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Cognitive and functional decline in Huntington's disease: Dementia criteria revisited

Authors

  • Guerry M. Peavy PhD,

    Corresponding author
    1. Department of Neurosciences, University of California, San Diego, California, USA
    • UCSD Shiley-Marcos Alzheimer's Disease Research Center, 8950 Villa La Jolla Dr. C-129, La Jolla, CA 92037
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  • Mark W. Jacobson PhD,

    1. Department of Psychology, Veterans Administration Medical Center, San Diego, California, USA
    2. Department of Psychiatry, University of California, San Diego, California, USA
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  • Jody L. Goldstein BS,

    1. Department of Neurosciences, University of California, San Diego, California, USA
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  • Joanne M. Hamilton PhD,

    1. Department of Neurosciences, University of California, San Diego, California, USA
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  • Amy Kane MS,

    1. Joint Doctoral Program in Clinical Psychiatry, San Diego State University/University of California, San Diego, California, USA
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  • Anthony C. Gamst PhD,

    1. Department of Neurosciences, University of California, San Diego, California, USA
    2. Department of Family and Preventive Medicine, University of California, San Diego, California, USA
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  • Stephanie L. Lessig MD,

    1. Department of Neurosciences, University of California, San Diego, California, USA
    2. Department of Neurology, Veterans Administration Medical Center, San Diego, California, USA
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  • J. C. Lee BA,

    1. Department of Psychiatry, University of California, San Diego, California, USA
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  • Jody Corey-Bloom MD, PhD

    1. Department of Neurosciences, University of California, San Diego, California, USA
    2. Department of Neurology, Veterans Administration Medical Center, San Diego, California, USA
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  • Potential conflict of interest: Nothing to report.

Abstract

The importance of designating criteria for diagnosing dementia lies in its implications for clinical treatment, research, caregiving, and decision-making. Dementia diagnosis in Huntington's disease (HD) is often based on criteria developed for Alzheimer's disease requiring memory loss. However, it is likely that other cognitive deficits contribute to functional impairment in HD before memory declines. The goal is to identify cognitive deficits that contribute to functional impairment to support dementia criteria that reflect HD neuropathology. Eighty-four HD mutation-positive subjects completed neuropsychological tests and the Unified Huntington's Disease Rating Scale Functional Independence Scale (FIS). Functional impairment was defined as 80 or below on the FIS. Speed of processing, initiation, and attention measures accounted for 70.0% of the variance in FIS ratings (linear regression) and correctly classified 91.7% of subjects as functionally impaired or intact (logistic regression). Measures of memory, motor impairment except dysarthria, neuroleptic use, and depressed mood did not improve prediction. A definition of HD dementia that includes cognitive impairment in at least two areas of cognition but does not require a memory deficit, in the context of impaired functional abilities and a deteriorating course, more accurately reflects HD neuropathology and could lead to improved research methods and patient care. © 2010 Movement Disorder Society

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