Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study
Identifieur interne : 000718 ( Istex/Corpus ); précédent : 000717; suivant : 000719Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study
Auteurs : Tanja Schmitz-Hübsch ; Mathieu Coudert ; Paola Giunti ; Christoph Globas ; Laszlo Baliko ; Roberto Fancellu ; Caterina Mariotti ; Alessandro Filla ; Maryla Rakowicz ; Perrine Charles ; Pascale Ribai ; Sandra Szymanski ; Jon Infante ; Bart P. C. Van De Warrenburg ; Alexandra Dürr ; Dagmar Timmann ; Sylvia Boesch ; Rafal Rola ; Chantal Depondt ; Ludger Schöls ; Elszbieta Zdzienicka ; Jun-Suk Kang ; Susanne Ratzka ; Berry Kremer ; Jörg B. Schulz ; Thomas Klopstock ; Bela Melegh ; Sophie Tezenas Du Montcel ; Thomas KlockgetherSource :
- Movement Disorders [ 0885-3185 ] ; 2010-04-15.
English descriptors
Abstract
Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society
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DOI: 10.1002/mds.22740
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title><author><name sortKey="Schmitz Bsch, Tanja" sort="Schmitz Bsch, Tanja" uniqKey="Schmitz Bsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</mods:affiliation></affiliation></author><author><name sortKey="Coudert, Mathieu" sort="Coudert, Mathieu" uniqKey="Coudert M" first="Mathieu" last="Coudert">Mathieu Coudert</name><affiliation><mods:affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name><affiliation><mods:affiliation>Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Globas, Christoph" sort="Globas, Christoph" uniqKey="Globas C" first="Christoph" last="Globas">Christoph Globas</name><affiliation><mods:affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name><affiliation><mods:affiliation>Department of Neurology and Stroke, County Hospital, Veszprém, Hungary</mods:affiliation></affiliation></author><author><name sortKey="Fancellu, Roberto" sort="Fancellu, Roberto" uniqKey="Fancellu R" first="Roberto" last="Fancellu">Roberto Fancellu</name><affiliation><mods:affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</mods:affiliation></affiliation></author><author><name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name><affiliation><mods:affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</mods:affiliation></affiliation></author><author><name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name><affiliation><mods:affiliation>Department of Neurology, University of Naples, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Rakowicz, Maryla" sort="Rakowicz, Maryla" uniqKey="Rakowicz M" first="Maryla" last="Rakowicz">Maryla Rakowicz</name><affiliation><mods:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Ribai, Pascale" sort="Ribai, Pascale" uniqKey="Ribai P" first="Pascale" last="Ribai">Pascale Ribai</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name><affiliation><mods:affiliation>Department of Neurology, St. Josef Hospital, University Hospital of Bochum, Bochum, Germany</mods:affiliation></affiliation></author><author><name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name><affiliation><mods:affiliation>Department of Neurology, University Hospital “Marqués de Valldecilla,” CIBERNED, Santander, Spain</mods:affiliation></affiliation></author><author><name sortKey="Van De Warrenburg, Bart P C" sort="Van De Warrenburg, Bart P C" uniqKey="Van De Warrenburg B" first="Bart P. C." last="Van De Warrenburg">Bart P. C. Van De Warrenburg</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Essen, Essen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation><mods:affiliation>Department of Neurology, University of Innsbruck, Innsbruck, Austria</mods:affiliation></affiliation></author><author><name sortKey="Rola, Rafal" sort="Rola, Rafal" uniqKey="Rola R" first="Rafal" last="Rola">Rafal Rola</name><affiliation><mods:affiliation>First Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Depondt, Chantal" sort="Depondt, Chantal" uniqKey="Depondt C" first="Chantal" last="Depondt">Chantal Depondt</name><affiliation><mods:affiliation>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium</mods:affiliation></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation><mods:affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Zdzienicka, Elszbieta" sort="Zdzienicka, Elszbieta" uniqKey="Zdzienicka E" first="Elszbieta" last="Zdzienicka">Elszbieta Zdzienicka</name><affiliation><mods:affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Kang, Jun Uk" sort="Kang, Jun Uk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name><affiliation><mods:affiliation>Department of Neurology, University of Frankfurt, Frankfurt/M, Germany</mods:affiliation></affiliation></author><author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name><affiliation><mods:affiliation>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Kremer, Berry" sort="Kremer, Berry" uniqKey="Kremer B" first="Berry" last="Kremer">Berry Kremer</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Schulz, Jorg B" sort="Schulz, Jorg B" uniqKey="Schulz J" first="Jörg B." last="Schulz">Jörg B. Schulz</name><affiliation><mods:affiliation>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name><affiliation><mods:affiliation>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</mods:affiliation></affiliation></author><author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name><affiliation><mods:affiliation>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</mods:affiliation></affiliation></author><author><name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name><affiliation><mods:affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:F565C9E4389796ABDE540475935DE23A74B8DFC4</idno><date when="2010" year="2010">2010</date><idno type="doi">10.1002/mds.22740</idno><idno