jujani in 11th century A.D. It was later discussed by Johannes Bausch in 1672.One of the first

complete description of TN was given by the English philosopher John Locke. In a letter

dated december 1677, he described his encounter with the wife of ambassador, the countess

59
of Northumberland, whom he asked to see. He reported that the countess experienced a fit of

violent & exquisite torment that extented over the right side of her face & mouth. Nicolas

Andre, in 1756, used the term tic douloureux (painful spasm) to describe the disorder. In

1773 Medical society of London, John Fothergill gave a full & accurate description of

trigeminal neuralgia14,16,17,18.

Epidemiology:

Trigeminal neuralgia is far the most frequently diagnosed form of neuralgia, with a

mean incidence of 4 per 100,000 population8,9,17,25.Early literature suggested a strong

preponderance in women; however, current data indicate that only approximately 60% of

patients with trigeminal neuralgia are female, with male female ratio ranging from 1:2 to 2:3.

The annual incidence for women is approximately 5.9 cases per 100,000 women; for men it is

approximately 3.4 cases per 100,000 men. However, among individuals older than 80 years,

men have a very high incidence of 45/100,000. Incidence increases with age with peak onset

between 50 and 70 years. The disorder can also occur in children with a youngest child

reported to have trigeminal neuralgia was 12 months old, whereas children between age 3 and

11 years have also experienced the condition16.

Anatomic and functional considerations:

The trigeminal nerve is the largest sensory cranial nerve. It also contains some motor fibers.

The principle function of the nerve is to carry information relating to light touch, temperature,

pain, &propioception from the face and head to the brain16.

Sensory fibers:

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The sensory fibers arise from the gasserian ganglion (lying near the petrous part of the

temporal bone in the duramater),pass backward,enter the pons,and divide into upper and

lower roots .The upper roots end in the principle or superior sensory nucleus and locus

caeruleus. The lower roots descend through the pons and medulla & terminate in the spinal

tract nucleus, which consists of sub nucleus oralis. The sub nucleus caudalis serves as the

principle brainstem relay site for orofacial pain, and the superior sensory nucleus and

subnucleus oralis are the sites relaying orofacial tactile information.

Electrophysiologic studies indicate that many of the nociceptive neurons in laminae of

the spinal dorsal horn have axons that project directly to the thalamus via the spinothalamic

tract. Likewise, nociceptive neurons in each part of the trigeminal brain stem complex have

axons that project directly or indirectly to the thalamus. Neurons similar to the dorsal horns

nociceptive neurons are found in the subnucleus caudalis has many features in common with

the spinal dorsal horn, including its layered appearance and cell types.

The response properties of cutaneous nociceptive neurons have been classified into

two main groups.

1. High threshold or nociceptive specific neurons, which respond exclusively to noxious

tactile stimuli such as a pinch or heat and.

2. Wide dynamic range neurons, which are excited by noxious and non-noxioustactile

stimuli.Many nociceptive neurons in the sub nucleus caudalis not only respond to noxious

stimulation of the orofacial tissues but also can be excited by noxious mechanical or chemical

stimulation of the temporomandibular joint,duramater and afferent nerves of the

jaw,tongue,and neck muscles.

Low threshold mechanoreceptive neurons do not respond to noxious tactile stimuli; they are

activated by light tactile stimuli applied to the skin, mucosa, or teeth. These neurons are

61
found mostly in laminae III and IV of the spinal cord and are similar to neurons in the

superior sensory nucleus16.

Motor fibers:

The motor fibers arise from superior and inferior nuclei. The motor fiber airsing from the

superior nucleus, also called mesocephalic roots, join with the motor fibers airising from the

lower nucleus and together they form the motor part of the trigeminal nerve. This part of the

nerve joins with the sensory root as it emerges from the side of the pons.

Principle divisions of the trigeminal nerve:

There are 3 main branches to the trigeminal nerve. The ophthalmic nerve (V1) is the smallest

division, containing only sensory fibers. It supplies branches to the cornea, ciliary body, iris,

lacrimal gland, conjunctiva, skin of the eyelid, eyebrows, forehead, and nose. The maxillary

nerve (V2) is the intermediate in size and is also sensory in nature. It supplies branches to the

side of the nose, upper eyelid, and upper lip. The mandibular nerve (V3) is the largest branch

and contains both sensory and motor fibers. It supplies branches to the teeth, gums, skin of

the temporal region, lower lip, lower part of the face, and muscles of mastication. Pain is

usually referred to a main division of the trigeminal nerve, but some cases, it may be felt over

the distribution of more than one division.

Branches of trigeminal nerve

Trigeminal
ganglion

62
 Ophthalmic
branch

Maxillary branch

Mandibular branch

Distribution of trigeminal neuralgia

Incidence of trigeminal neuralgia in different branches of trigeminal nerve.

Classification:

63
International headache society (IHS) classifies TN 15

Based on Etiology

1. Primary or Idiopathic TN

2. Secondary or Symptomatic TN

Based on Symptoms

1. Typical TN

2. Atypical TN

Pretrigeminal neuralgia:

Although a rare condition, pretrigeminal neuralgia has been recognized for some time. It is an

early form of TN. The presenting condition is typically an intermittent or continuous aching

or dull pain in the upper or lower jaw which eventually converts to lancinating and

paroxysmal pain, TN. The pain can mimic a toothache in the region where physical and

radiographic examinations reveal no abnormality and pain usually lasts hours to weeks.

Fromm et al. reported 18 patients with TN who had experienced pre-TN. In 8 of the 18

patients, pains triggered by chewing, drinking hot or cold liquids, brushing the teeth, yawning

or talking. in all these patients typical TN developed a few months to 12 years later and

involved the same branch of trigeminal nerve3,5,8,9,13,15,24.

Clinical characteristics3:

 Aching or burning pain

 Continuous or intermittent

 Unilateral location

 Local anesthesia of painful region temporarily arrests pain

64
  Neurologic examination will be normal

 No dentoalveolar cause found

 Frequently responsive to anticonvulsant therapy

Classical trigeminal neuralgia:

Classical TN (CTN) includes all cases without an established etiology, i.e. idiopathic, as

well as those with potential vascular compression of the fifth cranial nerve, whereas the

diagnosis of symptomatic TN (STN) is made in cases secondary to tumor, MS, structural

abnormalities of the skull base, and the like. It should be noted that categorization of TN into

typical and atypical forms is based on symptom constellation, and not etiology15.

Etiology of Trigeminal Neuralgia:

1. Neurovascular compression

Neurovascular compressionat the root entry zone is generally reported as the most

frequent cause. Hamlyn reviewed 31 articles of mainly microvascular decompression (MVD)

and reported that the median rate of patients without significant neurovascular compression

was 12%. However,this does not necessarily indicate that neurovascular compression at the

root entry zone accounts for more than 80% of TN cases7,8,9.

2. Multiple Sclerosis

TN is reported to occur in 0.9% to 4.5% of patients with MS.TN is diagnosed in 1-5%

of patients with MS. In patients with both TN and MS,TN is first symptom of MS in 9 to

14% of patients. TN due to MS is often accompanied by feelings of numbness or

paresthesia15,24.

3. Tumor & Cyst

65
 The reported range for patients with TN due to tumors is from 0.8% to 11.6%. Tumors

combined with aneurysms, angioma, or vascular malformation are reported as a cause of TN

in 5.7%, 10.7%, 13.4% respectively.Slowly growing tumors which distend the trigeminal root

rather than invade it, are usually found in TN. The pain is sometimes continuous in patients

with a tumor but may vary in intensity without a temporal pattern23.

4. Allergy

Hanes proposed a theory that the lack of sufficient gastric acid caused improper protein

digestion with resultant release of histamine like substance into the general circulation. It was

hypothesized that this led to an allergic response in the trigeminal nerve ganglion, which

caused TN. Of 75 TN patients and 1 patient with herpes zoster involving the trigeminal

nerve, 91% showed an absence or decrease in free HCL. Histamine desensitization with

repeated subcutaneous injection and the temporary oral use of antihistamine provided

complete relief in 70% of patients26.

5. Herpes simplex virus (HSV)

MVD reactivates HSV and HSV is thought to exist in the trigeminal ganglion. Ecker et al

have suggested that HSV is a causative agent for TN. Chang et al reported lesions suggestive

of previous viral neuritis at the root entry zone and in the pons which were thought to be a

cause of TN based on the findings of MRI26.

6. Other putative causes of TN

TN may also result from infarction of the root entry zone of the trigeminal nerve &

pontine. A herniated temporal lobe caused compression of the trigeminal nerve, resulting in

TN in a 22-year-old female. Congenital anomaly at the base of the skull can also produce

compression of the trigeminal nerve. Ohno reported that the cisternal space in the

66
cerebellopontine and prepontine cisterns of five young TN patients whose age ranged from

20 to 29 was extremely small.

Lee hypothesized that sagging of the hindbrain in the elderly patients could cause traction

on the trigeminal nerve at the petrous ridge, resulting in TN26.

7. Dental pathology and TN

Harris reported the occurrence of TN after dental surgery and after inferior dental

nerve injury due mandible fracture. Zurak et al reported that 63 patients with idiopathic

TN, except with isolated first branch TN, had numerous teeth extraction. The study

excluded erroneous extractions because of idiopathic TN. Of 229 patients with typical

TN, toothache, dental treatment, placement of bridge were attributed by 21 patients as the

cause of onset of pain. Eide et al reported 10 patients with trigeminal neuropathic pain

immediately after dental treatment. Dental pain and treatment are thought to trigger TN in

some patients, although the proportion in etiology of TN is not known.

PATHOGENESIS OF TRIGEMINAL NEURALGIA

Until recently, the mechanism of neuralgic pain was not known. Kerr and Millerhave

shown that significant pathologic change occurs in the myelin sheath of fibers in the

ganglion, dorsal root, or both. This change, recognizable by both light and electron

microscopy, consists of disintegration of the myelin sheath—ie, demyelination. There is some

evidence that demyelination results from a progressive degenerative process. There are likely

to be several reasons for demyelination of the nerve fiber. It may be associated with a

67
structural abnormality, whereby an adjacent structure applies constant force to the nerve root.

In the case of trigeminal neuralgia, the nerve root can be affected in the posterior fossa as it

exits the pons before reaching the gasserian ganglion. One source of such compression is an

aberrant loop of the superior cerebellar artery that lies on the nerve root and produces a

significant microvascular compression. This compression is thought to cause the

demyelination of the nerve7,8,9.

Other sources of compression that need to be considered are space occupying lesions in

this region of the brain, the most frequent of which is a cerebellopontine angle tumor.

Posterior fossa,tumors or basilar artery aneurysms are also possible sources, along with a

meningioma of Meckel's cavity or an epidermoid cyst. It is established that malignant tumors

that initiate pain are extramedullary9.

In TN the pain trigger is often innocuous stimulation that usually does not induce pain.

Following nerve injury there is an increased proportion of neurons with subthreshold

oscillations that bring the neuron close to its firing threshold. These neurons often generate

ectopic discharges spontaneously or following external stimuli (Amir et al 1999, Liu et al

2000). These ectopic discharges often last a number of seconds and are termed ‗after

discharge‘. Stimulation of peripheral nerve (particularly A beta fibers) or the dorsal root

produces a transient depolarization in passive neighbouring C fibresneurons in the same

ganglion (Amir and Devor 2000). These findings demonstrate a mechanism by which afferent

nociceptors could be stimulated by activity in low threshold mechanoreceptors particularly in

the event of nerve injury8,9.

Devor et al. proposed a ignition hypothesis for the mechanism of trigeminal neuralgia,

and any neuralgia, based on these findings. They suggested that the 'compression and

demyelination of the nerve root cause ectopic firing of a focal group of trigeminal ganglion

neurons. This causes cluster of neurons to become hyper excitable. When this activity is

68
supplemented by activity evoked from a peripheral trigger, especially light touch, the

aggregate focus activity produces a chain reaction spread of activity to passive neighboring

cells in the ganglion. After a brief period of autonomous firing, activity is quenched, and a

refractory period is initiated by an intrinsic suppressive (hyperpolarizing) process engaged as

a result of the rapid firing. Since the primary abnormality resides in the trigeminal ganglion

and nerve root, normal sensation is present in the periphery between periods of ectopic

paroxysmal discharge9,13.

Peripheral cause of TN:

Demyelination is thought to cause ectopic impulse generation, cross- talk between touch

sensation and pain sensation, and decrease central pain- gating mechanism resulting in TN.

However, it alone cannot account for rapid electrophysiologic recovery and pain relief after

MVD. Other factors, such as compression in addition to demyelination, may contribute to the

disorder.

Central nervous system (CNS) segment theory: De Ridder et al. hypothesized that

vascular compression syndrome did not arise from neurovascular compression at the root

entry zone, but from neurovascular compression along the CNS segment of the cranial nerve.

They reported that the peripheral nervous system segment was more resistant to compression

than the CNS segment based on a histological examination in humans. This theory explains

the lack of symptoms with neurovascular compression at root entry zone. The CNS segment

of the trigeminal nerve is 2-4 times longer than that of glossopharyngeal neuralgia. This

theory alone cannot explain this huge difference between the length of the CNS segment and

the incidence. This discrepancy has been explained by the assumption that a second factor,

such as a previous injury, is necessary for creating vascular compression symptoms such as

hemifacial spasm, TN and glossopharyngeal neuralgia.

69
 Ishikawa et al. speculated that arachanoid thickening or granulomatous adhesion between

the root and surrounding structures could cause TN. These abnormalities cause angulation or

torsion of the root, resulting in a tethering effect to the trigeminal root. This makes pulsatile

moment of the root more restricted, which could give the root an abnormally high stretching

force26.

Central cause of TN:

Peripheral causes alone cannot account for TN due MS. In an experimental study, injection

of certain substances into the trigeminal nucleus caudalis caused hypersensitivity of the face

that resembled TN. Kugelberg et al noted that the long summation times, the tendency of the

attack to be self-maintained, the long-lasting refractory period after an attack and efficacy of

antiepileptic drugs suggest that mechanism is situated centrally, probably in the brain stem in

structures related to the spinal trigeminal nucleus. Pagni hypothesized that TN was a sort of

sensory reflex epilepsy, whereas Duber et al explained the mechanisms of TN with the

neurophysiologic properties of wide dynamic range neuron26.

Significance of trigger points :

Trigger point has great significance in clinical examination that has great significance in

clinical examination that may probably indicate the presence of causative agent. Focal

vascular pulsating contact at DREZ(dorsal root entry zone) can be easily triggered by light,

non- noxious tactile stimuli. The tics are due to arteriosclerotic tortuous elongation of arterial

loop that is elicited clinically22.

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Clinical features8,23:

1. Location – TN is unilateral facial pain syndrome. Bilateral cases have been reported

in 1-4% of cases but one side usually precedes the onset of pain on the contralateral

side. Pain location is usually described according to the major branches of trigeminal

nerve, 36-42% of cases report pain in one branch.Most commonly the maxillary and

mandibular branches are affected together (35%) & all three branches are involved in

14% of patients.

2. Quality - Characterized by paroxysmal, sharpshooting, piercing, sudden, stabbing or

electrical in nature and severe. Some TN patients (4-35%) may describe two types of

pain: paroxysmal attacks of short sharp pain superimposed on a dull background pain

of varying duration (Katusic et al 1990, Rasmussen 1990).Pain severity is extreme,

rating 9 to 10 on a 10cm visual analogue scale (VAS).

3. Triggering - The most important feature of trigeminal neuralgia is existence of trigger

points often located in the skin of the lips, cheeks or gums on the side of the jaw,

underneath the eye ,in the eyelid or anywhere the trigeminal nerve reaches and when

touched may provoke a pain stimulus. A short gap between stimulation of trigger area

and pain onset may be observed & is termed as latency. The precipitating factors

includetalking(76%), touch(65%) ,temperature(cold 48%, heat 1%),movement of

head and bed rest (2%), psychological factors (2%), pressure by dentures (0.4%).

Washing face, brushing teeth, wind and shaving also triggers pain.

Trigger factors are distinct from trigger areas and include noise, lights and stress.

These may also occur in up to 60% of other orofacial pain patients but rapid, severe,

electric-like pain that occurs in TN should distinguish it from other pain syndromes.

71
 4. Temporal pattern – individual attack are characterized by a rapid onset and peak.

Attacks can last from 10 seconds to 2 minutes. This is followed by a refractory period

whose duration is related to the intensity of TN attack ,during this time pain is

impossible or extremely difficult to trigger.

5. Associated sensory & motor signs- sensory disturbances such as hypoaesthesia are

rare but occur in some patients with TN. Accompanying the pain of TN is a classic

contraction of the facial musculature, hence the term tic douloureux, tic convulsive.

6. Quality of life- the severity & progressive nature of TN has resulted in patients

committing suicide. Quality of life is much reduced in TN patients as either a direct

effect of pain or secondary to side effects of the drugs employed.

7. Eating can become almost impossible, and loss of weight is common among those

with the disorder.

8. During sleep there is no attack of TN.

International Headache Society criteria for trigeminal neuralgia11,12,14,19,25:

A. Paroxysmal attacks of facial or frontal pain which last a few seconds to less than 2 minutes

B. Pain should have at least 4 of the following characteristics:

1. Distribution along one or more divisions of the trigeminal nerve

2. Sudden, intense sharp, superficial, stabbing or burning in quality

3. Pain intensity severe

4. Precipitation from trigger areas, or by certain daily activities such as eating, talking,

washing the face or cleaning the teeth.

5. Between paroxysms the patient is entirely asymptomatic

72
C. No neurological deficit

D. Attacks are stereotyped in the individual patient

E. Exclusion of other causes of facial pain by history, physical examination, and special

investigation when necessary.

Diagnosis

HISTORY:

A thorough history and clinical evaluation with adequate radiographs of oral structures

are essential to rule out pathology. Patients with trigeminal neuralgia present with a primary

description of recurrent episodes of unilateral facial pain. Attacks last only seconds and may

recur infrequently or as often as hundreds of times each day; they rarely occur during sleep.

In trigger zones(small areas near the nose or mouth in patients with trigeminal neuralgia)

minimal stimulation initiates a painful attack. Patients with trigeminal neuralgia can pinpoint

these areas and will assiduously avoid any stimulation of them. Not all patients with

trigeminal neuralgia have trigger zones, but trigger zones are nearly pathognomonic for this

disorder8,9,25.

Table 1: Trigeminal Neuralgia: Clinical Diagnostic Criteria

1. Character Discharge, electric shock, excruciating,

superficial

2. Intensity Moderate to very severe

3. Duration Each pain episodes does not last more than 2

minutes, several episodes during the day

4. Periodicity Periods of weeks, months without pain but

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 also painless periods between attacks

5. Site Trigeminal nerve distribution area, in general

unilateral

6. Irradiation Within trigeminal nerve area or beyond

7. Triggering factors Innocuous stimulation such as eating

talking, bathing

8. Relieving factors Frequently sleep, anticonvulsant drugs

9. Associated factors Trigger zones, weight loss, low quality of life

and depression

PHYSICAL EXAMINATION:

The physical examination in patients with trigeminal neuralgia is generally normal.

Therefore, physical examination in patients with facial pain is most useful for identifying

abnormalities that point to other diagnosis. The physician should perform a careful

examination of the head and neck, with an emphasis on the neurologic examination. The ears,

mouth, teeth, and temporomandibular joint (TMJ) should be examined for problems that

might cause facial pain. The finding of typical trigger zones verifies the diagnosis of

74
trigeminal neuralgia. Patients with classical trigeminal neuralgia have a normal neurologic

examination. Sensory abnormalities in the trigeminal area, loss of corneal reflex, or evidence

of any weakness in the facial muscles should prompt the physician to consider symptomatic

trigeminal neuralgia or another cause of the patient‘s symptoms7,9,15,25

CHAIR SIDE INVESTIGATION:

Diagnostic block:

Protocol for diagnostic nerve block:

Inject 0.5 ml of normal saline at test sit

Wait 5 minute

Pain relieved Pain persists

Psychogenic pain likely Inject 0.5 ml of 0.5% lidocaine & wait for 5min

Pain relieved Pain persists

Small nociceptor fibers causing pain Inject inject 0.5 ml of 2%

direct therapy at them lidocaine & wait for 5min

Pain relieved Pain persists

large fibers initiating pain, consider Repeat protocol at more proximal

75
musculoskeletal origin of pain portion of nerve

Pain relieved Pain persists

Direct therapy at site where relief Consider central nervous system

occurred lesion or psychogenic pain

SPECIALIZED INVESTIGATIONS:

All patients who have TN should undergo imaging (CT or MRI) at least once during

diagnosis and therapy. Imaging techniques such as magnetic resonance tomographic

angiography (MRTA or MRA) may indicate vascular compression of the nerve root.More

sophisticated techniques, such as three-dimensional MRI with constructive interference in

steady state sequence, are superior to MRTA/MRA in detecting venular compressions

13,15,16,25
.

One recent study demonstrated that trigeminal reflex testing could distinguish classical from

symptomatic trigeminal neuralgia with a sensitivity of 96% and a specificity of 93%.

Trigeminal reflex testing involves electrical stimulation of the divisions of the trigeminal

nerve and measurement of the response with standard electromyography apparatus. This

testing is not readily available to most physicians, and its indications and clinical utility are

still unclear25.

Differential diagnosis25

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 A careful examination may disclose local findings indicative of otitis, sinusitis, dental

disorders, or TMJ dysfunction. A history of persistent pain or pain that occurs episodically in

attacks lasting longer than two minutes eliminates classical trigeminal neuralgia and should

leadto a search for other diagnoses. The pain of glossopharyngeal neuralgia, which may be

triggered by talking or swallowing, is located in the tongue and pharynx. Symptomatic

trigeminal neuralgia is usually caused by multiple sclerosis or by tumors arising near the

trigeminal nerve root.

1. Cluster headache: location of pain is retrobulbar, cheek, chin, and duration of attack is

20 min to hours. Shooting pain is superimposed on deep dull pain.

2. SUNCT: location of pain is forehead, retrobulbar and duration of attack is 5second to

several min. Shooting pain is present

3. Chronic paroxysmal hemicranias: location of pain is forehead, retrobulbar and

duration of attack is 2 to 45 min.

4. Cracked tooth syndrome: location of pain is upper jaw and lower jaw and duration of

attack is few seconds.

5. Jabs and joints syndrome: location of pain is anywhere in the head and duration of

attack is few seconds.

6. Post herpetic neuralgia: location of pain is forehead, eye, cheek (rarely) and duration

of attack is continuous.

