Journal
NEUROPSYCHOLOGY
Volume 25, Issue 1, Pages 1-14Publisher
AMER PSYCHOLOGICAL ASSOC
DOI: 10.1037/a0020937
Keywords
cognitive assessment; presymptomatic; neuropsychology; psychomotor; prediagnosis
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Funding
- National Institutes of Health, National Institute of Neurological Disorders and Stroke [NS40068]
- CHDI Foundation, Inc.
- NATIONAL CENTER FOR RESEARCH RESOURCES [UL1RR024979] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS040068] Funding Source: NIH RePORTER
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Objective: PREDICT-HD is a large-scale international study of people with the Huntington disease (HD) CAG-repeat expansion who are not yet diagnosed with HD. The objective of this study was to determine the stage in the HD prodrome at which cognitive differences from CAG-normal controls can be reliably detected. Method: For each of 738 HD CAG-expanded participants, we computed estimated years to clinical diagnosis and probability of diagnosis in 5 years based on age and CAG-repeat expansion number (Langbehn, Brinkman, Fa lush, Paulsen, & Hayden, 2004). We then stratified the sample into groups: NEAR, estimated to be <= 9 years; MID, between 9 and 15 years; and FAR, >= 15 years. The control sample included 168 CAG-normal participants. Nineteen cognitive tasks were used to assess attention, working memory, psychomotor functions, episodic memory, language, recognition of facial emotion, sensory-perceptual functions, and executive functions. Results: Compared with the controls, the NEAR group showed significantly poorer performance on nearly all of the cognitive tests and the MID group on about half of the cognitive tests (p = .05, Cohen's d NEAR as large as -1.17, MID as large as -0.61). One test even revealed significantly poorer performance in the FAR group (Cohen's d = -0.26). Individual tasks accounted for 0.2% to 9.7% of the variance in estimated proximity to diagnosis. Overall, the cognitive battery accounted for 34% of the variance; in comparison, the Unified Huntington's Disease Rating Scale motor score accounted for 11.7%. Conclusions: Neurocognitive tests are robust clinical indicators of the disease process prior to reaching criteria for motor diagnosis of HD.
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