Arch Neurol 2000 Mar;57(3):384-6

Effect of liver transplantation on neurological manifestations in Wilson
disease

Stracciari A, Tempestini A, Borghi A, Guarino M

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. 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