7. Giant cell arteritis: location of pain is forehead, neck, temple and duration of attack is

continuous

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 Table 2: Differential Diagnosis of Trigeminal Neuralgia

Prognosis:

Long-term follow up of patients who have TN shows that well-defined periods of pain attacks

are variably followed by periods of remission. However, TN bears a poor prognosis;

approximately 90% of patients who have TN report increased attack frequency and severity

accompanied by a progressive and increasing resistance to pharmacologic and surgical

treatment23.

Management of Trigeminal Neuralgia:

78
First medical management is advocated. If the patient dose not respond to it then only

surgical management is opted

Medical management

Historical overview of treatment in 1850s, Trousseau thought that painful attacks of TN

resembled epilepsy. Based on this idea, in 1942 Bergouignan described the successful use of

phenytoin in this condition very soon after the drug was first approved for use in epilepsy. In

the early 1960s, Blom reported that carbamazepine was more effective than phenytoin in

preventing the attacks28.

79
80
 Table 3: Antiepileptic drugs & dose schedules commonly used in the treatment of TN &

other painful neuropathies 23

Drug Initial Target / Dose increase Schedule

dose(mg) maximal dose (titration)a

(mg)a

Carbamazepine 100- 200 2400 100-200mg every 2 days 3-4 times/day

Carbamazepine 200-400 2400 Usually transfer from regular 2 times /day

CR format at equivalent dose

Oxcarbazepine 300 1200-2400 300-600mg/week 3times / day

Baclofen 5-15 30-60 5mg every 3 days 3times/day

Gabapentine 300 900-2400 300mg every 1-2 days 3times/day

Pregabalin 150 300-600 50mg every 2-3 days 2-3times/day

Lamotrigine * 25 400-600 25-50mg/week 1-2times/day

CR-controlled release
a
Titrate according to response and side effects,* lamotrigine has been tested as add on

therapy in TN

First line Approach

Carbamazepine:

It is a tricyclic imipramine antidepressant which is highly soluble in organic solvents. It

reduces sodium channel conductivity, stabilizing the membrane of pre and post-synaptic

neurons and making trigeminal mechanoreceptors less prone to respond to peripheral input.It

is almost completely absorbed from the gastrointestinal tract. Peak serum concentration is

81
achieved within 4-8 hours. It is extensively metabolized in liver with more than ten identified

metabolites.

Dosage: Preparations available are in the form of 100, 200 mg chewable tablets, 200 and 400

mg sustained release tablets. Dosage should be kept small to minimize adverse effects.

100mg two times a day for 2 weeks, then three times daily increasing by 100 mg every three

days to a maximum of 1000 mg/dayuntil either remission is achieved or side effects of

toxicity are unacceptable. Dosage may have to be increased after 20 days because of

autoinduction. Once complete pain relief has been maintained for one month, the drug can be

slowly titrated at the rate of 200 mg /day31.

Side effects: Visual blurring, dizziness, somnolence, skin rashes and ataxia and in rare cases

hepatic dysfunction, leucopenia, thrombocytopenia. Balance (disturbed – ataxia) is the

feature limiting the dose of carbamazepine

Investigations: Complete blood cell count and liver function tests should be done

periodically on patients treated for longer time.

Second line approach:

Baclofen:

Baclofen, an analogue of neurotransmitter aminobutyric acid, and it potentiates GABA. It

was first used in the treatment of trigeminal neuralgia in 1980. Initial dose is 5-10 mg thrice

daily which can be increased by 10 mg every alternate day till pain relief is achieved. The

usual maintenance dose is 50-60mg per day, however maximum dose of 20 mg four times a

day might be required.The side effects are postural hypotension, muscle weakness and

gastroenteric discomfort13.

Gabapentine :

82
It is structurally related to neurotransmitter gamma aminobutyric acid.it is given in 4 divided

doses a day. 1200 -3600 mg /day orally in q.i.d or b.i.d doses. The initial dose started from

300mg q.i.d initially and then titrated 300mg/day. The side effects are somnolence, dizziness,

ataxia, weakness, paraesthesia, fatigue, headache, nystagmus, diplopia. It should be used

with caution in the patients with renal or hepatic impairment31.

Oxycarbazepine:

Oxycarbazepine, a ketoanalogue of carbamazepine, gets metabolized to pharmacologically

active 10,11-dihydrocarbazepine. Its absorption is quick and complete within 1 to 2 hours.

The initial dose of 300-500mg which can be increased every 3 days of by 150 – 300mg upto

therapeutic dose of 1200mg thrice a day. The side effects -Hyponatraemia., somnolence and

fatigue, nausea and vomiting, headache and dizziness.Fluid restriction may be deemed

necessary in some patients to reduce the risk of precipitating seizures secondary to low serum

sodium.

Jorns TP, Zakrzewska JMstated that carbamazepine is still the first line drug for medical

management, but this should be changed to oxcarbazepine if there is poor efficacy and an

unacceptable side effect profile.

Gomez-Arguelles JM etalconducted a study to evaluate the efficacy and tolerability of

oxcarbazepine in trigeminal neuralgia (TN) unresponsive to treatment with the standard

antiepileptic drug, carbamazepine. They concluded that Oxcarbazepine was effective from

the first month of treatment. There was a significant reduction in pain frequency, leading to

improvements in patient satisfaction. In general, oxcarbazepine was well tolerated.

Oxcarbazepine appears to be an important alternative therapeutic approach for patients

affected by TN.

83
Phenytoin:

Phenytoin (5,5-diphenhydantoin) was first successfully used in the treatment of trigeminal

neuralgia in 1942. Peak concentration is achieved in 4 to 8 hours after oral administration. On

being absorbed, 90% of it gastro reversibly bound to serum proteins. Remaining 10% is the

component which is pharmacologically active. 100mg three times in a day is used initially.

Dosage is increased subsequently to as much as 800 mg a day. Neurophysiological studies

provide a rational basis for the use of phenytoin, thought to act principally by inhibiting

synaptic conduction in the brain stem, it also reduces the peripheral nerve conduction and

increases the threshold of electrical stimulation in animals. It can be used in combination with

carbamazepine or with baclofen. Theside effects include double vision, ataxia, liver disease,

gingival hyperplasia, hematologic disturbances, hirsutism, and memory defects.

Lamotrigine:

It is another antiepileptic drug which could be used in trigeminal neuralgia. It is seen to be

potent as carbamazepine in inactivating Na+ currents, with fewer side effects. 25 mg/day for

2weeks then 25 mg bid for 2 weeks with a maximum dose of 50 to 100 mg twice daily. The

side effects include skin rashes, dizziness, constipation, nausea and drowsiness

Third line of approach:

Clonazepam

Patients in whom carbamazepine is contraindicated clonazepam can be used.

Dosage: 4 to 8 mg/day. Results are reported of a preliminary trial with clonazepam in 19

patients with trigeminal neuralgia refractory to carbamazepine treatment.12Side effects –

drowsiness, ataxia.

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Topiramate :

Topiramate is a sulfamate-substituted monosaccharide. Its role in the management of

trigeminal neuralgia has been recently studied.

Dosage – starting dose 25mg/day, which can be increased by 25 mg every week up to a dose

of 100-400mg/day.

Side effects- fatigue, dizziness, nervousness, tremor, weight loss, difficulty with

concentration/attention.

Other drugs tried in treatment of TN:

Pimozide,2 to 12 mg/day has also been used in TN. Valproic acid is given in dose of 250 to

500mg qid. Careful monitoring is required in these patients due to side effects like tremors,

confusion, nausea, vomiting, weight gain, hepatoxicity. Pregabalin 150 to 600 mg daily and

amitriptyline 50 to 100mg/day depending on the frequency of attacks.Side effects -

drowsiness and dizziness.Levetiracetam (3–4 g/day) for 16 weeks as add-on therapy, after a

2-week baseline period may be effective and safe in trigeminal neuralgia treatment.

Other treatment modalities :

Botulinum toxin:

Botulinum toxin type A(Botox) has been used in somepatients. BoNT-A has been used for a

long time in the treatment of disorders characterised by pathologically increased muscle tone.

Early observations in dystonia also demonstrated an analgesic effect of BoNT-A, which led

tofurther investigation on its efficacy for painful conditions including neuropathic pain , low

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back pain and headaches . Evidence shows that BoNT-A reduces the local release of

nociceptive neuropeptides such as substance P, glutamate and calcitonin gene-related peptide

and inhibits peripheral sensitisation, which would result in decreased central sensitisation .

Hence it can be hypothesised that BoNT-A prevents peripheral sensitisation and subsequently

central sensitisation. There are reports of isolated cases of NT responding to BoNT-A. In

another open study, 13 patients showed significant relief from symptoms after treatment with

BoNT-A .

Subcutaneous sumatriptan:

Sumatriptan and zolmitriptan showed efficacy in controlling allodynic pain following nerve

injury in an animal model for trigeminal neuropathic pain. Both medications have agonistic

action on 5-HT1B, which results in inhibition of neurons of the trigeminal nucleus localised

in the medulla. Therefore, these drugs are thought to act on the central nervous system. These

data were confirmed in another clinical single-blind study of subcutaneous sumatriptan vs.

placebo. Results proved efficacy of sumatriptan on pain symptoms in patients with TN after

15 and 30 min compared to placebo. Benefits lasted for 7 hr on average and this limits the

clinical use of the drug. Potential side effects from subcutaneous injection of sumatriptan

include increased blood pressure, nausea and weakness, which are not dangerous.

Laser:

Laser treatment has also been used experimentally for trigeminal neuralgia. In a study,

human subjects received irradiation of the skin overlying peripheral nerves with a helium

neon laser for 20 seconds to each selected site. This treatment was accompanied by

irradiation of the skin overlying painful facial areas for 30 to 90 seconds. Laser was repeated

86
for 3 times weekly for 10 weeks. Subjects in the experimental group exhibited a statistically

significant reduction in the intensity and the frequency of the painful episodes16.

Eckerdal& Lehmann have observed from a double blind, placebo-controlled study that low

reactive level laser therapy (LLLT) is effective in the treatment of trigeminal neuralgia.

One recent study found that intranasal lidocaine (Xylocaine) significantly decreased second-

division trigeminal neuralgia pain for more than four hours. Topical preparations include

drugs like valproate sodium, racemic ketamine, proparacaine hydrochloride, and topical

capsaicin cream.

Proparacaine is a local anesthetic agent that anaesthetizes the eye and possibly the nerves

around it. Some studies have shown that anesthetic eye drops containing proparacaine can

give short term relief from some instances of trigeminal neuralgia. The treatment is usually

effective if the pain is in the ophthalmic division of the trigeminal nerve. Another study

suggest that the treatment is almost certainly ineffective for pain of classical trigeminal

neuralgia. Proparacaine can damage the eye if used extensively, thus, it cannot be considered

a long-term treatment.

Acupuncture:

Acupuncture exhibits diverse effects at various levels of the antinociceptive portions of the

nervous system. However, the traditional approaches to acupuncture therapy rely on a series

of quasi-metaphysical relationships dealing with energy flow and the relationship of the

human organism with environmental factors. These empirical relationships are based on

thousands of years of clinical observation, but lack sound scientific rationale. The validity of

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classical acupuncture applications based on traditional diagnostic and treatment protocols has

not been adequately studied. Adherence to traditional acupuncture diagnosis and treatment

paradigms has made assimilation of acupuncture into Western medical practice difficult.

Basic science studies are beginning to unravel the neurobiological basis of acupuncture

therapy, particularly for pain control. Two case reports that relied on the known

neuroanatomical and neurophysiological connections between classical acupuncture points

and the trigeminal system. It was these neurobiological relationships rather than traditional

acupuncture diagnosis that formed the basisof acupuncture point selection in cases of drug-

and surgery-resistant trigeminal neuralgia27.

Surgical management:

Microvascular decompression:

Since the original theory, outlined by Dandy in 1925, of vascular decompression as a

prominent feature of TN, it took almost half century until MVD was accepted as one of the

major surgical surgicalmethods. In MVD, the target area lies at the nerve pons junction. The

posterior fossa is approached through a sub-occipital craniotomy. After aspiration of the CSF,

the operator advances towards the nerve by gently retracting the supero-lateral margin of the

cerebellum. The most common finding is a segment of thesuperior cerebellar artery

compressing the nerve at the root entry zone. Less frequently, the anterior inferior cerebellar

artery or the superior petrosal veins are the cause of the compression. After the arachnoid is

dissected and vessel freed, the operator places a piece of shredded Teflon felt between the

vessel and the nerve to separate them13,31.

Radiofrequency gangliolysis;

Disillusionment with complication resulting from an uncontrolled spread of alcohol or

phenol injected into Meckel‘s cave for treatment of neuralgia led to the development of

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thermo coagulation of Gasserian ganglion in the 1930s. The concept was first explored by

Kirchner whose large series was published in 1942. In 1974, Sweet presented his method

which , by and large, is used today. Essentially, it involves a selective partial lesioning of the

affected ganglion or retrogasserian root13.

Glycerol gangliolysis:

The method was introduced by Hankinson after a fortuitous discovery, during the

development of stereotactic technique for gamma radiation, that glycerol mixed with

tantalum powder not only visualized the trigeminal cistern but also abolished pain in patients

with TN13.

Balloon compression:

This procedure was introduced in early 1980s by Mullan and Lichtor as a mechanical

destructive technique and it is performed under general anesthesia. Using fluoroscopic

control, a guide needle is inserted into the foramen ovale, but not beyond it. Through the

needle, Fogarty catheter is advanced until its tip lies in Meckel‘s cave and balloon is slowly

inflated with 0.5- 1.0ml of contrast dye until it occupies the cave, ensuring adequate

compression. Total compression times vary from 1-6 min. This produces only a mild sensory

loss with immediate pain relief in practically all patients13,30.

Stereotactic radiosurgery (gamma knife):

Well localized lesions in the trigeminal nerve can be generated also by stereo static

radiosurgery. Lars Leksell developed a radio surgical tool, ‗Gamma knife‘ to treat functional

brain disorders as an alternative to intracranial procedure.11 It is a minimally invasive

technique that precisely delivers radio surgical doses of 70 to 90 Gy to the trigeminal nerve

root at the point of vascular compression23.

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Cyber knife (CKR):

Stereotactic cyber knife radiosurgery has been proven effective treatment strategy for TN

using noninvasive head immobilization and the advance image guidance technology, the

robotic arm of CKR, dynamically tracks skull position and orientation during treatment,

thereby ensuring targeting accuracy throughout the entire procedure64.

Cryotherapy:

In 1976, Lloyd introduced cryotherapy, a surgical technique in which a peripheral branch of

three major divisions of the trigeminal nerve is exposed and frozen by direct application of a

cryoprobe with a tip temperature from -50 to -700.

GLOSSOPHARYNGEAL NEURALGIA:

Glossopharyngeal neuralgia is a distinctive syndrome, named by Wilfred Harris.

Historical perspective

The first description of glossopharyngeal neuralgia is credited to Theodore H.

Weisenburg in 1910.His patient presented with the classical symptoms of lancinating pain in

the ear and neck. Ten years later, Sicard and Robineau described 3 patients who had ‗algie

velo-pharyngeeessentielle‘ (pain in the distribution of the glossopharyngeal nerve of

unknown cause). Treatment with sedatives and physical agents failed; the patients became

suicidal. Sectioning the glossopharyngeal nerve through the neck was, however, successful in

relieving the pain in all 3 patients. A year later, Wilfred Harris coined the term

‗glossopharyngeal neuralgia‘43

Epidemiology:

GPN occurs much less frequently than trigeminal neuralgia; the ratio between them varies

from study to study, ranging from 5.9:1 to 100:1. The annual incidence rate of GPN,

according to a population-based study, is about 0.7 per 100,000. Overall, there is a higher

90
incidence of GPN in men in most series, but it never reached statistical significance. The

incidence of GPN increases with age; very rarely it affects persons younger than 20 and the

majority of patients start havingsymptoms after 50 years of age.

Etiology:

In regard to the etiology of GPN, all cases may be divided into two groups: those without

discernible cause, also called idiopathic or essential GPN, and those with an underlying

pathological process, such as neoplasm, inflammation or infection, or elongated styloid

process, in which the condition is referred to as secondary GPN.

Idiopathic GPN is thought by some authors to be caused by vascular compression of the

ninth cranial nerve as it exits or enters the brainstem. This theory is greatly supported by the

success of microvascular decompression (MVD) in elimination of symptoms, particularly for

the analogous condition of trigeminal neuralgia, in which this type of treatment is almost

uniformly successful.

In secondary GPN, various neoplasms of the pharynx, tongue, cerebellopontine angle, and

skull base are known to cause shooting pain in the ear and throat area, sometimes as a

presenting symptom. In fact, the first published description of GPN, given by Weisenburg in

1910, was a case of an infiltrating skull base tumor that involved multiple cranial nerves,

including the ninth.37 Some features that may allow one to suspect the secondary nature of

GPN are the presence of neurological deficits, such as numbness in distribution of the

glossopharyngeal nerve; absence of pain-free intervals between attacks, and spread of pain

outside the ninth nerve area. GPN was also encountered in patients with brainstem tumors as

well as after surgical resection of intramedullary tumors of the cervicomedullary junction.

Other causes of secondary GPN include traumatic injuries, vascular malformations such as

persistent hypoglossal artery and arteriovenous malformation, infectious processes including

parapharyngeal abscess, Chiari malformation, pontinelesion, and choroid plexus overgrowth.

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Anatomy and Pathogenesis:

The glossopharyngeal nerve has been called ‗the neglected cranial nerve‘, because it is small,

lies deep within the neck and is often unnoticed in surgical dissections. It is the nerve of the

third branchial arch. It exits the medulla laterally, just rostral to the vagus nerve. The

glossopharyngeal nerve receives sensory fibers from the posterior two thirds of the tongue,

including taste, and afferents from the carotid bodies that enter the nucleus of tractus

solitarius. Visceral pain passes to the spinal nucleus of V. It supplies parasympathetic fibers

to the parotid gland via the otic ganglion. Motor fibers supply the stylopharyngeus muscle

(from nucleus ambiguus) and the upper pharyngeal muscles.

Familial cases, and association with multiple sclerosis (MS), trauma and medullary

tumours have each been occasionally implicated, as have laryngeal and nasopharyngeal

tumours, Paget‘s disease and diverse tumours of the skull base. Oppenheim described

demyelinating plaques at the root entry zone as the potential cause of the closely related

trigeminal neuralgia in MS patients39.

Clinical features

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 1. Sudden, severe, brief, stabbing persistent or recurrent pain involving posterior tongue,

tonsillar fossa, and pharynx with radiation to deeper ear structures.

2. At times it may be life threatening as a result of associated cardiovascular consequences

like syncope probably mediated by functional central connections between visceral

afferents of cranial nerves (IX and X), and cardiac arrhythmias are common, particularly

bradycardia.

3. It can be severe debilitating disease with depression, suicidal tendencies, fear of

swallowing, loss of weight and malnutrition.

4. Pain is episodic and the time interval between the episodes reported is ½ to 9 years. Pain

usually lasts from 8 to 50 seconds but may continue for up to 40minutes or even recur in

rapid succession. Frequency of paroxysms may be 5 to 12 per day, reaching 150 to 200

per day.

5. GN trigger areas are located in the tonsillar region and posterior pharynx and are

activated through swallowing, especially cold fluids,chewing, yawning, talking and

moving the head can precipitate the pain. Sneezing, clearing the throat, touching gingival

or oral mucosa, blowing the nose, or rubbing the ear also trigger pain.

ICHD-II criteria for classical glossopharyngeal neuralgia45

Classical glossopharyngeal neuralgia:

A. Paroxysmal attacks of facial pain lasting from a fraction of a second to 2 minutes and

fulfilling criteria B and C.

B. Pain has all of the following characteristics:

1. Unilateral location.

2. Distribution within the posterior part of the tongue, tonsillar fossa, pharynx or beneath

the angle of the lower jaw and/or in the ear.

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 3. Sharp, stabbing and severe.

4. Precipitated by swallowing, chewing, talking, coughing and/or yawning.

C. Attacks are stereotyped in the individual patient.

D. There is no clinically evident neurological deficit.

E. Not attributed to another disorder.

Differential diagnosis:

The most common differential is Trigeminal Neuralgia, particularly when pain of GN spreads

to trigeminal dermatomes. Moreover, the co-occurrence of TN is reported in 10% to 12% of

patients who have GN. A significant association between GN and Multiple Schlerosis has

been reported. Regional infectious or inflammatory processes and cerebellopontine angle or

pontine lesions may cause GN like symptoms. Temporomandibular joint dysfunction and

temporal arteritis may cause pain poorly localised around the ear, but the signs, symptoms

and severity of the pain should clearly differentiate these conditions from glossopharyngeal

neuralgia of the otitic type. Atypical facial pain may provide diagnostic difficulties in that

depression often accompanies persistent facial pain, but the symptoms frequently have a

bizarre quality, and the area of distribution is not that of the glossopharyngeal nerve.Tonsillar

carcinoma invading the parapharyngeal space and other regional tumours (tongue,

oropharyngeal ) may mimic GN.

Pain in stylalgia is experienced on turning the head and radiography may demonstrate an

elongated styloid process. Elongated styloid process is an underlying cause of so-called Eagle

syndrome, a painful condition first described in 1937 in patients after tonsillectomy. The pain

in Eagle syndrome usually resembles that of GPN but in most cases is more constant and

duller in character. There are two distinct types of this syndrome: classic and carotid artery

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(syndrome). The first is characterized by a spastic and nagging pain in the pharynx; it is seen

in tonsillectomized patients and sometimes in un-operated patients with either significant

elongation of the styloid process, usually more than 3 to 3.5 cm in length, or ossification of

the stylohyoid ligament. The second arises with pain of the pharyngeal distribution that

becomes more prominent on head turning, headaches, and vertigo and is not related to

previous surgery. This is thought to be caused by pressure exerted by the elongated styloid

process on the carotid artery, especially when the head is turned. Although a part of the

differential diagnosis of GPN, Eagle syndrome is a distinct pathological entity.

In the secondary glossopharyngeal neuralgias, neurological abnormalities of cranial nerves

may be found and in some cases defects of cerebellar function will also be present.

Syringobulbia and tabetic crises must also be remembered as uncommon causes of pain in

this area46.

Investigations:

Imaging of brain, specifically, an MRI and MRA of brain focusing on the posterior cranial

fossa and upper cervical spine should be taken. Panoramic radiograph should be taken to rule

outEagle‘s syndrome. An ECG should be taken to rule out cardiac abnormalities45.

