A Case of Paroxysmal Dystonic Choreoathetosis

J Korean Neurol Assoc > Volume 9(1); 1991 > Article

Journal of the Korean Neurological Association 1991;9(1): 107-111.

가족력이 있는 Paroxysmal Dystonic Choreoathetosis 1예

나상옥, 황선출,김동훈.최문성,박규현,김상욱

메리놀병원 신경과. 부산대학교 신경과.

A Case of Paroxysmal Dystonic Choreoathetosis

Sang Ok Ra, M.D., Seon Chool Hwang, M.D., Dong Hun Kim, M.D., Mun Sung Choi, M.D. Kyu Hyun Park, M.D.*, Sang Wook Kim, M.D.*

Dep. Of Neurology, Maryknoll Hospital Dep. Of Neurology, Busan National University*

Abstract

Paroxysmal choreoathetosis(dyskinesia) is classified into two subtypes: paroxysmal kinesigenic choreoathetosis(PKC) and paroxysmal dystonic choreoathetosis(PDC). PDC consist of attacks of dystonia and /or choreoathetosis during which the patients are dysarthric or anarthric, have irregular clonic movement and dystonic posturing of extremities, and these not precipitated by sudden movement but rather by alocohol, coffee and stress. Attacks are longer (2ninute-4hours), but less frequently(3-4times/day) than PKC, they are not responsive to anticonvulsants(eg, phenytoin, carbamazepine) but controlled by clonazepam.This disease is rare, in the reported families, the transmission was clearly autosomal dominant with high penetrance.