What is the impact of education on Huntington's disease?
Identifieur interne : 003056 ( Ncbi/Checkpoint ); précédent : 003055; suivant : 003057What is the impact of education on Huntington's disease?
Auteurs : Jose Luis L Pez-Send N [Espagne] ; Ana Royuela ; Patricia Trigo ; Michael Orth ; Herwig Lange ; Ralf Reilmann ; Jennifer Keylock ; Hugh Rickards ; Silvia Piacentini ; Ferdinando Squitieri ; Bernhard Landwehrmeyer ; Marie-Noelle Witjes-Ane ; Caroline K. Jurgens ; Raymund A C. Roos ; Victor Abraira ; Justo G. De YébenesSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2011.
English descriptors
- KwdEn :
- Adult, Age of Onset, Cognition Disorders (etiology), Educational Status, Family Health, Female, Humans, Huntington Disease (complications), Huntington Disease (genetics), Huntington Disease (psychology), Male, Mental Disorders (etiology), Middle Aged, Phenotype, Regression Analysis, Severity of Illness Index, Trinucleotide Repeat Expansion (genetics).
- MESH :
- complications : Huntington Disease.
- etiology : Cognition Disorders, Mental Disorders.
- genetics : Huntington Disease, Trinucleotide Repeat Expansion.
- psychology : Huntington Disease.
- Adult, Age of Onset, Educational Status, Family Health, Female, Humans, Male, Middle Aged, Phenotype, Regression Analysis, Severity of Illness Index.
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.
DOI: 10.1002/mds.23385
PubMed: 21432905
Affiliations:
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Jurgens</name></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></author><author><name sortKey="Abraira, Victor" sort="Abraira, Victor" uniqKey="Abraira V" first="Victor" last="Abraira">Victor Abraira</name></author><author><name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G" last="De Yébenes">Justo G. De Yébenes</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2011">2011</date><idno type="doi">10.1002/mds.23385</idno><idno type="RBID">pubmed:21432905</idno><idno type="pmid">21432905</idno><idno type="wicri:Area/PubMed/Corpus">001368</idno><idno type="wicri:Area/PubMed/Curation">001368</idno><idno type="wicri:Area/PubMed/Checkpoint">000F93</idno><idno type="wicri:Area/Ncbi/Merge">003056</idno><idno type="wicri:Area/Ncbi/Curation">003056</idno><idno type="wicri:Area/Ncbi/Checkpoint">003056</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">What is the impact of education on Huntington's disease?</title><author><name sortKey="L Pez Send N, Jose Luis" sort="L Pez Send N, Jose Luis" uniqKey="L Pez Send N J" first="Jose Luis" last="L Pez-Send N">Jose Luis L Pez-Send N</name><affiliation wicri:level="3"><nlm:affiliation>Department of Neurology, Hospital Ramon y Cajal, Madrid, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Department of Neurology, Hospital Ramon y Cajal, Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Royuela, Ana" sort="Royuela, Ana" uniqKey="Royuela A" first="Ana" last="Royuela">Ana Royuela</name></author><author><name sortKey="Trigo, Patricia" sort="Trigo, Patricia" uniqKey="Trigo P" first="Patricia" last="Trigo">Patricia Trigo</name></author><author><name sortKey="Orth, Michael" sort="Orth, Michael" uniqKey="Orth M" first="Michael" last="Orth">Michael Orth</name></author><author><name sortKey="Lange, Herwig" sort="Lange, Herwig" uniqKey="Lange H" first="Herwig" last="Lange">Herwig Lange</name></author><author><name sortKey="Reilmann, Ralf" sort="Reilmann, Ralf" uniqKey="Reilmann R" first="Ralf" last="Reilmann">Ralf Reilmann</name></author><author><name sortKey="Keylock, Jennifer" sort="Keylock, Jennifer" uniqKey="Keylock J" first="Jennifer" last="Keylock">Jennifer Keylock</name></author><author><name sortKey="Rickards, Hugh" sort="Rickards, Hugh" uniqKey="Rickards H" first="Hugh" last="Rickards">Hugh Rickards</name></author><author><name sortKey="Piacentini, Silvia" sort="Piacentini, Silvia" uniqKey="Piacentini S" first="Silvia" last="Piacentini">Silvia Piacentini</name></author><author><name