Treatment of Paroxysmal Kinesigenic Dyskinesias

Abstract

Paroxysmal kinesigenic dyskinesia (PKD) is characterized by very brief (<60 s) but frequent (up to 100 times daily) attacks of dystonia and chorea triggered by sudden voluntary movements. PKD can be primary (idiopathic) or secondary (symptomatic); the former is associated with mutations in the gene encoding the proline-rich transmembrane protein 2 (PRRT2). Episodes are not painful, consciousness is always intact and between attacks patients are usually normal. PKD occurs isolated or in combination with other episodic disorders, namely, epilepsy and migraine, when associated with PRRT2 gene. Treatment efficacy and outcome depend on the aetiology of PKD. Idiopathic PKD can be successfully treated with low doses of carbamazepine, but several other antiepileptic drugs have also been described to be effective. In “symptomatic” PKD, treatment strategies should first target the underlying causative process.