What is the Impact of Education on Huntington's Disease?
Identifieur interne : 001910 ( Main/Merge ); précédent : 001909; suivant : 001911What is the Impact of Education on Huntington's Disease?
Auteurs : Jose Luis Lopez-Sendon [Espagne] ; Ana Royuela [Espagne] ; Patricia Trigo [Espagne] ; Michael Orth [Allemagne] ; Herwig Lange [Allemagne] ; Ralf Reilmann [Allemagne] ; Jennifer Keylock [Royaume-Uni] ; Hugh Rickards [Royaume-Uni] ; Silvia Piacentini [Italie] ; Ferdinando Squitieri [Italie] ; Bernhard Landwehrmeyer [Allemagne] ; Marie-Noelle Witjes-Ane [Pays-Bas] ; Caroline K. Jurgens [Pays-Bas] ; Raymund A. C. Roos [Pays-Bas] ; Victor Abraira [Espagne] ; Justo G. De Yebenes [Espagne]Source :
- Movement disorders [ 0885-3185 ] ; 2011.
Descripteurs français
- Pascal (Inist)
English descriptors
Abstract
Huntington's disease (HD) is a neurodegenerative disease caused by a cytosine adenosine guanine (CAG) expansion in the huntingtin gene. The length of the triplet repeat is the most important factor in determining age of onset and the severity of the disease, but substantial variability of these parameters is attributed to other factors. To investigate the relationship between the years of education and the age at onset and the severity of the phenotype in patients with HD, we applied multiple linear regression analysis to examine the impact of education on the age at onset and the severity of the clinical scores assessed by the Unified Huntington's Disease Rating Scale (UHDRS) of 891 patients with HD from the multinational observational study "Registry" conducted by the European Huntintgton's Disease Network. The model was adjusted for CAG repeat length and age at the time of assessment. Patients with lengthier education exhibited earlier estimated age at onset but less severe clinical scores (motor = -3.6, P = 0.006; cognitive = 27.0, P < 0.001; behavioral = -3.0, P < 0.001; and functional capacity = 1.1 points, P < 0.001) than those with shorter education, after controlling for age and number of CAG repeats. These differences persisted throughout all quartiles of disease severity. An earlier recognition of symptoms and manifestations among the more educated patients could explain the earlier estimated age at onset in this group. The link between better clinical UHDRS scores and higher education might reflect a beneficial effect of education or its covariates on the course of HD.
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<author><name sortKey="Jurgens, Caroline K" sort="Jurgens, Caroline K" uniqKey="Jurgens C" first="Caroline K." last="Jurgens">Caroline K. Jurgens</name>
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<author><name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G." last="De Yebenes">Justo G. De Yebenes</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">What is the Impact of Education on Huntington's Disease?</title>
<author><name sortKey="Lopez Sendon, Jose Luis" sort="Lopez Sendon, Jose Luis" uniqKey="Lopez Sendon J" first="Jose Luis" last="Lopez-Sendon">Jose Luis Lopez-Sendon</name>
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<s2>Madrid</s2>
<s3>ESP</s3>
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<country>Espagne</country>
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</placeName>
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<region nuts="2" type="region">Communauté de Madrid</region>
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<author><name sortKey="Royuela, Ana" sort="Royuela, Ana" uniqKey="Royuela A" first="Ana" last="Royuela">Ana Royuela</name>
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<author><name sortKey="Trigo, Patricia" sort="Trigo, Patricia" uniqKey="Trigo P" first="Patricia" last="Trigo">Patricia Trigo</name>
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<author><name sortKey="Orth, Michael" sort="Orth, Michael" uniqKey="Orth M" first="Michael" last="Orth">Michael Orth</name>
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<s2>Ulm</s2>
<s3>DEU</s3>
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<author><name sortKey="Lange, Herwig" sort="Lange, Herwig" uniqKey="Lange H" first="Herwig" last="Lange">Herwig Lange</name>
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<author><name sortKey="Reilmann, Ralf" sort="Reilmann, Ralf" uniqKey="Reilmann R" first="Ralf" last="Reilmann">Ralf Reilmann</name>
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<author><name sortKey="Keylock, Jennifer" sort="Keylock, Jennifer" uniqKey="Keylock J" first="Jennifer" last="Keylock">Jennifer Keylock</name>
<affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Department of Neurology, University of Birmingham</s1>
<s2>Birmingham</s2>
<s3>GBR</s3>
<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
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<region type="région" nuts="1">Midlands de l'Ouest</region>
</placeName>
<orgName type="university">Université de Birmingham</orgName>
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<author><name sortKey="Rickards, Hugh" sort="Rickards, Hugh" uniqKey="Rickards H" first="Hugh" last="Rickards">Hugh Rickards</name>
<affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Department of Neurology, University of Birmingham</s1>
<s2>Birmingham</s2>
<s3>GBR</s3>
<sZ>7 aut.</sZ>
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<placeName><settlement type="city">Birmingham</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Midlands de l'Ouest</region>
</placeName>
<orgName type="university">Université de Birmingham</orgName>
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<author><name sortKey="Piacentini, Silvia" sort="Piacentini, Silvia" uniqKey="Piacentini S" first="Silvia" last="Piacentini">Silvia Piacentini</name>
<affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Department of Neurology, University of Florence</s1>
<s2>Florence</s2>
<s3>ITA</s3>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>Italie</country>
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<author><name sortKey="Squitieri, Ferdinando" sort="Squitieri, Ferdinando" uniqKey="Squitieri F" first="Ferdinando" last="Squitieri">Ferdinando Squitieri</name>
<affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Neurogenetics and Rare Disease Centre, IRCCS Neuromed</s1>
<s2>Pozzili</s2>
<s3>ITA</s3>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>Italie</country>
<wicri:noRegion>Pozzili</wicri:noRegion>
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</author>
<author><name sortKey="Landwehrmeyer, Bernhard" sort="Landwehrmeyer, Bernhard" uniqKey="Landwehrmeyer B" first="Bernhard" last="Landwehrmeyer">Bernhard Landwehrmeyer</name>
<affiliation wicri:level="4"><inist:fA14 i1="05"><s1>Department of Neurology, University of Ulm</s1>
<s2>Ulm</s2>
<s3>DEU</s3>
<sZ>4 aut.</sZ>
<sZ>11 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
<placeName><region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Tübingen</region>
<settlement type="city">Ulm</settlement>
</placeName>
<orgName type="university">Université d'Ulm</orgName>
</affiliation>
</author>
<author><name sortKey="Witjes Ane, Marie Noelle" sort="Witjes Ane, Marie Noelle" uniqKey="Witjes Ane M" first="Marie-Noelle" last="Witjes-Ane">Marie-Noelle Witjes-Ane</name>
<affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Department of Neurology, Leiden University Medical Centre</s1>
<s2>Leiden</s2>
<s3>NLD</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
<sZ>14 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<placeName><settlement type="city">Leyde</settlement>
<region nuts="2" type="province">Hollande-Méridionale</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Jurgens, Caroline K" sort="Jurgens, Caroline K" uniqKey="Jurgens C" first="Caroline K." last="Jurgens">Caroline K. Jurgens</name>
<affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Department of Neurology, Leiden University Medical Centre</s1>
<s2>Leiden</s2>
<s3>NLD</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
<sZ>14 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<placeName><settlement type="city">Leyde</settlement>
<region nuts="2" type="province">Hollande-Méridionale</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Roos, Raymund A C" sort="Roos, Raymund A C" uniqKey="Roos R" first="Raymund A. C." last="Roos">Raymund A. C. Roos</name>
<affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Department of Neurology, Leiden University Medical Centre</s1>
<s2>Leiden</s2>
<s3>NLD</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
<sZ>14 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<placeName><settlement type="city">Leyde</settlement>
<region nuts="2" type="province">Hollande-Méridionale</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Abraira, Victor" sort="Abraira, Victor" uniqKey="Abraira V" first="Victor" last="Abraira">Victor Abraira</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CIBERESP</s1>
<s2>Madrid</s2>
<s3>ESP</s3>
<sZ>2 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
<placeName><settlement type="city">Madrid</settlement>
<region nuts="2" type="region">Communauté de Madrid</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G." last="De Yebenes">Justo G. De Yebenes</name>
<affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Neurology, Hospital Ramon y Cajal</s1>
<s2>Madrid</s2>
<s3>ESP</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>16 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
<placeName><settlement type="city">Madrid</settlement>
<region nuts="2" type="region">Communauté de Madrid</region>
</placeName>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>CIBERNED, ISCIII</s1>
<s2>Madrid</s2>
<s3>ESP</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>16 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
<placeName><settlement type="city">Madrid</settlement>
<region nuts="2" type="region">Communauté de Madrid</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="2011">2011</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Huntington disease</term>
<term>Nervous system diseases</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Chorée de Huntington</term>
