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


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.
Department of Gastroenterology, Salvador Zubiran National Institute of Medical Sciences and Nutrition, Mexico City, Mexico.
A
A
Duarte-Rojo
S
S
Zepeda-Gómez
J
J
García-Leiva
J M
JM
Remes-Troche
A
A
Angeles-Angeles
A
A
Torre-Delgadillo
M A
MA
Olivera-Martínez
D000293:Adolescent; D001921:Brain / Q000473:pathology; D002648:Child; D015331:Cohort Studies; D003300:Copper / Q000378:metabolism; D005260:Female; D006501:Hepatic Encephalopathy / Q000473:pathology / Q000601:surgery; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000209:etiology; D008800:Mexico; D009422:Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D011379:Prognosis; D055815:Young Adult
D003300:Copper