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

Identifieur interne : 003657 ( Main/Merge ); précédent : 003656; suivant : 003658

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

Auteurs : Bernard Ravina [États-Unis] ; Megan Romer [États-Unis] ; Radu Constantinescu [Suède] ; Kevin Biglan [États-Unis] ; Alicia Brocht [États-Unis] ; Karl Kieburtz [États-Unis] ; Ira Shoulson [États-Unis] ; Michael P. Mcdermott [États-Unis]

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.

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Pascal:08-0396181

Le document en format XML

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<author><name sortKey="Constantinescu, Radu" sort="Constantinescu, Radu" uniqKey="Constantinescu R" first="Radu" last="Constantinescu">Radu Constantinescu</name>
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<author><name sortKey="Mcdermott, Michael P" sort="Mcdermott, Michael P" uniqKey="Mcdermott M" first="Michael P." last="Mcdermott">Michael P. Mcdermott</name>
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<author><name sortKey="Kieburtz, Karl" sort="Kieburtz, Karl" uniqKey="Kieburtz K" first="Karl" last="Kieburtz">Karl Kieburtz</name>
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<author><name sortKey="Mcdermott, Michael P" sort="Mcdermott, Michael P" uniqKey="Mcdermott M" first="Michael P." last="Mcdermott">Michael P. Mcdermott</name>
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<series><title level="j" type="main">Movement disorders</title>
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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>
</front>
</TEI>
<affiliations><list><country><li>Suède</li>
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<name sortKey="Biglan, Kevin" sort="Biglan, Kevin" uniqKey="Biglan K" first="Kevin" last="Biglan">Kevin Biglan</name>
<name sortKey="Brocht, Alicia" sort="Brocht, Alicia" uniqKey="Brocht A" first="Alicia" last="Brocht">Alicia Brocht</name>
<name sortKey="Kieburtz, Karl" sort="Kieburtz, Karl" uniqKey="Kieburtz K" first="Karl" last="Kieburtz">Karl Kieburtz</name>
<name sortKey="Mcdermott, Michael P" sort="Mcdermott, Michael P" uniqKey="Mcdermott M" first="Michael P." last="Mcdermott">Michael P. Mcdermott</name>
<name sortKey="Romer, Megan" sort="Romer, Megan" uniqKey="Romer M" first="Megan" last="Romer">Megan Romer</name>
<name sortKey="Shoulson, Ira" sort="Shoulson, Ira" uniqKey="Shoulson I" first="Ira" last="Shoulson">Ira Shoulson</name>
</country>
<country name="Suède"><noRegion><name sortKey="Constantinescu, Radu" sort="Constantinescu, Radu" uniqKey="Constantinescu R" first="Radu" last="Constantinescu">Radu Constantinescu</name>
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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

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri