Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter Study
Identifieur interne : 000B45 ( PascalFrancis/Corpus ); précédent : 000B44; suivant : 000B46Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter 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.
Descripteurs français
- Pascal (Inist)
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.
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NO : | PASCAL 10-0233210 INIST |
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ET : | Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter Study |
AU : | SCHMITZ-HÜBSCH (Tanja); COUDERT (Mathieu); GIUNTI (Paola); GLOBAS (Christoph); BALIKO (Laszlo); FANCELLU (Roberto); MARIOTTI (Caterina); FILLA (Alessandro); RAKOWICZ (Maryla); CHARLES (Perrine); RIBAI (Pascale); SZYMANSKI (Sandra); INFANTE (Jon); VAN DE WARRENBURG (Bart P. C.); DÜRR (Alexandra); TIMMANN (Dagmar); BOESCH (Sylvia); ROLA (Rafal); DEPONDT (Chantal); SCHÖLS (Ludger); ZDZIENICKA (Elszbieta); KANG (Jun-Suk); RATZKA (Susanne); KREMER (Berry); SCHULZ (Jörg B.); KLOPSTOCK (Thomas); MELEGH (Bela); TEZENAS DU MONTCEL (Sophie); KLOCKGETHER (Thomas) |
AF : | Department of Neurology, University Hospital of Bonn/Bonn/Allemagne (1 aut., 29 aut.); Department of Biostatistics and Medical Informatics, Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux/Paris/France (2 aut., 28 aut.); Modeling in Clinical Research, University Pierre et Marie Curie/Paris/France (2 aut., 28 aut.); Department of Molecular Neuroscience, Institute of Neurology, Queen Square/London/Royaume-Uni (3 aut.); Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen/Tübingen/Allemagne (4 aut., 20 aut.); Department of Neurology and Stroke, County Hospital/Veszprém/Hongrie (5 aut.); Department of Biochemistry and Genetics, Fondazione-IRCCS, Istituto Neurologico Carlo Besta/Milan/Italie (6 aut., 7 aut.); Department of Neurology, University of Naples/Naples/Italie (8 aut.); Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology/Warsaw/Pologne (9 aut.); INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale/75013, Paris/France (10 aut., 11 aut., 15 aut.); AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière/75013, Paris/France (10 aut., 11 aut., 15 aut.); Department of Neurology, St. Josef Hospital, University Hospital of Bochum/Bochum/Allemagne (12 aut.); Department of Neurology, University Hospital "Marqués de Valldecilla," CIBERNED/Santander/Espagne (13 aut.); Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center/Nijnaegen/Pays-Bas (14 aut., 24 aut.); Department of Neurology, University Hospital of Essen/Essen/Allemagne (16 aut.); Department of Neurology, University of Innsbruck/Innsbruck/Autriche (17 aut.); First Department of Neurology, Institute of Psychiatry and Neurology/Warsaw/Pologne (18 aut.); Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles/Brussels/Belgique (19 aut.); Department of Genetics, Institute of Psychiatry and Neurology/Warsaw/Pologne (21 aut.); Department of Neurology, University of Frankfurt/Frankfurt/M/Allemagne (22 aut.); Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen/Göttingen/Allemagne (23 aut.); Department of Neurology, RWTH Aachen, University Medical Hospital/Aachen/Allemagne (25 aut.); Department of Neurology, Friedrich-Baur-Institute, University Hospital of Ludwig-Maximilians-University/München/Allem agne (26 aut.); Department of Medical Genetics and Child Development, University of Pécs/Pécs/Hongrie (27 aut.) |
DT : | Publication en série; Niveau analytique |
SO : | Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2010; Vol. 25; No. 5; Pp. 587-595; Bibl. 29 ref. |
LA : | Anglais |
EA : | 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. |
CC : | 002B17; 002B17A03 |
