No neurological improvement after liver transplantation for Wilson's disease.
Orthotopic liver transplantation (OLT) represents the sole etiological treatment for Wilson's disease (WD), but its efficacy in resolving the neurological symptoms is still a matter of debate. We present the case of a young male affected with WD with hepatic and neurological involvement, who underwent OLT for subacute liver failure. Clinical, neuropsychological and neuroradiological assessment were performed before and after OLT. OLT had no effect on neurological symptoms, suggesting that the basal ganglia damage may be irreversible. Nineteen months after OLT, the patient went on to develop new permanent extrapyramidal symptoms. We postulate a sequela of an early post-operative central pontine and extrapontine myelinolysis.
Guarino
M
M
Department of Neurology, S. Orsola-Malpighi Hospital, Bologna, Italy.
Stracciari
A
A
D'Alessandro
R
R
Pazzaglia
P
P
D000328:Adult; D014150:Antipsychotic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002531:Cerebellum / Q000503:physiopathology; D003072:Cognition Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000503:physiopathology; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007361:Intelligence Tests; D016031:Liver Transplantation; D008297:Male; D009069:Movement Disorders / Q000188:drug therapy / Q000209:etiology; D010868:Pimozide / Q000008:administration & dosage / Q000627:therapeutic use; D011149:Pons / Q000503:physiopathology
D014150:Antipsychotic Agents; D010868:Pimozide