Management:

Pharmacotherapies that may be used in glossopharyngeal neuralgia therapy42:

Generic name Trade name Dosage (mg/day)

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Carbamazepine Tegretol 100-2000

Carbitol

Duloxatine Cymbaita 20- 90

Gabapentin Neurontin 100-5000

Clonazepam Klonopin 0.5- 8

Lamotragine Lamictal 50-500

Baclofen Lioresal 10-80

Oxycarbazepine Trileptal 150- 2400

Phenytoin Dilantin 200-600

Pregabalin Lyrica

Topirimate Topamax 50-1000

Valproic acid Depakote 125-2500

Venlaflaxine Effexor 37.5-225

Until the introduction of carbamazepine, surgical division of the glossopharyngeal nerve

was the most effective method of treatment for severe cases. However, division of the nerve

through a pharyngeal or cervical incision led to a high incidence of recurrence of pain, and an

intracranial approach was adopted and described first by Dandy in 1927. It has since been

found that symptoms frequently recur unless the upper two rootlets of the vagus nerve are

also divided, and this method has become the standard surgical treatment.

Carbamazepine has been found effective in this condition as in the other primary

paroxysmal neuralgias, and one can thus frequently avoid submitting an elderly patient to

operation. The commonest disadvantage of this drug are that prolonged treatment which

causes unpleasant side-effects such as nausea, vomiting, skin rashes, paraesthesia, and ataxia.

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Bone marrow aplasia has resulted from its use and is not usually reversible when the drug is

withdrawn.

1. Pharmacotherapy for GN is based on drugs successfully used for TN. Carbamazepine or

gabapentin can be effective in suppressing painful paroxysms.

2. Glossopharyngeal nerve block with local anesthetic and methyl prednisolone in low

dose is safe effective form of therapy for GPN with pharmacological adjuvants.Local

anesthetic blocks are used for diagnostic and therapeutic purposes. Infiltration of the

parapharyngeal area with lidocaine solution, glossopharyngeal nerve block at the level

of the jugular foramen, or local application of cocaine solution to the throat usually

provides pain relief that lasts 1 to 2 hours; during this time the patient should be able to

eat, drink, and tolerate probing in the trigger zone without pain.

3. Injection of neurolytic substances (phenol, glycerin) has been proposed as a safe

alternative to more invasive procedures.

4. Surgical methods include nerve section, tractotomy or microvascular decompression.

Intracranial root section has been the most often employed and is generally effective but

additional section of the upper vagal rootlets is considered necessary in some cases.40

5. Microvascular decompression introduced by Jannetta can afford complete relief of pain

in 76% and substantial improvement in a further 16% as reported in a large series, with

a mean follow-up of 48 months.

6. More recently, endoscopy has been employed as the sole imaging modality in

glossopharyngeal nerve decompression. In the analogous trigeminal neuralgia, there is

93% success3 years after endoscopic surgery, but an annual recurrence rate of 3.5%42.

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 GN can be associated with cardiac syncope, which in most instances is caused by asystole

or bradycardia and, possibly, by a vasodepressor reaction. This syndrome may be referred to

as vagoglossopharyngeal neuralgia when the cardiac symptoms accompany pain attacks.

Several medical and surgical alternatives have been proposed for the treatment of GN

associated with syncope. Temporary and permanent pacemakers have been used to prevent

attacks of syncope with different results. Surgical treatment has also evolved over time with

very good results reported.

NERVUS INTERMEDIUS NEURALGIA

Also known as geniculate or Hunt‘s neuralgia11.

Introduction:

The sensory component of facial nerve that innervates the external auditory meatus, parts of

the pinna of the ear,& a small zone of skin beneath & behind the lobe of the ear is called the

nerve intermedius, also known as intermediate nerve of Wrisberg, CN14. When a paroxysmal

neuralgia affects this nerve, it is called nervus intermedius neuralgia. This condition is also

referred to as geniculate or seventh nerve neuralgia7,8,9,47.

Historic prospective:

In 1857, John Nottingham introduced the term ‗‗tic douloureux of the ear‘‘ to describe

sudden paroxysms of ear pain accompanied by flushing of the auricle. The absolute absence

of pain between attacks is a distinctive feature. In 1876, Webber described the pain

phenomena in six cases of facial palsy. In these cases, the pain was situated inside the ear and

the mastoid regions with atypical radiation to the face and occipital region. He thought that

the pain was mediated through the trigeminal and the auricular branches of the vagal nerve.

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Hunt introduced the term ‗‗geniculate neuralgia‘‘ to define a wide variety of painful

conditions of the face and head. This neuralgia was divided into primary, secondary and

reflex. Primary geniculate neuralgia consists of otalgic and prosopalgic, the latter intended as

a deep pain in the face, but may irradiate to the ear. Nevertheless, in the literature some

confusion was manifest between trigeminal, glossopharyngeal and nervus intermedius

neuralgia.

Etiology:

It is presumed that its etiology is the cross compression of the glossopalatinenerve at its

central peripheral myelin junction47

Clinical features:

The pain is felt in the tympanic membrane, walls of auditory canal, the external structures of

the ear. On occasions the pain may be felt in the palate, tongue, & even deeply in the facial

musculature. When the triggering is caused by touching the ear, topical anesthesia of external

auditory canal may arrest it9.

Since fibers of the facial nerve innervate the anterior two third of the tongue & part of the

soft palate, the actual propensity for numerous sites of pain & triggering is great indeed.

Nervus intermedius neuralgia may sometime be clinically confused with migraine variant.

At present, the HIS classification has set out obligatory requirements for the diagnosis

intermedius neuralgia, being the presence of both paroxysmal pain and a trigger area in the

posterior external auditory wall canal47.

The following guidelines may be helpful in differentiating these conditions:

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1. Neurovascular pain has characteristics of deep somatic pain, usually with a component of

throbbing. The pain distribution often follows the vascular arborization. Neuralgia is

neuropathic & has a precise, though widespread, distribution that makes anatomic sense.

2. Neurovascular pain is often accompanied by central excitatory effects not evident with

neuralgia.

3. If surgical approach is contemplated & positive identification of the affected nerve is

required, this can be done by a neurosurgical suboccipital exposure under local anesthesia

for direct stimulation of the nervus intermedius9.

Management:

Patients with geniculate neuralgia can be treated with anticonvulsant such as oxcarbazepine,

carbamazepine, and gabapentine. Patient who do not respond these medications may undergo

surgery for excision of the nervus intermedius and geniculate ganglion or microvascular

decompression.

POST HERPETIC NEURALGIA:

Definition:

Herpes zoster (HZ), also known as shingles, is a painful vesicular rash resulting from

reactivation of the virus that also causes chickenpox – Varicella zostervirus (VZV).

Typically, the rash runs its course in a matter of 4-5 weeks. The pain, however, may persist

months, even years, after the skin heals. This phenomenon is known as postherpetic neuralgia

(PHN).

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 Postherpetic neuralgia can be defined as neuropathic pain persisting more than 3 months

after crusting of Herpes zoster rash (scar tissue formation)48,50.

Introduction :

Herpes zoster (HZ) is more commonly known as shingles, from the Latin cingulum, for

―girdle.‖ This is because a common presentation of HZ involves a unilateral rash that can

wrap around the waist or torso like a girdle. Similarly, the name zoster is derived from

classical Greek, referring to a beltlike binding (known as a zoster) used by warriors to secure

armor22.

Varicella-zoster virus (VZV) is a member of the herpesvirus family that exclusively

infects humans and causes two distinct clinical syndromes. Primary infection presents as

varicella (or chickenpox), a highly contagious but typically benign illness that most often

occurs in epidemics among children. Upon resolution of varicella, the virus becomes latent in

cranial-nerve and dorsal-root ganglia and can reactivate decades later to produce zoster

(shingles) as cell-mediated immunity wanes with age. In immunocompetent patients herpes

zoster most often presents as a unilateral, dermatome-restricted rash accompanied by pain.

Both generally resolve after a duration of 2 to 3 weeks. In some patients, however, the pain

does not resolve when the rash heals but instead persists for weeks and sometimes for months

or years, a condition termed ―postherpetic neuralgia‖50

Not everyone who‘s had a reactivation of the virus develops postherpetic neuralgia. But

postherpetic neuralgia is a common complication of shingles in older adults. The greater your

age when the virus reactivates, the greater the chance you‘ll develop postherpetic neuralgia.

Postherpetic neuralgia (PHN) is the most common complication of herpes zoster among

immunocompetent patients and can lead to disabling and sometimes intractable chronic pain.

While a variety of definitions have been proposed by clinicians and investigators, recent

101
studies have suggested that the pain associated with herpes zoster can be divided into three

phases:

1. An acute herpetic neuralgia that accompanies the rash and lasts up to 30 days after the

onset of rash.

2. A subacute neuralgia that lasts between 30 and 120 days following the onset of rash.

3. PHN can cause substantial morbidity and, once established, can be difficult to manage

effectively, making this the most compelling indication for early treatment of herpes

zoster.

Pathophysiology:

The pathophysiology behind chronic zoster associated pain (i.e., PHN) is incompletely

understood. It is generally believed that PHN arises from inflammatory injury to sensory

nerves, ganglia, and nerve roots and associated maladaptive central responses to pain

signaling

MultipleMechanisms appear to be involved, including ectopic discharge from damaged

primary afferent neurons,central sensitization whereby central neurons develop functional

changes and a state ofhyperexcitability after primary afferent damage, and decreased

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inhibition of neuronal activity incentral structures due to loss of inhibitory

neurons.Noordenbos thought that pain and hyperesthesia in PHN were due to preferential

destruction of larger myelinated fibers in the peripheral nerve, leaving an excess of the small

myelinated and unmyelinated fibers. This would result in a loss of inhibitory (large-fiber)

input to the spinal cord with unopposed, nociceptive, afferent bombardment. Zacks etal found

no support for the role of persistent inflammation and were unable to correlate fibrosis and

pain, since they noted equal degrees of peripheral neural fibrosis in patients with and without

PHN. Evidence that ongoing small-fiber input might be important in painful nerve injuries

has been reported by Wall and Gutnick. They found evidence for preferential sprouting of

small-diameter primary afférents in experimental neuromas occurring after nerve transaction

in the rodent. These axons showed spontaneous activity, increased sensitivity to mechanical

stimuli, and sensitivity to a-adrenergic agonists and to sympathetic efferent activity. The

discovery of pathologic change in the dorsal horn originally described by Head and Campbell

and relief by dorsal root entry zone (DREZ) lesions support the concept of a derangement in

that area of possibly hypersensitive, deafferented neurons.

Interleukin-8 is associated with the pain induced by inflammatory reactions, and there are

high concentrations of interleukin-8 in the cerebrospinal fluid of patients who have

intractable postherpetic neuralgia. Furthermore, postmortem studies of patients who had

prolonged postherpetic neuralgia revealed marked inflammation around the spinal cord, with

massive infiltration and accumulation of lymphocytes. The inflammatory component of

postherpetic neuralgia suggests that suitably timed anti-inflammatory treatment may help

reverse the syndrome or at least limit its progression51

Subtypes of PHN

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PHN Due to Irritable Nociceptors:

Partial injury to peripheral nerve leads to sensitization and spontaneous activity in primary

afferents, including nociceptors. A nociceptor barrage produced by prolonged heat or

capsaicin sensitizes central nervous system (CNS) pain transmission neurons. Following

sensitization, activation of uninjured large-diameter primary afferents projecting to the

sensitized CNS neurons shifts from being perceived as nonpainful to painful. This perceptual

shift in the sensation produced by large fiber afferents extends beyond the stimulated area

(area of primary hyperalgesia). The new expanded area is referred to as the area of secondary

hyperalgesia. Cold application or local anesthetic infiltration of the primary zone terminates

the nociceptor barrage and the area of secondary hyperalgesia shrinks, proving that secondary

hyperalgesia requires ongoing nociceptor input for its maintenance.

In the absence of nerve damage, chronic inflammation could produce an irritable

nociceptor type of PHN. Acute zoster is accompanied by intense inflammation along the

affected peripheral nerve and skin that typically resolves in weeks to a few months. One of

the cases in Watson et al.'s postmortem series had pain of nearly 2 years' duration and

extensive inflammatory infiltrates throughout the peripheral nerve, DRG, and dorsal root.

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Likewise, Smith's series included a patient with pain of 1 year's duration and extensive

inflammatory infiltration in the DRG. Finally, Gilden and colleagues have described a few

PHN patients with evidence of continuing low-level varicella zoster virus (VZV) expression

whose pain responded to antiviral agents. Although present in only a small minority of

patients, chronic inflammation and/or continuing VZV expression could produce sensitization

and abnormal activity in the primary afferent system without requiring deafferentation.

Deafferentation PHN With and Without Allodynia:

Although they are a distinct minority, some PHN patients do not have allodynia. These

patients usually have a profound loss of all sensory modalities (i.e., anesthesia dolorosa).

Case series published by Watson and colleagues, and by Nurmikko and Bowsher, report that

less than 15% of PHN patients have no allodynia. The mechanism of pain in PHN patients

with marked sensory loss on examination who do not have allodynia is unknown. In our

experience, local anesthetic skin infiltration is ineffective. Possible mechanisms for the

continuing pain include deafferentation-induced hyperactivity of CNS pain transmission

neurons and/or disinhibition of CNS neurons due to predominant loss of pain inhibitory A-β

primary afferents or to less CNS inhibitory interneurons.

There is a third group of PHN patients who have a significant thermal sensory deficit, but

also have prominent allodynia. Animal studies suggest two possible explanations for this

combination of marked sensory loss and allodynia. In the first, primary afferent fibers that are

no longer connected to the skin could still be connected to their central targets, analogous to a

neuroma from a peripheral nerve injury. If those afferents are spontaneously active C

nociceptors, sensitization of the CNS would occur in the same manner as in the irritable

nociceptor type. Therefore, skin areas with primary afferents responsive to gentle mechanical

stimulation would exhibit allodynia through the secondary hyperalgesia mechanism. A

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second possibility is deafferentation-induced central reorganization. As described by

Shortland and Woolf in rats, second-order CNS pain transmission neurons in the substantia

gelatinosa that have lost their usual input from primary afferent nociceptors are contacted by

large-diameter myelinated afferents that sprout and grow in from their normal targets in the

deeper layers of the dorsal horn. These large-diameter afferents, when activated by gentle

mechanical stimulation, produce pain (allodynia) through their new direct connections to

CNS pain transmission cells. A third possibility is preserved islands of irritable nociceptors in

a region of deafferentation.

Clinical Presentation and Assessment of Herpes Zoster and PHN

Prodromal Phase:

A prodrome of herpes zoster commonly precedes rash onset by 48 to 72 hours, during which

patients often report headache, photophobia, and malaise, but rarely fever. Patients may

experience acute neuritis characterized by dermatome-restricted pain paresthesia, itching,

tingling or other abnormal skin sensations for days or even weeks before zoster rash onset.

The diagnosis of herpes zoster during the prodromal phase can be very challenging as the

rash, the pathognomonic feature of this disease, is absent at this stage. While a painful

prodrome is a risk factor for developing PHN, most studies do not support empiric zoster

treatment in the absence of a rash. For example, in a prospective study of 57 elderly patients

who presented to their general practitioner with unilateral pain of no obvious cause, only two

patients went on to develop herpes zoster over the next 28 days. Therefore, unless there is a

high index of suspicion that the patient has a zoster prodrome, it is probably best not to offer

empiric zoster treatment up front, but instead advise the patient to return promptly if a rash

appears49.

Rash Phase:

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The clinical diagnosis of herpes zoster is usually made during the eruptive or rash phase

when the typical erythematous maculopapular rash appears in a unilateral dermatomal

distribution. In the immunocompetent host, the rash does not cross the midline and most often

involves dermatomes from T3 to L3. While simultaneous involvement of multiple

noncontiguous dermatomes almost never occurs in immunocompetent patients, in up to 20%

of cases lesions overlap adjacent dermatomes. Moreover, the presence of a few lesions away

from the primary affected or adjacent dermatomes is neither uncommon nor prognostically

important in immunocompetent patients. Over the next 3 to 5 days, the rash quickly

progresses from maculopapular to clusters of clear vesicles, which then evolve through stages

of pustulation, ulceration, and crusting. Healing generally occurs over 2 to 4 weeks, and

frequently leaves some degree of scarring and permanent changes in pigmentation49.

In the immunocompetent host, the herpes zoster rash does not usually cross the midline.

It often involves the thoracic dermatome.

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Fig :Dermatomes

Nature of pain in PHN:

Lancinating pain and paresthesias sometimes associated with allodynia (pain after repeated

non noxious stimuli such as washing the face or brushing the hair) is noticed in postherpetic

neuralgia. As postherpetic pain can be very severe, even disturbing sleep and disabling

patients. The pain is often accompanied by a sensory deficit and there is a correlation

between the degree of sensory deficit and the severity of pain7,8,9,48,50,53.

MANAGEMENT OF PHN:

First line medications for PHN

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Tricyclic antidepressants:

Tricyclic antidepressants (TCAs) have been considered the first-line treatment for patients

with PHN. TCAs have a central analgesic effect that is separate from their antidepressant

effect, therefore, often used for patients with chronic pain syndromes. Multiple controlled

trials have now shown that TCAs are efficacious for treating PHN. As it is the best studied,

amitriptyline is the most commonly prescribed TCA for the treatment of PHN and other

chronic pain syndromes. However, amitriptyline is poorly tolerated and should be avoided in

elderly patients. In a randomized, double-blind trial of patients with PHN, nortriptyline was

compared to amitriptyline and found to be equally effective but better tolerated.

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Consequently, nortriptyline is now considered the preferred TCA for treatment of PHN.

Desipramine is a second option for patients who experience excessive sedation from

nortriptyline49.

Although TCAs lessen pain by inhibiting reuptake of serotonin and nor epinephrine, they

require atleast three months for positive effects. In a randomized trial of patients older than

60years, it was observed that 25mg amitriptyline administered within 48 hours of the rash

onset and continued for 90 days, yielded a 50 % reduction in pain at six months compared to

placebo. The use of TCAs such as amitriptyline, nortriptyline, doxepin & desipramine is well

established method of reducing the chronic burning pain characteristic of PHN.

Gabapentine :

It is structurally related to neurotransmitter gamma aminobutyric acid. It is given in 4 divided

doses a day. 1200 -3600 mg /day orally in q.i.d or b.i.d doses. The initial dose started from

300mg q.i.d initially and then titrated 300mg/day31,32.

Side effects: somnolence, dizziness, ataxia, weakness, paraesthesia, fatigue, headache,

nystagmus, diplopia

Precaution: should be used with caution in the patients with renal or hepatic impairment.

To reduce the side effects and increase patients compliance with treatment, gabapentine

should be initiated at low dosages (100-300mg in a single dose taken at bedtime or 100mg

taken 3times per day) and then titrated by 100mg 3 times per day .Gabapentin is the only

FDA-approved oral medication for treating PHN.

Recently gabapentin, was shown in two large controlled trials to be significantly superior to

placebo for the treatment of PHN. In the first study, a total of 229 patients with PHN were

randomly assigned to receive either gabapentin (titrated to a maximum dose of 3600 mg/day)

or placebo. The primary efficacy outcome of the study was change in daily pain score, based

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on an 11-point pain scale (0=no pain, 10=worse possible pain). After 4 weeks, the patients

who were given gabapentin exhibited a significant reduction in average daily pain score from

6.3 to 4.2 compared with a reduction of 6.5 to 6.0 for the placebo group. Secondary measures

of pain as well as changes in sleep interference also showed significant improvement with

gabapentin when compared to placebo.

A second multicenter, double-blind, placebocontrolled trial involved a total of 334 patients

with PHN. In this study, patients were randomly assigned to either gabapentin (either 1800 or

2400 mg/day in three divided doses) or placebo. After 7 weeks, the gabapentin treated

patients showed significant improvement in pain scores compared with those assigned to

placebo. The benefit above placebo was 18.8% for the 1800 mg dose and 18.7% for the 2400

mg dose.

Gabapentin treatment also resulted in significant improvements in sleep, mood and quality of

life.

Opioid analgesics:

The efficacy of opioid analgesics in patients with PHN was first demonstrated in a double-

blind study comparing intravenously administered morphine with placebo. By providing

evidence that PHN-associated pain could be temporarily relieved by infusions of opioid

analgesics, the results of this study suggested that longer-term oral treatment might also be

efficacious. In the studies, when compared with placebo, controlled-release oxycodone

titrated to a maximum dosage of 60 mg per day provided statistically significant benefits with

regard to pain, disability, and allodynia, and controlled-release morphine titrated to a

maximum dosage of 240 mg per day provided statistically significant benefits with regard to

pain and sleep but not physical functioning and mood. One of these studies was a 3-period

crossover trial in which opioid analgesics were compared with TCAs as well as placebo. In

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this trial, patients preferred treatment with opioid analgesics when compared with TCAs and

placebo, despite a greater incidence of side effects during opioid treatment.

Topical therapy:

Topical therapy includes the use of topical anesthetic agents, such as lidocaine 5%, or

analgesics, particularly capsaicin.

Capsaicin:

Capsaicin, an alkaloid derived from cayenne pepper that depletes the neurotransmitter

substance P when used topically, has been shown to be helpful in reducing the pain in

PHN.46,48 Capsaicin cream (0.025% and 0.075%) has been shown to be effective topical

medication for the relief of PHN when applied to the painful region. Application 2 to 3 times

daily of capsaicin depletes substance P, a neuropeptide contained in nociceptor C-fibers that

contributes to neurogenic inflammation and pain. Initial application of capsaicin may result in

burning sensation, but this is diminished after repeated use during the first 48 to 72 hours.

Lidocaine Patch:

As a topical preparation, the lidocaine patch 5% is well tolerated and has an excellent

safety profile. Mild skin reactions (e.g., erythema, rash) have been the only observed side

effects. However, there is minimal systemic uptake of lidocaine that must be considered for

patients taking oral class 1 antiarrhythmic drugs (i.e., mexiletine). The typical treatment

protocol involves applying a maximum of 3 patches per day for up to 12 hours total directly

to the area of maximum pain and/or allodynia. The patch should not be used in patients with

herpes zoster or those with other types of open skin lesions as the available formulation is not

sterile. In general, for most patients the effectiveness of the patch is apparent within 2 weeks,

so prolonged dose escalation is not necessary.

