Effect of Liver Transplant on Neurologic Manifestations and Brain Magnetic Resonance Imaging Findings in Wilson Disease.

Abstract

Wilson disease is an autosomal recessive disorder of copper metabolism. It leads to copper accumulation in various organs (liver, eye, brain) and deteriorates their functions. Symptoms usually appear in the second and third decades of life. Neurologic symptoms and manifestations may appear 2 to 5 years after liver involvement, and neurologic symptoms are usually movement disorders. The main treatment objective is to decrease accumulation of copper by increasing urinary copper excretion. With early diagnosis and treatment, the quality of life of patients with Wilson disease evolves. In this study, we aimed to evaluate the effects of liver transplant on neurologic manifestations and radiologic findings in patients with Wilson disease. Since 1988, our center has performed 642 liver transplant procedures. Fifty-three patients with Wilson disease received a liver transplant during this period, with 15 adults patients included in our study. All study patients were evaluated by the same neurologist and radiologist. Tremor was scored by the glass scale test. Radiologic evaluations were made by cranial magnetic resonance imaging. Before liver transplant, 4/15 study patients had tremor. In 1 patient, tremor was accompanied by dystonia; the patient's imaging findings and neurologic manifestations had regressed posttransplant. In the other 3 study patients with tremor, tremor decreased without any change in imaging findings. New-onset tremor was seen in 1 patient after liver transplant, but this patient had no observed imaging changes. This situation was correlated with immunosuppressive therapy. Neurologic recovery can be achieved in patients with Wilson disease with early diagnosis and treatment. Radiologic findings can be improved after therapy.