Executive functions in premanifest Huntington's disease.

S Christine You 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

Mov Disord

Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, California, USA.

Published: March 2014

This study aimed to evaluate whether specific executive function tests can serve as reliable markers for individuals with premanifest Huntington's disease (HD).
Fifteen premanifest HD participants were compared to 42 control subjects using the NIH EXAMINER battery, revealing significantly lower working memory scores in the HD group, along with correlations between executive scores and disease indicators.
Findings suggest that the overall executive function composite and working memory scores are effective in indicating cognitive deficits and disease progression in premanifest HD patients.

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.

Download full-text PDF	Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4029327	PMC
http://dx.doi.org/10.1002/mds.25762	DOI Listing

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