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Topical local anesthetic with a lidocaine patch (5%) has been shown to be effective in

providing pain relief in established PHN in two randomized, double-blind, vehicle-controlled

studies.In the more recent of these studies, lidocaine provided significantly greater pain relief

than did the vehicle control with patients remaining on the lidocaine patch 3.7-fold longer

than on the vehicle patch. At the completion of the study, 25/32 (78.1%) of subjects preferred

the lidocaine patch treatment as compared with 3/32 (9.4%) the placebo patch. On the bases

of these studies, the FDA has specifically approved the 5% lidocaine patch for the treatment

of PHN.

TENS: Use of TENS therapy has been beneficial in the management of PHN. In one review

the use of combination therapy consisting of amitriptyline, topical capsaicin and TENS was

recommended for the treatment of PHN over antiviral therapy.

Nutritional supplements:

Nutritional supplements like, L- lysine, vitamin C, vitamin E, vitamin B complex, zinc,

calcium and magnesium, can be used to support nerve health and provide protection from

free radicals. Herbals, such as green tea, used to provide antiviral, antioxidant and anti-

inflammatory support.

PROSPECTS FOR THE PREVENTION OF PHN:

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 Because PHN can be refractory not only to first- and second line treatment but also to all

other therapies, its prevention is a very important goal. In recent research, older age, greater

acute pain during herpes zoster, and greater rash severity have been identified by independent

groups of investigators as risk factors for PHN. These risk factors provide substantial support

for the conclusion that there is a greater risk of PHN in patients with more severe acute

infections, which are accompanied by greater neural damage. It has been proposed that this

neural damage in patients with herpes zoster contributes prominently to the development of

PHN, and that PHN might therefore be prevented by reducing the severity of the herpes

zoster infection.

One method of reducing the severity of the acute infection and limiting the degree of

neural damage it causes involves treatment with the antiviral agents acyclovir, famciclovir,

and valacyclovir. By inhibiting viral replication, these antiviral agents attenuate the severity

of the acute herpes zoster infection— specifically, the duration of viral shedding is decreased,

rash healing is hastened, and the severity and duration of acute pain is reduced. The results of

randomized, controlled trials and meta-analyses have also demonstrated that antiviral therapy

in herpes zoster significantly reduces the risk of prolonged pain. Although the results of each

of these studies taken singly can be challenged, the consistency of the findings provides

strong support for the use of antiviral agents in the treatment of herpes zoster. Antiviral

therapy has been recommended in several recently published literature reviews and treatment

guidelines for patients with herpes zoster who are older, have moderate or severe rash, have

moderate or severe pain, or have ophthalmic involvement.

Although the reduction in the risk of PHN that accompanies antiviral therapy in patients

with herpes zoster is both clinically and statistically significant, antiviral therapy does not

prevent PHN in all patients. Almost 20% of patients aged more than 50 years continue to

have pain 6 months after rash onset, despite treatment with famciclovir or valacyclovir

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beginning less than 72 hours after the onset of rash. Although it is possible that new antiviral

agents with greater efficacy will be developed, a different strategy for preventing PHN is to

supplement antiviral treatment. Unfortunately, the results of a number of studies that have

examined the long-term benefits of corticosteroids, TCAs, and nerve blocks in patients with

herpes zoster are either equivocal or in need of replication. Nevertheless, there are

compelling reasons to predict that combining antiviral therapy with effective relief of acute

herpes zoster pain (for example, by administration of opioid analgesics or gabapentin) will

further lessen the risk of PHN beyond that achieved by antiviral therapy alone. The basis for

this hypothesis is provided by the very well replicated relationship between acute pain

severity and PHN and by recent research on the pathophysiological mechanisms of PHN. But

even if there were no benefit with respect to the later development of PHN, the effective

relief of acute pain in patients with herpes zoster is clearly a very desirable treatment goal in

itself.

OCCIPITAL NEURALGIA

Introduction:

The greater occipital nerve is a continuation of the dorsal ramus of the second cervical

nerve. It moves superiorly between sternocleidomastoid and trapezius muscles to innervate

the posterior scalp. Compression of this nerve includes paresthesia or dysethesia in the back

of the head. True occipital neuralgia is rare, but when present, the paroxysmal pains are felt

in the posterior occipital region radiating up the back of the head. The pain may also be felt in

the cervical region.

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 While the parenchyma of the brain is insensate, the scalp, head muscles, periosteum, dura,

and blood vessels are all pain-sensitive; thus, there are many possible causes of head and face

pain. Occipital neuralgia is a headache syndrome that may be either primary or secondary.

 Primary headaches have no clear structural or disease-related cause, (eg, migraine,

tension, and cluster headaches). Primary headaches constitute the etiology of >90% of

head and facial pain and occipital neuralgia is often confused with other primary

headache syndromes, including migraine and cluster headaches.

 Secondary headaches have an underlying disease process that may include tumor,

trauma, infection, systemic disease, or hemorrhage.

Etiology:

Patients with occipital neuralgia may be divided into those with structural causes and those

with idiopathic causes. Structural causes include:

• Trauma to the greater and/or lesser occipital nerves

• Compression of the greater and/or lesser occipital nerves or C2 and/or C3 nerve roots by

degenerative cervical spine changes

• Cervical disc disease

• Tumors affecting the C2 and C3 nerve roots.

The greater occipital nerve receives sensory fibers from the C2 nerve root and the lesser

occipital nerve receives fibers from the C2 and C3 nerve roots. The third occipital nerve

(least occipital nerve) stems from the medial sensory branch of the posterior division of the

C3 nerve root and travels along the greater occipital nerve. It passes through the trapezius and

splenius capitus slightly medial to the greater occipital nerve. Clinically, the third occipital

nerve may also be involved in causing occipital neuralgia. Cervical spine changes include

spondylosis, arthritis of the upper cervical facet joints, and thickening of the ligaments in that

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area (particularly C1-4 levels). Some cases of presumed occipital neuralgia may in fact be C2

or C3 radiculopathies. Compression of the greater occipital nerve is possible as it travels up

the neck, passing through the semispinalis and trapezius muscles. Whiplash or

hyperextension injury may lead to this scenario. Other possible causes include localized

infections or inflammation, gout, diabetes, and blood vessel inflammation. Although it cannot

be quantified, most patients fall in the category of ―unknown cause,‖ when no identifiable

lesion is found.

Clinical features :

 Occipital neuralgia symptoms include aching, burning, and throbbing pain that is often

unilateral and continuous with intermittent, shocking, shooting pain.

 The pain usually originates in the suboccipital area and radiates to the posterior and/or

lateral scalp. Occasionally, patients report pain behind the eye on the affected side.

 Pain may also be perceived over the neck, temple, and frontal regions. Pressure over the

occipital nerves may amplify the pain, but there is usually no clear trigger.

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 Occasionally, neck movements (eg, extension and rotation) may trigger pain. At times,

patients with occipital neuralgia may experience symptoms similar to migraine or even

autonomic changes characteristic of cluster headaches.

 Associated symptoms include posterior scalp paresthesias, photophobia, and dizziness.

Many patients with occipital neuralgia report a cycle of pain-spasm-pain.

Diagnosis

Thorough history-taking and a complete physical and neurological examination are necessary

in diagnosing headache. A diagnosis is usually made based on the characteristic area of the

pain. In addition, finding tender areas that exacerbate the pain aids in diagnosis. It is

important to clarify whether the cause of occipital neuralgia is structural or idiopathic.

Abnormal findings on neurologic exam usually indicate a structural cause, in which case,

computed tomography (CT) or magnetic resonance imaging (MRI) of the head and cervical

spine may be indicated. The work-up of occipital neuralgia should include assessment for

atlanto-axial joint instability. Patients with a history of rheumatoid arthritis or trauma should

receive a thorough spine work-up. Diagnostic occipital nerve blockade also aids in diagnosis.

Occipital neuralgia often is confused with migraines and other headache syndromes . In

some cases, occipital neuralgia is misdiagnosed as fibrocytis or fibromyalgia, cervical spine

arthritis, or cervical disc disease.

According to the International Headache Society, the diagnostic criteria for occipital

neuralgia include the following (International Headache Society, 2005):

A. Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the

distribution(s) of the greater, lesser and/or third occipital nerves

B. Tenderness over the affected nerve

C. Pain is eased temporarily by local anaesthetic block of the nerve

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Diagnostic Occipital Nerve Blocks

Greater occipital nerve blocks have been advocated as a diagnostic test for cervicogenic

headache and occipital neuralgia. However, criteria and standards for diagnostic occipital

nerveblocks remain to be defined. There are no well-designed clinical trials that clearly

indicate thatinjection of the greater occipital nerve can be used as a specific diagnostic test

for headachesand occipital neuralgia.

Differential diagnosis:

1. Migraine headache:

Clinical features- Unilateral hemi cranial,pulsating, throbbing with sensitivity to light

and nausea. May have visual aura. Lasts 4-72 hours if untreated. It has peak incidence

25- 34 years old, 3-4 times more common in women than men. Family history of

migraine is common.

Pathophysiology – This is associated with cranial perivascular inflammation via

trigeminal nerve

2. Tension headache:

Clinical features – Usually bilateral, dull, pressing, squeezing, bandlike quality. May last

from 30 minutes to 7 days. Sensitivity to light and sound, but no nausea, may affect

frontal, frontal- occipital, orbital area. Most common headache affects both men women

equally.

Pathophysiology – Precise mechanism unknown, likely multifactorial. May be activation

of peripheral nociceptors within neck muscles or ligament.

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3. Cluster headache:

Clinical features – Excruciating, painful, drilling, boring quality that is often debilitating.

May be so severe that many patients contemplate suicide. Severe, unilateral orbital pain.

If untreated, may last from 15 to 180 minutes. Atleast one autonomic sign on painful

side (eg- lacrimation, nasal congestion, rhinorrhea, miosis, eye edema, ptosis,

conjunctival injection). May occur from once a day to 8 times a day in cycle from 1week

to every year.

Pathophysiology – Precise mechanism unknown. May be change in hypothalamic,

endocrine, brain stem, and central nervous system functioning.

4. Cervicogenic headache:

Clinical feature – May have similar presentation as occipital neuralgia, cluster tension,

and migraine headaches. Usually caused by neck movement or change in head position.

Ipsilateral shoulder, neck occiput, temple or per orbital region. Typical constant or

intermittent , but rarely throbbing or lacinating. May have associated nausea and

dizziness.

Pathophysiology – Various anatomic structures may transmit nociceptive signals.

Structure involved include, atlanto-occipital joint, atlanto-axial joint, C2-3 facet joint,

C2-3 disc suboccipital and upper cervical muscles, trapezius, and sternocleidomastoid

muscles.

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Management:

Medications:

Medications that may help relieve pain in occipital neuralgia include gabapentin 300-3600

mg/day, carbamazepine 400-1200 mg/day, phenytoin 300-600 mg/day, valproic acid 500-

2000 mg/day, and baclofen 40-120 mg/day. NSAIDs and opioids may also be beneficial.

Conservative treatment

Physical therapy, massage, acupuncture, and heat are other treatments that can be used for the

treatment of occipital neuralgia.

NERVE BLOCKS:

Nerve blocks consisting of steroids and local anesthetics may also be considered for

treatment of occipital neuralgia.

Occipital nerve block:

Occipital nerve block is indicated for the diagnosis or treatment of occipital neuralgia. The

greater occipital nerve is 2.5 to 3 cm lateral to the external occipital protuberance and medial

to the occipital artery. The third occipital nerve is medial to the greater occipital nerve and

the lesser occipital nerve is about 2.5 cm lateral to the artery.

The greater and third occipital nerves are blocked slightly above the superior nuchal line,

just medial to the occipital artery, which is easily palpated. After antiseptic preparation, a 25

gauge 11/2 inch needle attached to a 5 ml syringe is placed just medial to the artery at the

above location. For diagnostic indications, 1 ml of local anesthetic is injected. For treatment,

3-5 ml of local anesthetic combined with steroid is injected. Anesthesia in the region of the

greater occipital nerve usually occurs within 10 to 20 minutes. The most serious complication

is piercingthe occipital artery and bleeding. Compression of the occipital artery is usually

effective in avoiding any significant problems.

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BOTULINUM TOXIN

Clinical trials have shown that Botox injections for migraine headaches reduced the duration,

length, and severity of the headaches, as well as the intake of migraine medications. Botox

has been shown to be effective in the treatment of whiplash-associated disorders that often

cause occipital neuralgia. It improved the pain and increased the range of motion in these

patients. Because of its success in the treatment of muscle spasms and migraines, botulinum

toxin may prove to be a reasonable treatment option for occipital neuralgia in the future.

Botulinum Toxin Type A (Botox) is an accepted treatment for migraine headache and muscle

spasm related pain with relief up to 4 months. Botox was originally used to treat strabismus

and cervical dystonia. One trial demonstrated that Botox helped chronic daily headache and

appeared to have a cumulative effect with subsequent injections. Treatment with Botox is

generally well-tolerated; side effects are minimal and include minor discomfort or bleeding at

the time of injection.

SURGICAL MANAGEMENT:

Occipital neuralgia can occasionally be treated with micro vascular nerve decompression.

Surgical procedures such as epifacial electric stimulation, dorsal cervical rhizotomy,

neurolysis of the greater occipital nerve, and radiofrequency rhizotomy may also be

considered. Selective C2 and/or C3 dorsal rhizotomy is another option, although few papers

have been published assessing its utility. CT or fluoroscopy-guided percutaneous C2 and/or

C3 nerve block is also useful for confirmation of occipital neuralgia and as a preoperative

guide for dorsal cervical rhizotomy.

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RADIOFREQUENCY THERMOCOAGULATION

Radiofrequency thermo coagulation (RF) is another widely used method to treat occipital

neuralgia. It has many advantages, including safety, efficacy, a rapid recovery period, and no

permanent scarring. C2 ganglionotomy by RF lesion generator has also been performed and

resulted in cases of significant pain relief. Pulsed radiofrequency (PRF) is yet another

technique used to treat occipital neuralgia. In a case report, a patient was treated with PRF

and, after a 12-month follow-up, was pain-free. Recently, a new surgical treatment was

reported consisting of neurolysis of the greater occipital nerve and sectioning of the inferior

oblique muscle.

OCCIPITAL NERVE STIMULATOR IMPLANTATION

Surgical implantation of a subcutaneous electrode along the C1-C3 nerve level has been

shown to significantly reduce the pain of occipital neuralgia in patients who have failed

conservative therapies. In one study of 19 patients, 95% reported improvement in their

quality of life and would undergo the procedure again. In another study of 13 patients, 12

reported good-to-excellent pain control at up to 6 years of follow-up. The benefit of this

procedure is that it is minimally invasive and there is no permanent destruction of nerves or

other vital structures.

Another advantage is that patients can first undergo a percutaneous trial of temporary lead

placement for several days prior to permanent lead implantation. Depending on the results of

the temporary percutaneous trial, patients may or may not undergo the more invasive

permanent lead implantation. It has been postulated that a successful temporary percutaneous

lead trial, in combination with a successful diagnostic occipital nerve block, may predict a

highly effective permanent occipital nerve stimulator implantation.

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SUPERIOR LARYNGEAL NERVE NEURALGIA:

The superior laryngeal nerve is a branch of the vagus& innervates the cricothyroid muscle of

the larynx, which stretches, tenses, and adducts the vocal cord. Paralysis of this nerve causes

hoarseness and fatigued voice, with altered pitch. The clinical characteristics of superior

laryngeal neuralgia are periodic, unilateral submandibular pain radiating through the ear, eye

or shoulder. It is sometimes difficult to differentiate this condition from glossopharyngeal

neuralgia. The pain is paroxysmal, lasting momentarily, and may be provoked by swallowing,

straining the voice, turning the head, coughing, sneezing, yawning or blowing the nose,

occurs in attack of few minutes that reoccur up to 10- 30 times in 24hours with clustering of

different length and tendency to remission. The patient may report an irresistible urge to

swallow. The attack can be associated to an uncontrollable cough and can be triggered by

compression of the point of the entry through the hyoid membrane, sideways, and

immediately above the larynx.Trigger zone is frequently located just superior & lateral to the

thyroid cartilage.

SUPRAORBITAL NEURALGIA

Entrapments of the first-division of the trigeminal nerve can cause unilateral or bilateral

throbbing headaches, often just before menses or triggered by bright lights that cause

squinting. Supraorbital neuralgia can be mistaken for frontal sinusitis. It can be caused by

trauma to the face, such as when the head hits the windshield or after a punch to the face. The

headache might not present for many years until the scar cicatrix tightens enough around the

nerve to finally cause entrapment. There can be auras and unilateral or bilateral throbbing, as

well as photophobia, phonophobia, nausea and vomiting; and these headaches can meet all

the International Headache Society (IHS) criteria for migraines. Fluid retention, such as

before menses or with salt indiscretion (perhaps with red wine, monosodium glutamate, or

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cheeses) can trigger these ―migraines.‖ The supratrochlear nerve is also in this region and can

be injured by poor-fitting eyeglasses, presenting as a more midline forehead pain. We have

also seen patients with ―classic‖ cluster headaches (men, sudden onset, rhino rhea, scleral

injection, cyclic pattern) who have had instant and complete relief of their headaches with

injection of small (0.5 cc volume) of local anesthetic.

Management:

 Treatment (and diagnosis) involve injection of local anesthetic with steroid, preferably

during the headache initially. Small volumes need to be used to avoid increasing the

entrapment, and it has been dramatic how the headache resolves ―almost before the

needle is out,‖ with rapid relief of the nausea, photophobia, and other associated

symptoms.

 Cryoneurablation can give long-term relief by freezing the nerve at the supraorbital notch.

 Plastic surgeons using Botox for forehead wrinkles noted a dramatic decrease in

―migraines‖ in treated patients, suggesting that muscle entrapment of the supraorbital and

supratrochlear nerves may be a common pathology.

 Topical anti-inflammatory agents can also be very useful because of the thinness of the

skin in this area.

INFRAORBITAL NEURALGIA

This second division of the trigeminal nerve is also associated with headaches, often

misdiagnosed as maxillary sinusitis. Like the supraorbital nerve, it can be injured years

before the headaches start and can present as menstrual headaches or classical/common

migraines. The diagnosis is again made by injection, preferably during the headache; and

cryoneurablation (intraoral or extra oral) can be used.

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Neuralgia is a severe painful condition, most common being trigeminal neuralgia and

patients often visit multiple clinicians complaining of the orofacial pain. A thorough

understanding of the epidemiologic and etiologic aspects of dental, musculoskeletal,

neurovascular, and neuropathic orofacial pain conditions is essential to the practice of

evidence-based dentistry. So it is essential that oral physicians recognize the disease and

diagnose it correctly for the patients to receive appropriate referral and therapy for this

relatively treatable condition. Recent investigations have provided an explosion of knowledge

regarding pain mechanisms and pathways and an enhanced understanding of complexities of

many ramifications of the total pain experiences. Therefore, it is mandatory for the oral

physicians to develop the necessary clinical and scientific expertise on which he / she may

recommend diagnostic and management approaches. Optimum management can be achieved

only by determining an accurate and complete diagnosis and identifying all of the factors

associated with the underlying pathosis on a case specific basis.

Medical management remains the first step to treat neuralgias. Several new medications have

been tried in treating various types of neuralgia, and the recent advance in non invasive

therapy such as laser acupuncture have been tried with promising results in treatment of

neuralgias. Recent advances like gamma knife and cyber knife have been tried in treating

neuralgia

Many cases of neuropathic orofacial pain are best managed by a multidisciplinary team

involving dentists, neurologists, neurosurgeons clinical and health psychologists and other

health care disciplines. Being oral physicians, we must be aware of the existence of co

morbid conditions and address them appropriately to optimize treatment outcome

DIAGNOSIS AND TREATMENT FOR TRIGEMINAL NEURALGIA

126
127
Management Algorithm of Trigeminal neuralgia

FACIAL
PAIN

1. Paroxysmal
2. Trigger zones Further evaluation by
No
3. Unilateral inter disciplinary oral,
4. Restricted to area of facial & head pain center
trigeminal nerve
5. No sensory deficits

Neurology
Yes Lesion Neurosurgery
consult

MRI Demyelinating Neurology

Normal plaques consult

Gabapentin
Carbamazpine Vascular Imaging +
Lamotrigine abnormality Neurosurgery consult
Baclofen
Topiramate
topiramate
Not tolerated

Not effective

Pain Relief

Percutaneous
Yes Radiofrequency thermal
rhizotomy

Decrease Decrease
slowly dose
slowly

Recurrence of
pain

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BURNING MOUTH SYNDROME

Burning mouth syndrome is defined as a burning painful sensation in the mouth (oral

dysesthesia) with normal clinical examination and no obvious organic cause. BMS is

therefore a diagnosis of exclusion, made only after excluding all other causes of mouth pain.

Evidence suggests that this disorder has a multifactorial cause, with neurologic, psychogenic

and hormonal factors all contributing to the disease.

Prevalence/Incidence:

Prevalence rate is reported to be between 0.7% & 5% of the general population in one study

whereas other studies reported a prevalence between 3.7%- 18% and even upto 40%.It is

commonly reported in women between the ages of 50s and 70s. It is considered rare under

the age of 30. Female: male predilection ranges from 3:1 to 16:1.It is usually present 3yrs

before and 12 years after menopausal age in women.

Symptoms:

Persistent or constant oral mucosal pain daily Burning, scalding,numb feeling, tingling

 Location (one or more)-tongue, oral

mucosa, oropharyngeal areas,lips,nasal

mucosa

 Intensity -variable,weak to intense

 Pattern/timing-continuous not paroxysmal

 Localization-often bilateral, symmetric,

independent of nervous pathways.

Types:

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 Three patterns of oral pain have been identified

Type1: pain absent on waking and developing

during the day.

Type2: pain present day and night.

Type3: intermittent pain with pain-free days

Dysgeusia 70% Persistent taste, altered taste, metallic taste, bitter

taste

Xerostomia 46-67% With or without salivary gland hypofunction

Clinical examination

A thorough history is vital in arriving at a definitive diagnosis and should include a present

illness with previous treatments listed, a detailed past medical history, a complete list of

current medications, and a thorough review of systems. An exhaustive extra-oral and intraoral

examination should be performed following a detailed history. Local factorssuch as denture

fit and design, dental trauma,signs of parafunctional habits, mechanical or chemical

irritantinfection,hyposalivation,lesions and allergies should be the focus.

Diagnostic modalities:

The diagnosis of BMS is often complex due to the following multiple reasons:

 The diagnosis relies on the patient‘s presenting symptoms.

 The symptomatic triad is rarely present or overlapping of other contributing factors.

