Event-related brain potentials in paroxysmal kinesigenic choreoathetosis

Event-related brain potentials (ERPs) were recorded in a 30 year old female patient with familial kinesigenic choreoathetosis and a group of control subjects. In line with previous observations the patient exhibited an enhanced amplitude of the early component of the contingent negative variation of the ERP in a two stimulus paradigm. Moreover, a greatly enhanced P3 amplitude was found in auditory and visual classification (oddball) paradigms. These results suggest a general upregulation of widespread subcortical projection systems leading to both, abnormal movements and abnormally high amplitudes of ERPs.