Effect of liver transplantation on neurological manifestations in Wilson disease

Arch Neurol. 2000 Mar;57(3):384-6. doi: 10.1001/archneur.57.3.384.


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.

Observation: 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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / pathology
  • Cognition Disorders / etiology
  • Cognition Disorders / therapy*
  • Hepatolenticular Degeneration / complications
  • Hepatolenticular Degeneration / therapy*
  • Humans
  • Liver Transplantation*
  • Magnetic Resonance Imaging
  • Male
  • Motor Skills