Article

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.
CI - Copyright (c) 2013 Elsevier Masson SAS. All rights reserved.

Langue : ANGLAIS