You are viewing a javascript disabled version of the site. Please enable Javascript for this site to function properly.
Go to headerGo to navigationGo to searchGo to contentsGo to footer
In content section. Select this link to jump to navigation

Visual Working Memory Impairment in Premanifest Gene-Carriers and Early Huntington's Disease

Article type: Research Article

Authors: Dumas, Eve M. | Say, Miranda J. | Jones, Rebecca | Labuschagne, Izelle | O'Regan, Alison M. | Hart, Ellen P. | van den Bogaard, Simon J.A. | Queller, Sarah | Justo, Damian | Coleman, Allison | dar Santos, Rachelle C. | Dürr, Alexandra | Leavitt, Blair R. | Tabrizi, Sarah J. | Roos, Raymund A.C. | Stout, Julie C.

Affiliations: Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands | UCL Institute of Neurology, University College London, Queen Square, London, UK | Department of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK | School of Psychology and Psychiatry, Monash University, Clayton Campus, VIC, Australia | Queller Consulting, Dunedin, FL, USA | Department of Genetics and Cytogenetics, and INSERM UMR S679, APHP Hôpital de la Salpêtrière, Paris, France | Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada

Note: [] Correspondence to: Prof. Julie C. Stout, PhD, School of Psychology and Psychiatry, Monash University Clayton, Victoria 3800, Australia. Tel.: +61 3 99053987; Fax: +61 3 99053948; E-mail: [email protected]

Keywords: Huntington's disease, visual working memory, cognitive dysfunction, premanifest gene-carriers

DOI: 10.3233/JHD-2012-120010

Journal: Journal of Huntington's Disease, vol. 1, no. 1, pp. 97-106, 2012

Published: 2012
Get PDF open access

Abstract

Working memory deficits have been found in Huntington's disease (HD) and in a small group of premanifest (PreHD) gene-carriers. However, the nature and extent of these deficits are unknown. In a large cross-sectional study, we aimed to determine the degree of visuospatial working memory dysfunction across multiple stages of HD. Specifically, visuospatial working memory capacity and response times across various degrees of difficulty were examined, as well as the relationship between visuospatial working memory and motor dysfunction. We examined 62 PreHD-A gene-carriers (>10.8 years from estimated disease onset), 58 PreHD-B gene-carriers (<10.8 years from estimated disease onset), 77 stage-1 HD patients (HD1), 44 stage-2 HD patients (HD2), and 122 healthy controls. Participants viewed coloured squares (in sets of 3, 5 and 7) on a screen and were to decide whether on a subsequent screen the encircled square has changed colour. Accuracy and response times were recorded. Compared to controls, significant group differences in visuospatial working memory capacity (accuracy) were seen in PreHD-B, HD1 and HD2 groups across the difficulty levels. Significant group differences on response times were found for all groups (PreHD-A to HD2) compared to controls; the most difficult level producing the only group difference in speed between PreHD-A and controls. Accuracy and speed were positively correlated only in the HD groups. These findings suggest that visuospatial working memory impairments are detectable in both premanifest and manifest HD; the manifest HD showed evidence for a “worse-worse phenomenon” whereby reductions were present in both motor speed and accuracy.

Show less