title: Effect of liver transplantation on neurological manifestations in Wilson disease.
abstract: BACKGROUND
  Liver transplantation (LT) is the sole resolutive therapy for Wilson disease (WD) and is the treatment of choice for patients with WD who have fulminant hepatic failure or end-stage cirrhosis. Although its role in managing the neurological manifestations of WD is not yet conclusive, LT has recently been advocated as a therapy for neurologically affected patients with WD with stable liver function.
  
  
  OBJECTIVE
  To evaluate the effect of LT on the neurological manifestations of WD.
  
  
  METHODS
  A 44-year-old man with WD with cirrhosis and neurological symptoms (motor dysfunction and cognitive impairment) experienced a dramatic improvement in motor function early after LT, as well as normalization of copper balance and the disappearance of Kayser-Fleischer rings. Abnormalities seen on magnetic resonance imaging scans were reversed 18 months after LT. Cognitive testing 2 years after LT showed a moderate global improvement.
  
  
  CONCLUSIONS
  In this case, LT healed the neurological manifestations of WD. To date, this favorable result has been seen in almost 80% of cases. However, the decision to perform LT in patients with WD solely on the basis of neurological impairment must be considered experimental.
authors.affiliation: Neurological Service, S. Orsola-Malpighi Hospital, Bologna, Italy. neuro@orsola-malpighi.med.unibo.it
authors.forename: A
authors.initials: A
authors.lastname: Stracciari
authors.forename: A
authors.initials: A
authors.lastname: Tempestini
authors.forename: A
authors.initials: A
authors.lastname: Borghi
authors.forename: M
authors.initials: M
authors.lastname: Guarino
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000209:etiology / Q000628:therapy*; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000628:therapy*; D006801:Humans; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D009048:Motor Skills