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Executive functions in premanifest Huntington's disease

Authors

  • S. Christine You MS,

    Corresponding author
    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
    • Correspondence to: S. Christine You, 675 Nelson Rising Lane, Suite 190, Box 1207, San Francisco, CA 94158, USA; cyou@paloaltou.edu

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  • Michael D. Geschwind MD, PhD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Sharon J. Sha MD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Alexandra Apple BA,

    1. Department of Radiology and Biomedical Imaging, University of California, San Francisco, California, USA
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  • Gabriella Satris MS,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Kristie A. Wood BA,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Erica T. Johnson BA,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Jonathan Gooblar MA,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Jeanne S. Feuerstein BA,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Steven Finkbeiner MD, PhD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
    2. Gladstone Institute of Neurological Disease, San Francisco, California, USA
    3. Department of Physiology, University of California, San Francisco, California, USA
    4. Taube/Koret Center for Neurodegenerative Disease Research and the Hellman Program for Alzheimer's Disease Research, San Francisco, California, USA
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  • Gail A. Kang MD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Bruce L. Miller MD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Christopher P. Hess MD, PhD,

    1. Department of Radiology and Biomedical Imaging, University of California, San Francisco, California, USA
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  • Joel H. Kramer PsyD,

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Katherine L. Possin PhD

    1. Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA
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  • Funding agencies: This study was supported by the NINDS (HHSN271200623661C [to Joel H. Kramer]), the UC Discovery Research and Development Grant (ITLBIO 178688), the NIA (K23AG037566 [to Katherine L. Possin], R01AG032289 [to Joel H. Kramer], P50AG023501 [to Bruce L. Miller]), the Larry L. Hillblom Foundation (2007/2I [to Bruce L. Miller]), the Hellman Family Foundation, the Michael J. Homer Family Fund, the Taube/Koret Center, and the Huntington's Disease Society of America (made possible with a gift from the James E. Bashaw Family).

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

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. © 2013 International Parkinson and Movement Disorder Society

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