Paroxysmal Non-Kinesigenic Choreoathetosis

Abstract

Paroxysmal nonkinesigenic (dystonic) choreoathetosis (PNCA) is a rare disordercharacterised by choreoathetosic, dystonic movements which are seen spontaneously inparoxysmal character. PNCA differs from paroxysmal kinesigenic choreoathetosis (PKCA) by its younger age of onset, less frequent attacks and poor response to medical therapy. In contrast to PKCA, PNCA is nor provoked by sudden movements. We describe two patients with their clinical findings and their response to anticonvulsant therapy diagnosed as PNCA. In both patients paroxysms were noted in early infancy. Their motor and mental development was normal. Attacs, which were observed by awakening, excitement or at rest, continued 2 to 5 minutes and occured with a frequency of 1 to 4 times a day. EEGs and cerebral MRI were normal in both cases. Trials of mono and combined therapies with L-Dopa, carbamazepine, clonazepam, phenytoin and vigabatrin revealed no benefit.