FD : | Etat dépressif; Pathologie du système nerveux; Etat sanitaire; Ataxie spinocérébelleuse; Qualité de vie |
FG : | Maladie dégénérative; Maladie héréditaire; Pathologie du système nerveux central; Trouble de l'humeur |
ED : | Depression; Nervous system diseases; Health status; Spinocerebellar ataxia; Quality of life |
EG : | Degenerative disease; Genetic disease; Central nervous system disease; Mood disorder |
SD : | Estado depresivo; Sistema nervioso patología; Estado sanitario; Ataxia spinocerebelosa; Calidad vida |
LO : | INIST-20953.354000181079390100 |
ID : | 10-0233210 |
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Pascal:10-0233210Le document en format XML
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<author><name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
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<author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name>
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<author><name sortKey="Kremer, Berry" sort="Kremer, Berry" uniqKey="Kremer B" first="Berry" last="Kremer">Berry Kremer</name>
<affiliation><inist:fA14 i1="14"><s1>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center</s1>
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<author><name sortKey="Schulz, Jorg B" sort="Schulz, Jorg B" uniqKey="Schulz J" first="Jörg B." last="Schulz">Jörg B. Schulz</name>
<affiliation><inist:fA14 i1="22"><s1>Department of Neurology, RWTH Aachen, University Medical Hospital</s1>
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<s3>DEU</s3>
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<author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name>
<affiliation><inist:fA14 i1="23"><s1>Department of Neurology, Friedrich-Baur-Institute, University Hospital of Ludwig-Maximilians-University</s1>
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<author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name>
<affiliation><inist:fA14 i1="24"><s1>Department of Medical Genetics and Child Development, University of Pécs</s1>
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<s3>HUN</s3>
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</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Tezenas Du Montcel, Sophie" sort="Tezenas Du Montcel, Sophie" uniqKey="Tezenas Du Montcel S" first="Sophie" last="Tezenas Du Montcel">Sophie Tezenas Du Montcel</name>
<affiliation><inist:fA14 i1="02"><s1>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="03"><s1>Modeling in Clinical Research, University Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
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</author>
<author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<affiliation><inist:fA14 i1="01"><s1>Department of Neurology, University Hospital of Bonn</s1>
<s2>Bonn</s2>
<s3>DEU</s3>
<sZ>1 aut.</sZ>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter Study</title>
<author><name sortKey="Schmitz Hubsch, Tanja" sort="Schmitz Hubsch, Tanja" uniqKey="Schmitz Hubsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name>
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<author><name sortKey="Coudert, Mathieu" sort="Coudert, Mathieu" uniqKey="Coudert M" first="Mathieu" last="Coudert">Mathieu Coudert</name>
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</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="03"><s1>Modeling in Clinical Research, University Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name>
<affiliation><inist:fA14 i1="04"><s1>Department of Molecular Neuroscience, Institute of Neurology, Queen Square</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>3 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Globas, Christoph" sort="Globas, Christoph" uniqKey="Globas C" first="Christoph" last="Globas">Christoph Globas</name>
