Hepatorenal syndrome, hepatopulmonary syndrome, and now, hepatospinal syndrome?

Background & Aims: Hepatic myelopathy is a rare complication of chronic liver disease, causing progressive spastic paraparesis. Today, no therapy of this disorder has been established. Commonly used therapeutic strategies for hepatic encephalopathy aiming at the reduction of plasma ammonia levels such as protein restriction, oral neomycin, lactulose, or ornithine aspartate fail to improve the symptoms of hepatic myelopathy. The aim of this study was to find out whether orthotopic liver transplantation (OLT) may improve hepatic myelopathy. Methods: Follow-up examinations of 3 patients with severe hepatic myelopathy before and after OLT. Results: In all 3 patients, the neurologic status improved significantly after liver transplantation. The grade of improvement was related to the time interval between onset of the first symptoms of hepatic myelopathy and liver transplantation. Conclusions: Early recognition of hepatic myelopathy is important because timely liver transplantation as an established therapy for end-stage liver disease offers the chance of complete recovery from hepatic paraparesis.