Effect of liver transplantation on neurological manifestations in Wilson disease.
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.
Stracciari
A
A
Neurological Service, S. Orsola-Malpighi Hospital, Bologna, Italy. neuro@orsola-malpighi.med.unibo.it
Tempestini
A
A
Borghi
A
A
Guarino
M
M
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