Acquired Hepatocerebral Degeneration Presenting with Downbeat Nystagmus and Ataxia: A Case Report.
- Author:
Chang Min LEE
1
;
Ho Jun YU
;
Jeong Am RYU
;
Jae Il KIM
Author Information
1. Department of Neurology, Dankook University College of Medicine, Cheonan, Korea. nrdoc@dku.edu
- Publication Type:Case Report
- Keywords:
Acquired hepatocerebral degeneration;
Downbeat nystagmus;
Ataxia
- MeSH:
Ataxia*;
Atrophy;
Brain;
Esophageal and Gastric Varices;
Extremities;
Female;
Gait Ataxia;
Hepatolenticular Degeneration*;
Humans;
Korea;
Liver Cirrhosis, Biliary;
Magnetic Resonance Imaging;
Mesencephalon;
Middle Aged
- From:Journal of the Korean Neurological Association
2007;25(1):101-104
- CountryRepublic of Korea
- Language:Korean
-
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.