<affiliation><inist:fA14 i1="05"><s1>Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen</s1>
<s2>Tübingen</s2>
<s3>DEU</s3>
<sZ>4 aut.</sZ>
<sZ>20 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name>
<affiliation><inist:fA14 i1="06"><s1>Department of Neurology and Stroke, County Hospital</s1>
<s2>Veszprém</s2>
<s3>HUN</s3>
<sZ>5 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Fancellu, Roberto" sort="Fancellu, Roberto" uniqKey="Fancellu R" first="Roberto" last="Fancellu">Roberto Fancellu</name>
<affiliation><inist:fA14 i1="07"><s1>Department of Biochemistry and Genetics, Fondazione-IRCCS, Istituto Neurologico Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name>
<affiliation><inist:fA14 i1="07"><s1>Department of Biochemistry and Genetics, Fondazione-IRCCS, Istituto Neurologico Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name>
<affiliation><inist:fA14 i1="08"><s1>Department of Neurology, University of Naples</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>8 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Rakowicz, Maryla" sort="Rakowicz, Maryla" uniqKey="Rakowicz M" first="Maryla" last="Rakowicz">Maryla Rakowicz</name>
<affiliation><inist:fA14 i1="09"><s1>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>9 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<affiliation><inist:fA14 i1="10"><s1>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="11"><s1>AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Ribai, Pascale" sort="Ribai, Pascale" uniqKey="Ribai P" first="Pascale" last="Ribai">Pascale Ribai</name>
<affiliation><inist:fA14 i1="10"><s1>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="11"><s1>AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name>
<affiliation><inist:fA14 i1="12"><s1>Department of Neurology, St. Josef Hospital, University Hospital of Bochum</s1>
<s2>Bochum</s2>
<s3>DEU</s3>
<sZ>12 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name>
<affiliation><inist:fA14 i1="13"><s1>Department of Neurology, University Hospital "Marqués de Valldecilla," CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>13 aut.</sZ>
</inist:fA14>
</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><inist:fA14 i1="14"><s1>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center</s1>
<s2>Nijnaegen</s2>
<s3>NLD</s3>
<sZ>14 aut.</sZ>
<sZ>24 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<affiliation><inist:fA14 i1="10"><s1>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="11"><s1>AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name>
<affiliation><inist:fA14 i1="15"><s1>Department of Neurology, University Hospital of Essen</s1>
<s2>Essen</s2>
<s3>DEU</s3>
<sZ>16 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
<affiliation><inist:fA14 i1="16"><s1>Department of Neurology, University of Innsbruck</s1>
<s2>Innsbruck</s2>
<s3>AUT</s3>
<sZ>17 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Rola, Rafal" sort="Rola, Rafal" uniqKey="Rola R" first="Rafal" last="Rola">Rafal Rola</name>
<affiliation><inist:fA14 i1="17"><s1>First Department of Neurology, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>18 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Depondt, Chantal" sort="Depondt, Chantal" uniqKey="Depondt C" first="Chantal" last="Depondt">Chantal Depondt</name>
<affiliation><inist:fA14 i1="18"><s1>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles</s1>
<s2>Brussels</s2>
<s3>BEL</s3>
<sZ>19 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name>
<affiliation><inist:fA14 i1="05"><s1>Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen</s1>
<s2>Tübingen</s2>
<s3>DEU</s3>
<sZ>4 aut.</sZ>
