Affiliations: LUNAM Université, Laboratoire de Psychologie des Pays de la Loire, Université de Nantes et Angers, France | Neuropsychology and Auditory Cognition, Department of Psychology, University of Lille, France | Laboratoire Epsylon, EA 4556, Université Paul-Valery, Montpellier III, France | Radboud University Nijmegen, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands | Rehabilitation Medical Centre Groot Klimmendaal, SIZA Support and Rehabilitation, Arnhem, The Netherlands | Centre Mémoire de Ressources et de Recherches, CHU Angers, France
Note:  Correspondence to: Mohamad El Haj, Neuropsychology and Auditory Cognition, Department of Psychology, University of Lille 3, Domaine Universitaire du Pont de Bois, BP 60149, 59653 Villeneuve d'Ascq cedex, France. Tel.: +33 6 73 08 78 76; Fax: +33 3 20 41 63 24; E-mail: [email protected]
Abstract: Background: Huntington's disease (HD) is characterized by episodic memory deterioration. Objective: Our paper investigates the cognitive mechanisms that might underlie this decline. To this aim, we tested two executive hypotheses, the binding and the inhibition hypotheses. Methods: Fifteen HD patients (Mean Cytosine–Adenine–Guanine repeats = 44.93, SD = 2.82), and eighteen controls matched for age, gender and education were assessed with a neuropsychological battery tapping episodic memory and several executive functions, including binding and inhibition. Results: Episodic decline in patients with HD was only related to binding performance. Conclusions: Our study shows that HD patients suffer from a perturbation of the associative or integrative mechanisms responsible for the combination of different memory features into complex episodic representations. Damage to frontal-hippocampal circuitry in HD is likely to be responsible for this impairment.