 The diagnosis is obtained after eliminating other potential causes for oral burning.

 A careful evaluation of any structure in the head and neck complex that may potentially

cause oral pain should be performed. The following additional evaluations may be

necessary:

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 Sialometry to evaluate the oral dryness

Unstimulated whole saliva >0.1mg/min

Stimulated whole saliva > 0.7mg/min

 Biopsy of minor salivary glands if Sjogren syndrome is suspected

 Biopsy or cytology if any mucosal lesions will be included in differential diagnosis

 Culture of oral samples ,to rule out fungal, viral and bacterial infections

 Haematological test that may include complete and differential blood counts, fasting

blood sugar levels, thyroid panel , nutritional factors to rule out deficiencies such as iron,

folate and B12), autoimmune panel (ANA, Rf,Anti-SSA,Anti-SSB)

 Skin patch test to rule out allergic reaction

 Magnetic resonance imaging if BMS is associated with neuralgia or trigeminal nerve

neuropathy is included in the differential and to rule out systemic conditions

 Trial of discontinuation of medications such as angiotensin- converting enzyme inhibitors

known to cause symptoms may be considered

 Psychometric tests including symptoms checklist 90 revised, multidimensional pain

inventory, hospital anxiety and depression scale ,Beck depression Inventory can be

considered to evaluate the influence of psychological factors

 Gastric reflex studies may also be considered

Classification of BMS

Source, year Diagnostic criteria

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Fortuna etal ,2013 Suggests rename as complex oral sensitivity disorder(COSD)

and defines as an oropharyngeal discomfort due to one or more

symptoms for which no specific cause of any type can be

identified in the following criteria

1. Any type of oropharyngeal symptom that can be persistent

or intermittent with possible phases of remission /

exacerbation during the day

2. Absence of any clinically and instrumentally detectable

oropharyngeal lesion

3. Absence of any type of local and/or systemic factors such

as oral diseases, drugs, trauma, hypersensitivity reactions,

physical/chemical agents

ICHD II , 2004 Describes as an imtra-oral burning sensation for which no

medical or dental cause can be found and the diagnostic criteria

as follows

A. Pain in the mouth present daily and presenting most of

the day.

B. Oral mucosa is of normal appearance

C. Local and systemic diseases have been excluded

D. Pain may be confined to the tongue (glossodynia) with or

without xerostomia, paresthesia and dysgeusia

Scala etal , 2003 1. Primary BMS or essential /idiopathic BMS with no

organic or systemic cause

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 2. Secondary BMS resulting from local/systemic cause

Muzyka & De Rossi,1999 Type percentage symptoms

1 35% Awaken without symptoms

Progress throughout the day

Present daily

Food/drink relieve symptoms

2 55% Awaken with symptoms

Progress daily

Food/drink relieve symptoms

3 10% Occasional symptoms

Worsen with food/ drink

Unusual oral sites affected

Increases incidence of contact allergy

International association Defined as ―all forms of burning sensation in the mouth

for the study of pain including complaints described as stingling sensation or pain, in

(IASP)(Merksey and association with an oral mucosa that appears clinically normal in

Bogduk) , 1994 the absence of local or systemic diseases or alterations

Lamey &lewis ,1989 Type 1: Progressive pain throughout the day

Type 2: constant throughout the day

Type 3: symptoms are intermittent and there are some symptom-

free days

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Management

Establishing a definitive diagnosis- Separating oral burning from BMS, ruling out local and

systemic causes of oral burning that is not pertinent to definitive diagnosis of BMS

Understanding the local, systemic and psychological factors that may be responsible for oral

burning associated with secondary BMS and therefore a foundation for diagnosing primary

BMS

Establishing a treatment plan based on the presenting symptoms and clinical presentations in

the initial visit and treatment modifications based on investigations and prior treatment

outcomes in the following visits.

Pharmacological strategies

BMS is a multifactorial chronic neuropathic condition that requires therapeutic strategies that

include pharmacological interventions directly relating to the symptoms and/or treating the

underlying local, systemic, and or psychological factors. These strategies target different

factors that may be directly related to the symptoms and signs or to subclinical neuropathic

condition.

Treatment strategy can be based on the following:

Palliative

Symptomatic

Therapeutic

Combinations of the above

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Step1: Diagnose and manage local and systemic cofactors related to secondary BMS

 Local

o Oral examination

o Salivary hypo function

o Parafunctional habits

o Contact allergies

 Systemic

o Hematological parameters

o Nutritional deficiencies

o Hormonal disturbances

o Side effects from medications

 Psychological factors

Step2: Multidisciplinary management of primary or idiopathic BMS

Based on published randomized clinical trials:

 Topical

o Clonazepam

 Systemic

o Alpha-lipoic acid

o Selective serotonin reuptake inhibitors(paroxetine, sertraline)

o Amisulpride

o Anticonvulsants (gabapentin)

 Cognitive behavioral therapy

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Based on expert opinion and common clinical practice but not yet evaluated

 Topical

o Capsaicin

o Doxepin

o Lidocaine

 Systemic

o Tricyclic anti depressants

o Serotonin- norepinephrine reuptake inhibitors

o Anti-convulsants

o Opioids

o Benzodiazepins - Clonazepam, Alprazolam

TOPICAL MEDICATIONS

Medications Specific examples Dose Directions

Benzodiazepins Klonazepam wafer/orally 0.25mg- 0.25 mg at

disintegrating tablets 2mg/d bedtime;increase dosage

by 0.25 mg every 4 to 7

days until oral burning is

relieved or side effects

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 occur;as dosage increases,

medication is taken as full

dose or in 3 divided doses

Anesthetic Lidocaine 2% viscous gel Variable Applied PRN on the oral

mucosa/tongue

Atypical analgesic Capsacin cream Variable Rinse mouth with 1

teaspoon of a 1:2 dilution

(or higher) of hot pepper

and water.increase the

strength of capsaicin as

tolerated to a maximum

of 1:1 dilution

Anti-depressant Doxepin 5%cream Variable Apply every 4-6 hrs

Nonsteroidal anti- Benzydamine oral rinse Variable Dispense 5ml, swish for

inflammatory 30sec and spit tid

Anti-microbial Lactoperoxidase oral rinse Variable Dispense 5ml, swish for

30sec and spit bid

Mucosal-protectant Sucralfate oral rinse Variable Dispense 5ml, swish for

30sec and spit tid

SYSTEMIC MEDICATIONS

Medications Specific examples Dose Directions

Benzodiazepine (low dose) Clonazepam 0.5-2mg/day 0.25 mg at

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 bedtime; increase

dosage by 0.25 mg

every 4to7days

until oral burning is

relieved or side

effects occur; as

dosage increases,

medication is taken

as full dose or in 3

divided doses

Chlordiazepoxide 10-30mg/day 5mg at bed time;

Increase dosage by

5mg every 4to 7

days

until oral burning is

relieved or side

effects occur; as

dosage increases,

medication is taken

a in 3 divided doses

Anti-convulsants Gabapentin 300-1600mg/day 100mg at

bedtime;increase

dosage by 100mg

every 4 to 7 days

until oral burning is

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 relieved or side

effects occur;as

dosage increases

medication is taken

in 3 divided doses

Pregabalin 25-300mg 25mg at bedtime;

increase dosage by

25 mg every 4 to 7

days until oral

burning is relieved

or side effects

occur;as dosage

increases

medication is taken

in 3 divided doses

Antidepressants Amitriptyline 10-150 mg/day 10mg at

(low dose) bedtime;increase

dosage by 10 mg

every 4 to 7 days

until oral burning is

relieved or side

effects occur

Nortriptyline 10-150mg/day 10mg at bedtime;

increase dosage by

10 mg every 4 to 7

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 days until oral

burning is relieved

or side effects

occur

Selective serotonin reuptake Paroxetine 20-50mg/day 10-150 QAM

inhibitors Sertraline 50-200mg/day Start 50mg PO

QD,may increase

25-50 mg every 4

to 7 days until oral

burning is relieved

or side effects

occur

Trazodone 100-400mg/day Start 50 mg PO

BID/TID, may

increase 50 mg

every 4 to 7 days

until oral burning is

relieved or side

effects occur

Selective norepinephrine Milnacipran 100mg/d 50 mg BID, start

reuptake inhibitors with 12.5mg then

12.5 BID every 4

to 7 days until oral

burning is relieved

or side effects

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 occur,

max 200 mg/d

Duloxetine 60-120mg/d 60mg-120mg PO

QD

Antioxidant Alpha lipoic acid 600-1200mg 300mg/600mg BID

Atypical antipsychotic Olanizipine 5-20 mg/d 5-20mg PO QPM

Dopamine agonist Pramipexole 0.125-0.5mg Start 0.125mg PO

PQ QPM QPM, may increase

0.125mg/d q4-7

max, 0.5mg/d,2-3

hrs before bedtime

Herbal supplement Hypericum 300mg to 1800 300mg tid

perforatum( mg/d in divided

StJohns wort) doses

Salivary stimulants Pilocarpine 15-40 mg/day 5mg/10mg

cevimiline 90-120 mg/day TID/QID

30mg TID/QID

Non pharmacological strategies

Cessation of parafunctional habits such as clenching, bruxism, tongue protrusion that may

contribute to oral burning is advised. Desensitizing appliances can be used to reduce oral

burning and can also be used as a habit-breaking appliance. Modification of oral care

products, such as alcohol- free mouthwashes, andregular oral care products without flavoring

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agents can be considered. Patients with influence of psychological factors couldbe counseled

for stress management

Symptoms associated with BMS can be quite varied and can have a negative impact on oral

health related quality of life. Management of BMS can be challenging for clinicians, as the

treatment is aimed at the relief of symptoms without a definitive cure.

PRIMARY HEADACHE DISORDERS

Primary headache disorders include migraine, tension-type headaches, and the trigeminal

autonomic cephalgias (TACs). "Primary" refers to a lack of clear underlying causative

pathology, trauma, or systemic disease. The TACs include cluster headache, paroxysmal

hemicrania, and short-lasting neuralgiform headache attacks with conjunctival injection and

tearing (SUNCT). Hemicrania continua, although classified separately by the International

Headache Society, shares many features of both migraine and the TACs

MIGRAINE

Migraine is the most common of the vascular headaches, which may occasionally also cause

pain of the face and jaws. It may be triggered by foods such as nuts, chocolate, and red wine,

stress, sleep deprivation, or hunger.

EPIDEMIOLOGY

In the Global Burden of Disease Study2010 (GBD2010), it was ranked as the third most

prevalent disorder in the world. In GBD2015, it was ranked third–highest cause of disability

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worldwide in both males and females under the age of 50 years.Migraine is more common in

women.

MWA is an inherited disorder affecting the young, with an onset before the age of 20

years in about half of the cases.

ETIOLOGY AND PATHOGENESIS

The classic theory is that migraine is caused by vasoconstriction of intracranial vessels

(which causes the neurologic symptoms), followed by vasodilatation (which results in

pounding headache).

Newer research techniques suggest a series of factors, including the triggering of neurons in

the midbrain that activate the trigeminal nervous system in the medulla resulting in the

release of neuropeptides such as substance P. These neurotransmitters activate receptors on

the cerebral vessels walls, causing vasodilatation and vasoconstriction.

Phases of migraine presentation

Migraine presentation may be divided into phases and each phase may occur alone or in

combination with each other. The headache is identical in both MA and MWA. The phases

are divided into four.

Prodrome: this phase includes the premonitory signs and symptoms occurring days or hours

before some or all headaches.it consists ofnonspecific neurologic / autonomic signs, and

constitutional symptoms, tiredness, difficulty in concentration and stiff neck.

Aura (MA): focal neurologic signs or symptoms such as visual(flashing lights), sensory (pins

and needles), and motor (speech) are present in this phase.it develops over 5 to 20 minutes

and last for less than 60 minutes.it is followed in about 10 minutes by a typical headache.

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Headache phase:It is typically unilateral with no side preference. Side-locked migraine in

upto half of migraineurs is seen. In some patients it is bilaterally seen. It is usually seen in

ocular, temporal and frontal regions along with occipital and neck regions. It has a throbbing

or pulsating nature.it have moderate to severe intensity.Sharp periorbital ―ice-pick‖ pains

interictally has been observed. Routine physical activity aggravates pain.Moving the head or

coughing will accentuates headaches.Headache is insidious may take 0.5-2.0 hours.periodic

typically lastingupto 4 to 72 hours. Frequency is in most cases less than 1 per month but may

vary from upto 2-12 headaches per month. Vast majority report nausea and photophobia or

phonophobia.50% vomit during an attack. Autonomic signs includes usually

lacrimation(=50%)

Postdrome: In this depression, irritability and tiredness occurs.

TYPES OF MIGRAINE

Migraine with aura(classic migraine)

Migraine without aura(common migraine)

Chronic migraine

CLINICAL MANIFESTATIONS

Classic migraine starts with a prodromal aura that is usually visual but that may also be

sensory or motor. The visual aura that commonly precedes classic migraine includes flashing

lights or a localized area or depressed vision (scotoma). Sensitivity to light, hemi anesthesia,

aphasia, or other neurologic symptoms may also be part of the aura, which commonly lasts

from 20 to 30 minutes. The aura is followed by an increasingly severe unilateral throbbing

headache that is frequently accompanied by nausea and vomiting. The patient

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characteristically lies down in a dark room and tries to fall asleep. Headaches

characteristically last for hours up to 2 or 3 days

Common migraine is not preceded by an aura, but patients may experience irritability or

other mood changes. The pain of common migraine resembles the pain of classic migraine

and is usually unilateral, pounding, and associated with sensitivity to light and noise. Nausea

and vomiting are also common.

Basilar migraine is most common in young women

The symptoms are primarily neurologic and include aphasia, temporary blindness, vertigo,

confusion, and ataxia. These symptoms may be accompanied by an occipital headache

Facial migraine (Carotodynia) causes a throbbing and / or sticking pain in the neck or jaw.

The pain is associated with involvement of branches of the carotid artery rather than the

cerebral vessels. The symptoms of facial migraine usually begin in individuals who are 30 to

50 years of age. Patients often seek dental consultation, but unlike the pain of a toothache,

facial migraine pain is not continuous but lasts minutes to hours and recurrent tenderness of

the carotid artery. Face and jaw pain may be the only manifestation or migraine, or it may be

an occasional pain in patients who usually experience classic or common migraine.

Chronic migraine

Some migraine sufferers may have a clinically progressive disease in which migraine

episodes increases in frequency over time .a proportion of migraineurs ( 15.6%) describe

daily or near daily headaches and approximately 2.5% of the general population has chronic

migraine. The risk to progress from episodic to chronic migraine increases significantly in

whites with obesity and a high baseline headache frequency.

It is bilateral mainly in fronto temporal region upto half may be strictly unilateral. Mostly

mild to moderate. Dull pressing quality . occurs more than 15 days permonth(.3months) with

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no medication over. Truly continuous headache in fewer than half of patients . superimposed,

severe typical migraine attacks occur.night time arousals due to headache reported,

particularly by women. Most cases seem to begin as episodic migraine that at approximately

age 30 to 40 became increasingly frequent.particularly in women, CM is still accompanied by

mild migrainous features.menstrual relation and other triggers may still be prominent.

Anxiety and depression seems to be common in patients with CM , affecting one-third to

nearly 90% of patients hypo thalamic dysfunction has been found in CM

Migraine triggers

 Several factors have been reported as initiators of individual attacks in migraineurs

termed triggers or precipitating factors and reported by 90% of migraineurs.

 Anxiety and stress

 Fatigue, sleeping difficulties

o Occasionally woken from sleep by a migraine, early morning

o Interestingly, sleeping may abolish headache

 Foods and drinks

 Menstruation

o Hormone variations associated with migraine onset and patterns

o A quarter of women report menstrual related migraine, more so in

clinic based populations

o Improvement or resolution of migraine headaches during late

pregnancy

 Weather changes

 Smells, smoke and light

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Differential diagnosis

 Tension-type headache(TTH)

o Clinical overlap with mild MWA is prominent

 Oral/dental

o Neurovascular pain in the lower face/oral cavity reported

o Termed facial migraine, lower half facial migraine or neuro vascular orofascial

pain

 Sinusitis

Extremely common misdiagnosis, particularly in migraine with midface pain

 Vascular disorders

Transient ischemic attacks,thromboembolic stoke, intracranial

hematoma,subarachnoid hemorrhage, and arterial hypertension may cause migraine

like headaches.

 Intracranial tumors, infections and regional trauma may induce migraine like

headaches.

 Some are sudden -onset headaches or are accompanied by atypical neurological signs

and symptoms

 Cervicogenic headache may clinically resemble headache

TREATMENT

Although there is no cure, adequate control can be achieved for most migraineurs

Non pharmacological treatment

Patient education

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Patients with migraine should be carefully assessed to determine common food triggers.

Attempts to minimize reactions to the stress of everyday living by using relaxation techniques

may also be helpful to some patients.

Pharmacological treatment

Drug therapy may be used either prophylactically to prevent attacks in patients who

experience frequent headaches or acutely at the first sign of an attack.

Abortive (acute,symptomatic)

It aims to rapidly relieve headache with no recurrence or side effects. It is used when fewer

than 4 to 8 attacks per month or to supplement prophylactic regimens.Drugs that are useful in

aborting migraine include ergotamine and sumatriptan, which can be given orally, nasally,

rectally or parentally. These drugs must be used cautiously since they may cause

hypertension and other cardiovascular complications.Methylsergide or monoamine oxidase

inhibitors such as phenelzine can be used to manage difficult cases that do not respond to

safer drugs. Complementary treatments such as butterbur, feverfew and coenzyme-Q may

also be effective.

Some of the common abortive treatments of migraine

Class Drugs Initial oral dose,mg

Analgesics Aspirin 500-1000mg

Combinations Aspirin +paracetamol 500-600+200-400+50-200

+caffeine

Paracetamol+ codeine 400+25

Ergot alkaloids Dihydroergotamine NS 2

NSAIDs

Non-specific Naproxen sodium 550-825mg

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 Ibuprofen 400-800mg

Diclofenac 50-100mg

Selective COX-2 inhibitors Rofecoxib 25-50mg

Triptans (5HT agonist) Sumatriptan 50-100

Sumatriptan NS 20(1 NS metered dose)

Sumatriptan SC 6

Naratriptan 2.5

Eletriptan 40

Rizatriptan 10

Zolmitriptan 2.5

Zolmitriptan NS 2.5(1 NS metered dose)

Frovatriptan 2.5

Opiods Butorphanol NS 1-2 metered doses

Preventive (chronic,prophylactic)

It aims to reduce attack frequency, severity and duration. It is used in frequent (>4-8 attacks

monthly) or debilitating attacks. Drugs with high efficacy and mild to moderate adverse

events such as beta blockers, amitriptyline, divalproex(good in chronic migraine),topiramate

are used taking medical contraindications or comorbidities such as insomnia, depression and

hypertension into consideration. Drugs with lower efficacy and mild to moderate adverse

events such as selective serotonin reuptake inhibitors , calcium channel antagonists,

gabapentin, riboflavin and NSAIDs.

Choice of migraine preventive treatment

Drug Dose, mg Adverse effects Contraindications Relative

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 indications

Propanalol (SR) 80-240 Bradycardia Asthma Hypertension

Hypotension Depression Angina

Fatigue Cardiac failure

Sleep disturbances Raynaud disease

Dyspepsia Diabetes

Depression

Amitriptyline 10-50 Sedation Mania Insomnia

Weight gain Urinary retension Anxiety

Dry mouth Heart block Depression

Blurred vision TTH

Constipation Other chronic

Urinary retension pains

Postural

hypotension

Sodium 500-1000 Nausea, vomiting Liver disease Mania

valproate Alopecia, Respiratory Epilepsy

tremor, disorders Anxiety

weight gain/loss Glaucoma

Topiramate 25-200 Dizziness, Renal disease Over weight

confusion, Respiratory

language problems, disorders

Paresthesias, Glaucoma

nausea, anorexia,

diplopia

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Treatment outcome: About two thirds of patients will experience a 50% reduction in

headache frequency on most preventive therapies with sodium valproate having better

prognosis.

Migraine is a debilitating syndrome that in mild or atypical forms is often

misdiagnosed. Correct diagnosis allows the control of most headaches.

Trigeminal autonomic cephalgias (TAC)

TACs are primary headaches with a common clinical phenotype consisting of trigeminal

pain, rhythmicity (particularly in CH) and autonomic signs. It consists of cluster headache,

paroxysmal hemicrania,short-lasting neuralgiform headache attacks with conjunctival

injection and tearing (SUNCT) and hemicrania continua

Pathophysiology

Any pathophysiological construct for TACs must account for the three major clinical features

characteristic of the various conditions that comprise this group: Trigeminal distribution pain;

ipsilateral autonomic features; and, the distinct circadian and circannual periodicity,

especially in CH

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The pain-producing innervation of the cranium projects through branches of the trigeminal

and upper cervical nerves to the trigeminocervical complex, from where the nociceptive

pathways project to higher centers. This implies an integral role for the ipsilateral trigeminal

nociceptive pathways in TACs. The ipsilateral autonomic features suggest cranial

parasympathetic activation (lacrimation, rhinorrhoea, nasal congestion and eyelid edema) and

sympathetic hypofunction (ptosis and miosis). Goadsby and Lipton have suggested that the

pathophysiology of the TACs revolves around the trigeminal-autonomic reflex. There is

considerable experimental animal literature to document that stimulation of trigeminal

afferents can result in cranial autonomic outflow, the trigeminal-autonomic reflex. In fact,

some degree of cranial autonomic symptomatology is a normal physiologic response to

cranial nociceptive input, and patients with other headache syndromes often report these

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symptoms. The distinction between the TACs and other headache syndromes is the degree of

cranialautonomic activation and not its presence.

The cranial autonomic symptoms may be prominent in the TACs due to a central

disinhibition of the trigeminal-autonomic reflex.Supporting evidence is emerging from

functional imaging studies: Positron emission tomography studies in CH and PH, and

functional MRI studies in SUNCT syndrome have demonstrated hypothalamic activation.

Importantly, the involvement of posterior hypothalamic structures may account for the

rhythmicity or periodicity that is such a hallmark of cluster headache. Hypothalamic

activation is not seen in experimental trigeminal distribution head pain.There are direct

hypothalamic-trigeminal connections. There is abundant evidence for a role of the

hypothalamus in mediating anti-nociceptive and autonomic responses. In fact, there is direct

evidence from animal experimental studies for hypothalamic activation when intracranial

pain structures are activated. Moreover, the hypothalamic peptides Orexin A and B can elicit

pro-nociceptive and anti-nociceptive effects in the trigeminal system.These data have led to

the suggestion that the TACs are probably due to an abnormality in the hypothalamus with

subsequent trigeminovascular and cranial autonomic activation.

