APPROACH TO

MOVEMENT DISORDERS

Presented by:
Dr. Milan Mori
 INTRODUCTION

 Involuntary movements unaccompanied by weakness

 Movement disorders disrupt motor function by abnormal , involuntary ,

unwanted movements or may curtail the amount of normal free flowing
and fluid movement.

 They occur due to defect in Basal Ganglia Pathways

Basal Ganglia

 There are five nucleus in basal ganglia which include:

1) Caudate nucleus : along lateral side of each lateral ventricle
2) Putamen
3) Globus pallidus (Gpi & Gpe)
4) Subthalamic nucleus : small structure on border between
brain
stem & cerebrum , lateral & inferior to hypothalamus
5) Substantia nigra (SNc& SNr ) : Histologically- 2 portions –
SNc &
SNr
Basal Ganglia
Basal Ganglia

 3 biochemical pathways-
 Nigrostriatal Dopaminergic System
 Intrastriatal Cholinergic System
 GABAergic system projecting from Striatum to Globus pallidus and substantia
nigra
 Parkinson
Hypokinetic
Disease

Myoclonus

Jerky
CLASSIFICATIO Movements
Chorea

N Hyperkineti
Tic
Disorders
c

Dystonia
Non Jerky
movements
Tremor
Clinico-pathologic correlation

 Parkinsonism substantia nigra

 U/l hemiballismus subthalamic nucleus
 Chorea caudate
nucleus
 Athetosis , dystonia putamen or thalamus
 Myoclonus cerebellar cortex /
thalamus
 Rhythmic palatal/facial myoclonus central tegmental tract , inf
olivary nuc ,
olivodentate fib
Hypokinetic Disorders

 Parkinson's disease (PD): Archetype of hypokinetic movement disorders

 Hypokinetic movement disorders are usually called Akinetic-rigid

syndromes and About 80% of akinetic-rigid syndrome are due to PD.
Parkinsonian Tremor

 Resting, static, or non-intention tremor slow and coarse in nature

 Onset is usually in one hand; it may later involve the contralateral upper
limb or ipsilateral lower limb.
 The rate vary from 2 to 6 Hz, averaging 4 to 5 Hz
 Movement consists of alternate contractions of agonist and antagonist,
involving the flexors, extensors, abductors, and abductors of the fingers
and thumb, together with motion of the wrist and arm
Parkinson Disease

Cardinal Features-
 Resting Tremors
 Rigidity
 Bradykinesia
 Gait Dysfunction
Spectrum of Hyperkinetic Disorders

Myoclonus Ballismus Chorea Athetosis

Dystonia

Movements become - Less violent / explosive / jerky

Smoother and more flowing
More sustained
They differ from tics in that they cannot be suppressed by voluntary
control
Myoclonus

 Single or repetitive, abrupt, brief, rapid, lightning-like, jerky, arrhythmic,

involuntary contractions involving portions of muscles, entire muscles,
or groups of muscles

 Movements are quicker than chorea

 Seen principally in the muscles of the extremities and trunk, but the
involvement is often multifocal, diffuse, or widespread

 May involve the facial muscles, jaws, tongue, pharynx, and larynx
 Classification of Myoclonus

 Myoclonus has been classified in numerous ways, including the following:

positive (muscle contraction) vs negative (loss of muscular tone-asterixis);

stimulus sensitive (reflex) vs spontaneous;

rhythmic vs arrhythmic;

anatomically (peripheral, spinal, segmental ,brainstem or cortex)

by aetiology (physiologic, essential, epileptic, and symptomatic)

 may be sporadic or familial (hereditary essential myoclonus,
paramyoclonus multiplex)
 Treatment: Valproic acid is drug of choice May respond to
benzodiazepines
Ballism

 Greek word ballismos, which means a jumping movement

 They are usually unilateral- Hemiballismus

 Rarely, they may involve bilateral Upper Limbs or lower limbs

(paraballismus) or a single extremity (monoballismus)
Hemiballismus

 Dramatic neurologic syndrome of wild, flinging (forceful), incessant

(uninterrupted or continuous) movements that occur on one side of the
body

 Due to infarction or hemorrhage in the region of the contralateral

subthalamic nucleus

 Results in disinhibition of the motor thalamus and the cortex, resulting

in contralateral hyperkinetic movements
Hemiballismus

 Movements are involuntary and purposeless. More rapid and forceful

 Involve the proximal portions of the extremities

 When fully developed, there are continuous, violent, swinging, flinging,

rolling, throwing, flailing(thrashing) movements of the involved
extremities

 Hemiballismus is difficult to treat, incredibly disabling.

