Progression of neurovisceral storage disease with supranuclear ophthalmoplegia following orthotopic liver transplantation.
A 7-year-old girl with progressive ataxia, spasticity, supranuclear ophthalmoplegia, and sea-blue histiocytes in her bone marrow underwent orthotopic liver transplantation for hepatocellular carcinoma. After an initial period of stabilization, she has shown progression of neurologic symptoms with recurrence of storage material in the transplanted liver.
D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D008064:Lipidoses / Q000150:complications*; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery; D016031:Liver Transplantation; D009886:Ophthalmoplegia / Q000150:complications; D012008:Recurrence