Q J Med 1987 Aug;64(244):685-91

Reversal of severe neurological manifestations of Wilson's disease followin
orthotopic liver transplantation

Polson RJ, Rolles K, Calne RY, Williams R, Marsden D
Liver Unit, King's College Hospital, Denmark Hill, London.

Abstract
Experience with liver transplantation for patients with Wilson's disease who
have major neurological impairment is limited, and this report describes the
results obtained in two such patients. The first was a 30-year-old man with a
14-month history of hepatic and neurological impairment. In spite of treatment
with d-penicillamine, he developed increasing dysarthria, dysphagia, akinesia
and rigidity of all four limbs, and required continuous nursing care. Following
transplantation, liver function was almost normal from four weeks onwards, but
recovery of neurological function was much slower and was not seen until two to
three months after surgery. By four months he was sufficiently mobile to be
discharged, and when he returned for assessment at eight months, no abnormal
neurological signs were detectable. The second patient was a 27-year-old woman
with worsening liver dysfunction for eight years; one year previously she had
developed dysarthria, akinesia, a fine tremor and moderate rigidity of all limbs
as well as marked psychological impairment. There was no improvement on
treatment with d-penicillamine or trientine, but as liver function returned to
normal two months after liver grafting, her neurological and psychological
function began to improve so that by three months she could be discharged