Cluster headache

It is the archetypal TAC with severe pain and major autonomic activation. The precise

genetics of CH are unclear. CH is likely to have an autosomal dominant gene with low

penetrance.

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Epidemiology

CH typically appears between the ages of 20-29yrs. It occurs 53 cases per 100,000 and may

reach upto 120-300 per 100,000 and seems to affect men than women.

Clinical features

A unique feature of episodic Chis the distinctive circadian and circannual periodicity.

Episodic CH commonly occurs atleast once daily for a period of weeks at the same time of

day or night. Active periods (6-12 weeks) are followed by a period of temporary remission

that may last from weeks to years (average 12 months). Attacks tend to be shorter and less

severe at the beginning and towards the end of each cluster period. At its initial onset , CH

active periods are seasonal occurring around spring or autumn.

There are 2 distinctive temporal presentations of CH

Most (80-85% ) suffer from the episodic type characterized by atleast 2 cluster periods

separated by pain free periods of 1 month or more over 7 to 365 days.

In chronic CH , repeated attacks recur over more than a year without remissions or with

remission periods lasting less than 1 month. Interictal pain is also present between attacks or

between clusters. Of the 15% of the patients with CH , in two-thirds it usually begins as such

And in the remaining evolves from the episodic form. Upto half of the patientswith chronic

CH reports transition to an episodic pattern over the course of disease , attack duration tends

to lengthen in both episodic and chronic CH.

Surprisingly for such a dramatic syndrome, the interval until final

diagnosis was 3-6 years. Among factors that increased the diagnostic delay were referral

patterns, the presence of migrainous features, an episodic pattern, and a young age at onset.

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Clinical features

IHS requires atleast 5 attacks that meet the criteria outlined

Periorbital or ocular pain

 lower and upper subtypes of CH

o Upper CH: forehead, temporal and parietal regions

o Lower CH : temporal,and suboccipital with radiation to the teeth,jaws,neck,teeth and

cheeks.

Pain is usually unilateral. In 20% of cases, it may change sides. It attacks alternate sides;

more common between clusters than between attacks in the same cluster.Pain has

excruciating severity: rated as 8 -10 on a 10-point visual analog scale (VAS) by more than

85% of patients and some report considering suicide.Pain is nonspecific; throbbing or boring,

burning, stabbing. ―Hot poker‖ or a ―stabbing‖ feeling in the eye.Sudden jabs of intense pain

is often felt.it is often accompanied by atleast 1 of the following ipsilateral autonomic signs

such as a)conjunctival injection/ lacrimation, b) nasal conjestion/rhinorrhea c)eyelid edema

d)forehead/facial sweating e) miosis and ptosis and f)restlessness(not a local autonomic sign

but frequent(>80%). Patient appears to be agitated, continually moving around particularly

during severe attacks in sharp contrast to the quiet-seeking behavior observed in migraine. It

lasts in 15-80 minutes. The peak intensity is usually rapid and occurs within 3 minutes but

may take upto 9-10 minutes. The longlasting attacks and may last from 3-48 hours.the

frequency of occurrence varies from 1-8 per day.

Nocturnal CH is particularly highly prevalent(51-73%). Pain awakens patients within 90

minutes with the onset of rapid eye movement(REM) sleep. The association between episodic

chronic CH and REM sleep is less established. Patients with CH significantly suffer from

obstructive sleep apnea. Alcohol may precipitate CH attacks during active cluster periods.

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Some patients with chronic CH report high alcohol/ tobacco consumption.CH prodromes

include autonomic signs such as blurred vision, sensitivity to smells, nausea, dyspepsia,

hunger, irritability, tiredness and tenseness and mild pain or nonpainful sensations in the area

that subsequently becomes painful. The premonitory symptoms may predict CH days before

onset which is present in 40% of CH cases and similar to those experienced by migraineurs.

―Aura like‖ symptoms are present in 14% of cases.

Autonomic signs: Ipsilateral lacrimation is the most frequent AS occurring in approximately

90% of cases. It is common and pronounced in CH. Rarely ptosis and miosis (partial horner

syndrome) may persist. Intensity of AS may be related to pain severity. The migrainous

features are common in CH such as photophobia, phonophobia, nausea and vomiting in upto

half of cases. Photophobia and phonophobia are unilateral in CH and bilateral in migraine.CH

is associated with transient hemiparesis, visual symptoms, photophobia, phonophobia, and

nausea with astriking similarity to side-locked migraine.

Secondary CH

Dental/sinus pathology

It may be related to referral patterns and occurrence of lower CH. It may occur as a result of

rare pathologies such as vascular lesions, multiple sclerosis, pituitary tumor, trauma etc.

Secondary TACs have no ―typical‖ presentation and mimic primary TAC. Neuroimaging

must be performed for all TACs or atypical TAC like syndromes.

Treatment

Non-pharmacological treatment

Patient education. Based on the attack patterns, patients should avoid daytime naps.

Alcoholic drinks and other triggers are to be avoided. Altitude hypoxaemia may trigger an

attack during active periods, but may be pharmacologically prevented.

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Pharmacological treatment

Abortive (first line)

Rapid symptomatic relief with oxygen inhalation

Useful diagnostic test

In resistant cases try higher flow rates(15l/min)

Subcutaneous sumatriptan if medically fit.

Agent Dose Comments Sideeffects

Oxygen (inhaled via 5-10L/min First line but None

face mask) 15 min cumbersome

15L/min may be tried Hyperbaric oxygen

also efficacious but

impractical

Sumatriptan 6-12 mg SC First line, fast, and Contraindicated in

efficacious; 12 mg as CV diseases. Fatigue,

effective as mg but nausea/vomiting

with more side Chest symptoms

effects. Skin reactions over

It is marginally less puncture wound.

20 mg IN effective in chronic Contraindicated in

CH CV disease

Less effective but IN<SC

easier to use.

Zolmitriptan 5-10mg IN Limited efficacy Contraindicated in

Alternative to IN CV disease

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 sumatriptan Better in episodic CH

Dihydroergotamine 0.5-1.0mhIN Reduces severity but Contraindicated in

(bilateral) not frequency. Risk CV disease. Do not

of rebound use with a triptan.

Lignocaine 1 ml of 4%-10% Pain is decreased but Bitter taste

solution applied IN not enough studies. It

on cotton pledget needs to be deep

bilaterally inserted near

pterygopalatine

foramen.

Transitional and prophylactic

Rapid transitional prophylaxis may be attained with corticosteroids for a limited period in

selected patients. Long term prophylaxis usually with verapamil in both episodic and chronic

CH is done. Topiramate is used as a second-line therapy

Agent Target dose Comments Side effects

Verapamil 180-480 mg/d(PO) First line treatment Hypotension

Perform baseline and Bradycardia

6 monthly ECGs. Heart block

Dizziness

Fatigue

Prednisone 80mg (PO) Good for initial and Increased appetite,

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 transitional therapy Nervousness,

until eg. Verapamil Hyperglycemia,

takes effect. Insomnia,

Prolonged use not Headache

recommended

because of side

effects. Taper over

10-21 days

Topiramate 25-200 mg/d (PO) Increase by 25mg/d Cognitive effects

every 5d Paresthesia

Dizziness

Valproic acid 600-2000 mg/d (PO) Efficacious in Nausea

patients with Dizziness

pronounced Dyspepsia

migrainous features. Thrombocytopenia

Monitor liver

function.

Gabapentin 900mg/d (PO) Few studies but Drowsiness

promising results

Melatonin 9-10 mg/d nocte Few studies None

(PO)

Long term prophylaxis usually with verapamil in both episodic and chronic CH is done.

Although there are many side effects, lithium carbonate may also be considered.

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Agent Target dose Comments Side effects

Verapamil 360-480mg/d (PO) First line treatment Hypotension

Perform baseline Bradycardia

ECG Heartblock

Dizziness

Fatigue

Lithium carbonate 300-900 mg(PO) Requiring monitoring Weakness

of renal and thyroid Nausea

function, and of Tremor

serum concentrations Slurred speech

(best at 0.4- Blurred vision

0.8mEq/L) Side effects >

verapamil

Surgical

Surgical treatments are done for carefully selected recalcitrant cases.

Recent reports indicate that medication overuse headache (MOH) is a possible complication

in patients with CH and patients with other TACs. Remission periods in many patients may

increase with time and beyond the age of 65-75. Active CH is rare.

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Paroxysmal hemicrania

PH is a rare entity

Epidemiology

Estimated prevalence of PH is 2-20 per 100,000. The mean age of onset is usually 34-41

years. But children aged 6 and adults aged 81 years have been reported with average illness

duration of 13 years. The episodic PH is considered to have an earlier mean age of onset

(27 years) than the chronic form(37years).Only 20% of PH behave episodically and many of

these develop eventually to chronic form.

Clinical features

It is unilateral with severe orbital or periorbital pain.it may rarely become bilateral. It may

also temporal, periauricular, maxillary and rarely occipital areas. It can be referred to

shoulder, neck and arm which is quite common. The strong pain may cross midline. The vast

majority of attacks donot change sides. Pain last 2-30 minutes.it is more usually 13-29

minutes, but may last nearly an hour. Pain onset is rapid and mostly peaks in less than 5

minutes. Pain is sharp and excruciating. It can also be throbbing,stabbing, sharp, or boring. It

is accompanied by atleast one of the following ipsilateral autonomic signs such as

conjunctival injection/ lacrimation, nasal congestion/rhinorrhea, eyelid edema,

forehead/facial sweating, miosis and ptosis. Usually 8-30 attacks occur per 24 hours. The

seasonal pattern of attacks in PH patients has been described. The temporal similarity of CH

behaviour has led to the term ―modified cluster pattern‖. 30% report REM-related nocturnal

attacks that wake. It has an absolute response to indomethacin.Autonomic signs may occur

bilaterally but are more pronounced on the symptomatic side. The most commonly seen are

ipsilateral lacrimation, nasal congestion, conjunctival injection and rhinnorrhea.

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Secondary paroxysmal hemicrania

Clinical features include malignancy, central nervous system disease and benign tumors.

Parotid gland epidermoid carcinoma with cerebral metastasis can be present. Systemic

diseases can also be seen. All PH cases require imaging.

Treatment

The response of PH to indomethacin is absolute. Dosage is 75-225mg/d (PO). Most cases

respond within 24 hours, but 3 days at 75 mg followed, if needed by 150mg for a further 3

days is recommended as trial therapy. High persistent dosage requirements may indicate

underlying pathology. Prognosis in PH is good and long-term remission has been reported.

Indomethacin – resistant PH may respond to topiramate.

Short-lasting , Unilateral, Neuralgiform headache attacks with Cranial autonomic

features (SUNCT)

SUNCT syndrome is a unilateral headache/facial pain characterized by brief paroxysmal

attacks accompanied by ipsilateral local AS, usually conjunctival injection and lacrimation.

The similarities of this syndrome to trigeminal neuralgia (TN) are particularly the triggering

mechanism and many believe SUNCT to be a TN variant.

Epidemiology

It is suggested to be as common as PH. It has slightly more male predilection with a mean

onset at approximately 50 years. SUNCT occurring in siblings has recently been presented as

―familial SUNCT‖. The HIS requires atleast 20 attacks that meet the criteria outlined.

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Clinical features

It is usually unilateral, ocular/periocular pain, but may involve most head areas. Pain

spreading across the midline or changing sides are rare. Pain is usually moderate to severe;

less severe than TN. Pain is accompanied by ipsilateral conjunctival injection and

lacrimation. Pain is usually stabing or pulsating; sometimes electric or burning. It lasts from

5-240 seconds. Usually 15-120 seconds 9mean 1 minute). Longer attacks of upto10 minutes

and even 2-3 hours reported. ‖SUNCT status‖ is the rare pain that occurs most of the day for

1-3 days. Three patterns of attacks described such as a)classical single attacks, b)groups of a

number stabs/attacks and c)a saw-tooth pattern with numerous stabs/attacks lasting minutes.

Frequency is from 3-200 daily. It is inconsistent and irregular with an average of 28 per day.

A bimodal distribution of attacks occurring in the morning and late afternoon has been

observed. Fewer than 2% of attacks occur at night. a ―cluster-like‖ pattern has been reported

with active and inactive periods.

Pain in SUNCT may be triggered by light mechanical stimuli in the areas innervated by the

trigeminal nerve but with a short latency until pain onset. Extra trigeminal triggers including

neck movements have also been shown to precipitate attacks. No refractory period has been

demonstrated in SUNCT. SUNCT is accompanied by marked ipsilateral conjunctival

injection and lacrimation that appear rapidly with onset of pain. Nasal stuffiness and

rhinorrhea are common. Sweating may accompany attacks but is rare and often subclinical.

SUNA

SUNA can be differentiated from SUNCT by 2 criteria. It can be accompanied by an

autonomic sign (eg. nasal congestion), and attack duration has been extended upto 10

minutes. There will be less prominent or absent conjunctival injection and lacrimation.A

seasonal pattern has been reported in SUNA.

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SUNCT/SUNA treatment

Lamotrigine is the drug of choice. Initial dosage is 25 mg per day; increase very slowly,

reach target in 7 or more weeks.SUNCT may respond to steroids. Anticonvulsant drugs may

produce some improvement. Carbamazepine, topiramate, and gabapentin (see Table 6). Case

reports of successful surgical microvascular decompression and percutaneous trigeminal

ganglion compression for SUNCT. Remissions have been observed and may last for several

months.

Hemicrania Continua

HC is increasingly considered a TAC variant.

Clinical features

Unilateral headache present for more than 3 months. Pain in the frontal and temporal regions

and periorbitally.Although very rare, pain can also change sides. Few bilateral cases are also

been reported. Daily and continuous pain is seen. Severity is moderate (VAS 4.7). It is

characterized (74%) by fluctuations in pain severity. Exacerbations are totally disabling in

about 40% of patients.Exacerbations result in severe pain (VAS 9.3) lasting 30 minutes to 10

hours and even up to 2 to 5 days.During exacerbation, HC is almost indistinguishable from

migraine. Patients may report a sharp pain similar to the condition of ―jabs and jolts.‖ Some

patients (18%) describe a distinct ocular sensation mimicking a foreign body (or sand), that

may accompany or precede the headaches. Pain is throbbing (one-third of cases); may appear

as pain intensity increases.Complete response to indomethacin. During exacerbations,

accompanied by at least 1 of the following ipsilateral autonomic phenomena/signs:

Conjunctival injection/lacrimation, Nasal congestion/rhinorrhea Miosis and ptosis

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Two forms of HC have been described: remitting and continuous. The remitting form is

characterized by headache that can last for some days followed by a pain-free period lasting

from 2 to 15 days.One-third of remitting cases become continuous following a mean duration

of 7.8 years.Nocturnal attacks were reported in up to half of patients. HC is not usually

accompanied by notable pathology or other abnormalities.Most published cases of HC with

computerized scanning of the head, neurologic and other physical examination, hematology,

and serum biochemistry were all normal. Cases of HC secondary to pathology or systemic

disease have been reported. There is usually a paucity of AutonomicSigns (AS) in HC.

However, during exacerbation, AS commonly appear singly or in various combinations, but

are still relatively mild. This strengthens the hypothesis that activation of AS is dependent on

pain severity. The most common signs present in 30% to 40% of patients are photophobia,

nausea, conjunctival injection, phonophobia, and tearing. During exacerbations up to 60% of

patients display qualities such as photophobia, phonophobia, nausea, and, more rarely,

vomiting. HC with aura has also been described, further linking HC to migraine

pathophysiology. More rarely (15%–18%) nasal stuffiness or rhinorrhea, vomiting, or ptosis

may also be reported. These features establish the HC phenotype as straddling both TACs

and migraine. Secondary hemicrania continua Medication abuse.Mesenchymal tumor in the

sphenoid bone has been reported. Head trauma and surgery.

Treatment

Indomethacin is totally effective. Relief occurs within hours or 1 to 2 days. Other NSAIDs

are less effective.Piroxicam-beta-cyclodextrin is a good alternative for selected cases.

Differential diagnosis of TACs

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Fig :Pain location in TACs and migraine66.

TACs are characterized by orbital and periorbital pain. In paroxysmal hemicrania and

hemicrania continua there are large adjacent areas affected. Migraine is largely unilateral but

may be bilateral in up to 30% of cases (this has been marked by a lighter-shaded area

contralaterally). The two-headed arrow above the diagram indicates side shift, which occurs

in specific headache66.

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Given the predominate sensory system involved, referral patterns of TACs often involve

orofacial structures and at times may primarily present in intraoral or unusual facial sites.

Thus, CH and PH have caused misdiagnosis as dental pain leading to unnecessary dental

interventions. Cluster headaches are often seen by ear, nose, and throat surgeons and

erroneously diagnosed as sinus pathology.

TensionTypeHeadacheSyndrome

The IHS subclassifies TTH into episodic (infrequent and frequent), chronic, and probable

TTH. The individual attacks in these subentities have similar clinical features with some

subtle differences; severity and the occurrence of mild nausea tend to increase with

frequency. Pericranial muscle tenderness is an extremely common feature in patients with

TTH, but because some patients do not demonstrate this feature, the IHS subclassifies TTH

as with or without pericranial tenderness.

TTH is extremely common, and most individuals will have experienced one in their

lifetime.TTH has a 1-year prevalence in adults of more than 80%, higher than migraine.

Infrequent episodic TTH (IETTH), which occurs on average once per month, is most

common (48%–59%) but does not usually require medical attention.One-year prevalence of

frequent episodic TTH (FETTH) is 18% to 43% and 10% to 25% report weekly

headaches.TTH, in particular chronic TTH (CTTH), is thought to account for more than 10%

of disease-related absenteeism.The average onset age of TTH is 20 to 30 years with peak

167
prevalence in the third to fifth decades.However, up to 25% of school children report having

TTH, and in the older population (>60 years) the prevalence is 20% to 30%. Genetic studies

reveal that first-degree relatives of CTTH sufferers are 3 times as likely to also suffer

headaches relative to the population.FETTH is significantly affected by environmental

factors, with evidence for only a minor genetic contribution.

Episodic Tension-Type Headache

Clinical features

Bilateral in >90%. Often occurs in Occipital, parietal, temporal or frontal areas. ―Bandlike‖

or ―caplike.‖ Site may vary with intensity.Pressurelike, dull or tight. Throbbing is rare and

related to severity.Intensity is mild to moderate. Increases with headache frequency.Duration

is 30 minutes to 7 days.25% of episodic TTH (ETTH) evolves into CTTH. Sleep

disturbances may occur. Mild to moderate anorexia is seen in some cases. Occasional and

mild photophobia (10%) or phonophobia (7%) are noticed.Many patients will suffer both

migraines and TTH, which may further affect quality of life. Interestingly, ETTH in migraine

sufferers responds to sumatriptan, a migrainespecific drug, whereas in patients who do not

suffer from migraine, it does not. This may suggest that mild migraines may phenotypically

be very similar to ETTH. ETTH is commonly precipitated by a number of factors: stress,

fatigue, disturbed meals, menstruation, alcohol, and a lack of sleep. Although TTH is usually

not aggravated by physical activity, there are reports that in some patients with TTH, exercise

may aggravate pain. Other than in location, TTH is very similar to masticatory myofascial

pain.

Chronic Type Tension Headache

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CTTH is one of the subtypes of a group simply termed ―chronic daily headaches,‖ based on

the daily or near daily occurrence of headaches.

Clinical features of CTTH

Classically, the patient with CTTH is middle-aged and female, with a long headache history

that began with episodic headaches 10 to 20 years previously and slowly increased in

frequency. The clinical features of CTTH are largely similar to those in FETTH, with

differences in accompanying features, treatment response, and impact on quality of life.

CTTH is bilateral involvingfrontal, temporal, or frontotemporal regions.Pressurelike mostly

moderate pain is noticed.

Disorders of the Masticatory Muscles

Muscle disorders involving the masticatory muscles have been considered analogous to

skeletal muscle disorders throughout the body. However, emerging research has shed new

light on the varied etiology, clinical presentation, diagnosis, and treatment of myofascial pain

and masticatory muscle disorders. Mechanisms behind masticatory muscle pain include

overuse of a normally perfused muscle or ischemia of a normally working muscle,

sympathetic reflexes that produce changes in vascular supply and muscle tone, and changes

in psychological and emotional states. Neurons mediating pain from skeletal muscle are

subject to strong modulatory influences. Bradykinin, serotonin, substance P, prostaglandins,

and neuropeptides sensitize nociceptors and can easily sensitize nociceptive endings. Painful

conditions of muscle often result in increased sensitivity of peripheral nociceptors and

hyperexcitability in the central nervous system with hyperalgesia. Muscle disorders can be

divided into regional disorders, such as myalgia associated with temporomandibular joint

(TMJ) disorder, and systemic disorders, such as pain associated with fibromyalgia. The

paucity of data on the etiology and pathophysiology of muscle pain limits the ability to

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clearly delineate all groups of muscle disorders. Frequently the clinician must rely on clinical

judgment to establish a diagnosis. It is clear that well-designed controlled trials and additional

research is necessary for the development of validated diagnostic criteria and treatment

protocols.

CLASSIFICATION OF MASTICATORY MUSCLE DISORDERS

Chronic myalgia of the muscle of mastication (MOM) is one aspect of temporomandibular

disorders (TMDs). Historically, clinicians and researchers have subclassified TMDs into

intracapsular disorders and masticatory muscle disorders such as local myalgia, myofascial

pain, centrally mediated myalgia, myospasm, myositis, myofibrotic contracture, and

masticatory muscle neoplastic disease.9 Conflicting classification schemes and terminology

have led to significant confusion among clinicians, and perhaps inaccurate diagnosis and

treatment of patients. In fact, many studies continue to group muscle pain and painful TMJ

disorders together under the term TMD, although these entities are pathophysiologically and

clinically distinct. Although the most common feature of most masticatory muscle disorders

is pain, mandibular dysfunction such as difficulty chewing and mandibular dysfunction may

also occur. The clinician needs to differentiate masticatory muscle disorders from the primary

TMDs such as those that involve pain associated with osteoarthritis, disc displacement, or

jaw dysfunction.

The clinical features of masticatory muscle disorders are as follows.