type="url">https://api.istex.fr/document/F565C9E4389796ABDE540475935DE23A74B8DFC4/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">000718</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title><author><name sortKey="Schmitz Bsch, Tanja" sort="Schmitz Bsch, Tanja" uniqKey="Schmitz Bsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</mods:affiliation></affiliation></author><author><name sortKey="Coudert, Mathieu" sort="Coudert, Mathieu" uniqKey="Coudert M" first="Mathieu" last="Coudert">Mathieu Coudert</name><affiliation><mods:affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name><affiliation><mods:affiliation>Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Globas, Christoph" sort="Globas, Christoph" uniqKey="Globas C" first="Christoph" last="Globas">Christoph Globas</name><affiliation><mods:affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name><affiliation><mods:affiliation>Department of Neurology and Stroke, County Hospital, Veszprém, Hungary</mods:affiliation></affiliation></author><author><name sortKey="Fancellu, Roberto" sort="Fancellu, Roberto" uniqKey="Fancellu R" first="Roberto" last="Fancellu">Roberto Fancellu</name><affiliation><mods:affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</mods:affiliation></affiliation></author><author><name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name><affiliation><mods:affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</mods:affiliation></affiliation></author><author><name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name><affiliation><mods:affiliation>Department of Neurology, University of Naples, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Rakowicz, Maryla" sort="Rakowicz, Maryla" uniqKey="Rakowicz M" first="Maryla" last="Rakowicz">Maryla Rakowicz</name><affiliation><mods:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Ribai, Pascale" sort="Ribai, Pascale" uniqKey="Ribai P" first="Pascale" last="Ribai">Pascale Ribai</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name><affiliation><mods:affiliation>Department of Neurology, St. Josef Hospital, University Hospital of Bochum, Bochum, Germany</mods:affiliation></affiliation></author><author><name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name><affiliation><mods:affiliation>Department of Neurology, University Hospital “Marqués de Valldecilla,” CIBERNED, Santander, Spain</mods:affiliation></affiliation></author><author><name sortKey="Van De Warrenburg, Bart P C" sort="Van De Warrenburg, Bart P C" uniqKey="Van De Warrenburg B" first="Bart P. C." last="Van De Warrenburg">Bart P. C. Van De Warrenburg</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name><affiliation><mods:affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Essen, Essen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation><mods:affiliation>Department of Neurology, University of Innsbruck, Innsbruck, Austria</mods:affiliation></affiliation></author><author><name sortKey="Rola, Rafal" sort="Rola, Rafal" uniqKey="Rola R" first="Rafal" last="Rola">Rafal Rola</name><affiliation><mods:affiliation>First Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Depondt, Chantal" sort="Depondt, Chantal" uniqKey="Depondt C" first="Chantal" last="Depondt">Chantal Depondt</name><affiliation><mods:affiliation>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium</mods:affiliation></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation><mods:affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Zdzienicka, Elszbieta" sort="Zdzienicka, Elszbieta" uniqKey="Zdzienicka E" first="Elszbieta" last="Zdzienicka">Elszbieta Zdzienicka</name><affiliation><mods:affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Kang, Jun Uk" sort="Kang, Jun Uk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name><affiliation><mods:affiliation>Department of Neurology, University of Frankfurt, Frankfurt/M, Germany</mods:affiliation></affiliation></author><author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name><affiliation><mods:affiliation>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Kremer, Berry" sort="Kremer, Berry" uniqKey="Kremer B" first="Berry" last="Kremer">Berry Kremer</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Schulz, Jorg B" sort="Schulz, Jorg B" uniqKey="Schulz J" first="Jörg B." last="Schulz">Jörg B. Schulz</name><affiliation><mods:affiliation>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</mods:affiliation></affiliation></author><author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name><affiliation><mods:affiliation>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</mods:affiliation></affiliation></author><author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name><affiliation><mods:affiliation>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</mods:affiliation></affiliation></author><author><name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name><affiliation><mods:affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</mods:affiliation></affiliation><affiliation><mods:affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation><mods:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-04-15">2010-04-15</date><biblScope unit="vol">25</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="587">587</biblScope><biblScope unit="page" to="595">595</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">F565C9E4389796ABDE540475935DE23A74B8DFC4</idno><idno type="DOI">10.1002/mds.22740</idno><idno type="ArticleID">MDS22740</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>EQ‐5D</term><term>depression</term><term>quality of life</term><term>spinocerebellar ataxia</term><term>subjective health rating</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Tanja Schmitz‐Hübsch MD</name><affiliations><json:string>Department of Neurology, University