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Cognitive Abilities of Children With Neurological and Liver Forms of Wilson Disease

Favre, Emilie*,†; Lion-François, Laurence; Canton, Marie§; Vanlemmens, Claire||; Bonneton, Marjorie; Bouillet, Lise#; Brunet, Anne-Sophie*; Lachaux, Alain*,**

Journal of Pediatric Gastroenterology and Nutrition: March 2017 - Volume 64 - Issue 3 - p 436–439
doi: 10.1097/MPG.0000000000001346
Short Communication: Hepatology

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.

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*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.

Address correspondence and reprint requests to Emilie Favre, PhD, Centre National de Référence pour la Maladie de Wilson, Service de Gastroentérologie, Hépatologie et Nutrition Pédiatrique HFME, 59 Boulevard Pinel, 69677 Bron Cedex, France (e-mail:

Received 16 March, 2016

Accepted 23 July, 2016

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The authors have no conflicts of interest.

© 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,