Features of Local Myalgia

Sore MOM with pain in cheeks and temples on chewing, wide opening, and often on

waking (eg, nocturnal bruxism)

Bilateral

Described as stiff, sore, aching, spasm, tightness, or cramping

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 Sensation of muscle stiffness, weakness, fatigue

Possible reduced mandibular range of motion

Differential diagnosis: myositis, myofascial pain, neoplasm, fibromyalgia

Features of Myofascial Pain

Regional dull, aching muscle pain

Trigger points present and pain referral on palpation with/without autonomic symptoms

Referred pain often felt as headache

Trigger points can be inactivated with local anesthetic injection

Sensation of muscle stiffness and/or malocclusion not verified clinically

Otologic symptoms including tinnitus, vertigo, and pain

Headache or toothache

Decreased range of motion

Hyperalgesia in region of referred pain

Differential diagnosis:

arthralgia, myositis, local myalgia, neoplasia, fibromyalgia

Diagnostic criteria for masticatory muscle disorders

Disorder Etiology Diagnostic Criteria

Centrally mediated chronic Chronic generalized muscle History of prolonged and

muscle pain pain associated with a continuous muscle pain

comorbid disease Regional dull, aching pain at

rest

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 Pain aggravated by function

of affected muscles

Pain aggravated by palpation

Myalgia (local) Acute muscle pain Regional dull, aching pain

Protective muscle splinting during function

Postexercise soreness No or minimal pain at rest

Muscle fatigue Local muscle tenderness on

Pain from ischemia palpation

Absence of trigger points

and pain referral

Myofascial pain Chronic regional muscle pain Regional dull, aching pain at

rest

Pain aggravated by function

of affected muscles

Provocation of trigger points

alters pain complaint and

reveals referral pattern

>50% reduction of pain with

vapocoolant spray or local

anesthetic injection to trigger

point followed by stretch

Myofibrotic contracture Painless shortening of Limited range of motion

muscles Firmness on passive stretch

(hard stop)

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 Little or no pain unless

involved muscle is forced to

lengthen

Myositis Inflammation secondary to Continuous pain localized in

direct trauma or infection muscle area following injury

or infection

Diffuse tenderness over

entire muscle

Pain aggravated by function

of affected muscles

Moderate to severe decreased

range of motion due to pain

and swelling

Neoplasia Benign or malignant May or may not be painful

Anatomic and structural

changes Imaging and biopsy

needed

Myospasm Acute involuntary and Acute onset of pain at rest

continuous muscle and during function

contraction Markedly decreased range of

motion due to continuous

involuntary muscle

contraction Pain aggravated

by function of affected

muscles

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 Increased electromyographic

activity higher than at rest

Sensation of muscle

tightness, cramping, or

stiffness

(Data from de Leeuw R. Orofacial pain: guidelines for assessment, classification, and

management. The American Academy of Orofacial Pain. 4th edition. Chicago: Quintessence

Publishing Co, Inc; 2008.)

Features of Centrally Mediated Myalgia

Trigger points and pain referral on palpation. Sensation of muscle stiffness, weakness, and/or

fatigue. Sensation of malocclusion not verified clinically.Otologic symptoms including

tinnitus, vertigo, and pain .Decreased range of motion. Hyperalgesia. No response to

treatment directed at painful muscle tissue

Features of Myospasm

Sudden and involuntary muscle contraction is noticed.Acute malocclusion may be present

(dependent on muscles involved).Decreased range of motion and pain on function and at

rest.Myospasm is relatively a rare disorder in orofacial pain population

Features of Myositis

History of trauma to muscle or source of infection.Often continuous pain affecting entire

affected muscle.Pain aggravated by function .Severe limited range of motion

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Features of Myofibrotic Contracture

Not usually painful.Often follows long period of limited range of motion or disuse (eg,

intermaxillary fixation). History of infection or trauma is common

Differential diagnosis: TMJ ankylosis, coronoid hypertrophy

Features of Masticatory Muscle Neoplasia

Pain may or may not be present. Anatomic and structural changes: tumors may be in

muscles or masticatory spaces.Swelling, trismus, paresthesias, and pain referred to

teeth.Positive findings on imaging or biopsy

Some clinicians have stressed classifying myogenic disorders based on an anatomic system

allowing for a simpler diagnostic process, because evaluation of the patient involves careful

palpation of the masticatory muscles and joints.The clinician needs to determine the etiology

and pathophysiology that occur with the various masticatory muscle disorders, such as

disorders caused by trauma. A thorough history and clinical examination, an understanding of

pain neuroanatomy and neurophysiology, and an in-depth knowledge of research on muscle

pain are important. Recently a new term, persistent orofacial muscle pain (POMP), has been

introduced, to more accurately reflect the interplay between peripheral nociceptive sources in

muscles, faulty central nervous system components, and decreased coping ability.POMP

likely shares mechanisms with tension-type headache, regional myofascial pain, and

fibromyalgia, and has genetically influenced traits that determine pain modulation and

pharmacogenomics interacting with psychological traits to affect disease onset, clinical

progression, and pain experience.To date, these factors cannot be identified in the individual

patient sufficiently enough to tailor focused, mechanism-based treatment. POMP is consistent

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with the condition often referred to as centrally mediated myalgia and, as such, treatment

needs to be redirected from local and regional therapies to systemic and central ones.

Etiology of myogenous pain

Etiology Criteria

Focal myalgia from direct trauma History of trauma preceding pain onset

Subjective pain in muscles with function Pain

reproduced on palpation

Primary myalgia due to parafunction No history of trauma

Subjective pain in muscle with function

Pain reproduced on palpation

No trigger points

Secondary myalgia due to active local History of recent joint, oral soft tissue, or

pathology or recent medications pulpal disease or medication (eg, serotonin-

selective reuptake inhibitors) that coincides

with muscle pain

Subjective pain in muscle with function

Pain reproduced on palpation

Myofascial pain No history of recent trauma

Subjective pain in muscles with function

Pain reproduced on palpation

Trigger points and pain referral

Diffuse chronic muscle pain and Subjective pain in multiple sites aggravated

fibromyalgia by function Widespread pain involving more

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 than 3 body quadrants >3 mo duration Strong

pain on palpation in 11 of 18 body sites

(Data from Clark GT, Minakuchi H. Oral appliances. In: Laskin DM, Greene CS, Hylander

WL, editors. Temporomandibular disorders: an evidence-based approach to diagnosis and

treatment. Chicago: Quintessence; 2006.)

CLINICAL EXAMINATION OF THE PATIENT

The most effective approach for the diagnosis of masticatory muscle pain involves careful

review of the chief complaint, the history of the present illness, the dental, medical, and

psychosocial behavioral histories and a comprehensive evaluation of the head and neck

including a cranial nerve assessment. In addition, imaging modalities may be important in

ruling out other conditions. No one physical finding can be relied on to establish a diagnosis;

rather, a pattern of abnormalities may suggest the source of the problem and diagnosis.

However, masticatory muscle tenderness on palpation is the most consistent examination

feature present in TMDs.

In fact, the clinical features that distinguish patients from non-TMD or masticatory muscle

pain most consistently reported in the literature are: restricted passive mouth opening without

pain; masticatory muscle tenderness on palpation; limited maximal mouth opening; and an

uncorrected deviation on maximum mouth opening and tenderness on muscle or joint

palpation.

History of the present illness : pain

characteristics

Quality Common patient descriptors: dull, sharp,

tight, aching, tired, etc

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Location Unilateral vs bilateral

Pain confined to a single muscle or referred

to a distant area

Intensity On a scale of 1–10

Mild, moderate, or severe

Onset, duration, pattern How long has the pain been present? What if

anything caused the pain? (eg, trauma) What

has been the course of pain since its onset?

(eg, episodic, constant, fluctuating)

Modifiers What exacerbates or diminishes the pain?

Does anything you do or use help or worsen

pain?

Chronicity How long has the pain been present?

Comorbid symptoms and signs Are there any other conditions or symptoms

associated with pain? (eg, depression, acute

anxiety, nausea/vomiting, tearing, visual

changes, dizziness, numbness/tingling,

weakness, generalized pain)

Questions regarding oral behavior and parafunction

DO YOU: Clench or grind your teeth when asleep? Sleep in a position that puts pressure on

your jaw? (eg, side, stomach) Clench or press teeth together while awake? Touch or hold

teeth together while eating? Hold, tighten, or tense muscles without clenching or touching

teeth together? Hold out or jut jaw forward or to side? Press tongue between teeth? Bite,

chew, or play with tongue, cheeks, or lips? Hold jaw in rigid or tense position to brace or

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protect jaw? Bite or hold objects between teeth (eg, pens, pipe, hair, fingernails, and so

forth)? Use chewing gum? Play musical instruments that involve mouth or jaw? Lean with

hand on jaw or chin? Chew food on one side only? Eat between meals (food requiring lots of

chewing)? Talk at length? Sing? Yawn excessively? Hold telephone between head and

shoulder?

(Data from Ohrbach R, Markiewicz M, McCall WD Jr. Oral Behaviors Checklist:

performance validity of targeted behaviors [abstract]. J Dent Res 2004;83:(Spec Issue A):

Objective determination of the presence or absence of parafunctional jaw behavior is

challenging.Although the presence of these behaviors may not have proven diagnostic

validity, their assessment remains important because it provides potential causative or

perpetual factors and/or effects on the masticatory system. An oral behavior checklist is a

useful instrument for determining the presence or awareness of parafunctional behaviors.

Interincisor separation (plus or minus the incisor overlap in centric occlusion) provides the

measure of mandibular movement. Maximum interincisal opening (MIO) should be measured

using a ruler without pain, as wide as possible with pain, and after opening with clinician

assistance. Mouth opening with assistance is accomplished by applying mild to moderate

pressure against the upper and lower incisors with the thumb and index finger. Passive

stretching often allows the clinician to assess and differentiate the limitation of opening

caused by a muscle or joint problem by comparing assisted opening with active opening. This

action provides the examiner with the quality of resistance at the end of the movement. Often,

muscle restrictions are associated with a soft end-feel and result in an increase of more than 5

mm above the active opening (wide opening with pain), whereas joint disorders such as acute

nonreducing disc displacements have a hard end-feel and characteristically limit assisted

opening to less than 5 mm (normal MIO is w40 mm; range 35–55 mm). Measurements of

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lateral movement are made with the teeth slightly separated, measuring the displacement of

the lower midline from the maxillary midline, and adding or subtracting the lower-midline

displacement at the start of movement. Protrusive movement is measured by adding the

horizontal distance between the upper and lower central incisors and adding the distance the

lower incisors travel beyond the upper incisors; normal lateral and protrusive movements are

approximately 7 mm.

The primary finding related to masticatory muscle palpation is pain; however, the methods

for palpation are not standardized in clinical practice. The amount of pressure to apply and

the exact sites that are most likely associated with TMD are unknown. Some clinicians have

recommended attempting to establish a baseline (to serve as a general guide or reference) by

squeezing a muscle between the index finger and thumb or by applying pressure in the center

of the forehead or thumbnail to gauge what pressure becomes uncomfortable.

The Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD) guidelines

recommend 1 lb (0.45 kg) of pressure for the joint and 2 lb (0.9 kg) of pressure for the

muscles. Palpation should be accompanied by: asking the patient about the presence of pain

at the palpation site; whether palpation produces pain spread or referral to a distant site; and

whether palpation reproduces the pain the patient has been experiencing. Reproducing the

site and the character of the pain during the examination procedure helps identify the

potential source of the pain. The distant origin of referred pain can also be identified by

palpation. Palpation of the muscles for pain should be done with the muscles in a resting

state. There are no standardized methods of assessing the severity of palpable pain, and the

patient should be asked to rate the severity by using a scale (eg, a numeric scale from 1 to 10,

a visual analog scale, or a ranking such as none, mild, moderate, or severe).

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The RDC/TMD recommends using the categories of pressure only, mild pain, moderate pain,

and severe pain. These ratings may also be useful in assessing treatment progress in addition

to asking patients what percentage of improvement they may feel. The lateral pterygoid is in

a position that does not allow access for adequate palpation examination, even though there

are examination protocols and descriptions for palpating this muscle. Patients with TMDs

often have musculoskeletal problems in other regions (neck, back, and so forth). The upper

cervical somatosensory nerves send branches that synapse in the spinal trigeminal nucleus,

which is one proposed mechanism to explain referral of pain from the neck to the orofacial

region and masticatory muscles. The sternocleidomastoid and trapezius muscles are often part

of cervical muscle disorders, and may refer pain to the face and head. Other cervical muscle

groups to include in the palpation examination include the paravertebral (scalene) and

suboccipital muscles. Injections of anesthetics into the TMJ or selected masticatory muscles

may help confirm a diagnosis. Elimination of or a significant decrease in pain and improved

jaw motion should be considered a positive test result. Diagnostic injections may also be

helpful in differentiating pain arising from joints or muscle. In situations where a joint

procedure is being considered, local anesthetic injection of the joint may confirm the joint as

the source of pain. Injecting trigger points or tender areas of muscle should eliminate pain

from the site and should also eliminate referred pain associated with the injected trigger

point. Interpretation of injections in the context of all the diagnostic information is vital,

because a positive result does not ensure a specific diagnosis. Recently, the use of botulinum

toxin (Botox) has been advocated for trigger-point injections and for the management of

tension-type headache. In several case-control studies and randomized trials, descriptive

analysis showed that improvements in both objective (range of mandibular movements) and

subjective (pain at rest; pain during chewing) clinical outcome variables were higher in

Botox-treated groups than in the placebo-treated subjects. Patients treated with Botox had a

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higher subjective improvement in their perception of treatment efficacy than placebo-treated

subjects.

TREATMENT OF MASTICATORY MUSCLE DISORDERS

It is important for the clinician treating patients with TMDs to distinguish clinically

significant disorders that require therapy from incidental findings in a patient with facial pain

attributable to other causes.TMJ abnormalities are often discovered on routine examination,

and may not require treatment such as with asymptomatic clicking of the TMJ. The need for

treatment is largely based on the level of pain and dysfunction as well as the progression of

symptoms. With respect to disorders of MOM, the principles of treatment are based on a

generally favorable prognosis and an appreciation of the lack of clinically controlled trials

indicating the superiority, predictability, and safety of treatments presently available. The

literature suggests that many treatments have some beneficial effect, although this effect may

be nonspecific and not directly related to the particular treatment. According to the American

Association of Dental Research, it is strongly recommended that, unless there are specific and

justifiable indications to the contrary, treatment of TMD patients, including those with

disorders of MOM, initially should be based on the use of conservative, reversible, and

evidence-based therapeutic modalities. Studies of the natural history of many TMDs suggest

that they tend to improve or resolve over time. Although no specific therapies have been

proved to be uniformly effective, many of the conservative modalities have proved to be at

least as effective as most forms of invasive treatment in providing symptomatic relief.

Because such modalities do not produce irreversible changes, they present much less risk of

producing harm. Professional treatment should be augmented with a home-care program

whereby patients are taught about their disorder and how to manage their symptoms.

Treatments that are relatively accessible, not prohibitive owing to expense, safe, and

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reversible should be given priority, for example: education; self-care; physical therapy;

intraoral appliance therapy; and short-term pharmacotherapy, behavioral therapy, and

relaxation techniques. There is evidence to suggest that multimodal therapy and combining

treatments produces a better outcome. Occlusal therapy continues to be recommended by

some clinicians as an initial treatment or as a requirement to prevent recurrent symptoms.

However, research does not support occlusal abnormalities as a significant etiologic factor in

TMD including masticatory muscle disorders.

Avoidance therapy and cognitive awareness plays a vital role in patient care but has little

scientific evidence to support its use. Generally speaking, common sense dictates that if

something hurts, it should be avoided.

Four behaviors should be avoided in the patient with masticatory muscle pain:

1. Avoidance of clenching by reproducing a rest position where the patient‘s lips are closed

but teeth are slightly separated

2. Avoidance of poor head and neck posture

3. Avoidance of testing the jaw or jaw joint clicking

4. Avoidance of other habits such as nail biting, lip biting, gum chewing, and so forth (Box

2).

Many patients report benefit from heat or ice packs applied to painful MOM. The local

application of heat can increase circulation and relax muscles, whereas ice may serve as an

anesthetic for painful muscles. In addition, stretch therapy must be part of a self-care

program. Stretches should be done multiple times daily to maximize effectiveness. The most

effective stretching exercise is passive stretching.

Physiotherapy helps to relieve musculoskeletal pain and restore normal function by altering

sensory input; reducing inflammation; decreasing, coordinating, and strengthening muscle

activity; and promoting the rehabilitation of tissues.A licensed professional therapist is

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recommended for treatment. Despite the absence of well-controlled clinical trials,

physiotherapy is a well-recognized effective and conservative therapy for many disorders of

the MOM.

Physical Therapy Techniques

Posture training

Exercises

Mobilization

Physical Agents and Modalities

Electrotherapy and transcutaneous electrical nerve stimulations (TENS)

Ultrasound

Iontophoresis

Vapocoolant spray

Trigger-point injections with local anesthetic or Botox

Acupuncture

Laser treatment

Initial treatment of masticatory muscle disorders Treatment Component Description

Education Explanation of the diagnosis and treatment Reassurance about the generally good

prognosis for recovery and natural course Explanation of patient‘s and doctor‘s roles in

therapy Information to enable patient to perform self-care Self-care Eliminate oral habits (eg,

tooth clenching, chewing gum) Provide information on jaw care associated with daily

activities Physical therapy Education regarding biomechanics of jaw, neck, and head posture

Passive modalities (heat and cold therapy, ultrasound, laser, TENS) Range of motion

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exercises (active and passive) Posture therapy Passive stretching, general exercise and

conditioning program Intraoral appliance therapy Cover all the teeth on the arch the appliance

is seated on Adjust to achieve simultaneous contact against opposing teeth Adjust to a stable

comfortable mandibular posture Avoid changing mandibular position Avoid long-term

continuous use Pharmacotherapy NSAIDs, acetaminophen, muscle relaxants, antianxiety

agents, tricyclic antidepressants Behavioral/relaxation techniques Relaxation therapy

Hypnosis Biofeedback Cognitive-behavioral therapy Abbreviations: NSAIDs, nonsteroidal

anti-inflammatory drugs; TENS, transcutaneous electrical nerve stimulation.

Patient‘s exercise instructions

Certain exercises can help you relieve the pain that comes from tired, cramped muscles. They

can also help if you have difficulty opening your mouth. The exercises described work by

helping you relax tense muscles and are referred to as ―passive stretching.‖ The more often

you do these exercises, the more you‘ll relax the muscles that are painfully tense. Do these

exercises 2 times daily:

1. Ice down both sides of the face for 5 to 10 minutes before beginning (ice cubes in

sandwich bags or packs of frozen vegetables work well for this).

2. Place thumb of one hand on the edge of the upper front teeth and the index and middle

fingers of the other hand on the edge of the lower front teeth, with the thumb under the chin.

3. The starting position for the stretches is with the thumb of the one hand and index finger of

the other hand just touching.

4. Gently pull open the lower jaw, using the hand only, until you feel a passive stretch, not

pain, hold for 10 seconds, then allow the lower jaw to close until the thumb and index finger

are once again contacting; it is crucial that when doing these exercises not to use the jaw

muscles to open and close, but rather manual manipulation only (the fingers do all the work!).

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5. Repeat the above stretching action 10 times, performing 2 to 3 sets per day, 1 in the

morning and 1 or 2 in the evening

6. When finished with the exercises, one can place moist heat to both sides of the face for 5 to

10 minutes (heating a wet washcloth in the microwave for about 1 minute works well for

this).

Patient‘s instructions for self-care

 Be aware of habits or patterns of jaw use.

o Avoid tooth contact except during chewing and swallowing.

o Notice any contact the teeth make.

o Notice any clenching, grinding, gritting, or tapping of teeth, or any tensing or

rigid holding of the jaw muscles.

o Check for tooth clenching while driving, studying, doing computer work,

reading, or engaging in athletic activities; when at work or in social situations;

and when experiencing overwork, fatigue, or stress.

o Position the jaw to avoid tooth contacts.

o Place the tip of the tongue behind the top teeth and keep the teeth slightly apart;

maintain this position when the jaw is not being used for functions such as

speaking and chewing.

 Modify your diet.

o Choose softer foods and only those foods that can be chewed without pain.

o Cut foods into smaller pieces; avoid foods that require wide mouth opening and

biting off with the front teeth, or foods that are chewy and sticky and that require

excessive mouth movements.

o Do not chew gum.

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  Do not test the jaw.

 Do not open the mouth wide or move the jaw around excessively to assess pain or

motion.

 Avoid habitually maneuvering the jaw into positions to assess its comfort or range.

 Avoid habitually clicking the jaw if a click is present.

 Avoid certain postures.

o Do not lean on or cup the chin when performing desk work or at the dining table.

o Do not sleep on the stomach or in postures that place stress on the jaw.

 Avoid elective dental treatment while symptoms of pain and limited opening are

present.

 During yawning, support the jaw by providing mild pressure underneath the chin

with the thumb and index finger or with the back of the hand.

 Apply moist hot compresses to the sides of the face and to the temple areas for 10 to

20 minutes twice daily.

SPLINT THERAPY

Splints, orthotics, orthopedic appliances, bite guards, nightguards, or bruxing guards are used

in TMD treatment, and often for disorders of masticatory muscles. Their use is considered to

be a reversible part of initial therapy. Several studies on splint therapy have demonstrated a

treatment effect, although researchers disagree as to the reason for the effect. Splints appear

to be better than no treatment, but only as effective as other active interventions

formyofascial pain. A systematic review and meta-analysis by Ebrahim and colleagues

reviewed 11 eligible studies of 1567 patients, and demonstrated promising results for pain

reduction, very low evidence for an effect on quality of life, and significant research

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The appliance most commonly used in splint therapy is described as a stabilization appliance

or muscle relaxation splint. It is designed to cover a full arch and adjusted to avoid altering

jaw position or placing orthodontic forces on the teeth. It should be adjusted to provide

bilateral, even contact with the opposing teeth on closure and in a comfortable mandibular

posture. It should be reexamined periodically and readjusted as necessary to accommodate

changes in mandibular posture or muscle function that may affect the opposing tooth contacts

on the appliance. At the beginning of appliance therapy, a combination of appliance use

during sleep and for periods during waking hours is appropriate. Factors such as tooth

clenching when driving or exercising, or pain symptoms that tend to increase as the day

progresses, may be better managed by increasing splint use during these times .To avoid the

possibility of occlusal change, no appliance should not be worn continuously (ie, 24 hours per

day) over prolonged periods . Full-coverage appliance therapy during sleep is a common

practice to reduce the effects of bruxism and is not usually associated with occlusal change

PHARMACOLOGIC THERAPY

Both clinical and controlled experimental studies suggest that medications may promote

patient comfort and rehabilitation when used as part of comprehensive treatment. Although

there is a tendency for clinicians to rely on ―favourite‖ agents, no single medication has

proved to be effective for the entire spectrum of TMDs.With respect to pain associated with

disorders of the MOM, analgesics, nonsteroidal anti-inflammatory agents, corticosteroids,

benzodiazepines, muscle relaxants, and low-dose antidepressants have shown efficacy. Many

of the medications used for fibromyalgia can be used for patients with masticatory muscle

disorders. These agents are versatile and effective at treating the multiple symptoms

associated with chronic muscle pain. The medications used for myofascial pain and

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masticatory muscle disorders are discussed in greater detail elsewhere in this issue by Nasri-

Heir and colleagues.