Hospital of Bonn, Bonn, Germany</json:string></affiliations></json:item><json:item><name>Mathieu Coudert MSc</name><affiliations><json:string>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</json:string><json:string>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</json:string></affiliations></json:item><json:item><name>Paola Giunti MD</name><affiliations><json:string>Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Christoph Globas MD</name><affiliations><json:string>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</json:string></affiliations></json:item><json:item><name>Laszlo Baliko MD</name><affiliations><json:string>Department of Neurology and Stroke, County Hospital, Veszprém, Hungary</json:string></affiliations></json:item><json:item><name>Roberto Fancellu MD</name><affiliations><json:string>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</json:string></affiliations></json:item><json:item><name>Caterina Mariotti MD</name><affiliations><json:string>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</json:string></affiliations></json:item><json:item><name>Alessandro Filla MD</name><affiliations><json:string>Department of Neurology, University of Naples, Naples, Italy</json:string></affiliations></json:item><json:item><name>Maryla Rakowicz MD, PhD</name><affiliations><json:string>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland</json:string></affiliations></json:item><json:item><name>Perrine Charles MD, PhD</name><affiliations><json:string>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</json:string><json:string>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</json:string></affiliations></json:item><json:item><name>Pascale Ribai MD</name><affiliations><json:string>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</json:string><json:string>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</json:string></affiliations></json:item><json:item><name>Sandra Szymanski MD</name><affiliations><json:string>Department of Neurology, St. Josef Hospital, University Hospital of Bochum, Bochum, Germany</json:string></affiliations></json:item><json:item><name>Jon Infante MD</name><affiliations><json:string>Department of Neurology, University Hospital “Marqués de Valldecilla,” CIBERNED, Santander, Spain</json:string></affiliations></json:item><json:item><name>Bart P.C. van de Warrenburg MD</name><affiliations><json:string>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</json:string></affiliations></json:item><json:item><name>Alexandra Dürr MD, PhD</name><affiliations><json:string>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</json:string><json:string>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</json:string></affiliations></json:item><json:item><name>Dagmar Timmann MD</name><affiliations><json:string>Department of Neurology, University Hospital of Essen, Essen, Germany</json:string></affiliations></json:item><json:item><name>Sylvia Boesch MD</name><affiliations><json:string>Department of Neurology, University of Innsbruck, Innsbruck, Austria</json:string></affiliations></json:item><json:item><name>Rafal Rola MD, PhD</name><affiliations><json:string>First Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland</json:string></affiliations></json:item><json:item><name>Chantal Depondt MD, PhD</name><affiliations><json:string>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium</json:string></affiliations></json:item><json:item><name>Ludger Schöls MD</name><affiliations><json:string>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</json:string></affiliations></json:item><json:item><name>Elszbieta Zdzienicka MD</name><affiliations><json:string>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland</json:string></affiliations></json:item><json:item><name>Jun‐Suk Kang MD</name><affiliations><json:string>Department of Neurology, University of Frankfurt, Frankfurt/M, Germany</json:string></affiliations></json:item><json:item><name>Susanne Ratzka MD</name><affiliations><json:string>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</json:string></affiliations></json:item><json:item><name>Berry Kremer MD</name><affiliations><json:string>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</json:string></affiliations></json:item><json:item><name>Jörg B. Schulz MD</name><affiliations><json:string>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</json:string></affiliations></json:item><json:item><name>Thomas Klopstock MD</name><affiliations><json:string>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</json:string></affiliations></json:item><json:item><name>Bela Melegh MD</name><affiliations><json:string>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</json:string></affiliations></json:item><json:item><name>Sophie Tezenas du Montcel MD, PhD</name><affiliations><json:string>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</json:string><json:string>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</json:string></affiliations></json:item><json:item><name>Thomas Klockgether MD</name><affiliations><json:string>Department of Neurology, University Hospital of Bonn, Bonn, Germany</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>spinocerebellar ataxia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>subjective health rating</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>quality of life</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>EQ‐5D</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>depression</value></json:item></subject><language><json:string>eng</json:string></language><abstract>Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society</abstract><qualityIndicators><score>7.328</score><pdfVersion>1.3</pdfVersion><pdfPageSize>612 x 810 