Cerebral manifestation of Wilson's disease successfully treated with liver transplantation.
The main indication for orthotopic liver transplantation (OLTx) in Wilson's disease (WD) is severe hepatic decompensation. Our 15-year-old patient is the second case to date in whom OLTx was performed because of neurologic manifestations resulting from WD. His initial condition involving recurrent headaches, tremor, and athetoid hand movements progressively deteriorated during therapy with D-penicillamine, zinc sulfate, and trientine until he was severely dysarthric, unable to walk, and bedridden. After OLTx, his neurologic condition became almost normal.
Bax
R T
RT
Department of Pediatrics, Technische Universität München, Germany.
Hässler
A
A
Luck
W
W
Hefter
H
H
Krägeloh-Mann
I
I
Neuhaus
P
P
Emmrich
P
P
D000293:Adolescent; D001927:Brain Diseases / Q000150:complications / Q000628:therapy*; D003300:Copper / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000378:metabolism / Q000628:therapy*; D006801:Humans; D016031:Liver Transplantation; D008297:Male
D003300:Copper