title: Liver Transplantation in Wilson's Disease with Neurological Impairment: Evaluation in 4 Patients.
abstract: The aim of this work is to report our early experiences about the benefits of liver transplantation (LT) in the treatment of persistent neurological symptoms in Wilson's disease (WD) patients.
  
  
  
  We describe our findings in 4 WD patients with neurological impairment or symptoms treated by LT: 2 patients had transplants due to worsening of neurological symptoms despite long-term appropriate medical treatment. The other 2 required LT because of symptoms associated with liver failure. Patients were evaluated using the modified Rankin scale and the Unified Wilson's Disease Rating Scale (UWDRS).
  
  
  
  The 4 patients experienced neurological improvement after LT. The pre-LT Rankin score of the 2 patients transplanted due to neurological impairment was 4 compared to 3 and 2, respectively, post LT. The pre-LT Rankin scores of the 2 WD cases transplanted because of hepatic failure were 1 and 2, respectively, compared to 0 in both cases post LT. UWDRS score improved in 2 cases and remained stable in 1 less severely impaired case. Brain MRI abnormalities proved partially reversible in 3 patients and remained stable for 1 patient.
  
  
  
  These results suggest that LT could be envisaged for neurologically impaired WD patients.
authors.affiliation: Service de Neurologie C, Hôpital Neurologique, Bron, France.
authors.forename: Chloé
authors.initials: C
authors.lastname: Laurencin
authors.forename: Anne Sophie
authors.initials: AS
authors.lastname: Brunet
authors.forename: Jérôme
authors.initials: J
authors.lastname: Dumortier
authors.forename: Laurence
authors.initials: L
authors.lastname: Lion-Francois
authors.forename: Stéphane
authors.initials: S
authors.lastname: Thobois
authors.forename: Jean Yves
authors.initials: JY
authors.lastname: Mabrut
authors.forename: Rémi
authors.initials: R
authors.lastname: Dubois
authors.forename: France
authors.initials: F
authors.lastname: Woimant
authors.forename: Aurélia
authors.initials: A
authors.lastname: Poujois
authors.forename: Olivier
authors.initials: O
authors.lastname: Guillaud
authors.forename: Alain
authors.initials: A
authors.lastname: Lachaux
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Broussolle
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology* / Q000473:pathology; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged