Madden J W, Ironside J W, Triger D R, Bradshaw J P
Q J Med. 1985 Apr;55(216):63-73.
A man of 61 with a 26-year history of progressive cerebellar ataxia was admitted to hospital. He was found to have chronic liver disease and died 22 days after admission. A diagnosis of hepatolenticular degeneration (Wilson's disease) was supported by clinical investigations and confirmed at autopsy, when tissue copper studies were performed. Several unusual features were present, including a unilateral Kayser-Fleischer ring, a hepatocellular carcinoma, peripheral neuropathy, pontine demyelination and calcification of neurones in the medulla. The significance of these findings is discussed with a review of the relevant literature.
一名61岁男性因进行性小脑共济失调26年入院。他被发现患有慢性肝病，入院22天后死亡。临床检查支持肝豆状核变性（威尔逊病）的诊断，并在尸检时通过组织铜研究得以证实。存在一些不寻常的特征，包括单侧凯-弗环、肝细胞癌、周围神经病变、脑桥脱髓鞘和延髓神经元钙化。结合相关文献综述对这些发现的意义进行了讨论。