<sZ>20 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Zdzienicka, Elszbieta" sort="Zdzienicka, Elszbieta" uniqKey="Zdzienicka E" first="Elszbieta" last="Zdzienicka">Elszbieta Zdzienicka</name>
<affiliation><inist:fA14 i1="19"><s1>Department of Genetics, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>21 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
<affiliation><inist:fA14 i1="20"><s1>Department of Neurology, University of Frankfurt</s1>
<s2>Frankfurt/M</s2>
<s3>DEU</s3>
<sZ>22 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name>
<affiliation><inist:fA14 i1="21"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen</s1>
<s2>Göttingen</s2>
<s3>DEU</s3>
<sZ>23 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Kremer, Berry" sort="Kremer, Berry" uniqKey="Kremer B" first="Berry" last="Kremer">Berry Kremer</name>
<affiliation><inist:fA14 i1="14"><s1>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center</s1>
<s2>Nijnaegen</s2>
<s3>NLD</s3>
<sZ>14 aut.</sZ>
<sZ>24 aut.</sZ>
</inist:fA14>
</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><inist:fA14 i1="22"><s1>Department of Neurology, RWTH Aachen, University Medical Hospital</s1>
<s2>Aachen</s2>
<s3>DEU</s3>
<sZ>25 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name>
<affiliation><inist:fA14 i1="23"><s1>Department of Neurology, Friedrich-Baur-Institute, University Hospital of Ludwig-Maximilians-University</s1>
<s2>München</s2>
<s3>DEU</s3>
<sZ>26 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name>
<affiliation><inist:fA14 i1="24"><s1>Department of Medical Genetics and Child Development, University of Pécs</s1>
<s2>Pécs</s2>
<s3>HUN</s3>
<sZ>27 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Tezenas Du Montcel, Sophie" sort="Tezenas Du Montcel, Sophie" uniqKey="Tezenas Du Montcel S" first="Sophie" last="Tezenas Du Montcel">Sophie Tezenas Du Montcel</name>
<affiliation><inist:fA14 i1="02"><s1>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation><inist:fA14 i1="03"><s1>Modeling in Clinical Research, University Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<affiliation><inist:fA14 i1="01"><s1>Department of Neurology, University Hospital of Bonn</s1>
<s2>Bonn</s2>
<s3>DEU</s3>
<sZ>1 aut.</sZ>
<sZ>29 aut.</sZ>
</inist:fA14>
</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="2010">2010</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>Depression</term>
<term>Health status</term>
<term>Nervous system diseases</term>
<term>Quality of life</term>
<term>Spinocerebellar ataxia</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Etat dépressif</term>
<term>Pathologie du système nerveux</term>
<term>Etat sanitaire</term>
<term>Ataxie spinocérébelleuse</term>
<term>Qualité de vie</term>
</keywords>
</textClass>
</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.</div>
</front>
</TEI>
<inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>0885-3185</s0>
</fA01>
<fA03 i2="1"><s0>Mov. disord.</s0>
</fA03>
<fA05><s2>25</s2>
</fA05>
<fA06><s2>5</s2>
</fA06>
<fA08 i1="01" i2="1" l="ENG"><s1>Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter Study</s1>
</fA08>
<fA11 i1="01" i2="1"><s1>SCHMITZ-HÜBSCH (Tanja)</s1>
</fA11>
<fA11 i1="02" i2="1"><s1>COUDERT (Mathieu)</s1>
</fA11>
<fA11 i1="03" i2="1"><s1>GIUNTI (Paola)</s1>
</fA11>
<fA11 i1="04" i2="1"><s1>GLOBAS (Christoph)</s1>
</fA11>
<fA11 i1="05" i2="1"><s1>BALIKO (Laszlo)</s1>
</fA11>
<fA11 i1="06" i2="1"><s1>FANCELLU (Roberto)</s1>
</fA11>
<fA11 i1="07" i2="1"><s1>MARIOTTI (Caterina)</s1>
</fA11>
<fA11 i1="08" i2="1"><s1>FILLA (Alessandro)</s1>
</fA11>
<fA11 i1="09" i2="1"><s1>RAKOWICZ (Maryla)</s1>
</fA11>
<fA11 i1="10" i2="1"><s1>CHARLES (Perrine)</s1>
</fA11>
<fA11 i1="11" i2="1"><s1>RIBAI (Pascale)</s1>
</fA11>
<fA11 i1="12" i2="1"><s1>SZYMANSKI (Sandra)</s1>
</fA11>
