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KMID : 0359720070250010101
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Journal of the Korean Neurological Association
2007 Volume.25 No. 1 p.101 ~ p.104
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Acquired Hepatocerebral Degeneration Presenting with Downbeat Nystagmus and Ataxia: A Case Report
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Lee Chang-Min
Yu Ho-Jun
Ryu Jeong-Am
Kim Jae-Il
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Abstract
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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.
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KEYWORD
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Acquired hepatocerebral degeneration, Downbeat nystagmus, Ataxia
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