sortKey="Squitieri, Ferdinando" sort="Squitieri, Ferdinando" uniqKey="Squitieri F" first="Ferdinando" last="Squitieri">Ferdinando Squitieri</name></author><author><name sortKey="Landwehrmeyer, Bernhard" sort="Landwehrmeyer, Bernhard" uniqKey="Landwehrmeyer B" first="Bernhard" last="Landwehrmeyer">Bernhard Landwehrmeyer</name></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></author><author><name sortKey="Jurgens, Caroline K" sort="Jurgens, Caroline K" uniqKey="Jurgens C" first="Caroline K" last="Jurgens">Caroline K. Jurgens</name></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></author><author><name sortKey="Abraira, Victor" sort="Abraira, Victor" uniqKey="Abraira V" first="Victor" last="Abraira">Victor Abraira</name></author><author><name sortKey="De Yebenes, Justo G" sort="De Yebenes, Justo G" uniqKey="De Yebenes J" first="Justo G" last="De Yébenes">Justo G. De Yébenes</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2011" type="published">2011</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Age of Onset</term><term>Cognition Disorders (etiology)</term><term>Educational Status</term><term>Family Health</term><term>Female</term><term>Humans</term><term>Huntington Disease (complications)</term><term>Huntington Disease (genetics)</term><term>Huntington Disease (psychology)</term><term>Male</term><term>Mental Disorders (etiology)</term><term>Middle Aged</term><term>Phenotype</term><term>Regression Analysis</term><term>Severity of Illness Index</term><term>Trinucleotide Repeat Expansion (genetics)</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Cognition Disorders</term><term>Mental Disorders</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Huntington Disease</term><term>Trinucleotide Repeat Expansion</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Age of Onset</term><term>Educational Status</term><term>Family Health</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Phenotype</term><term>Regression Analysis</term><term>Severity of Illness Index</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 = 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.</div></front></TEI><affiliations><list><country><li>Espagne</li></country><region><li>Communauté de Madrid</li></region><settlement><li>Madrid</li></settlement></list><tree><noCountry><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 Yébenes">Justo G. De Yébenes</name><name sortKey="Jurgens, Caroline K" sort="Jurgens, Caroline K" uniqKey="Jurgens C" first="Caroline K" last="Jurgens">Caroline K. Jurgens</name><name sortKey="Keylock, Jennifer" sort="Keylock, Jennifer" uniqKey="Keylock J" first="Jennifer" last="Keylock">Jennifer Keylock</name><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="Orth, Michael" sort="Orth, Michael" uniqKey="Orth M" first="Michael" last="Orth">Michael Orth</name><name sortKey="Piacentini, Silvia" sort="Piacentini, Silvia" uniqKey="Piacentini S" first="Silvia" last="Piacentini">Silvia Piacentini</name><name sortKey="Reilmann, Ralf" sort="Reilmann, Ralf" uniqKey="Reilmann R" first="Ralf" last="Reilmann">Ralf Reilmann</name><name sortKey="Rickards, Hugh" sort="Rickards, Hugh" uniqKey="Rickards H" first="Hugh" last="Rickards">Hugh Rickards</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><name sortKey="Royuela, Ana" sort="Royuela, Ana" uniqKey="Royuela A" first="Ana" last="Royuela">Ana Royuela</name><name sortKey="Squitieri, Ferdinando" sort="Squitieri, Ferdinando" uniqKey="Squitieri F" first="Ferdinando" last="Squitieri">Ferdinando Squitieri</name><name sortKey="Trigo, Patricia" sort="Trigo, Patricia" uniqKey="Trigo P" first="Patricia" last="Trigo">Patricia Trigo</name><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></noCountry><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="L Pez Send N, Jose Luis" sort="L Pez Send N, Jose Luis" uniqKey="L Pez Send N J" first="Jose Luis" last="L Pez-Send N">Jose Luis L Pez-Send N</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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