<fA11 i1="13" i2="1"><s1>INFANTE (Jon)</s1>
</fA11>
<fA11 i1="14" i2="1"><s1>VAN DE WARRENBURG (Bart P. C.)</s1>
</fA11>
<fA11 i1="15" i2="1"><s1>DÜRR (Alexandra)</s1>
</fA11>
<fA11 i1="16" i2="1"><s1>TIMMANN (Dagmar)</s1>
</fA11>
<fA11 i1="17" i2="1"><s1>BOESCH (Sylvia)</s1>
</fA11>
<fA11 i1="18" i2="1"><s1>ROLA (Rafal)</s1>
</fA11>
<fA11 i1="19" i2="1"><s1>DEPONDT (Chantal)</s1>
</fA11>
<fA11 i1="20" i2="1"><s1>SCHÖLS (Ludger)</s1>
</fA11>
<fA11 i1="21" i2="1"><s1>ZDZIENICKA (Elszbieta)</s1>
</fA11>
<fA11 i1="22" i2="1"><s1>KANG (Jun-Suk)</s1>
</fA11>
<fA11 i1="23" i2="1"><s1>RATZKA (Susanne)</s1>
</fA11>
<fA11 i1="24" i2="1"><s1>KREMER (Berry)</s1>
</fA11>
<fA11 i1="25" i2="1"><s1>SCHULZ (Jörg B.)</s1>
</fA11>
<fA11 i1="26" i2="1"><s1>KLOPSTOCK (Thomas)</s1>
</fA11>
<fA11 i1="27" i2="1"><s1>MELEGH (Bela)</s1>
</fA11>
<fA11 i1="28" i2="1"><s1>TEZENAS DU MONTCEL (Sophie)</s1>
</fA11>
<fA11 i1="29" i2="1"><s1>KLOCKGETHER (Thomas)</s1>
</fA11>
<fA14 i1="01"><s1>Department of Neurology, University Hospital of Bonn</s1>
<s2>Bonn</s2>
<s3>DEU</s3>
<sZ>1 aut.</sZ>
<sZ>29 aut.</sZ>
</fA14>
<fA14 i1="02"><s1>Department of Biostatistics and Medical Informatics, Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</fA14>
<fA14 i1="03"><s1>Modeling in Clinical Research, University Pierre et Marie Curie</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>28 aut.</sZ>
</fA14>
<fA14 i1="04"><s1>Department of Molecular Neuroscience, Institute of Neurology, Queen Square</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>3 aut.</sZ>
</fA14>
<fA14 i1="05"><s1>Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen</s1>
<s2>Tübingen</s2>
<s3>DEU</s3>
<sZ>4 aut.</sZ>
<sZ>20 aut.</sZ>
</fA14>
<fA14 i1="06"><s1>Department of Neurology and Stroke, County Hospital</s1>
<s2>Veszprém</s2>
<s3>HUN</s3>
<sZ>5 aut.</sZ>
</fA14>
<fA14 i1="07"><s1>Department of Biochemistry and Genetics, Fondazione-IRCCS, Istituto Neurologico Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</fA14>
<fA14 i1="08"><s1>Department of Neurology, University of Naples</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>8 aut.</sZ>
</fA14>
<fA14 i1="09"><s1>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>9 aut.</sZ>
</fA14>
<fA14 i1="10"><s1>INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</fA14>
<fA14 i1="11"><s1>AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière</s1>
<s2>75013, Paris</s2>
<s3>FRA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
<sZ>15 aut.</sZ>
</fA14>
<fA14 i1="12"><s1>Department of Neurology, St. Josef Hospital, University Hospital of Bochum</s1>
<s2>Bochum</s2>
<s3>DEU</s3>
<sZ>12 aut.</sZ>
</fA14>
<fA14 i1="13"><s1>Department of Neurology, University Hospital "Marqués de Valldecilla," CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>13 aut.</sZ>
</fA14>
<fA14 i1="14"><s1>Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center</s1>
<s2>Nijnaegen</s2>
<s3>NLD</s3>
<sZ>14 aut.</sZ>
<sZ>24 aut.</sZ>
</fA14>
<fA14 i1="15"><s1>Department of Neurology, University Hospital of Essen</s1>
<s2>Essen</s2>
<s3>DEU</s3>
<sZ>16 aut.</sZ>
</fA14>
<fA14 i1="16"><s1>Department of Neurology, University of Innsbruck</s1>
<s2>Innsbruck</s2>
<s3>AUT</s3>
<sZ>17 aut.</sZ>
</fA14>
<fA14 i1="17"><s1>First Department of Neurology, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>18 aut.</sZ>
</fA14>
<fA14 i1="18"><s1>Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles</s1>
<s2>Brussels</s2>
<s3>BEL</s3>
<sZ>19 aut.</sZ>
</fA14>
<fA14 i1="19"><s1>Department of Genetics, Institute of Psychiatry and Neurology</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>21 aut.</sZ>
</fA14>
<fA14 i1="20"><s1>Department of Neurology, University of Frankfurt</s1>
<s2>Frankfurt/M</s2>
<s3>DEU</s3>
