[Eleven cases of paroxysmal kinesigenic choreoathetosis; correlation with benign infantile convulsions]

We report 11 subjects, consisting of sibling cases from 5 unrelated families, with paroxysmal kinesigenic choreoathetosis (PKC). In these subjects the PKC attacks started between the ages of 5 and 17 years and were well controlled with anti-epileptic drugs or subsided spontaneously. In 8 cases from 4 families with PKC, infantile convulsions occurred between the ages of 3 and 8 months and an excellent prognosis was obtained in all but one male. Four of the 8 subjects had complex partial seizures which were characterized by staring, eye deviation, apnea, or loss of consciousness. The nature of these convulsions shared some of the clinical features of benign infantile epilepsies, which have been recently advocated as a new category of epilepsy. There were no differences in the clinical between the cases with and without infantile convulsions.