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1420</abstractCharCount><pdfWordCount>5270</pdfWordCount><pdfCharCount>36537</pdfCharCount><pdfPageCount>9</pdfPageCount><abstractWordCount>194</abstractWordCount></qualityIndicators><title>Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title><genre><json:string>Serial article</json:string></genre><host><volume>25</volume><pages><total>9</total><last>595</last><first>587</first></pages><issn><json:string>0885-3185</json:string></issn><issue>5</issue><subject><json:item><value>Research Article</value></json:item></subject><genre></genre><language><json:string>unknown</json:string></language><title>Movement Disorders</title><doi><json:string>10.1002/(ISSN)1531-8257</json:string></doi></host><publicationDate>2010</publicationDate><copyrightDate>2010</copyrightDate><doi><json:string>10.1002/mds.22740</json:string></doi><id>F565C9E4389796ABDE540475935DE23A74B8DFC4</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/F565C9E4389796ABDE540475935DE23A74B8DFC4/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/F565C9E4389796ABDE540475935DE23A74B8DFC4/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/F565C9E4389796ABDE540475935DE23A74B8DFC4/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><availability><p>Wiley Subscription Services, Inc., A Wiley Company</p></availability><date>2010</date></publicationStmt><notesStmt><note type="content">*Potential conflict of interest: Nothing to report.</note><note>European Union (TS‐H, CG, PG, RF, CM, AF, MR, BPCW, SB, RR, LS, EZ, J‐SK, JBS, BM, STM, TK) - No. EUROSCA/LSHM‐CT‐2004‐503304; No. GeneMove/01 GM 0503;</note><note>German Ministry of Education and Research (DT, LS, J‐SK, JBS, TK, TK) - No. 3 PO5B 019 24;</note><note>Polish Ministry of Scientific Research and Information Technology (RR)</note><note>EUROSCA - No. LSHM‐CT‐2004‐503304;</note><note>Institute of Neurology, UCl, Queen Square WC1N 3BG London UK</note><note>Robert‐Bosch Foundation (Forschungskolleg Geriatrie)</note><note>Fondazione IRCCS Neurological Institute Carlo Besta, Milan, Italy</note><note>Italian Ministry of Health - No. RF74, PSNeuro, ex art. 59/2005/11;</note><note>AIFA (Agenzia Italiana Farmaco) - EudraCT No. 2007‐003357‐85; No. AIFA FARM6H95MJ;</note><note>Italian Ministry of University (MUR, hereditary spastic paraplegias)</note><note>Italian Medicine Agency (AIFA, amyotrophic lateral sclerosis)</note><note>Italian Ataxia Association (AISA, Friedreich)</note><note>Ma‐Je‐R Ltd, Warsaw, Poland</note><note>Department of Clinical Neurophysiology</note><note>Institute of Psychiatry and Neurology, Warsaw, Poland</note><note>Private Neurological Office, Warsaw, Poland</note><note>Fondo de Investigación Sanitaria (FIS) - No. PI070014;</note><note>PHRC program, funded by AP‐HP - No. AOM03059‐R05129DD;</note><note>Era‐Net program, funded by EU and the French National Agency for Research - No. R07207HS;</note><note>HI‐Q - No. C08D004;</note><note>French National Agency for Research - No. A09057DS; No. A05221DS;</note><note>GeNeMove - No. 01GM0603;</note><note>Deutsche Forschungsgemeinschaft - No. DFG Ti 239/8‐1; No. DFG TI 239/9‐1;</note><note>Bernd Fink Foundation</note><note>German Heredoataxia Foundation</note><note>IFTZ (MUI, Austria)</note><note>Polish Ministery of Science - No. P05.B.190.29;</note><note>Santhera Pharmaceuticals</note><note>Deutsche Forschungsgemeinschaft - No. SCHO754/3‐1; No. SCHO754/4‐1;</note><note>German Research Council (BMBF)</note><note>Leukonet - No. 01GM0644;</note><note>E‐RARE - No. EUROSPA (01GM0807); No. RISCA (01GM0820);</note><note>Volkswagen Foundation - No. I/80711;</note><note>HSP‐Selbsthilfegruppe Deutschland eV</note><note>Department of Clinical Genetics</note><note>Bezirkskrankenhaus Augsburg and Klinikum Augsburg, Germany</note><note>NGFNplus and Competence Network Degenerative Dementias</note><note>Friedreich ataxia and Leber hereditary optic neuropathy</note><note>Hungarian Science Foundation (OTKA) - No. T 73430;</note><note>Science Advisory Board of the Biobanking and Biomolecular Resources Infrastructure (BBMRI)</note><note>pan‐European biobank harmonizing EU7 funded - No. INFRA‐2007‐2.2.1.16;</note><note>Deutsche Forschungsgemeinschaft (DFG) - No. KFO177;</note><note>Clinical Research Group (Innate Immunity in Chronic Neurodegeneration) - No. E‐Rare 01GM0819 RISCA;</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title><author><persName><forename type="first">Tanja</forename><surname>Schmitz‐Hübsch</surname><roleName type="degree">MD</roleName></persName><note type="correspondence"><p>Correspondence: Department of Neurology, University Hospital of Bonn, Sigmund‐Freud‐Str 25, D‐53105 Bonn, Germany</p></note><affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</affiliation></author><author><persName><forename type="first">Mathieu</forename><surname>Coudert</surname><roleName type="degree">MSc</roleName></persName><affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</affiliation><affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</affiliation></author><author><persName><forename type="first">Paola</forename><surname>Giunti</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, United Kingdom</affiliation></author><author><persName><forename type="first">Christoph</forename><surname>Globas</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</affiliation></author><author><persName><forename type="first">Laszlo</forename><surname>Baliko</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Stroke, County Hospital, Veszprém, Hungary</affiliation></author><author><persName><forename type="first">Roberto</forename><surname>Fancellu</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</affiliation></author><author><persName><forename type="first">Caterina</forename><surname>Mariotti</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</affiliation></author><author><persName><forename