A Case of Hereditary Progressive Dystonia With Diurnal Fluctuation

J Korean Neurol Assoc > Volume 8(2); 1990 > Article

Journal of the Korean Neurological Association 1990;8(2): 344-348.

증상의 일중 변화를 보이는 Hereditary Progressive Dystonia 1 예

김동훈, 나상옥,황선출,최문성,박규현,김상욱,정대수

메리놀병원 신경과. 부산대학교 신경과. 춘해병원 신경과.

A Case of Hereditary Progressive Dystonia With Diurnal Fluctuation

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

Dep. Of Neurology, Maryknoll Hospital

Abstract

Hereditary progressive dystonia develope in the first decade of ?life and is characterized by marked diurnal fluctuation of symptoms(dystonic postures and movements),? i.e.symptoms aggrevated towards evening and alleviated after sleep. These symptoms and signs responded dramatically to levodopa. ?With the high incidence of familial cases this disorder is considers to be hereditary, but ?inheritance is not clear yet. Segawa regarded this disorder as a dystonia different from Parkinson's disease and ?other types of dystonic movement disorders, thereafter it has been reported under name of ?hereditary progressive dystonia with marked diurnal fluctuation.