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Movement disorders*

* Except Parkinsonism and tremor


Submitted to
AskTheNeurologist.Com
in 2007
Author Anon.
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
Types of abnormal movement
Myoclonus
Ballismus
Chorea
Athetosis
Dystonia
Tics
Tremor ( discussed elsewhere )
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 / segmental restricted to particular part of body
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 )

- renal failure

- hyponatremia.. And others
Segmental Myoclonus
Can arise from lesions anywhere in the
CNS
Can arise from many of the processes that
can cause generalised myoclonus
Example is palatal myoclonus which
arises due to any lesion that interrupts
pathway between red nucleus,
cerebellum and olivary nucleus.
Treatment of Myoclonus
Valproic acid is drug of choice


May respond to benzodiazepines
e.g. clonazepam
Ballismus, Chorea, Athotosis
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



WHY?
Because..
They often co-exist
Even neurologists may often not be
able to agree as to how a particular
movement should be classified!
They often ( with some notable
exceptions ) have the same
significance in terms of aetiology.
The spectrum
Ballismus Dystonia Chorea 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
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 contralateral
subthalamic nucleus
Chorea ( dance in Greek)
Rapid irregular muscle jerks
May affect limbs, head, face and tongue

In the limbs chorea refers more to distal
movements ( as proximal movements usually
called ballismus)

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
Athetosis changeable in Greek
Slow, flowing, often twisting
movements
Occurs mainly distally ( hands, fingers)
Can also affect face and tongue
Often use term choreoathetosis due
to overlap between syndromes ( chorea
referring to less smooth , more jerky
movements)
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 with
choreoathetosis may perform an involuntary
movement in which his hand and fingers are twisted
behind his head. He may hold this position for a few
moments before his hand moves back in front of his
body.
The part of the movement when the limb was held,
unmoving, in an abnormal position would be
considered a dystonia ( may occur alone).
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 hand
used to perform specific tasks e.g.
writing, playing piano
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 muscles
responsible for focal dystonias, allowing
relief for up to several months
Requires expertise; can cause more
extensive paralysis than intended.
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 torsion
dystonia
- Focal dystonias
Hereditary
Huntingtons disease

Wilsons disease

Neuroacanthocytosis

Hereditary dystonias - idiopathic torsion dystonia
- dopa - responsive dystonia

Wilsons disease
Autosomal recessive defect of copper excretion in which there is
defective copper-binding to ceruloplasmin
Leads to copper deposition in:
- liver causing cirrhosis
- brain ( especially basal ganglia )
leading to movement disorders and
other abnormalities
- Cornea leading to the appearance of rusty
brown Kayser-Fleischer rings around
cornea (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 diagnose
as it is TREATABLE:- Penicillamine is given to chelate the copper
and promote excretion.
Idiopathic torsion dystonia
Hereditary and sporadic forms
Variable inheritence
High incidence in Ashkenazi Jews
Onset may be in childhood / adulthood
Affects limbs, trunk, neck, face
Trial of L-dopa usually initiated
Level of disability variable
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
Drug-induced
Drugs acting on dopaminergic system
- Antiparkinsonian
- Antipsychotic
Lithium

Phenytoin

Oral contraceptives
Anoxic brain damage ( post CPR )
Systemic lupus erythematosis
Hepatic failure
Endocrine - Thyrotoxicosis
- Addisons
Electrolyte - Low Ca, Mg,
- High Na
Polycythemia rubra vera
Secondary to medical disorders
(A SHEEP)
Sydenhams chorea
Mainly children / adolescents
Complication of previous group A
streptococcal infection
Usually no recent history of infection
Acute / subacute onset
May have behavioural problems
Usually remits spontaneously
Chorea gravidarum
Chorea of any cause that begins in
pregnancy
May represent recurrence of
Sydenhams chorea.
Most commonly associated with anti-
phospholipid syndrome +/- SLE
Usually resolves spontaneously
Tics
Recurrent, sterotyped abnormal movements

May be suppressed voluntarily or with
distraction

Voluntary suppression leads to anxiety and a
build-up of internal unrest.

Worsen under stress
Summary
Movement disorders are often difficult to define precisely, but
have similar differential diagnoses.

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

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

In some cases treatment includes treatment of underlying
cause e.g. Wilsons disease
Submitted to
AskTheNeurologist.Com
in 2007
Author Anon.

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