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Measuring Executive Dysfunction Longitudinally and in Relation to Genetic Burden, Brain Volumetrics, and Depression in Prodromal Huntington Disease
Journal article   Open access   Peer reviewed

Measuring Executive Dysfunction Longitudinally and in Relation to Genetic Burden, Brain Volumetrics, and Depression in Prodromal Huntington Disease

Kathryn V. Papp, Peter J. Snyder, James A. Mills, Kevin Duff, Holly J. Westervelt, Jeffrey D. Long, Spencer Lourens and Jane S. Paulsen
Archives of clinical neuropsychology, Vol.28(2), pp.156-168
03/01/2013
DOI: 10.1093/arclin/acs105
PMCID: PMC3569950
PMID: 23246934
url
https://doi.org/10.1093/arclin/acs105View
Published (Version of record) Open Access

Abstract

Executive dysfunction (ED) is a characteristic of Huntington disease (HD), but its severity and progression is less understood in the prodromal phase, e.g., before gross motor abnormalities. We examined planning and problem-solving abilities using the Towers Task in HD mutation-positive individuals without motor symptoms (n 781) and controls (n 212). Participants with greater disease progression (determined using mutation size and current age) performed more slowly and with less accuracy on the Towers Task. Performance accuracy was negatively related to striatal volume while both accuracy and working memory were negatively related to frontal white matter volume. Disease progression at baseline was not associated with longitudinal performance over 4 years. Whereas the baseline findings indicate that ED becomes more prevalent with greater disease progression in prodromal HD and can be quantified using the Towers task, the absence of notable longitudinal findings indicates that the Towers Task exhibits limited sensitivity to cognitive decline in this population.
Psychology Psychology, Clinical Social Sciences

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