title: Cognitive Abilities of Children With Neurological and Liver Forms of Wilson Disease.
abstract: Cognitive impairment in adult patients experiencing Wilson disease is now more clearly described, even in liver forms of the disease. Although this condition can appear during childhood, the cognitive abilities of children have not yet been reported in a substantial case series. This retrospective study included 21 children with Wilson disease who had undergone general cognitive assessment. The results argue in favor of a poor working memory capacity in the liver form of the disease, and more extensive cognitive impairments in its neurological form. Extensive neuropsychological investigations on all children experiencing Wilson disease are thus required.
authors.affiliation: *Department of Pediatric Gastroenterology, Hepatology and Nutrition, French National Centre for Wilson's Disease, Children and Mother's Hospital of Lyon, Bron †Department of Neurology C, Pierre Wertheimer Neurological Hospital of Lyon, Bron ‡Department of Pediatric Neurology, Children and Mother's Hospital of Lyon, Bron §Reference Centre for Inherited Metabolic Disorder, Children's Hospital of Nancy, Vandoeuvre-lès-Nancy ||Department of Hepatology, Competency Centre for Wilson's Disease, Jean Minjoz Hospital, Besançon ¶Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Nancy, Vandoeuvre-lès-Nancy #Regional Centre for Learning and Language Disorders of Children, Jean Minjoz Hospital, Besançon **Department of Medicine, University Claude Bernard Lyon 1, Vileurbanne, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Favre
authors.forename: Laurence
authors.initials: L
authors.lastname: Lion-François
authors.forename: Marie
authors.initials: M
authors.lastname: Canton
authors.forename: Claire
authors.initials: C
authors.lastname: Vanlemmens
authors.forename: Marjorie
authors.initials: M
authors.lastname: Bonneton
authors.forename: Lise
authors.initials: L
authors.lastname: Bouillet
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Brunet
authors.forename: Alain
authors.initials: A
authors.lastname: Lachaux
mesh_terms: D000293:Adolescent; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000523:psychology*; D006801:Humans; D007361:Intelligence Tests; D008297:Male; D008570:Memory, Short-Term; D009483:Neuropsychological Tests; D012189:Retrospective Studies