type="first">Alessandro</forename><surname>Filla</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University of Naples, Naples, Italy</affiliation></author><author><persName><forename type="first">Maryla</forename><surname>Rakowicz</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation></author><author><persName><forename type="first">Perrine</forename><surname>Charles</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation></author><author><persName><forename type="first">Pascale</forename><surname>Ribai</surname><roleName type="degree">MD</roleName></persName><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation></author><author><persName><forename type="first">Sandra</forename><surname>Szymanski</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, St. Josef Hospital, University Hospital of Bochum, Bochum, Germany</affiliation></author><author><persName><forename type="first">Jon</forename><surname>Infante</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University Hospital “Marqués de Valldecilla,” CIBERNED, Santander, Spain</affiliation></author><author><persName><forename type="first">Bart P.C.</forename><surname>van de Warrenburg</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</affiliation></author><author><persName><forename type="first">Alexandra</forename><surname>Dürr</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation></author><author><persName><forename type="first">Dagmar</forename><surname>Timmann</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University Hospital of Essen, Essen, Germany</affiliation></author><author><persName><forename type="first">Sylvia</forename><surname>Boesch</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University of Innsbruck, Innsbruck, Austria</affiliation></author><author><persName><forename type="first">Rafal</forename><surname>Rola</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>First Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation></author><author><persName><forename type="first">Chantal</forename><surname>Depondt</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium</affiliation></author><author><persName><forename type="first">Ludger</forename><surname>Schöls</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</affiliation></author><author><persName><forename type="first">Elszbieta</forename><surname>Zdzienicka</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation></author><author><persName><forename type="first">Jun‐Suk</forename><surname>Kang</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University of Frankfurt, Frankfurt/M, Germany</affiliation></author><author><persName><forename type="first">Susanne</forename><surname>Ratzka</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</affiliation></author><author><persName><forename type="first">Berry</forename><surname>Kremer</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</affiliation></author><author><persName><forename type="first">Jörg B.</forename><surname>Schulz</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</affiliation></author><author><persName><forename type="first">Thomas</forename><surname>Klopstock</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</affiliation></author><author><persName><forename type="first">Bela</forename><surname>Melegh</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</affiliation></author><author><persName><forename type="first">Sophie Tezenas</forename><surname>du Montcel</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</affiliation><affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</affiliation></author><author><persName><forename type="first">Thomas</forename><surname>Klockgether</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</affiliation></author></analytic><monogr><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="pISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><idno type="DOI">10.1002/(ISSN)1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-04-15"></date><biblScope unit="vol">25</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="587">587</biblScope><biblScope unit="page" to="595">595</biblScope></imprint></monogr><idno type="istex">F565C9E4389796ABDE540475935DE23A74B8DFC4</idno><idno type="DOI">10.1002/mds.22740</idno><idno type="ArticleID">MDS22740</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2010</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>spinocerebellar ataxia</term></item><item><term>subjective health rating</term></item><item><term>quality of life</term></item><item><term>EQ‐5D</term></item><item><term>depression</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Research Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2009-02-12">Received</change><change when="2009-07-11">Registration</change><change when="2010-04-15">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/F565C9E4389796ABDE540475935DE23A74B8DFC4/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName><publisherLoc>Hoboken</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1531-8257</doi><issn type="print">0885-3185</issn><issn type="electronic">1531-8257</issn><idGroup><id type="product" value="MDS"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title><title type="short">Mov. Disord.