PAROXYSMAL KINESIGENIC CHOREOATHETOSIS IN SINGAPORE AND ITS RELATIONSHIP TO EPILEPSY

Objectives: To study the clinical characteristics of paroxysmal kinesi genic choreoathetosis (PKC) in our local population and its relationsh ip to epilepsy. Methods: We reviewed retrospectively 15 patients who w ere managed by neurologists in our department from 1982 to 1996. The l iterature was also reviewed to study the association between PKC and e pilepsy. Results: In our study, all the cases were idiopathic. The mal e to female ratio was 14:1 with all major races represented. Sixty per cent of our patients suffered dystonic posturing rather than chorea, d uring the attacks. Twenty-one percent had a family history of a simila r disorder which appeared to be of autosomal dominant inheritance. The sporadic form (79%) predominated in Singapore. One had a history of f ebrile fits while two had a history of epilepsy. We reviewed the avail able 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 an d 400 mg/day. Conclusion: PKC affected all three major races in our po pulation with a high male to female ratio of 14:1. Seventy-nine percen t of our cases were sporadic and 60% suffered dystonic posturing durin g attacks. Of the cases reviewed, 8% of patients with idiopathic PKC a lso had epilepsy. (C) 1998 Elsevier Science B.V. All rights reserved.