Chorea (latin- choreus- dance)
 Jerky semi-purposive uncontrollable movements of limbs, face & trunk,
increase with anxiety & disappear during sleep.

 Patients often attempt to conceal involuntary movements by

superimposing voluntary movements onto them e.g., an involuntary
movement of arm towards face may be adapted to look-like an attempt
to look at watch
 Causes of chorea:

 Drugs : levodopa in Parkinson’s patients , oral contraceptive pill

 Vascular disease of the basal ganglia : atheroma
 Systemic lupus erythematosus
 Degenerative diseases : Huntington’s disease
 Post-infectious : Sydenham’s chorea
 Chorea gravidarum
 Chorea associated with NMDA Receptor positive encephalitis
 Chorea-acanthocytosis
 Other causes : Thyrotoxicosis
 Chorea

Childhood Onset Adult Onset

(<16 Years, AAO) (>16 Years, AAO)

Sporadic Autosomal Recessive Autosomal Dominant X – Linked

Mitochondrial

Martinez-Ramirez, D, et al. Review of Hereditary and Acquired Rare Choreas. Tremor and Other Hyperkinetic Movements. 2020;
 Evaluation of Chorea

•Family History •Other Neurological features

•Time Course •Other Medical conditions

•Phenomenology •Medications

•Exacerbating / reliving factors •Neuroimaging and Lab workup

 Autosomal Dominant Chorea’s

•Huntington’s disease •Benign Heriditary chorea

•C9orf72 expansions •Neuroferritionpathy
•HDL1
•Fahr’s disease (SLC20A1,
•HDL2
PDGFRB, PDFGB…)
•SCA (1,2,3,8, 17)
•Paroxymal dyskinesias
•DRPLA
 Sporadic Chorea – Structural
lesions

CNS Lymphoma Non ketotic Hyperglycemia Metabolic BSPDC

Metabolic Multiple sclerosis Infections Meningioma

• Movement Disorders
 Sporadic Chorea – Structural
lesions
• Stroke Basal Ganglia Necrosis

• Tumor
• AV malformation
Caudate Atrophy Recent Infection
• BG Calcification

Lactate / Pyruvate Increased Post Infectious Striatal

necrosis

Workup for Inherited

metabolic diseases; organic /
Infantile bilateral striatal Leigh’s Syndrome & Other
aminoacids, lysosomal
necrosis Mitochondrial Disorders
enzymes, skin biopsy ect

If NEGATIVE workup for

GENETIC DISORDERS
 Sporadic Chorea – Iron Deposition Disorders

Aceruloplasmenia MPAN

PANK BPAN PLAN

 Sporadic Chorea – Iron Deposition Disorders
PANK Mutation

PANK Serum Ceruloplasmin

Normal Reduced

PLA2G6 Mutation Aceruloplasmineia

Phospholipase associated FAHN

Neurodegeneration
BPAN Genetic Workup

MPAN
 Medications Causing
Chorea
• Dopamine Receptor Blockers • Antiparkinosonian Medications
• Phenothiazines • Levodopa
• Butyrophenones • Dopamine Agonists
• Benzamides • Anticholinergics

• Antiepileptics • Calcium Channel Blockers

• Phenytoin • Cinnarizine
• Carbamaz • Flunarizine
epine
• Verapamil
• Valproic
acid • Others
• Lithium, Baclofen, Digoxin
• Psyhcostimul
ants • TCA, Cyclosporine
• Ampheta • Steroids, Oral contraceptives, Theophylline
mines
 Sporadic Chorea – Normal MRI
Medication / Drug Induced

Yes / Most Likely Metabolic Screening

Acute Tardive Level -1 : Electrolytes, Glucose, Pregnancy Test, TSH

Diagnostic Level -2 : APLA, SLE, Autoimmune, Polyathemia vera, thrombocythemia, Celiac,