Medications used for fibromyalgia that may be beneficial for masticatory muscle pain

Medication Class Effect

Tricyclic antidepressants (TCAs Moderately helpful for pain

More side effects (xerostomia, fatigue)

Serotonin-selective reuptake Fewer side effects than TCAs

inhibitors More effective for anxiety/

depression than for pain

Muscle relaxants Moderately helpful for local muscle pain

More side effects (xerostomia, sedation)

Serotonin-norepinephrinereuptake Moderately helpful for fibromyalgia-

inhibitors related pain

Low-potency opioids

Moderately helpful for fibromyalgia-

related pain

NSAIDs Helpful for acute inflammatory pain but

not chronic muscle pain or fibromyalgia-

related pain

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It is clear that there are several types of disorders of the masticatory muscles, each of which

may have a complex etiology, clinical course, and response to therapy. Masticatory muscle

disorders include both regional and centrally mediated problems. Host susceptibility plays a

role at several stages of these disorders, including pain modulation and response to therapy.

Disorders of the masticatory muscles must be accurately identified and differentiated from

primary TMJ disorders such as those involving pain from osteoarthritis, disc displacement, or

jaw dysfunction

ATYPICAL FACIAL PAIN

Atypical facial pain (AFP) is a constant chronic orofacial discomfort or pain, defined by the

International Headache Society as facial pain not fulfilling other criteria. Therefore, like

burning mouth syndrome (see below), it is also a diagnosis reached only by the exclusion of

organic disease; there are no physical signs, investigations are all negative and it is an MUS.

Atypical facial pain is fairly common, affecting probably around 1-2% of the population. It is

sometimes termed persistent idiopathic facial pain.

Atypical facial pain is often of a dull boring or burning type character and ill-defined location

and there is: • a total lack of objective signs • a negative result from all investigations • no

clear explanation as to cause • poor response to treatment. Patients are often middle-aged or

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older and 70% or more are females. Most sufferers from AFP are otherwise normal

individuals who are or have been, under extreme stress such as bereavement, or concern

about cancer. There are often recent adverse life-events, such as bereavement or family

illness and/or dental or oral interventive procedures.

Clinical features

History findings in AFP include pain mainly in the upper jaw, of distribution unrelated to the

anatomical distribution of the trigeminal nerve, poorly localised, and sometimes crossing the

midline to involve the other side or moving to another site. Pain is often of a deep, dull boring

or burning, chronic discomfort, and persists for most or all of the day but does not waken the

patient from sleep. However the patient may report difficulty sleeping. There may also be

multiple oral and/or other psychogenic related complaints, such as dry mouth, bad or altered

taste, thirst, headaches, chronic back pain, irritable bowel syndrome or dysmenorrhoea.

Patients only uncommonly use analgesics to try and control the pain but there is a high level

of use of health care services. There have often already been multiple consultations and

attempts at treatment. Pain is accompanied by altered behaviour, anxiety or depression. Over

50% of such patients are depressed or hypochondriacal, and some have lost or been separated

from parents in childhood. Many lack insight and will persist in blaming organic diseases (or

health care professionals) for their pain. Clinical examination is unremarkable with a total

lack of objective physical (including neurological) signs. All imaging studies and blood

investigations are negative.

Diagnosis of AFP

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Diagnosis of atypical facial pain is clinical through careful examination of the mouth, perioral

structures, and cranial nerves, and imaging (tooth/jaw/sinus radiography and MRI/CT scan)

to exclude organic disease such as space-occupying or demyelinating diseases (Table 4).

MANAGEMENT OF PATIENTS SUFFERING ATYPICAL FACIAL PAIN OR PAIN

WITH A PSYCHOGENIC BASIS

Few patients with AFP have spontaneous remission and thus treatment is usually indicated (.

Reassurance and attention to any factors such as the dentures or haematinic deficiencies may

be indicated, but active dental or oral surgical treatment, or attempts at ‗hormone

replacement‘, or polypharmacy in the absence of any specific indication, should be avoided.

Do not repeat examinations or investigations at subsequent appointments, since this only

serves to reinforce abnormal illness behaviour and health fears. Avoid attempts at relieving

pain by operative intervention — since these are rarely successful; indeed, active dental

measures such as restorative treatment, endodontics or oral surgical treatment, in the absence

of any specific indication, should be avoided as they may simply reinforce the patient‘s

perception that the pain has an organic basis.

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However, it is important where possible, to identify and relieve factors which lower the pain

threshold (fatigue, anxiety and depression). Simple analgesics such as NSAIDs should be

tried initially, before embarking on more potent preparations. Patient information is a very

important aspect in management. Cognitive-behavioural therapy (CBT) or a specialist referral

may be indicated. It is important to clearly acknowledge the reality of the patient‘s symptoms

and distress and never attempt to trivialise or dismiss them. Try to explain the psychosomatic

background to the problem, ascribing the symptoms to causes for which the patient cannot be

blamed Set goals which include helping the patient cope with the symptoms rather than

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attempting any impossible cure Offer referral to a specialist or a trial of antidepressants,

explaining that these agents are being used to treat the symptoms not depression, that some

antidepressants have analgesic activity and that antidepressants have been shown in

controlled trials to be effective for this problem, even in non-depressed persons.

Temporomandibular joint pains

Temporomandibular disorders are among the most misdiagnosed and mistreated maladies in

medicine.In the past, disorders of the masticatory system were generally treated as one

condition or syndrome, with no attempt to differentiate subtypes of muscle and joint

disorders.The term TMD are collective term embracing a member of clinical problems that

involve the masticatory musculatures, the TMJ & associated structure or both

Definition

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Okeson J defined TMD as a collective term embracing a number of clinical problems that

involve the masticatory musculature, the temporomandibular joint and associated structures

or both.

Acc to Schiffman, Haley, Shapiro (1990)the TMD encompasses many disorders of the

masticatory musculature (i.e.myositis, muscle spasm, muscle contracture, & myofascial pain

syndrome)and TMJ (internal derangements with or without reduction and degenerative joint

disease)

The AAOP (in 1993 & 1996) refined TMD as a collective term embracing a number of

clinical problems that involve the masticatory musculature, the temporomandibular joint and

associated structures or both.

EPIDEMIOLOGY

Temporomandibular disorders (TMD) are a broad group of clinical problems involving the

masticatory musculature, the temporomandibular joint, surrounding bony and soft tissue

components, and combinations of these problems. Symptoms of TMD include decreased

mandibular range of motion, pain in the muscles of mastication, temporomandibular joint

(TMJ) pain, associated joint noise with function, generalized myofascial pain, and a

functional limitation or deviation of the jaw opening. The prevalence of TMD is thought to be

greater than 5% of the population.Lipton and colleagues showed that about 6% to 12% of the

population experience clinical symptoms of TMD. Patients with TMD symptoms present

over a broad age range; however, there is a peak occurrence between 20 and 40 years of age

TMD is thought to be a multifactorial process secondary to muscle hyperfunction or

parafunction, traumatic injuries, hormonal influences, and articular changes within the joint.

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Various investigators have found correlations between occlusion and TMJ symptoms. Mohlin

and Koppetal showed an association between occlusal interferences and myofascial pain and

dysfunction. They found links between posterior crossbite with muscular discomfort. Patients

with deep bites, class II malocclusion, and anterior open bites may also be predisposed to

myofascial pain.Masticatory-muscle hyperactivity progressing to a ―vicious cycle‖ has been

proposed as the cause of myofascial pain. The diagnostic terms of ―myospasm,‖ ―muscle

spasm,‖ and ―reflex splinting‖ have been used to describe these conditions. The lack of a

clear single cause has resulted in the proposal of a multifactorial etiology.

This disorders are characterized by

1. Facial pain in the region of the TMJ & for the muscle of mastication.

2. Limitation or deviation in the mandibular range of motion.

3. TMJ sounds during jaw movements & function.

Etiology

Some of the factors proposed are the following:

1. Parafunctional habits (eg, nocturnal bruxing, tooth clenching, lip or cheek biting).

2. Emotional distress

3. Acute trauma from blows or impacts

4. Trauma from hyperextension (dental procedures,oral intubation for general anesthesia,

yawning, hyperextension associated with cervical trauma)

5. Instability of maxillomandibular relationships

6. Laxity of the joint

7. Comorbidity of other rheumatic or musculoskeletal disorders

8. Poor general health and an unhealthy lifestyle

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In general, TMD can be divided into articular and nonarticular disorders. These disorders are

synonymous with intracapsular and extracapsular conditions, respectively.

Most nonarticular disorders present as myofascial pain focused to the muscles of mastication.

In fact, more than 50% of TMD is myofascial pain. Other nonarticular disorders include

chronic conditions, such as fibromyalgia, muscle strain, and myopathies. Myofascial pain and

dysfunction is theorized to arise from clenching, bruxism, or other parafunctional habits. The

result is masticatory musculature strain, spasm, pain, and functional limitation

Articular disorders (internal derangement) can be divided into inflammatory and

noninflammatory arthropathies. Inflammatory articular disorders include rheumatologic

processes, such as rheumatoid arthritis (RA), seronegative spondylopathies, such as

ankylosing spondylitis, psoriatic arthritis, gout, and infectious arthritis. Noninflammatory

articular disk disorders include osteoarthritis, joint damage from prior trauma or surgery, or

other cartilage or bone disorders. Mechanistically, articular disorders occur as a result of an

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altered balance of anabolic and catabolic cytokines. This cytokine imbalance creates an

inflammatory milieu, which leads to oxidative stress, free radicals, and ultimately joint

damage. Internal derangement equates to changes in the disk-condyle relationship.

Disk displacements are categorized as disk displacement with reduction or disk displacement

without reduction. The fibrocartilage disk is typically displaced anteromedially but rarely

may be displaced laterally or posteriorly. Anatomically, disk displacement with reduction is

interference between the mandibular condyle with the articular disk during jaw opening or

closing. This interference may generate clicking, popping, or crepitus in the joint, which can

be associated with discomfort. Clicking alone, however, is not diagnostic of articular disk

displacement. During disk displacement with reduction, the condyle meets the posterior

aspect of the disk, which then reduces to its proper position between the condyle and glenoid

fossa.Articular disk displacement is associated with TMD. One study found magnetic

resonance imaging (MRI) evidence of disk displacement in 84% of symptomatic patients

with TMD versus 33% of asymptomatic patients. MRI findings, however, should not solely

dictate treatment because disk displacement may occur in asymptomatic patients.

Disk displacement without reduction results in a closed lock whereby the condylar movement

is physically blocked by the anteriorly displaced disk.

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Acute closed clock is associated with limited mandibular opening and severe pain. Physical

examination should include a general assessment of the head and neck, palpation of the

masticatory muscles, occlusal analysis, examination of the jaw opening and closing, and

palpation of the TMJ. Palpation of the muscles of mastication may elicit mild to severe pain.

Masseters are palpated with fingers positioned over the angle of the mandible. The temporalis

muscles are palpated along the temple with the jaw relaxed and clenched. The pterygoid

muscles are palpated intraorally along the medial aspect of the mandibular ramus between the

tonsillar pillars.

In general, articular disorders are classified according to the Wilkes‘ Staging Classification

for Internal Derangement of the TMJ (stages I–V). Wilkes‘ classification is based on clinical,

radiologic, and anatomic findings

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For research purposes, a more detailed diagnostic classification is used. This classification is

known as the Research Diagnostic Criteria for TMD (RDC/TMD). The RDC/TMD

classification system is divided into 3 axes: axis I (muscle disorders), axis 2 (disk disorders),

and axis 3 (arthralgias).

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Diagnosis

Diagnosing TMD requires a focused history and physical examination. Pain and limited

range of motion are accepted symptoms of TMJ dysfunction. Radiographic studies can also

be used as supplemental diagnostic tools. Periapical radiographs can be used to rule out

dental pathologies as a cause of referred pain. Cone beam computed tomography scans and

panoramic radiographs will provide detailed imaging of the joint‘s bony structures but not the

articular disk. MRI is the modality of choice for examining the disk position and morphology

(gold standard). MRI may also show degenerative bony changes. MRI findings should not

alone dictate treatment strategies. One must combine patients‘ clinical presentation, signs,

and symptoms along with TMJ imaging when developing a treatment plan. On MRI, joint

effusions are radiographic signs of inflammation. Inflammation indicates a transition from

adaptive to pathologic changes within the joint. The MRI diagnosis of anterior disk

displacement uses the most superior aspect of the condyle (12-o‘clock position) as a

reference point. Anterior disk displacement is defined radiographically when the posterior

disk tissue is located anterior to the 12-o‘clock condylar position. Disk displacement may

occur in asymptomatic patients such that all radiographic findings must be placed in clinical

context before beginning TMJ treatments.

TREATMENT

The treatment of TMJ osteoarthrosis and internal derangement can be divided into 3 broad

categories: noninvasive, minimally invasive, and invasive management. The specific

management plan can vary depending on the specific diagnosis and severity of TMJ disorder;

however, the underlying principles of treatment apply universally.

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1. Multidisciplinary approach involving multiple specialties, including general dentistry, oral

medicine, orofacial pain, orthodontics, oral surgery, physical therapy, and psychiatry may be

necessary to fully address the problem from all angles.

2. There is progression of treatment only after failure of more conservative modalities. The

least invasive and most reversible treatments should be tried first. Only after a failure to alter

the disease process and clinical symptoms should more invasive and often nonreversible

treatments be initiated.

Goals of treatment

1. Decreasing joint pain

2. Increasing joint function and opening

3. Preventing further joint damage

4. Improving overall quality of life and reducing disease-related morbidities

NONINVASIVE TREATMENT OPTIONS

Occlusal or Stabilization Splints

Physicians have used various types of splints since the eighteenth century for the treatment of

TMJ disorders. Today the use of splints has become one of the most common in office initial

treatments for TMD-associated pain. Since their inception, splints are thought to work by

unloading the condyle and in effect protecting the TMJ and articular disk from degeneration

and excessive articular strain. Although there are varying designs, they all function similarly

to disengage the condylar head from the fossa and articular disk

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.

A recent meta-analysis of randomized controlled trials evaluating intraoral orthopedic

appliances for TMD showed that hard stabilization appliances have good evidence of modest

efficacy in the treatment of temporomandibular joint dysfunction (TMJD) pain compared

with nonoccluding appliances and no treatment. Other types of appliances, including soft

stabilization appliances, anterior positioning appliances, and anterior bite appliances, have

some evidence of efficacy in reducing TMD pain. However, a Cochrane Database review of

stabilization splint therapy for TMJ pain revealed that there is insufficient evidence either for

or against the use of stabilization splint therapy.Clearly further randomized controlled studies

with larger sample sizes and longer duration of follow-up are needed to study the

effectiveness of splint therapy for TMD pain.

Pharmacotherapy

Pharmacologic therapy in conjunction with other treatment modalities often plays an

important role in the management of articular disk and TMJ disorders. The aim of

pharmacotherapy can be divided into 2 main goals:

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1. Treatment of the underlying disease process

2. Alleviation of disease associated symptoms, such as pain and swelling

There are various classes of medications that function to target each of the 2 treatment goals .

Oftentimes it is necessary to use a combination of medications to treat both the pain as well

as the inflammatory disease process, depending on the severity of disease. However, care

must be taken to avoid the prolonged use of certain medications, in particular analgesics, to

prevent drug tolerance and dependency. The health provider‘s ultimate goal should be

symptomatic relief for a period of time in the hopes that this will break the disease cycle and

lead to permanent improvement. Despite the frequent use of pharmacologic agents, numerous

review articles have shown insufficient evidence to support or not support the effectiveness

of pharmacologic interventions for pain in patients with TMJ disorders. There is an obvious

need for further randomized controlled trials to study the effectiveness of pharmacologic

interventions to treat pain associated with TMD

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Physical Therapy

Physical therapy is commonly used in the outpatient setting to relieve musculoskeletal pain,

reduce inflammation, and restore oral motor function. Physical therapy plays an adjunctive

role in virtually all TMJ disorders treatment regimens. Various physical therapy modalities

are available to the outpatient health provider.

MINIMALLY INVASIVE TREATMENT OPTIONS

Intra-articular Injections

Different therapeutic solutions can be injected directly into the TMJ space and allow for the

targeted treatment of inflammation and joint degeneration (see Table 3). The TMJ has 2

unconnected cavities, superior and inferior, partitioned by the articular disk. The superior

space injection is the commonly used technique. However, a recent review article showed

that an inferior space injection, or simultaneous upper and lower spaces injections, seemed to

be more effective with increasing mouth opening and decreasing TMJ-associated pain

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Arthrocentesis/Arthroscopy

Arthrocentesis and arthroscopy are safe and quick minimally invasive procedures that are

used in patients who are resistant to more conservative treatment modalities. Oftentimes they

are combined with immediate postoperative intra-articular injections and the use of occlusal

splints, pharmacotherapy, and physical therapy during the recovery period

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Arthroscopy for TMD treatment

Arthroscopy involves insertion of an arthroscope and inspection of the TMJ under fluid

distention under general anesthesia; allows for irrigation of joint space, lysis of these

adhesions, and mobilization of the joint under direct visualization

Indication

Limited opening and pain secondary to internal derangement

TMJ hypomobility secondary to fibrosis or adhesions

Degenerative osteoarthritis

Contraindications

TMJ with severe bony or fibrous ankylosis

Extracapsular source of pain

Patients who have not undergone noninvasive treatment modalities

Practitioner with lack of open joint surgery experience

Efficacy

A large multicenter study reports more than 90% success rate as defined as improved

mobility, pain, and function. Arthroscopy led to greater improvement in opening after 12

months than arthrocentesis; however, there was no difference in pain

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INVASIVE TREATMENT OPTIONS

Arthroplasty

TMJ arthroplasty involves the reshaping of the articular surface to remove osteophytes,

erosions, and irregularities found in osteoarthritis refractory to other treatment modalities.

These patients frequently also present with articular disk degeneration or displacement, which

can be repositioned, repaired, or entirely removed. All such procedures should be performed

by an experienced oral surgeon under general anesthesia, and this is done using an open

surgical approach through a periauricular skin incision. Complications are rare but can

include wound infection, facial nerve injury, permanent occlusal changes, relapsing joint

pain, and life-threatening vascular injuries.As with all TMJ-related surgeries, early

postoperative physical therapy and range-of-motion exercises are vital to achieving long-term

functional improvements.

Disk repositioning: Reposition the disk back to its normal anatomic position in patients with

internal derangement. This procedure is most effective in disks that are normal appearing

(white, firm, shiny) with minimal displacement.

Disk repair: Small disk perforations can be repaired with a tension-free primary closure.

Discectomy alone: Removal of the articular disk is indicated in patients with severe disk

perforation, complete loss of disk elasticity, and who are persistently symptomatic even after

disk repositioning. Although studies have shown theris generally an improvement in pain and

maximal mouth opening following the surgical removal of the disk, patients also exhibited

signs of fibrous adhesions, narrowing of joint space, and osteophyte formation on MRI.44–46

Discectomy with graft replacement: The placement of a graft is thought to protect the joint

from further degeneration and prevent the formation of fibrous adhesions. The use of

autogenous sources, such as temporalis flaps, auricular cartilage, and dermal grafts, results in

superior clinical outcomes compared with alloplastic grafts. Studies have showed that

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autogenous grafts actually did not prevent remodeling of the joint but may help to reduce the

onset of crepitus resulting from discectomy alone. However, it was shown that discectomy

with dermis graft replacement does result in a statistically significant improvement in pain,

chewing, and general health .

Total Joint Replacement

TMJ replacement is intended primarily at restoration of form and function, and any pain

relief gained is only a secondary benefit.The need for TMJ replacement typically indicates

severely damaged joints with end-stage disease that has failed all other more conservative

treatment modalities. Autogenous costochondral bone grafts have been frequently used in

TMJ reconstruction in the past because of its gross anatomic similarity to the mandibular

condyle, ease of adaptation to the recipient site, and its demonstrated growth potential in

juveniles. However, because of potential harvest-site morbidity and failure during the

transplantation process or from functional loading, the use of alloplastic materials has

become increasingly more popular in the adult population.

Indications and relative contraindications for TMJ alloplastic replacement

Indications

1. Ankylosis or reankylosis with severe anatomic abnormalities 2. Failure of autogenous

grafts in patients who underwent multiple operations 3. Destruction of autogenous graft tissue

by pathosis 4. Severe inflammatory joint disease that results in anatomic mutilation of the

total joint components and functional disability 5. Failure of Proplast-Teflon implant (Vitek

Inc, Houston, Texas) 6. Failure of Vitek-Kent total or partial joints (Vitek, Inc, Houston,

Texas)

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Relative contraindications

1. Pediatric patients with immature facial skeleton 2. Patients with unrealistic expectations or

lack of understanding of procedure 3. Uncontrolled systemic disease 4. Allergy to implant

material 5. Active infection at implantation site

Currently, various custom and stock titanium joint designs are available, which consist of

both a fossa and a condylar component held in place by screw fixation. Studies have shown

that both custom and stock alloplastic TMJ replacements resulted in statistically significant

improvement in pain level, jaw function, and incisal opening.

CONCLUSION

Orofacial pain is a common complaint of patients seen by dental professionals. Even if this

complaint is not the primary reason for a patient seeking treatment, or the pain is

nonodontogenic in origin, it is still important to acknowledge the problem to find an

appropriate route to proceed. Whether this involves treatment of the condition or referral to

another practitioner for assessment, formulating an inclusive differential diagnosis is a crucial

step in the process. A broad range of possibilities prevents the provider from making a

diagnosis too quickly, and most importantly assures that more serious causes are not

overlooked or mistaken for common, less threatening condition.

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