Movement Disorders (revue)

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Ten-Year Rate of Longitudinal Change in Neurocognitive and Motor Function in Prediagnosis Huntington Disease

Identifieur interne : 001C23 ( PascalFrancis/Curation ); précédent : 001C22; suivant : 001C24

Ten-Year Rate of Longitudinal Change in Neurocognitive and Motor Function in Prediagnosis Huntington Disease

Auteurs : Andrea C. Solomon [États-Unis] ; Julie C. Stout [États-Unis, Australie] ; Marjorie Weaver [États-Unis] ; Sarah Queller [États-Unis] ; Allison Tomusk [États-Unis] ; Kathryn Burr Whitlock [États-Unis] ; Siu L. Hui [États-Unis] ; Jeanine Marshall [États-Unis] ; Jacqueline Gray Jackson [États-Unis] ; Eric R. Siemers [États-Unis] ; Xabier Beristain [États-Unis] ; Joanne Wojcieszek [États-Unis] ; Tatiana Foroud [États-Unis]

Source :

RBID : Pascal:08-0522177

Descripteurs français

English descriptors

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.
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A11 06  1    @1 BURR WHITLOCK (Kathryn)
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A11 08  1    @1 MARSHALL (Jeanine)
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A14 03      @1 Department of Medical and Molecular Genetics, Indiana University School of Medicine @2 Indianapolis, Indiana @3 USA @Z 3 aut. @Z 8 aut. @Z 9 aut. @Z 13 aut.
A14 04      @1 Department of Medicine, Division of Biostatistics, Indiana University School of Medicine, Regenstrief Institute @2 Indianapolis, Indiana @3 USA @Z 7 aut.
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C01 01    ENG  @0 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.
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C03 02  X  FRE  @0 Pathologie du système nerveux @5 02
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C07 04  X  SPA  @0 Enfermedad hereditaria @5 40
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C07 05  X  ENG  @0 Central nervous system disease @5 41
C07 05  X  SPA  @0 Sistema nervosio central patología @5 41
N21       @1 343
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Pascal:08-0522177

Le document en format XML

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<title xml:lang="en" level="a">Ten-Year Rate of Longitudinal Change in Neurocognitive and Motor Function in Prediagnosis Huntington Disease</title>
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<div type="abstract" xml:lang="en">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.</div>
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