Abstract:
Objectives. This study sought to evaluate the effect of liver transplantation on the
neuropsychological manifestations of Wilson disease.
Materials and Methods. Nine of 42 Wilson disease patients had neuropsychological
symptoms before liver transplantation. They were 7 male and 2 female subjects with a
median age of 19 years (range 10 to 25). They were analyzed for their preoperative and
postoperative hepatic, neurological, and psychological scores described by the Unified
Wilson Disease Rating Scale after a mean 36.6 months of follow-up.
Results. Preoperative mean Model for End-Stage Liver Disease and Child-Pugh scores
were 18.3 (range 15 to 26) and 8.9 (range 6 to 12), respectively. One patient had acute
postoperative ischemic stroke unrelated to Wilson disease and was excluded from the
statistical analysis. Preoperative and postoperative hepatic, neurological, and
psychological scores of the remaining 8 patients were 7.4 2.3 vs 2.4 1.3 (P ¼ .0005),
17.7 11.7 vs 12.7 12.5 (P ¼ .055), and 9.0 1.7 vs 7.0 2.1 (P ¼ .033).
Conclusions. Liver transplantation for Wilson disease can provide some improvement of
the neuropsychological symptoms in addition to the hepatic recovery.