HIV, B12, TPHA, Lead, Paraneoplastic, Infectious (Bacterial, Viral, spirochetal)

Friedrich’s Ataxia
Ataxin Normal LFT Abnormal
AT, AOA-1, AOA-2

Increased CPK

Genetic Workup Acquired Hepatolenticular

Normal
Degeneration
 Chorea – Genetic Forms
Adult Onset Childhood Onset

AD AR AD AR X-linked / mitochondrial
• Friedreich’s Ataxia
• Huntington’s Disease • NPC • BHC • Pelizaeus
• Ataxia Telangiectasia
Merzbacher
• BSPDC • Chorea- • Tuberous
• AOA-1
Sclerosis • Organic acidurias
• Neuroferritionpathy acanthocytosis • AOA-2
(GA-1, MMA,
• ADCY5 • FOXG1, GNAO1, GRIN1,
• HDL1 • McLeod homocytinuria,
FRRS1L, IRF2bPL
Syndorome …)
• C9orf72 • Xeroderma
Pigmentosum
• Aceruloplasmin • GLUT1 deficiency
• Familial Prion • HDL-3
emia • Sulfite oxidase
• SPG-58
• SCA (1, 2, 3, 7, 8, 12, def
• Wilson Disease • PDE10A
17, 48) • Leighs’Pyruvate
• GPR88

• DRPLA carboxylase def

Martinez-Ramirez, D, et al. Review of Hereditary and Acquired Rare Choreas. Tremor and Other Hyperkinetic Movements. 2020;
Athetosis

 Athetosis means “without fixed position”

 Involuntary, irregular, coarse, somewhat rhythmic, and writhing or

squirming in character (twisting)

 movements are characterized by any combination of flexion, extension,

abduction, pronation, and supination, often alternating and in varying
degrees
Athetosis

 Affected limbs are in constant motion

 Disappear in sleep

 Voluntary movements are impaired, and coordinated action may be

difficult or impossible

 Choreoathetosis refers to movements that lie between chorea and

athetosis in rate and rhythmicity, and may represent a transitional form
Tics

 Stereotyped character of recurrent movements

 Differentiating feature- preceded by rising discomfort or urge (sensory

tic), that is relieved by the actual movement (itch and scratch analogy)

 Can be suppressed by an effort of will, may lead to rebound of tics

afterwards

 Predominantly involve face, upper arms and neck

 Classification-

 I- Simple Tics- eye blinking, nose wrinkling, throat clearing

 II- Complex Tics- touching things, smelling objects, echopraxia(mimicking movements)

 A- Motor Tics- stereotyped head jerks

 B- Phonic Tics- grunting, echolalia (repeating other people’s words), palilalia (repeating
one’s own words), coprolalia (expression of obscene words)
Tourette’s Syndrome

 Affects males> females

 Multiple motor tics accompanied by phonic tics.

 Age group- 2-15 years (mean 7 years)

 Mostly disappear in adulthood

Tremor

Tremor is an involuntary, rhythmic, oscillatory movement of a body part

Axis 1- emphasizing the clinical features, history, and tremor characteristics

Axis 2- emphasizing the potential etiologies of tremor

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

Axis 1

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

 Tremor syndromes

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

Activation conditions for tremor examination

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

Tremors on the basis of activation
pattern
 Axis 2

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

History in a case of tremor

 Can you tell me about your tremor? or What type of tremor do you
have? or When do you notice tremor?
 Does your hand shake when you are writing?
 Does your hand shake when you are trying to eat something?
 The body areas that seem to be shaking (eg, arms, head, voice)?
 The limb positions that bring on the tremor and, conversely, those that
seem to lessen it ?
 The age at which tremor began ?
 How the tremor has changed over the years ?
History in a case of tremor

 The presence of other involuntary movements ?

