Movement Disorders 4335

8/11/2019 Movement Disorders 4335

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Movement disorders** Except Parkinsonism and tremor

Submitted toAskTheNeurologist.Com

in 2007

Author Anon.
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What is a Movement disorder?

A disorder which impairs the regulation of voluntary motor

activity without directly affecting strength, sensation or cerebellar

function

Sometimes also known as extrapyramidal disorders

Many neurologists restrict the term extrapyramidal to refer only

to Parkinsonism
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Types of abnormal movement

Myoclonus

Ballismus

Chorea

Athetosis

Dystonia

Tics

Tremor ( discussed elsewhere )
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Myoclonus

sudden, brief, shock-like involuntary movements

May be caused by active muscle contraction

- positive myoclonus

May be caused by inhibition of ongoing muscle activity

- negative myoclonus ( eg. Asterixis )

Generalised - widespread throughout body

Focal / segmentalrestricted to particular part of body
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Generalised myoclonus

Physiologic - Nocturnal ( usually on falling asleep )- Hiccups

Essential - Occurs in the absence of other

abnormality- Benign and sometimes inherited

Epileptic - Demonstrable cortical source Symptomatic i.e secondary to disease process

- Neurodegenerative eg. Wilsons disease

- Infectious e.g CJD, Viral encephalitis- Toxic e.g. penicillin, antidepressants

- Metabolic - anoxic brain damage

- hypoglycemia

- hepatic failure ( asterixis )
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Segmental Myoclonus

Can arise from lesions anywhere in theCNS

Can arise from many of the processes thatcan cause generalised myoclonus

Example is palatal myoclonus which

arises due to any lesion that interruptspathway between red nucleus,

cerebellum and olivary nucleus.


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Treatment of Myoclonus

Valproic acid is drug of choice

May respond to benzodiazepinese.g. clonazepam
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Ballismus, Chorea, Athotosis

and DystoniaThese should NOT be thought of asseparate entities amenable to specific

definition but rather as a SPECTRUM ofmovements that blend into one-another

WHY?
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Because..

They often co-exist Even neurologists may often not be

able to agree as to how a particularmovement should be classified!

They often ( with some notable

exceptions ) have the samesignificance in terms of aetiology.
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The spectrum

Ballismus DystoniaChorea Athetosis

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
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Ballismus

Violent flinging movement of entire limb Almost always unilateral and therefore

use term HEMIBALLISMUS Involves proximal musculature and is

sometimes thought of as a

proximal unilateral chorea Usually due to a CVA in contralateralsubthalamic nucleus
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Chorea ( dance in Greek)

Rapid irregular muscle jerks May affect limbs, head, face and tongue

In the limbs chorea refers more to distalmovements ( as proximal movements usuallycalled ballismus)

Patients often attempt to conceal involuntarymovements by superimposing voluntarymovements onto them e.g. an involuntarymovement of arm towards face may be adapted

to look-like an attempt to look at watch
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Athetosis changeable in Greek

Slow, flowing, often twistingmovements

Occurs mainly distally ( hands, fingers) Can also affect face and tongue Often use term choreoathetosis due

to overlap between syndromes ( choreareferring to less smooth , more jerkymovements)
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Dystonia

condition in which the patient assumes a sustained,abnormal posture or limb position

Due to co-contraction of agonist and antagonist

muscles in part of body Can be thought of as an athetoid movement that

gets stuck for a period of time; thus, a patient withchoreoathetosis may perform an involuntarymovement in which his hand and fingers are twisted

behind his head. He may hold this position for a fewmoments before his hand moves back in front of hisbody.

The part of the movement when the limb was held,

unmoving, in an abnormal position would beconsidered a dystonia ( may occur alone).
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Examples of focal dystonias

Blepharospasm:- involuntary forceful closure of eyes

Torticollis:- Tendency of neck to twist to one side.

Writers cramp:

- Dystonic posturing of arm when handused to perform specific tasks e.g.writing, playing piano


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Botulinum toxin

Derived from Clostridium botulinum Prevents pre-synaptic release of Ach

vesicles and therefore blocks neuro-muscular junction transmission

May be injected selectively into musclesresponsible for focal dystonias, allowingrelief for up to several months

Requires expertise; can cause moreextensive paralysis than intended.


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Causes of Chorea, Dystonia and

athetosis Hereditary Static Encephalopathy ( Cerebral Palsy ) Drugs

Cerebrovascular ( ischaemia, haemorrhage ) Structural lesions ( subthalamic nucleus) Secondary to medical disorders Miscellaneous - Sydenhams chorea

- Chorea Gravidarum

- Sporadic idiopathic torsiondystonia

- Focal dystonias
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Hereditary

Huntingtons disease

Wilsons disease

Neuroacanthocytosis

Hereditary dystonias - idiopathic torsion dystonia- dopa - responsive dystonia


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Wilsons disease

Autosomal recessive defect of copper excretion in which there isdefective copper-binding to ceruloplasmin

Leads to copper deposition in:- liver causing cirrhosis

- brain ( especially basal ganglia )leading to movement disorders andother abnormalities

- Cornea leading to the appearance of rustybrown Kayser-Fleischer rings aroundcornea (usually only seen with slit-lamp).

Diagnosis is via low serum ceruloplasmin, increased urinary Cu,liver biopsy ( excessive Cu) and brain MRI changes

Although it is rare it is very important to think-of and diagnoseas it is TREATABLE:- Penicillamine is given to chelate the copper

and promote excretion.
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Idiopathic torsion dystonia

Hereditary and sporadic formsVariable inheritence

High incidence in Ashkenazi Jews Onset may be in childhood / adulthoodAffects limbs, trunk, neck, face Trial of L-dopa usually initiated Level of disability variable


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Dopa-responsive dystonia

Autosomal dominant inheritence

Onset usually in childhood Girls more severely affected Some parkinsonian features - rigidity

- bradykinesia

Low levels of L-Dopa very effective


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Drug-induced

Drugs acting on dopaminergic system- Antiparkinsonian

- Antipsychotic Lithium

Phenytoin

Oral contraceptives
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Anoxic brain damage ( postCPR ) Systemic lupus erythematosis

Hepatic failure Endocrine - Thyrotoxicosis

- Addisons

Electrolyte - Low Ca, Mg,- High Na

Polycythemia rubra vera

Secondary to medical disorders(A SHEEP)
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Sydenhams chorea

Mainly children / adolescents Complication of previous group A

streptococcal infection Usually no recent history of infectionAcute / subacute onset

May have behavioural problems Usually remits spontaneously


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Chorea gravidarum

Chorea of any cause that begins inpregnancy

May represent recurrence ofSydenhams chorea.

Most commonly associated with anti-

phospholipid syndrome +/- SLE Usually resolves spontaneously


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Tics

Recurrent, sterotyped abnormal movements

May be suppressed voluntarily or withdistraction

Voluntary suppression leads to anxiety and a

build-up of internal unrest.

Worsen under stress
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Summary

Movement disorders are often difficult to define precisely, buthave similar differential diagnoses.

They are often a manifestation of a more widespreadneurological or internal medical problem.

Other than the specific treatments mentioned, most details oftherapy are beyond the scope of this course.

In some cases treatment includes treatment of underlyingcause e.g. Wilsons disease
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Movement Disorders 4335