Autosomal dominant paroxysmal kinesigenic choreoathetosis. An electroneurophysiological study.

A case of an 11-year-old boy with an autosomal dominant form of paroxysmal kinesigenic choreoathetosis is presented. Routine EEG, sleep EEG recording, and registration of visual evoked potentials and somatosensory evoked potentials were normal. EEG with videomonitoring and registration of event-related potentials, however, showed abnormalities, which are discussed in detail. Our data provide further arguments in support of the hypothesis that paroxysmal kinesigenic choreoathetosis is the expression of a dysbalance in the cortico-striopallidal-thalamic loop, and has an extrapyramidal genesis.