 The presence of other neurologic symptoms aside from tremor ?
 The presence of pulling sensations or discomfort in the body part that is
shaking ?
 The use of medications that seem to produce or exacerbate tremor?
 Dietary factors that exacerbate tremor (eg, coffee and other forms of
caffeine) ?
 Symptoms of thyroid diseases (eg, weight loss, heat intolerance) ?
 Family history of “shaking” or tremor?
Conditions aggravating the tremor

Conditions aggravating tremors

Stressors Anxiety, fright, sleep deprivation, muscle fatigue, fever, cold weathe
Medications Stimulants: caffeine, nicotine, amphetamines, methylphenidate, ephedrine,
pseudoephedrine Bronchodilators: albuterol and β-agonists, theophylline
and other catecholamine-like agents
Mood stabilizers/antidepressants: lithium, tricyclic antidepressants, SSRIs
Cardiac medications: amiodarone
Thyroid hormone
Immunosuppressant and anticancer drugs: corticosteroids, tacrolimus,
cyclosporine, vincristine, cisplatin, paclitaxel, doxorubicin, cytosine
arabinoside, ifosfamide, 5-fluorouracil, methotrexate
Hypoglycemic agents: sulfonylureas
Antiepileptics: sodium valproate, carbamazepine, phenytoin
Dopamine-blocking or -depleting agents: typical and atypical antipsychotic
medications, antiemetics (metoclopramide), tetrabenazine
Toxins Mercury, lead, manganese, arsenic, cyanide, naphthalene, toluene, lindane
Metabolic Nephrotic or liver failure, hyperthyroidism, thyrotoxicosis, hypo- or
disorders hyperglycemia, pheochromocytoma
Bhatia KP, et al. Mov Disord. (2018) 33:75–87
Jankovic J et al Ann Intern Med 1980;93(03): 460–465
 Clinical examination of tremors
Posture /Position
Rest Tremor LYING POSITION
1. Head
2. Trunk
3. Legs
SITTING POSITION
1. Hands (upper extremity fully
supported on the armrest of the chair)
2. Head
3. Face/Chin/Jaw
4. Voice (aa,ee o)
5. Tongue

Bhatia KP, et al. Mov Disord. (2018) 33:75–87)

Lenka A et al Front. Neurol. 12:684835
 Protocol for examination of tremors

Posture /Position
Action Tremor 1. Tongue ( protruded)
2. Hands outstretched with elbow extension
3. Hands outstretched with elbow flexon
4. Right leg extension
5. Left leg extension
6. FNF (right hand)
7. FNF (left hand)
8. Sitting to standing position

Bhatia KP, et al. Mov Disord. (2018) 33:75–87

Lenka A et al Front. Neurol. 12:684835
Dystonic tremor: Video
Primary writing tremor:Video
Dystonia

 Sustained or intermittent muscle contraction of antagonist muscle causing

abnormal often repetitive movements and postures.

 Pathophysiology
co contracting synchronous bursts

agonist and antagonist muscle leads

to recruitment of the muscle groups
which is not required for a given movement
Types of dystonia

 Focal dystonia

cervical Blepharospasm Limp Spasmodic Oromandibular

 Generalized- onset mainly in childhood or
adolescence , often
hereditary in nature.

 Combined- associated with other movement

disorder like
parkinsonism and myoclonus.
 Complex- dystonia is part of a syndrome like
Wilson disease ,
Huntington disease, Lesch Nyhan
syndrome.
 Treatment-
 Trihexyphenidyl
 Baclofen
 Botulinum toxin
 DBS of pallidum
 Ballism, Chorea, Athetosis and
Dystonia
 These should NOT be thought of as separate entities amenable to
specific definition but rather as a SPECTRUM of movements that blend
into one-another.

 They often co-exist. Even neurologists may often not be able to agree as
to how a particular movement should be classified.
Tardive dyskinesia

 Involuntary movements as facial grimacing , chewing movements,

tongue movements (oro-bucco-mandibular dyskinesia).

 Appear after weeks, generally years of exposure to dopamine receptor

blocking drugs. Older or classical ‘typical’ antipsychotics
chlorpromazine, haloperidol

 Thought to be due to receptors super sensitivity to these

drugs .Changes in synapse number and dendrite arborization also occur.
Tardive dyskinesia

 Newer ‘atypical’ drugs (e.g. clozapine, quetiapine,

aripiprazole)
How to approach to a patient of
movement disorders?
 The key to diagnosing movement disorders is establishing the
phenomenology of the clinical syndrome.

 The phenomenology is determined from specific combination of

dominant movement disorder, presence of any additional abnormal
movements, and any further neurological and non neurological
abnormalities.
 A range of conditions, both neurological and non neurological, can mimic
various movement disorders, and it is important not to miss these
lookalikes.

 A systematic approach is recommended when clinicians see patients

who present with one or more types of movement disorder.
Any approach begins with . . .

 A good history
 A good physical exam
 Keen sense of observation
 A systematic differential diagnosis
Elements of the history

 Time course/functional disability/effect upon quality of life

 Past medical history, including infections and toxin exposures
 Drug history (current, previous, recreational)eg- Typical Antipsychotics,
Metoclopromide, Phenytoin, Fluoxetine, Lithium
 Alcohol responsiveness
 Family history
 Neuropsychiatric features
 Autonomic symptoms
 Sleep problems
Other elements of history

 Do specific actions provoke the movement?

 Do the movements occur in relation to other actions?
 Do the movements occur during sleep?
 Are there any associated sensory symptoms?
 Can the movements be suppressed?
 Are there aggravating or alleviating factors?
Observation

 Rhythmic vs. arrhythmic

 Sustained vs. nonsustained
 Paroxysmal vs. Nonparoxysmal
 Slow vs. fast
 Amplitude
 At rest vs. action
 Patterned vs. non-patterned
 Combination of varieties of movements
 Suppressibility
Rhythmic vs. arrhythmic

Rhythmic Arrhythmic
 Tremor Akathitic movements
 Dystonic tremor Athetosis
 Dystonic myorhythmia Ballism
 Myoclonus (segmental) Chorea
 Myoclonus (oscillatory) Dystonia
 Moving toes/fingers Tics
 Periodic movements of sleep
 Tardive dyskinesia (stereotypy)
Points to remember on exam

 Observe casually during history:

 Any involuntary movements and their distribution
 Utterances and vocalizations
 Blink frequency
 Excessive sighing
 Cognitive assessment
 Gait, axial tone
 Eye movements (range, speed)
 Limb examination (writing, hand posture)
 Tremors/postures
 Tone
 Power and co-ordination
 Fine finger and rapid alternating movements
 Reflexes/plantars
Diagnostic
Algorithm
Movement Disorder Mimics

 Mimics of parkinsonism-
 Depression
 Obsessive slowness
 Hypothyroidism
 Spasticity
 Frozen Shoulder
 Catatonia
Movement Disorder Mimics

 Mimics of craniocervical Dystonia

 Retropharyngeal abscess
 Atlanto-axial subluxation
 Congenital Muscular Torticollis
Movement Disorder Mimics

 Mimics of Limb Dystonia

 Contracture
 Spasticity
 Abnormal posture due to paresis or atrophy
 Stiff-person syndrome
 Tonic Spasms
 Seizures
Movement Disorder Mimics

 Mimics of myoclonus
 Tics
 Fasciculations (spontaneous contractions of muscle fibers supplied by a single
motor unit that are too small to cause movement across a joint)
 Myokymia ( involuntary, subtle, continuous, rippling quivering of muscles,
which does not produce movement across a joint)
 Chorea
Systemic Disorders
presenting with
dyskinesias
Hyperkinesias that persist during sleep

 Secondary palatal myoclonus

 Ocular myoclonus
 Oculofaciomasticatory myorhythmia
 Moving toes
Psychogenic Movement Disorders

 Tremors affecting upper limbs- most common psychogenic movement

disorder

 Acute in onset, patterned, and are inconsistent with known movement

disorders

 Diagnosis is based on non organic quality of movement with absence of

findings of any disease

 Characteristic feature- Variability and Distractibility

Psychogenic Movement Disorders

 Magnitude increases with attention and diminishes after being asked to

perform different tasks

 Tremor frequency- variable and it responds to placebo

 It may or maynot be associated with other psychiatric disorders.

Take Home Message

 Establishing the phenomenology of a movement disorder is essential in

the diagnostic pathway

 Most of the movement disorders are diagnosed clinically and

investigations play only a supportive role

 Many conditions- both neurological and non neurological, can mimic

various movement disorders and it is vital not to miss these lookalikes
THANK YOU