</title></titleGroup></publicationMeta><publicationMeta level="part" position="50"><doi origin="wiley" registered="yes">10.1002/mds.v25:5</doi><numberingGroup><numbering type="journalVolume" number="25">25</numbering><numbering type="journalIssue">5</numbering></numberingGroup><coverDate startDate="2010-04-15">15 April 2010</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="100" status="forIssue"><doi origin="wiley" registered="yes">10.1002/mds.22740</doi><idGroup><id type="unit" value="MDS22740"></id></idGroup><countGroup><count type="pageTotal" number="9"></count></countGroup><titleGroup><title type="articleCategory">Research Article</title><title type="tocHeading1">Research Articles</title></titleGroup><copyright ownership="thirdParty">Copyright © 2010 Movement Disorder Society</copyright><eventGroup><event type="manuscriptReceived" date="2009-02-12"></event><event type="manuscriptRevised" date="2009-06-29"></event><event 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Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af22" countryCode="DE" type="organization"><unparsedAffiliation>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af23" countryCode="DE" type="organization"><unparsedAffiliation>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af24" countryCode="HK" type="organization"><unparsedAffiliation>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">spinocerebellar ataxia</keyword><keyword xml:id="kwd2">subjective health rating</keyword><keyword xml:id="kwd3">quality of life</keyword><keyword xml:id="kwd4">EQ‐5D</keyword><keyword xml:id="kwd5">depression</keyword></keywordGroup><fundingInfo><fundingAgency>European Union (TS‐H, CG, PG, RF, CM, AF, MR, BPCW, SB, RR, LS, EZ, J‐SK, JBS, BM, STM, TK)</fundingAgency><fundingNumber>EUROSCA/LSHM‐CT‐2004‐503304</fundingNumber><fundingNumber>GeneMove/01 GM 0503</fundingNumber></fundingInfo><fundingInfo><fundingAgency>German Ministry of Education and Research (DT, LS, J‐SK, JBS, TK, TK)</fundingAgency><fundingNumber>3 PO5B 019 24</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Polish Ministry of Scientific Research and Information Technology (RR)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>EUROSCA</fundingAgency><fundingNumber>LSHM‐CT‐2004‐503304</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Institute of Neurology, UCl, Queen Square WC1N 3BG London UK</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Robert‐Bosch Foundation (Forschungskolleg Geriatrie)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Fondazione IRCCS Neurological Institute Carlo Besta, Milan, Italy</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Italian Ministry of Health</fundingAgency><fundingNumber>RF74, PSNeuro, ex art. 59/2005/11</fundingNumber></fundingInfo><fundingInfo><fundingAgency>AIFA (Agenzia Italiana Farmaco)</fundingAgency><fundingNumber>EudraCT No. 2007‐003357‐85</fundingNumber><fundingNumber>AIFA FARM6H95MJ</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Italian Ministry of University (MUR, hereditary spastic paraplegias)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Italian Medicine Agency (AIFA, amyotrophic lateral sclerosis)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Italian Ataxia Association (AISA, Friedreich)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Ma‐Je‐R Ltd, Warsaw, Poland</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Department of Clinical Neurophysiology</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Institute of Psychiatry and Neurology, Warsaw, Poland</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Private Neurological Office, Warsaw, Poland</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Fondo de Investigación Sanitaria (FIS)</fundingAgency><fundingNumber>PI070014</fundingNumber></fundingInfo><fundingInfo><fundingAgency>PHRC program, funded by AP‐HP</fundingAgency><fundingNumber>AOM03059‐R05129DD</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Era‐Net program, funded by EU and the French National Agency for Research</fundingAgency><fundingNumber>R07207HS</fundingNumber></fundingInfo><fundingInfo><fundingAgency>HI‐Q</fundingAgency><fundingNumber>C08D004</fundingNumber></fundingInfo><fundingInfo><fundingAgency>French National Agency for Research</fundingAgency><fundingNumber>A09057DS</fundingNumber><fundingNumber>A05221DS</fundingNumber></fundingInfo><fundingInfo><fundingAgency>GeNeMove</fundingAgency><fundingNumber>01GM0603</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Deutsche Forschungsgemeinschaft</fundingAgency><fundingNumber>DFG Ti 239/8‐1</fundingNumber><fundingNumber>DFG TI 239/9‐1</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Bernd Fink Foundation</fundingAgency></fundingInfo><fundingInfo><fundingAgency>German Heredoataxia Foundation</fundingAgency></fundingInfo><fundingInfo><fundingAgency>IFTZ (MUI, Austria)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Polish Ministery of Science</fundingAgency><fundingNumber>P05.B.190.29</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Santhera Pharmaceuticals</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Deutsche Forschungsgemeinschaft</fundingAgency><fundingNumber>SCHO754/3‐1</fundingNumber><fundingNumber>SCHO754/4‐1</fundingNumber></fundingInfo><fundingInfo><fundingAgency>German Research Council (BMBF)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Leukonet</fundingAgency><fundingNumber>01GM0644</fundingNumber></fundingInfo><fundingInfo><fundingAgency>E‐RARE</fundingAgency><fundingNumber>EUROSPA (01GM0807)</fundingNumber><fundingNumber>RISCA (01GM0820)</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Volkswagen Foundation</fundingAgency><fundingNumber>I/80711</fundingNumber></fundingInfo><fundingInfo><fundingAgency>HSP‐Selbsthilfegruppe Deutschland eV</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Department of Clinical Genetics</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Bezirkskrankenhaus Augsburg and Klinikum Augsburg, Germany</fundingAgency></fundingInfo><fundingInfo><fundingAgency>NGFNplus and Competence Network Degenerative Dementias</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Friedreich ataxia and Leber hereditary optic neuropathy</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Hungarian Science Foundation (OTKA)</fundingAgency><fundingNumber>T 73430</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Science Advisory Board of the Biobanking and Biomolecular Resources Infrastructure (BBMRI)</fundingAgency></fundingInfo><fundingInfo><fundingAgency>pan‐European biobank harmonizing EU7 funded</fundingAgency><fundingNumber>INFRA‐2007‐2.2.1.16</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Deutsche Forschungsgemeinschaft (DFG)</fundingAgency><fundingNumber>KFO177</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Clinical Research Group (Innate Immunity in Chronic Neurodegeneration)</fundingAgency><fundingNumber>E‐Rare 01GM0819 RISCA</fundingNumber></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn6"><p>Potential conflict of interest: Nothing to report.