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Ten‐year rate of longitudinal change in neurocognitive and motor function in prediagnosis Huntington disease
Authors:Andrea C Solomon PhD  Julie C Stout PhD  Marjorie Weaver MS  Sarah Queller PhD  Allison Tomusk PhD  Kathryn Burr Whitlock MS  Siu L Hui PhD  Jeanine Marshall MS  Jacqueline Gray Jackson BS  Eric R Siemers MD  Xabier Beristain MD  Joanne Wojcieszek MD  Tatiana Foroud PhD
Institution:1. Department of Psychological and Brain Sciences, Indiana University, Bloomington, Indiana, USA;2. School of Psychology, Psychiatry, and Psychological Medicine, Monash University, Clayton, Victoria, Australia;3. Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, Indiana, USA;4. Department of Medicine, Division of Biostatistics, Indiana University School of Medicine, Regenstrief Institute, Indianapolis, Indiana, USA;5. Department of Neurology, Indiana University School of Medicine, Indianapolis, Indiana, USA;6. Lilly Corporate Center, Indianapolis, Indiana, USA
Abstract:Longitudinal studies of neurocognitive function in prediagnosis Huntington disease (pre‐HD) have been few, and duration of follow‐up has been brief. In this study, 155 individuals at‐risk for HD completed a battery of cognitive and motor tasks at two study visits ~10 years apart. Participants were classified as: (1) at‐risk, without the CAG expansion (healthy controls, NC; n = 112), or (2) CAG expanded (CAG+; n = 43). To examine the rate of decline at different stages of the pre‐HD period, participants in the CAG+ group were further characterized as converters (i.e., individuals who developed manifest HD over the course of the study; n = 21) or nonconverters (n = 22), and their performances were compared. The CAG+ group exhibited faster rates of neurocognitive decline over the course of the study, relative to the NC group. In addition, more rapid decline was associated with closer proximity to estimated age of disease onset in the CAG+ group. Faster rates of motor and psychomotor decline were observed in the CAG+ converter group, relative to the nonconverters. These findings suggest that neurocognitive decline in pre‐HD, particularly in motor and psychomotor domains, begins insidiously and accelerates as individuals approach disease onset. © 2008 Movement Disorder Society
Keywords:Huntington disease  prediagnosis  longitudinal  neurocognitive
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