Craniocervical Segmental Dystonia in the Spinocerebellar Ataxia Type 2

J Korean Neurol Assoc > Volume 25(2); 2007 > Article

Journal of the Korean Neurological Association 2007;25(2): 232-235.

두경부 분절성 근긴장 이상증을 보인 제2형 척수 소뇌성 실조증 2예

노지훈, 고성범 김지현 이대희 박건우

고려대학교 의과대학 신경과학교실

Craniocervical Segmental Dystonia in the Spinocerebellar Ataxia Type 2

Jee Hoon Roh

Department of Neurology, Korea University College of Medicine, Seoul, Korea

Abstract

The spinocerebellar ataxia type 2 (SCA 2) is an autosomal dominant cerebellar ataxia that commonly presents with cerebellar ataxia, hyporeflexia, and slow saccades. Recent clinical series described movement disorder in the SCA 2 such as Parkinsonism or dystonia. Dystonia can be observed in and even be the presenting feature of the SCA 2. We report two patients with genetically confirmed SCA 2 displaying a slowly progressive syndrome combined with cerebellar ataxia and craniocervical segmental dystonia. KeyWords:Spinocerebellar ataxia type 2, Carniocervical segmental dystonia