In Wilson's disease, liver transplantation can constitute the only option for patients presenting with fulminant hepatic failure or decompensated liver disease unresponsive to drug therapy. We report the case of a 29-year-old woman receiving a liver transplant for end-stage Wilson's disease who developed neurological complications after transplantation. After an accurate evaluation of possible differential causes of neurological complications developing as the result of liver transplantation, moyamoya disease was diagnosed. Moyamoya disease is a rare cerebrovascular disease of unknown etiology. However, data exist supporting a possible role for some immunosuppressive regimens in determining the peculiar vascular alterations observed in moyamoya disease. To the best of our knowledge, the association with post-transplantation state for Wilson's disease has not been previously described.
© 2011 The Japan Society of Hepatology.