<term>Pathologie du système nerveux</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Huntington's disease (HD) is a neurodegenerative disease caused by a cytosine adenosine guanine (CAG) expansion in the huntingtin gene. The length of the triplet repeat is the most important factor in determining age of onset and the severity of the disease, but substantial variability of these parameters is attributed to other factors. To investigate the relationship between the years of education and the age at onset and the severity of the phenotype in patients with HD, we applied multiple linear regression analysis to examine the impact of education on the age at onset and the severity of the clinical scores assessed by the Unified Huntington's Disease Rating Scale (UHDRS) of 891 patients with HD from the multinational observational study "Registry" conducted by the European Huntintgton's Disease Network. The model was adjusted for CAG repeat length and age at the time of assessment. Patients with lengthier education exhibited earlier estimated age at onset but less severe clinical scores (motor = -3.6, P = 0.006; cognitive = 27.0, P < 0.001; behavioral = -3.0, P < 0.001; and functional capacity = <sub>1</sub>
.1 points, P < 0.001) than those with shorter education, after controlling for age and number of CAG repeats. These differences persisted throughout all quartiles of disease severity. An earlier recognition of symptoms and manifestations among the more educated patients could explain the earlier estimated age at onset in this group. The link between better clinical UHDRS scores and higher education might reflect a beneficial effect of education or its covariates on the course of HD.</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Espagne</li>
<li>Italie</li>
<li>Pays-Bas</li>
<li>Royaume-Uni</li>
</country>
<region><li>Angleterre</li>
<li>Bade-Wurtemberg</li>
<li>Communauté de Madrid</li>
<li>District de Tübingen</li>
<li>Hollande-Méridionale</li>
<li>Midlands de l'Ouest</li>
</region>
<settlement><li>Birmingham</li>
<li>Leyde</li>
<li>Madrid</li>
<li>Ulm</li>
</settlement>
<orgName><li>Université d'Ulm</li>
<li>Université de Birmingham</li>
</orgName>
</list>
<tree><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="Lopez Sendon, Jose Luis" sort="Lopez Sendon, Jose Luis" uniqKey="Lopez Sendon J" first="Jose Luis" last="Lopez-Sendon">Jose Luis Lopez-Sendon</name>
</region>
<name sortKey="Abraira, Victor" sort="Abraira, Victor" uniqKey="Abraira V" first="Victor" last="Abraira">Victor Abraira</name>
<name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G." last="De Yebenes">Justo G. De Yebenes</name>
<name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G." last="De Yebenes">Justo G. De Yebenes</name>
<name sortKey="Lopez Sendon, Jose Luis" sort="Lopez Sendon, Jose Luis" uniqKey="Lopez Sendon J" first="Jose Luis" last="Lopez-Sendon">Jose Luis Lopez-Sendon</name>
<name sortKey="Royuela, Ana" sort="Royuela, Ana" uniqKey="Royuela A" first="Ana" last="Royuela">Ana Royuela</name>
<name sortKey="Royuela, Ana" sort="Royuela, Ana" uniqKey="Royuela A" first="Ana" last="Royuela">Ana Royuela</name>
<name sortKey="Trigo, Patricia" sort="Trigo, Patricia" uniqKey="Trigo P" first="Patricia" last="Trigo">Patricia Trigo</name>
<name sortKey="Trigo, Patricia" sort="Trigo, Patricia" uniqKey="Trigo P" first="Patricia" last="Trigo">Patricia Trigo</name>
</country>
<country name="Allemagne"><region name="Bade-Wurtemberg"><name sortKey="Orth, Michael" sort="Orth, Michael" uniqKey="Orth M" first="Michael" last="Orth">Michael Orth</name>
</region>
<name sortKey="Landwehrmeyer, Bernhard" sort="Landwehrmeyer, Bernhard" uniqKey="Landwehrmeyer B" first="Bernhard" last="Landwehrmeyer">Bernhard Landwehrmeyer</name>
<name sortKey="Lange, Herwig" sort="Lange, Herwig" uniqKey="Lange H" first="Herwig" last="Lange">Herwig Lange</name>
<name sortKey="Reilmann, Ralf" sort="Reilmann, Ralf" uniqKey="Reilmann R" first="Ralf" last="Reilmann">Ralf Reilmann</name>
</country>
<country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Keylock, Jennifer" sort="Keylock, Jennifer" uniqKey="Keylock J" first="Jennifer" last="Keylock">Jennifer Keylock</name>
</region>
<name sortKey="Rickards, Hugh" sort="Rickards, Hugh" uniqKey="Rickards H" first="Hugh" last="Rickards">Hugh Rickards</name>
</country>
<country name="Italie"><noRegion><name sortKey="Piacentini, Silvia" sort="Piacentini, Silvia" uniqKey="Piacentini S" first="Silvia" last="Piacentini">Silvia Piacentini</name>
</noRegion>
<name sortKey="Squitieri, Ferdinando" sort="Squitieri, Ferdinando" uniqKey="Squitieri F" first="Ferdinando" last="Squitieri">Ferdinando Squitieri</name>
</country>
<country name="Pays-Bas"><region name="Hollande-Méridionale"><name sortKey="Witjes Ane, Marie Noelle" sort="Witjes Ane, Marie Noelle" uniqKey="Witjes Ane M" first="Marie-Noelle" last="Witjes-Ane">Marie-Noelle Witjes-Ane</name>
</region>
<name sortKey="Jurgens, Caroline K" sort="Jurgens, Caroline K" uniqKey="Jurgens C" first="Caroline K." last="Jurgens">Caroline K. Jurgens</name>
<name sortKey="Roos, Raymund A C" sort="Roos, Raymund A C" uniqKey="Roos R" first="Raymund A. C." last="Roos">Raymund A. C. Roos</name>
</country>
</tree>
</affiliations>
</record>
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