Paroxysmal kinesigenic choreoathetosis in Singapore and its relationship to epilepsy

To study the clinical characteristics of paroxysmal kinesigenic choreoathetosis (PKC) in our local population and its relationship to epilepsy.

We reviewed retrospectively 15 patients who were managed by neurologists in our department from 1982 to 1996. The literature was also reviewed to study the association between PKC and epilepsy.

In our study, all the cases were idiopathic. The male to female ratio was 14:1 with all major races represented. Sixty percent of our patients suffered dystonic posturing rather than chorea, during the attacks. Twenty-one percent had a family history of a similar disorder which appeared to be of autosomal dominant inheritance. The sporadic form (79%) predominated in Singapore. One had a history of febrile fits while two had a history of epilepsy. We reviewed the available literature and found five other patients with idiopathic PKC also suffering from epilepsy. Of the 83 patients reviewed, 8% had epilepsy. This further strengthens the relationship between the two conditions. All our patients responded well to phenytoin at doses between 100 and 400 mg/day.

PKC affected all three major races in our population with a high male to female ratio of 14:1. Seventy-nine percent of our cases were sporadic and 60% suffered dystonic posturing during attacks. Of the cases reviewed, 8% of patients with idiopathic PKC also had epilepsy.