Abstract

Liver transplantation for neuropsychiatric Wilson Disease

Although neuropsychiatric manifestations are prominent in some patients with Wilson disease, there is little published information regarding the efficacy of liver transplantation for these patients. A 22-year-old male with advanced neurological impairment and prominent psychiatric manifestations due to Wilson disease who underwent liver transplantation is presented. After transplantation, the ceruloplasmin and copper studies normalized and eventually the Kayser-Fleischer rings disappeared. Neurological recovery was very slow and incomplete, and his behavioural and personality disorder was entirely unaffected. He committed suicide 43 months post-transplantation. A review of the small number of related published cases in the English language literature shows variable neurological recovery post-transplantation, but the course of psychiatric manifestations is virtually never described. This case suggests that one must be cautious regarding liver transplantation for Wilson disease in patients with prior psychiatric manifestations. Aggressive medical management is likely to be preferable in most cases.