<sZ>22 aut.</sZ>
</fA14>
<fA14 i1="21"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen</s1>
<s2>Göttingen</s2>
<s3>DEU</s3>
<sZ>23 aut.</sZ>
</fA14>
<fA14 i1="22"><s1>Department of Neurology, RWTH Aachen, University Medical Hospital</s1>
<s2>Aachen</s2>
<s3>DEU</s3>
<sZ>25 aut.</sZ>
</fA14>
<fA14 i1="23"><s1>Department of Neurology, Friedrich-Baur-Institute, University Hospital of Ludwig-Maximilians-University</s1>
<s2>München</s2>
<s3>DEU</s3>
<sZ>26 aut.</sZ>
</fA14>
<fA14 i1="24"><s1>Department of Medical Genetics and Child Development, University of Pécs</s1>
<s2>Pécs</s2>
<s3>HUN</s3>
<sZ>27 aut.</sZ>
</fA14>
<fA20><s1>587-595</s1>
</fA20>
<fA21><s1>2010</s1>
</fA21>
<fA23 i1="01"><s0>ENG</s0>
</fA23>
<fA43 i1="01"><s1>INIST</s1>
<s2>20953</s2>
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</fA43>
<fA44><s0>0000</s0>
<s1>© 2010 INIST-CNRS. All rights reserved.</s1>
</fA44>
<fA45><s0>29 ref.</s0>
</fA45>
<fA47 i1="01" i2="1"><s0>10-0233210</s0>
</fA47>
<fA60><s1>P</s1>
</fA60>
<fA61><s0>A</s0>
</fA61>
<fA64 i1="01" i2="1"><s0>Movement disorders</s0>
</fA64>
<fA66 i1="01"><s0>USA</s0>
</fA66>
<fC01 i1="01" l="ENG"><s0>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.</s0>
</fC01>
<fC02 i1="01" i2="X"><s0>002B17</s0>
</fC02>
<fC02 i1="02" i2="X"><s0>002B17A03</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE"><s0>Etat dépressif</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG"><s0>Depression</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA"><s0>Estado depresivo</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE"><s0>Pathologie du système nerveux</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG"><s0>Nervous system diseases</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA"><s0>Sistema nervioso patología</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE"><s0>Etat sanitaire</s0>
<s5>09</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG"><s0>Health status</s0>
<s5>09</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA"><s0>Estado sanitario</s0>
<s5>09</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE"><s0>Ataxie spinocérébelleuse</s0>
<s2>NM</s2>
<s5>10</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG"><s0>Spinocerebellar ataxia</s0>
<s2>NM</s2>
<s5>10</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA"><s0>Ataxia spinocerebelosa</s0>
<s2>NM</s2>
<s5>10</s5>
</fC03>
<fC03 i1="05" i2="X" l="FRE"><s0>Qualité de vie</s0>
<s5>11</s5>
</fC03>
<fC03 i1="05" i2="X" l="ENG"><s0>Quality of life</s0>
<s5>11</s5>
</fC03>
<fC03 i1="05" i2="X" l="SPA"><s0>Calidad vida</s0>
<s5>11</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE"><s0>Maladie dégénérative</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG"><s0>Degenerative disease</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE"><s0>Maladie héréditaire</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG"><s0>Genetic disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Pathologie du système nerveux central</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Central nervous system disease</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0>
<s5>39</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Trouble de l'humeur</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Mood disorder</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Trastorno humor</s0>
<s5>41</s5>
</fC07>
<fN21><s1>158</s1>
</fN21>
<fN44 i1="01"><s1>OTO</s1>
</fN44>
<fN82><s1>OTO</s1>
</fN82>
</pA>
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<server><NO>PASCAL 10-0233210 INIST</NO>
<ET>Self-Rated Health Status in Spinocerebellar Ataxia-Results from a European Multiceriter Study</ET>
