MovDisordV3, PascalFrancis, Corpus, bibRecord, 001202

The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease

Identifieur interne : 001202 ( PascalFrancis/Corpus ); précédent : 001201; suivant : 001203

The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease

Auteurs : Bernard Ravina ; Megan Romer ; Radu Constantinescu ; Kevin Biglan ; Alicia Brocht ; Karl Kieburtz ; Ira Shoulson ; Michael P. Mcdermott

Source :

Movement disorders [ 0885-3185 ] ; 2008.

RBID : Pascal:08-0396181

Descripteurs français

Pascal (Inist)
- Chorée de Huntington, Pathologie du système nerveux.

English descriptors

KwdEn :
- Huntington disease, Nervous system diseases.

Abstract

The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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A03		`1`		`@0 Mov. disord.`
A05				`@2 23`
A06				`@2 9`
A08	`01`	`1`	`ENG`	`@1 The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease`
A11	`01`	`1`		`@1 RAVINA (Bernard)`
A11	`02`	`1`		`@1 ROMER (Megan)`
A11	`03`	`1`		`@1 CONSTANTINESCU (Radu)`
A11	`04`	`1`		`@1 BIGLAN (Kevin)`
A11	`05`	`1`		`@1 BROCHT (Alicia)`
A11	`06`	`1`		`@1 KIEBURTZ (Karl)`
A11	`07`	`1`		`@1 SHOULSON (Ira)`
A11	`08`	`1`		`@1 MCDERMOTT (Michael P.)`
A14	`01`			`@1 Department of Neurology, University of Rochester School of Medicine and Dentistry @2 Rochester, New York @3 USA @Z 1 aut. @Z 4 aut. @Z 5 aut. @Z 6 aut. @Z 7 aut.`
A14	`02`			`@1 Department of Statistics, Pennsylvania State University @2 State College, Pennsylvania @3 USA @Z 2 aut.`
A14	`03`			`@1 Department of Neurology, University of Goteborg @2 Goteborg @3 SWE @Z 3 aut.`
A14	`04`			`@1 Department of Biostatistics and Computational Biology, University of Rochester School of Medicine and Dentistry @2 Rochester, New York @3 USA @Z 8 aut.`
A20				`@1 1223-1227`
A21				`@1 2008`
A23	`01`			`@0 ENG`
A43	`01`			`@1 INIST @2 20953 @5 354000196264160040`
A44				`@0 0000 @1 © 2008 INIST-CNRS. All rights reserved.`
A45				`@0 27 ref.`
A47	`01`	`1`		`@0 08-0396181`
A60				`@1 P`
A61				`@0 A`
A64	`01`	`1`		`@0 Movement disorders`
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C01	`01`		`ENG`	@0 The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.
C02	`01`	`X`		`@0 002B17`
C02	`02`	`X`		`@0 002B17G`
C03	`01`	`X`	`FRE`	`@0 Chorée de Huntington @5 01`
C03	`01`	`X`	`ENG`	`@0 Huntington disease @5 01`
C03	`01`	`X`	`SPA`	`@0 Corea Huntington @5 01`
C03	`02`	`X`	`FRE`	`@0 Pathologie du système nerveux @5 02`
C03	`02`	`X`	`ENG`	`@0 Nervous system diseases @5 02`
C03	`02`	`X`	`SPA`	`@0 Sistema nervioso patología @5 02`
C07	`01`	`X`	`FRE`	`@0 Pathologie de l'encéphale @5 37`
C07	`01`	`X`	`ENG`	`@0 Cerebral disorder @5 37`
C07	`01`	`X`	`SPA`	`@0 Encéfalo patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Syndrome extrapyramidal @5 38`
C07	`02`	`X`	`ENG`	`@0 Extrapyramidal syndrome @5 38`
C07	`02`	`X`	`SPA`	`@0 Extrapiramidal síndrome @5 38`
C07	`03`	`X`	`FRE`	`@0 Maladie dégénérative @5 39`
C07	`03`	`X`	`ENG`	`@0 Degenerative disease @5 39`
C07	`03`	`X`	`SPA`	`@0 Enfermedad degenerativa @5 39`
C07	`04`	`X`	`FRE`	`@0 Maladie héréditaire @5 40`
C07	`04`	`X`	`ENG`	`@0 Genetic disease @5 40`
C07	`04`	`X`	`SPA`	`@0 Enfermedad hereditaria @5 40`
C07	`05`	`X`	`FRE`	`@0 Pathologie du système nerveux central @5 41`
C07	`05`	`X`	`ENG`	`@0 Central nervous system disease @5 41`
C07	`05`	`X`	`SPA`	`@0 Sistema nervosio central patología @5 41`
N21				`@1 259`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

Format Inist (serveur)

