paroxysmal choreoathetosis

Epidemiology

• frequently begins in early adolescence

Genetics

• may occur in several members of a family, or in a single family member
• same gene is also associated with epilepsy

Clinical manifestations

• episodes of involuntary movements of the limbs, trunk, & facial muscles.
• tightening of muscles may occur as a prodrome
• involuntary movements precipitate some attacks
• alcohol, caffeine, tiredness or stress can precipitate episodes
• episodes can last from 10 seconds to over an hour
• lingering muscle tightness may remain after an attack.

Management

• carbamazepine has been very successful in reducing or eliminating attacks of paroxysmal choreoathetosis
• other anticonvulsants have been used with good results
• prognosis
  • generally lessens with age
  • many adults have a complete remission
  • prognosis generally good because pharmaceutical therapy is effective

More general terms

• choreoathetosis