Paroxysmal kinesigenic choreoathetosis: a report of 26 patients

Paroxysmal kinesigenic choreoathetosis (PKC) is a neurological condition wh ich results in abnormal involuntary movements that are precipitated by sudd en movement. Because of its rarity, large case series of PKC have not been published. We studied 26 patients with PKC, which represents the largest se ries thus reported. We reviewed our cases with respect to attack characteri stics, aetiology, family history, and treatment response. Our population co nsisted of 23 men and 3 women. Seven patients had a family history of parox ysmal dyskinesia. None of our patients had clear evidence of symptomatic PK C. Two-thirds of our patients had attacks lasting between 30-60 s, and over one-half experienced one to ten attacks per day. Attack distribution varie d widely, and most experienced pure dystonia rather than choreodystonic mov ements. Most patients responded very well to anticonvulsant therapy. We als o report the PET results from two of our patients and Bereitschaftspotentia l abnormalities recorded from two others.