Paroxysmal kinesigenic dyskinesia is a neurological disorder characterized by brief episodes of dystonia and choreathetosis triggered by sudden voluntary movement. The attacks typically last less than one minute and may occur even several times a day.
Besides familiar idiopathic forms with autosomal dominant inheritance, sporadic cases may occur, or symptomatic forms may be associated with some CNS diseases and may manifest with the same type of paroxysms. At the beginning we review current knowledge on this disorder, including the historical development of the concept of paroxysmal dyskinesia.
Then we present the case of a young woman who has suffered from sporadic idiopathic paroxysmal kinesigenic dyskinesia since she was 13 years old. Diagnosis was proved during video-EEG monitoring and her paroxysms can be characterised as alternating hemidystonia.
She has been successfully treated with 300mg of lamotrigine per day.