Acquired Hepatocerebral Degeneration Presenting with Downbeat Nystagmus and Ataxia: A Case Report

J Korean Neurol Assoc > Volume 25(1); 2007 > Article

Journal of the Korean Neurological Association 2007;25(1): 101-104.

하향안진과 운동실조를 보인 후천성 간뇌변성

이창민, 유호준 유정암 김재일

단국대학교 의과대학 신경과학교실

Acquired Hepatocerebral Degeneration Presenting with Downbeat Nystagmus and Ataxia: A Case Report

Chang-Min Lee

Department of Neurology, Dankook University College of Medicine, Cheonan, Korea

Abstract

Nystagmus or ataxia is a rare manifestation of acquired hepatocerebral degeneration (AHCD). A 49-year-old woman presented with downbeat nystagmus and limb and gait ataxia. She was diagnosed as primary biliary cirrhosis with a gastric varix. Brain MRI showed cerebellar vermian atrophy and characteristic T1 high-signal intensities in bilateral globus pallidi and ventral midbrain. We report a rare case of AHCD manifesting prominent cerebellar symptoms. This has not yet been reported in Korea.KeyWords:Acquired hepatocerebral degeneration, Downbeat nystagmus, Ataxia