NO :	PASCAL 08-0396181 INIST
ET :	The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease
AU :	RAVINA (Bernard); ROMER (Megan); CONSTANTINESCU (Radu); BIGLAN (Kevin); BROCHT (Alicia); KIEBURTZ (Karl); SHOULSON (Ira); MCDERMOTT (Michael P.)
AF :	Department of Neurology, University of Rochester School of Medicine and Dentistry/Rochester, New York/Etats-Unis (1 aut., 4 aut., 5 aut., 6 aut., 7 aut.); Department of Statistics, Pennsylvania State University/State College, Pennsylvania/Etats-Unis (2 aut.); Department of Neurology, University of Goteborg/Goteborg/Suède (3 aut.); Department of Biostatistics and Computational Biology, University of Rochester School of Medicine and Dentistry/Rochester, New York/Etats-Unis (8 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2008; Vol. 23; No. 9; Pp. 1223-1227; Bibl. 27 ref.
LA :	Anglais
EA :	The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.
CC :	002B17; 002B17G
FD :	Chorée de Huntington; Pathologie du système nerveux
FG :	Pathologie de l'encéphale; Syndrome extrapyramidal; Maladie dégénérative; Maladie héréditaire; Pathologie du système nerveux central
ED :	Huntington disease; Nervous system diseases
EG :	Cerebral disorder; Extrapyramidal syndrome; Degenerative disease; Genetic disease; Central nervous system disease
SD :	Corea Huntington; Sistema nervioso patología
LO :	INIST-20953.354000196264160040
ID :	08-0396181

Links to Exploration step

Pascal:08-0396181

Le document en format XML

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<author><name sortKey="Biglan, Kevin" sort="Biglan, Kevin" uniqKey="Biglan K" first="Kevin" last="Biglan">Kevin Biglan</name>
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<front><div type="abstract" xml:lang="en">The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.</div>
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<fA14 i1="01"><s1>Department of Neurology, University of Rochester School of Medicine and Dentistry</s1>
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<fA14 i1="02"><s1>Department of Statistics, Pennsylvania State University</s1>
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<fA14 i1="03"><s1>Department of Neurology, University of Goteborg</s1>
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<fA14 i1="04"><s1>Department of Biostatistics and Computational Biology, University of Rochester School of Medicine and Dentistry</s1>
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<fC01 i1="01" l="ENG"><s0>The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.</s0>
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<s5>01</s5>
</fC03>
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<s5>02</s5>
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</fC07>
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<s5>37</s5>
</fC07>
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<s5>38</s5>
</fC07>
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<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Extrapiramidal síndrome</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Maladie dégénérative</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Degenerative disease</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0>
<s5>39</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Maladie héréditaire</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Genetic disease</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0>
<s5>40</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE"><s0>Pathologie du   système nerveux central</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG"><s0>Central nervous system disease</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0>
<s5>41</s5>
</fC07>
<fN21><s1>259</s1>
</fN21>
<fN44 i1="01"><s1>OTO</s1>
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<fN82><s1>OTO</s1>
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<server><NO>PASCAL 08-0396181 INIST</NO>
<ET>The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease</ET>
<AU>RAVINA (Bernard); ROMER (Megan); CONSTANTINESCU (Radu); BIGLAN (Kevin); BROCHT (Alicia); KIEBURTZ (Karl); SHOULSON (Ira); MCDERMOTT (Michael P.)</AU>
<AF>Department of Neurology, University of Rochester School of Medicine and Dentistry/Rochester, New York/Etats-Unis (1 aut., 4 aut., 5 aut., 6 aut., 7 aut.); Department of Statistics, Pennsylvania State University/State College, Pennsylvania/Etats-Unis (2 aut.); Department of Neurology, University of Goteborg/Goteborg/Suède (3 aut.); Department of Biostatistics and Computational Biology, University of Rochester School of Medicine and Dentistry/Rochester, New York/Etats-Unis (8 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2008; Vol. 23; No. 9; Pp. 1223-1227; Bibl. 27 ref.</SO>
<LA>Anglais</LA>
<EA>The objective of this study was to examine the relationship between CAG repeat length (CAGn) and clinical progression in patients with Huntington's disease (HD). There are conflicting reports about the relationship between CAGn and clinical progression of HD. We conducted an analysis of data from the Coenzyme Q10 and Remacemide Evaluation in Huntington's Disease (CARE-HD) clinical trial. We modeled progression over 30 months on the Unified Huntington's Disease Rating Scale (UHDRS) and supplemental neuropsychological and behavioral tests using multiple linear regression. Mean subject age was 47.9 ± 10.5 years and mean CAGn was 45.0 ± 4.1. Multiple linear regression revealed statistically significant associations between CAGn and worsening on several motor, cognitive, and functional outcomes, but not behavioral outcomes. Many effects were clinically important; 10 additional CAG repeats were associated with an 81% increase in progression on the Independence Scale. These associations were not observed in the absence of age adjustment. Age at the time of assessment confounds the association between CAGn and progression. Adjusting for age shows that longer CAGn is associated with greater clinical progression of HD. This finding may account for the variable results from previous studies examining CAGn and progression. Adjusting for CAGn may be important for clinical trials.</EA>
<CC>002B17; 002B17G</CC>
<FD>Chorée de Huntington; Pathologie du   système nerveux</FD>
<FG>Pathologie de l'encéphale; Syndrome extrapyramidal; Maladie dégénérative; Maladie héréditaire; Pathologie du   système nerveux central</FG>
<ED>Huntington disease; Nervous system diseases</ED>
<EG>Cerebral disorder; Extrapyramidal syndrome; Degenerative disease; Genetic disease; Central nervous system disease</EG>
<SD>Corea Huntington; Sistema nervioso patología</SD>
<LO>INIST-20953.354000196264160040</LO>
<ID>08-0396181</ID>
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	Movement Disorders (revue)
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Movement Disorders (revue)

The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease

The Relationship Between CAG Repeat Length and Clinical Progression in Huntington's Disease

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