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Self‐Rated Health Status in SCA</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Self‐rated health status in spinocerebellar ataxia—Results from a European multicenter study</title></titleInfo><name type="personal"><namePart type="given">Tanja</namePart><namePart type="family">Schmitz‐Hübsch</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</affiliation><description>Correspondence: Department of Neurology, University Hospital of Bonn, Sigmund‐Freud‐Str 25, D‐53105 Bonn, Germany</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Mathieu</namePart><namePart type="family">Coudert</namePart><namePart type="termsOfAddress">MSc</namePart><affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</affiliation><affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Paola</namePart><namePart type="family">Giunti</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christoph</namePart><namePart type="family">Globas</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Laszlo</namePart><namePart type="family">Baliko</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Stroke, County Hospital, Veszprém, Hungary</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Roberto</namePart><namePart type="family">Fancellu</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Caterina</namePart><namePart type="family">Mariotti</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Biochemistry and Genetics, Fondazione‐IRCCS, Istituto Neurologico Carlo Besta, Milan, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Alessandro</namePart><namePart type="family">Filla</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University of Naples, Naples, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Maryla</namePart><namePart type="family">Rakowicz</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Perrine</namePart><namePart type="family">Charles</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pascale</namePart><namePart type="family">Ribai</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Sandra</namePart><namePart type="family">Szymanski</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, St. Josef Hospital, University Hospital of Bochum, Bochum, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jon</namePart><namePart type="family">Infante</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University Hospital “Marqués de Valldecilla,” CIBERNED, Santander, Spain</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Bart P.C.</namePart><namePart type="family">van de Warrenburg</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Alexandra</namePart><namePart type="family">Dürr</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale, F‐75013, Paris, France</affiliation><affiliation>AP‐HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière F‐75013, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Dagmar</namePart><namePart type="family">Timmann</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University Hospital of Essen, Essen, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Sylvia</namePart><namePart type="family">Boesch</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University of Innsbruck, Innsbruck, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Rafal</namePart><namePart type="family">Rola</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>First Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Chantal</namePart><namePart type="family">Depondt</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Ludger</namePart><namePart type="family">Schöls</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Elszbieta</namePart><namePart type="family">Zdzienicka</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jun‐Suk</namePart><namePart type="family">Kang</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University of Frankfurt, Frankfurt/M, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Susanne</namePart><namePart type="family">Ratzka</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen, Göttingen, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Berry</namePart><namePart type="family">Kremer</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jörg B.