<AU>SCHMITZ-HÜBSCH (Tanja); COUDERT (Mathieu); GIUNTI (Paola); GLOBAS (Christoph); BALIKO (Laszlo); FANCELLU (Roberto); MARIOTTI (Caterina); FILLA (Alessandro); RAKOWICZ (Maryla); CHARLES (Perrine); RIBAI (Pascale); SZYMANSKI (Sandra); INFANTE (Jon); VAN DE WARRENBURG (Bart P. C.); DÜRR (Alexandra); TIMMANN (Dagmar); BOESCH (Sylvia); ROLA (Rafal); DEPONDT (Chantal); SCHÖLS (Ludger); ZDZIENICKA (Elszbieta); KANG (Jun-Suk); RATZKA (Susanne); KREMER (Berry); SCHULZ (Jörg B.); KLOPSTOCK (Thomas); MELEGH (Bela); TEZENAS DU MONTCEL (Sophie); KLOCKGETHER (Thomas)</AU>
<AF>Department of Neurology, University Hospital of Bonn/Bonn/Allemagne (1 aut., 29 aut.); Department of Biostatistics and Medical Informatics, Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux/Paris/France (2 aut., 28 aut.); Modeling in Clinical Research, University Pierre et Marie Curie/Paris/France (2 aut., 28 aut.); Department of Molecular Neuroscience, Institute of Neurology, Queen Square/London/Royaume-Uni (3 aut.); Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen/Tübingen/Allemagne (4 aut., 20 aut.); Department of Neurology and Stroke, County Hospital/Veszprém/Hongrie (5 aut.); Department of Biochemistry and Genetics, Fondazione-IRCCS, Istituto Neurologico Carlo Besta/Milan/Italie (6 aut., 7 aut.); Department of Neurology, University of Naples/Naples/Italie (8 aut.); Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology/Warsaw/Pologne (9 aut.); INSERM, UMR_S679 Neurologie and Thérapeutique Expérimentale/75013, Paris/France (10 aut., 11 aut., 15 aut.); AP-HP, Department of Genetics and Cytogenetics, Hôpital de la Salpêtrière/75013, Paris/France (10 aut., 11 aut., 15 aut.); Department of Neurology, St. Josef Hospital, University Hospital of Bochum/Bochum/Allemagne (12 aut.); Department of Neurology, University Hospital "Marqués de Valldecilla," CIBERNED/Santander/Espagne (13 aut.); Department of Neurology, Donders Centre for Neuroscience, Radboud University Nijmegen Medical Center/Nijnaegen/Pays-Bas (14 aut., 24 aut.); Department of Neurology, University Hospital of Essen/Essen/Allemagne (16 aut.); Department of Neurology, University of Innsbruck/Innsbruck/Autriche (17 aut.); First Department of Neurology, Institute of Psychiatry and Neurology/Warsaw/Pologne (18 aut.); Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles/Brussels/Belgique (19 aut.); Department of Genetics, Institute of Psychiatry and Neurology/Warsaw/Pologne (21 aut.); Department of Neurology, University of Frankfurt/Frankfurt/M/Allemagne (22 aut.); Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University Hospital of Göttingen/Göttingen/Allemagne (23 aut.); Department of Neurology, RWTH Aachen, University Medical Hospital/Aachen/Allemagne (25 aut.); Department of Neurology, Friedrich-Baur-Institute, University Hospital of Ludwig-Maximilians-University/München/Allem agne (26 aut.); Department of Medical Genetics and Child Development, University of Pécs/Pécs/Hongrie (27 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2010; Vol. 25; No. 5; Pp. 587-595; Bibl. 29 ref.</SO>
<LA>Anglais</LA>
<EA>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.</EA>
<CC>002B17; 002B17A03</CC>
<FD>Etat dépressif; Pathologie du système nerveux; Etat sanitaire; Ataxie spinocérébelleuse; Qualité de vie</FD>
<FG>Maladie dégénérative; Maladie héréditaire; Pathologie du système nerveux central; Trouble de l'humeur</FG>
<ED>Depression; Nervous system diseases; Health status; Spinocerebellar ataxia; Quality of life</ED>
<EG>Degenerative disease; Genetic disease; Central nervous system disease; Mood disorder</EG>
<SD>Estado depresivo; Sistema nervioso patología; Estado sanitario; Ataxia spinocerebelosa; Calidad vida</SD>
<LO>INIST-20953.354000181079390100</LO>
<ID>10-0233210</ID>
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