Liver transplantation for neurologic Wilson's disease: reflections on two cases within a mexican cohort.

ABSTRACT

BACKGROUND: Liver transplantation (LT) in Wilson s disease (WD) is mostly indicated when progressing liver disease or acute liver failure occurs. In patients with neurological manifestations, this procedure has not gained wide acceptance based on previous reports of dismal prognosis. OBJECTIVE: To describe a Mexican cohort of pa- tients with WD with special focus on LT in patients with deteriorating neurological manifestations. MATERIAL AND METHODS: Patients with confirmed WD and their first-degree relatives were evaluated at the hepatology clinic of a tertiary referral hospital. Attention was placed on therapy and outcome. RESULTS: Eleven patients were followed for a period of up to 80 months (7 probands and 4 affected family members), 10 patients having hepatic manifestations and 4 having neuro psychia- tric phenomena. Pharmacologic treatment was uniform in most patients, and LT was indicated in 2 cases because of deteriorating neurological status. These patients had total remission of their neurological manifestations with marked improvement on imaging studies. CONCLUSIONS: Follow-up and pharmacologic treatment was flawed by several adverse conditions present in our population. Patients with progressing neurological disease had a favorable outcome after LT, a similar response to the one reported by several authors. In conclusion, LT should be strongly considered for the treatment of these patients.