일치 내사시를 동반한 치아적핵담창구루이체위축증
(DRPLA)

J Korean Neurol Assoc > Volume 27(4); 2009 > Article

Journal of the Korean Neurological Association 2009;27(4): 428-431.

Dentatorubropallidoluysian Atrophy (DRPLA) With Comitant Esotropia

유재국, 구용서 권도영 박문호 박건우

고려대학교 의과대학 신경과학교실

일치 내사시를 동반한 치아적핵담창구루이체위축증 (DRPLA)

Jae-Kook Yoo

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

Abstract

The possibility of a central origin should be considered for late-onset concomitant esotropia. Concomitant esotropia has been reported to occur with spinocerebellar ataxia types 1, 2, and 3, but not with other degenerative cerebellar ataxia disorders. We report on a 28-year-old woman with ataxia in whom a detailed ophthalmologic examination revealed concomitant esotropia. She was subsequently diagnosed with dentatorubropallidoluysian atrophy (DRPLA). We suggest that the presence of concomitant esotropia could be used to differentiate DRPLA from other hereditary ataxias. Key Words: Dentatorubropallidoluysian atrophy (DRPLA), Concomitant esotropia