Journal article
Neurocognitive Signs in Prodromal Huntington Disease
Neuropsychology, Vol.25(1), pp.1-14
01/2011
DOI: 10.1037/a0020937
PMCID: PMC3017660
PMID: 20919768
Abstract
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, Falush, 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.
Details
- Title: Subtitle
- Neurocognitive Signs in Prodromal Huntington Disease
- Creators
- Julie C Stout - School of Psychology, Psychiatry, and Psychological Medicine, Monash UniversityJane S Paulsen - The Roy J. and Lucille A. Carver College of Medicine, University of IowaSarah Queller - Department of Psychological and Brain Sciences, Indiana UniversityAndrea C Solomon - Department of Neurology, University of Alabama at BirminghamKathryn B Whitlock - Department of Psychological and Brain Sciences, Indiana UniversityJ. Colin Campbell - Department of Psychological and Brain Sciences, Indiana UniversityNoelle Carlozzi - Kessler Foundation Research Center, West Orange, NJKevin Duff - Department of Psychiatry, University of IowaLeigh J Beglinger - Department of Psychiatry, University of IowaDouglas R Langbehn - Department of Psychiatry, University of IowaShannon A Johnson - Department of Psychology, Dalhousie UniversityKevin M Biglan - Clinical Trials Coordination Center, University of RochesterElizabeth H Aylward - Department of Radiology, University of WashingtonPREDICT-HD Investigators and Coordinators of the Huntington Study Group
- Contributors
- Stephen M Rao (Editor)
- Resource Type
- Journal article
- Publication Details
- Neuropsychology, Vol.25(1), pp.1-14
- DOI
- 10.1037/a0020937
- PMID
- 20919768
- PMCID
- PMC3017660
- NLM abbreviation
- Neuropsychology
- ISSN
- 0894-4105
- eISSN
- 1931-1559
- Publisher
- American Psychological Association
- Grant note
- DOI: 10.13039/100000002, name: National Institutes of Health, award: NS40068; DOI: 10.13039/100005725, name: CHDI Foundation, Inc.
- Language
- English
- Date published
- 01/2011
- Academic Unit
- Psychiatry; Psychological and Brain Sciences; Iowa Neuroscience Institute
- Record Identifier
- 9984003947302771
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