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. 2014 Mar;29(3):405-9.
doi: 10.1002/mds.25762. Epub 2013 Dec 27.

Executive functions in premanifest Huntington's disease

S Christine You  1 , Michael D Geschwind, Sharon J Sha, Alexandra Apple, Gabriella Satris, Kristie A Wood, Erica T Johnson, Jonathan Gooblar, Jeanne S Feuerstein, Steven Finkbeiner, Gail A Kang, Bruce L Miller, Christopher P Hess, Joel H Kramer, Katherine L Possin
Affiliations

Executive functions in premanifest Huntington's disease

S Christine You et al. Mov Disord. 2014 Mar.

Abstract

Background: We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).

Methods: Fifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.

Results: The premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.

Conclusions: The NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD.

Keywords: Huntington's disease; disease burden; executive function; striatal volume; working memory.

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Figures

Figure 1
Figure 1
Scatterplots of Executive Composite and Working Memory Score to Striatal Volumes and CAPS
See this image and copyright information in PMC

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