title: Cognitive profile in Wilson's disease: a case series of 31 patients.
abstract: BACKGROUND
  Wilson's disease (WD) is a rare autosomal recessive disorder of copper metabolism. If untreated, WD, which is initially a liver disease, can turn into a multi-systemic disease with neurological involvement. Very few studies have described cognitive impairment in WD. The aim of this study is to report the cognitive profile of 31 treated WD patients.
  
  
  METHODS
  Patients were classed into two groups using the Unified Wilson Disease Rating Scale (UWDRS): WD patients without neurological signs (WD-N(-)) (n=13), and WD patients with neurological signs (WD-N(+)) (n=18). The patients participated in a neuropsychological assessment evaluating memory, executive function and visuo-spatial abilities.
  
  
  RESULTS
  Both groups performed well for verbal intelligence and episodic memory skills. However, the majority of these patients exhibited altered performance for at least one cognitive test, particularly in the executive domain. The WD-N(+) group performed less well than the WD-N(-) group on cognitive tests involving rapid motor function, abstract thinking, working memory and top-down inhibitory control.
  
  
  CONCLUSIONS
  Cognitive impairment in treated WD patients essentially affects executive function involving fronto-striatal circuits. Verbal intelligence and episodic memory abilities seem to be remarkably preserved. Neuropsychological assessment is a valuable tool to evaluate the presence and the consequences of these cognitive impairments in WD patients with or without neurological signs in the course of this chronic disease.
authors.affiliation: French national reference centre for Wilson's disease, neurology department, Lariboisière hospital, 2, rue Ambroise-Paré, 75010 Paris cedex 10, France. Electronic address: emilie.wenisch@lrb.aphp.fr.
authors.forename: E
authors.initials: E
authors.lastname: Wenisch
authors.forename: A
authors.initials: A
authors.lastname: De Tassigny
authors.forename: J-M
authors.initials: JM
authors.lastname: Trocello
authors.forename: J
authors.initials: J
authors.lastname: Beretti
authors.forename: N
authors.initials: N
authors.lastname: Girardot-Tinant
authors.forename: F
authors.initials: F
authors.lastname: Woimant
mesh_terms: D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D003071:Cognition; D056344:Executive Function; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000523:psychology*; D006801:Humans; D008297:Male; D008568:Memory; D008875:Middle Aged; D009483:Neuropsychological Tests; D011597:Psychomotor Performance; D013028:Space Perception; D014706:Verbal Learning; D014796:Visual Perception; D014888:Wechsler Scales; D055815:Young Adult
chemical_list: D002614:Chelating Agents
keywords: Cognition; Examen neuropsychologique; Executive function; Fonctions exécutives; Maladie de Wilson; Maladie métabolique; Metabolic disease; Neuropsychology; Wilson's disease