B Y B A D E R A L M A S A A D

Movement Disorders

Introduction

Spectrum

Abnormalities in muscle tone, in coordination and apraxias are now classified under movement disorders

Clinical sign, not diagnosis.

Approach is different:

what class is the movement disorder.

Is it primary or secondary

Classes

Tremor

Dystonia

Chorea

Tardive dyskinesia

Ballism

Tics

Myoclonus

others

Tremor

Rythmic oscillation of a body part, produced by either

alternating or synchronous contractions of reciprocally innervated antagonistic muscles

Fixed frequency

Amplitude can vary widely

Rest

Postural

Intention

Rest Tremor

occurs with body part in complete repose, and often dampens or subsides entirely with action.

Onset is most commonly in arms, often asymetrically

Can affect the face ( lips and jaw)

Usually starts distally.

Usually disappears during sleep

Distraction often brings out the tremor

Resting tremor

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Causes of Rest Tremor

Parkinsons disease and other parkinsoniansyndromes

Midbrain or rubral tremor (holmes tremor)

Wilsons disease

Essential tremor

Postural Tremor

Tremor that is present on maintenance of a posture

Many are mistaken as having “bad nerves”

Can be associated with terminal accentuation

Anxiety and stress worsen the tremor

Postural tremor

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Causes of Postural Tremor

Physiological tremor

Essential tremor

Anxiety, fatigue, emotion, stress, hypoglycemia, thyrotoxicosis, phaeochromocytoma, drugs etc

Primary writing tremor

Orthostatic tremor

PD (re-emergent tremor)

Cerebellar

Midbrain tremor

Psychogenic

And many others

Head Tremor

Essential tremor

Cervical dystonia

Cerebellar outflow disease

Head tremor

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Dystonia

Disorder dominated by sustained muscle contractions, which often cause twisting and repetitive

movements or abnormal postures.

Slow and twisting

Rapid (myoclonus like)

Superimposed rythmic movements

Stress and anxiety aggravate the movements

Sensory tricks

Can be task specific

Dystonia

Primary ( pure, no other signs)

Secondary ( dystonia-plus syndrome)

Examples:

Blepharospasm

Oromandibular or lingual dystonia

Laryngeal dystonia

Cervical dystonia

Axial dystonia

Dystonia

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Chorea

Greek choreia= dance

Irregular, unpredictable, brief, jerky movements that flow randomly from one part of the body to another.

Choreoathetosis is slow chorea.

Aggravated by standing and walking

Motor impersistence (esp. in HD)

Chorea

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Chorea

Acute or subacute onset?1. Cerebral infarcts, Sydenham chorea, hyperthyroidism, SLE,

neuroleptic withdrawal, chorea gravidarum

Slow and insidious onset?1. Huntingtons disease, benign hereditary chorea(TITF1 gene)

Relapsing remitting pattern?1. SLE, Drugs

Family history!

Tardive dyskinesia

repetitive stereotypical movements which are most pronounced in the orolingual region.

Speed and amplitude can increase markedly with distraction.

Patients have voluntary control over movements and can suppress them

Tongue protrusion is manageable, with no motor impersistence

Gait is often normal

Tardive dyskinesia

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Tardive dyskinesia

Causes:

Dopamine receptor blockers (antipsychotics, metoclopramide)

Dopamine agonists, anticholinergics, antihistamines all cause a similar type of dyskinesia

Multiple infarctions in the basal ganglia

Cerebellar vermis lesions

NMDAR encephalitis associated with ovarian or testicular tumours

Idiopathic oromandibular dystonia

Ballism

Uncommon

Greek = “to throw”

High amplitude movements, violent, flinging in nature

Rapid and nonpatterned

Involve more proximal muscles

Usually involves one side of body, hemiballismus

Commonly associated with other movement disorders such as chorea and dystonia

Cause: lesion in contralateral subthalamic nucleus or striatum ( eg. Vascular, tumors, infections, NKHS, drugs)

Ballism

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Tics

Brief, intermittent movements (motor tics) or sounds (phonic tics)

Sudden, transitory, often repetitive and stereotypical that may mimic fragments of normal behaviour

Inner urge to make movement, or local premonitory sensation temporarily relieved by the tic

Can be suppressed voluntarily, however patients feel mounting tension inside to perform the tic

Increase with stress, increase at rest! Simple tics and complex tics Negative tics

Tics

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Tics

Causes:

Primary; Tourette syndrome, Huntington disease, Neuroacanthocytosis

Secondary; infections, drugs, toxins, head trauma, stroke

Myoclonus

Sudden, brief, shocklike involuntary movement possibly caused by active muscle contraction (positive myoclonus) or inhibition of ongoing muscle activity (negative myoclonus)

Clinical patterns vary widely; frequency, amplitude, distribution

Usually arrhythmic and irregular, but can sometimes be rhythmic (eg. Oscillatory myoclonus)

Myoclonus

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Myoclonus Causes

Physiological myoclonus (normal subjects)

• Sleep jerks (hypnagogic jerks), anxiety or exercise induced, hiccup

Essential myoclonus (no known cause or neurological deficit)

Sporadic or hereditary

Epileptic myoclonus (seizures dominate and no encephalopathy)

Fragments of epilepsy, childhood myoclonic epilepsies, progressive myoclonic epilepsies

Symptomatic myoclonus (progressive or static encephalopathy

dominates) Storage diseases, spinocerebellar degeneration, basal ganglia degeneration, mitochondrial

encephalopathies, dementias, viral encephalopathies, metabolic, toxic, focal CSN damage

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Others

Restless leg syndrome; 14% of women and 7% of men older than 50.

Psychogenic movement disorder; Common Clues are:

Somatic and psychiatric complaints Movement disorders not categorizable or typical of a certain class or showing a

combination of many classes Sudden onset(often in relation to physical or emotional trauma) Secondary gain Variable frequency of tremor Distractibility Exaggeration of symptoms

Final note

Always, when applicable, rule out Wilsons Disease!