Liver transplantation for Wilson disease: review and a case report of an unexpected neurological complication

Abstract

Wilson’s disease is a rare autosomal recessive inherited disorder of copper metabolism, which results in copper accumulation in several tissues, especially with liver injury and failure. Orthotopic liver transplantation (OLT) can be lifesaving for patients with hepatic complications of Wilson’s disease- fulminant liver failure or unresponsiveness to medical therapy in chronic liver disease, with or without neurological manifestations. We report the case of a 19-year-old woman receiving a liver transplant for acute liver failure caused by Wilson’s disease, who developed headache as the only symptom of a serious neurological complication after transplantation. The clinical course and neuroimaging demonstrating aneurismal subarachnoid hemorrhage are presented. The neurological complications and the difficulties of medical management in an immunosuppressed patient are discussed. Headache in a recently transplanted patient for acute liver failure may be a sign of a serious cerebral complication, subarachnoid hemorrhage. Early recognition and proper management may prevent life-threatening neurologic complications but chronic immunosuppression may impede a favourable outcome.

Keywords

• wilson’s disease
• acute liver failure
• liver transplantation
• subarachnoid hemorrhage