</namePart><namePart type="family">Schulz</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, RWTH Aachen, University Medical Hospital, Aachen, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Thomas</namePart><namePart type="family">Klopstock</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Friedrich‐Baur‐Institute, University Hospital of Ludwig‐Maximilians‐University, München, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Bela</namePart><namePart type="family">Melegh</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Sophie Tezenas</namePart><namePart type="family">du Montcel</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié‐Salpêtrière, Assistance Publique‐Hôpitaux, Paris, France</affiliation><affiliation>Modeling in Clinical Research, University Pierre et Marie Curie, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Thomas</namePart><namePart type="family">Klockgether</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">article</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2010-04-15</dateIssued><dateCaptured encoding="w3cdtf">2009-02-12</dateCaptured><dateValid encoding="w3cdtf">2009-07-11</dateValid><copyrightDate encoding="w3cdtf">2010</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">1</extent><extent unit="tables">3</extent><extent unit="references">29</extent><extent unit="words">7308</extent></physicalDescription><abstract lang="en">Patient‐based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ‐5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient‐reported health status was compromised in patients of all genotypes (EQ‐5D visual analogue scale (EQ‐VAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ‐VAS variance in the whole sample and might be extrapolated to other SCA genotypes. © 2010 Movement Disorder Society</abstract><note type="content">*Potential conflict of interest: Nothing to report.</note><note type="funding">European Union (TS‐H, CG, PG, RF, CM, AF, MR, BPCW, SB, RR, LS, EZ, J‐SK, JBS, BM, STM, TK) - No. EUROSCA/LSHM‐CT‐2004‐503304; No. GeneMove/01 GM 0503; </note><note type="funding">German Ministry of Education and Research (DT, LS, J‐SK, JBS, TK, TK) - No. 3 PO5B 019 24; </note><note type="funding">Polish Ministry of Scientific Research and Information Technology (RR)</note><note type="funding">EUROSCA - No. LSHM‐CT‐2004‐503304; </note><note type="funding">Institute of Neurology, UCl, Queen Square WC1N 3BG London UK</note><note type="funding">Robert‐Bosch Foundation (Forschungskolleg Geriatrie)</note><note type="funding">Fondazione IRCCS Neurological Institute Carlo Besta, Milan, Italy</note><note type="funding">Italian Ministry of Health - No. RF74, PSNeuro, ex art. 59/2005/11; </note><note type="funding">AIFA (Agenzia Italiana Farmaco) - EudraCT No. 2007‐003357‐85; No. AIFA FARM6H95MJ; </note><note type="funding">Italian Ministry of University (MUR, hereditary spastic paraplegias)</note><note type="funding">Italian Medicine Agency (AIFA, amyotrophic lateral sclerosis)</note><note type="funding">Italian Ataxia Association (AISA, Friedreich)</note><note type="funding">Ma‐Je‐R Ltd, Warsaw, Poland</note><note type="funding">Department of Clinical Neurophysiology</note><note type="funding">Institute of Psychiatry and Neurology, Warsaw, Poland</note><note type="funding">Private Neurological Office, Warsaw, Poland</note><note type="funding">Fondo de Investigación Sanitaria (FIS) - No. PI070014; </note><note type="funding">PHRC program, funded by AP‐HP - No. AOM03059‐R05129DD; </note><note type="funding">Era‐Net program, funded by EU and the French National Agency for Research - No. R07207HS; </note><note type="funding">HI‐Q - No. C08D004; </note><note type="funding">French National Agency for Research - No. A09057DS; No. A05221DS; </note><note type="funding">GeNeMove - No. 01GM0603; </note><note type="funding">Deutsche Forschungsgemeinschaft - No. DFG Ti 239/8‐1; No. DFG TI 239/9‐1; </note><note type="funding">Bernd Fink Foundation</note><note type="funding">German Heredoataxia Foundation</note><note type="funding">IFTZ (MUI, Austria)</note><note type="funding">Polish Ministery of Science - No. P05.B.190.29; </note><note type="funding">Santhera Pharmaceuticals</note><note type="funding">Deutsche Forschungsgemeinschaft - No. SCHO754/3‐1; No. SCHO754/4‐1; </note><note type="funding">German Research Council (BMBF)</note><note type="funding">Leukonet - No. 01GM0644; </note><note type="funding">E‐RARE - No. EUROSPA (01GM0807); No. RISCA (01GM0820); </note><note type="funding">Volkswagen Foundation - No. I/80711; </note><note type="funding">HSP‐Selbsthilfegruppe Deutschland eV</note><note type="funding">Department of Clinical Genetics</note><note type="funding">Bezirkskrankenhaus Augsburg and Klinikum Augsburg, Germany</note><note type="funding">NGFNplus and Competence Network Degenerative Dementias</note><note type="funding">Friedreich ataxia and Leber hereditary optic neuropathy</note><note type="funding">Hungarian Science Foundation (OTKA) - No. T 73430; </note><note type="funding">Science Advisory Board of the Biobanking and Biomolecular Resources Infrastructure (BBMRI)</note><note type="funding">pan‐European biobank harmonizing EU7 funded - No. INFRA‐2007‐2.2.1.16; </note><note type="funding">Deutsche Forschungsgemeinschaft (DFG) - No. KFO177; </note><note type="funding">Clinical Research Group (Innate Immunity in Chronic Neurodegeneration) - No. E‐Rare 01GM0819 RISCA; </note><subject lang="en"><genre>Keywords</genre><topic>spinocerebellar ataxia</topic><topic>subjective health rating</topic><topic>quality of life</topic><topic>EQ‐5D</topic><topic>depression</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><subject><genre>article category</genre><topic>Research Article</topic></subject><identifier type="ISSN">0885-3185</identifier><identifier type="eISSN">1531-8257</identifier><identifier type="DOI">10.1002/(ISSN)1531-8257</identifier><identifier type="PublisherID">MDS</identifier><part><date>2010</date><detail type="volume"><caption>vol.</caption><number>25</number></detail><detail type="issue"><caption>no.</caption><number>5</number></detail><extent unit="pages"><start>587</start><end>595</end><total>9</total></extent></part></relatedItem><identifier type="istex">F565C9E4389796ABDE540475935DE23A74B8DFC4</identifier><identifier type="DOI">10.1002/mds.22740</identifier><identifier type="ArticleID">MDS22740</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2010 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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