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paroxysmal choreoathetosis
Epidemiology:
- frequently begins in early adolescence
Genetics:
1) may occur in several members of a family, or in a single family member
2) same gene is also associated with epilepsy
Clinical manifestations:
1) episodes of involuntary movements of the limbs, trunk, & facial muscles.
2) tightening of muscles may occur as a prodrome
3) involuntary movements precipitate some attacks
4) alcohol, caffeine, tiredness or stress can precipitate episodes
5) episodes can last from 10 seconds to over an hour
6) lingering muscle tightness may remain after an attack.
Management:
1) carbamazepine has been very successful in reducing or eliminating attacks of paroxysmal choreoathetosis
2) other anticonvulsants have been used with good results
3) prognosis
a) generally lessens with age
b) many adults have a complete remission
c) prognosis generally good because pharmaceutical therapy is effective
General
choreoathetosis
References
- NINDS Paroxysmal Choreoathetosis Disease Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Paroxysmal-Choreoathetosis-Information-Page