Correlation of neurological manifestations and MR images in a patient with Wilson's disease after liver transplantation

Acta Neurol Scand. 2000 Aug;102(2):135-9. doi: 10.1034/j.1600-0404.2000.102002135.x.


Orthotopic liver transplantation (OLT) has been applied to patients with Wilson's disease (WD) for correction of irreversible liver cirrhosis. However, the neurological outcome and the correlation between clinical manifestations and neuroimage findings after OLT remain uncertain. We present a WD patient who showed an improvement in both liver functions and neurological manifestations after OLT. Serum levels of ceruloplasmin and copper returned to normal rapidly after the operation. His ataxic gait was improved 5 months later and dysmetria and tremor disappeared 11 months later. The high signal intensities on T2-weighted brain magnetic resonance images regressed at bilateral thalami 5 months later and disappeared in bilateral thalami and red nuclei 16 months after OLT. We conclude that the neurological improvement could be expected in WD patients after OLT. The improvement was correlated with the MRI changes in red nuclei and bilateral thalami.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / pathology*
  • Dyskinesias / etiology*
  • Dyskinesias / pathology
  • Dyskinesias / physiopathology
  • Dyskinesias / therapy*
  • Hepatolenticular Degeneration / complications
  • Hepatolenticular Degeneration / pathology
  • Hepatolenticular Degeneration / physiopathology
  • Hepatolenticular Degeneration / surgery*
  • Humans
  • Liver Function Tests
  • Liver Transplantation*
  • Magnetic Resonance Imaging
  • Male
  • Recovery of Function
  • Red Nucleus / pathology
  • Thalamus / pathology
  • Treatment Outcome