Chronic acquired hepatocerebral degeneration presenting with Meige’s syndrome and behavioral symptoms fully reversed by liver transplantation

NEUROLOGICAL SCIENCES(2021)

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摘要
Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of movement disorders and cognitive\behavioral changes. Its pathogenesis is debated and the symptoms are poorly responsive to medical therapy. Meige’s syndrome is a form of cranial dystonia, considered an idiopathic form of adult onset dystonia. We report a 60-year-old man with HCV-related liver cirrhosis and hepatocarcinoma who developed Meige’s syndrome associated with cognitive and behavioral manifestations, unrelated to acute metabolic derangement. CAHD was diagnosed. Liver transplantation reversed the clinical picture and MR abnormalities, reinforcing the idea that CAHD is a potentially reversible syndrome, which may be healed by liver transplantation and should not be considered a contraindication for this operation.
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关键词
Acquired hepatocerebral degeneration,Movement disorders,Cognitive impairment,Meige’s syndrome,Psychosis,Liver transplantation
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