title: An unusual case of Wilson's disease.
abstract: 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.
authors.forename: J W
authors.initials: JW
authors.lastname: Madden
authors.forename: J W
authors.initials: JW
authors.lastname: Ironside
authors.forename: D R
authors.initials: DR
authors.lastname: Triger
authors.forename: J P
authors.initials: JP
authors.lastname: Bradshaw
mesh_terms: D001921:Brain / Q000473:pathology*; D006528:Carcinoma, Hepatocellular / Q000150:complications; D003300:Copper / Q000032:analysis; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000150:complications; D008297:Male; D008875:Middle Aged
chemical_list: D003300:Copper