Abstract
The paroxysmal dyskinesias are a heterogeneous group of rare movement disorders, characterized by the abrupt onset of involuntary hyperkinetic movements with or without trigger factors and of variable duration. Interictal periods are marked by relative normality, although there is evidence for an association between some genotypes and migraine, episodic ataxia, and seizure disorders. Three genes have been identified that are associated with the three most common syndromes; however, these do not account for some cases with an otherwise typical history. The clinical phenotype continues to evolve with increasing characterization of genetically proven cases. Paroxysmal kinesigenic dyskinesia responds well to carbamazepine therapy.
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