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Detailed information of FAMILIAL PAROXYSMAL CHOREOATHETOSIS
FAMILIAL PAROXYSMAL CHOREOATHETOSIS
DEFINITION:
A rare movement disorder characterized by episodes of chorea,
dystonia, or tonic posturing provoked by sudden physical activity.
EPIDEMIOLOGY:
- incidence: rare
- age of onset:
- 1st and 2nd decades of life
- risk factors:
- familial - autosomal dominant and sporadic forms
CLINICAL FEATURES:
- episodes of chorea, dystonia, or tonic posturing provoked by
- sudden physical activity
- hyperventilation
- profound emotions
- stress
- episode characteristics
- usually last from 2-5 minutes (but can last for hours)
- followed by an absolute refractory period for 5-20 minutes
- bilateral or unilateral
- not associated with loss of consciousness
- frequency varies from few episodes/year to 100's/day
- in some pateints associated with a sensory prodrome
- numbness or tingling of face or limbs prior to attack
INVESTIGATIONS:
- all tests (CT, MRI, EEG, etc) are normal
MANAGEMENT:
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Pediatric Database - FAMILIAL PAROXYSMAL CHOREOATHETOSIS
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