Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)
Identifieur interne : 000707 ( Main/Corpus ); précédent : 000706; suivant : 000708Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)
Auteurs : Felix Geser ; Gregor K. Wenning ; Klaus Seppi ; Michaela Stampfer-Kountchev ; Christoph Scherfler ; Martin Sawires ; Carolin Frick ; Jean-Pierre Ndayisaba ; Hanno Ulmer ; Maria T. Pellecchia ; Paolo Barone ; Hee T. Kim ; Juzar Hooker ; Niall P. Quinn ; Adriana Cardozo ; Eduardo Tolosa ; Michael Abele ; Thomas Klockgether ; Karen Stergaard ; Erik Dupont ; Nicole Schimke ; Karla M. Eggert ; Wolfgang Oertel ; Ruth Djaldetti ; Werner PoeweSource :
- Movement Disorders [ 0885-3185 ] ; 2006-02.
English descriptors
Abstract
The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P < 0.0001) and UMSARS I scores by 35.6% (P < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society
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DOI: 10.1002/mds.20678
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Wenning</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Stampfer Ountchev, Michaela" sort="Stampfer Ountchev, Michaela" uniqKey="Stampfer Ountchev M" first="Michaela" last="Stampfer-Kountchev">Michaela Stampfer-Kountchev</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Scherfler, Christoph" sort="Scherfler, Christoph" uniqKey="Scherfler C" first="Christoph" last="Scherfler">Christoph Scherfler</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Sawires, Martin" sort="Sawires, Martin" uniqKey="Sawires M" first="Martin" last="Sawires">Martin Sawires</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Frick, Carolin" sort="Frick, Carolin" uniqKey="Frick C" first="Carolin" last="Frick">Carolin Frick</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Ndayisaba, Jean Ierre" sort="Ndayisaba, Jean Ierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Ulmer, Hanno" sort="Ulmer, Hanno" uniqKey="Ulmer H" first="Hanno" last="Ulmer">Hanno Ulmer</name><affiliation><mods:affiliation>Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Pellecchia, Maria T" sort="Pellecchia, Maria T" uniqKey="Pellecchia M" first="Maria T." last="Pellecchia">Maria T. Pellecchia</name><affiliation><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><affiliation><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Kim, Hee T" sort="Kim, Hee T" uniqKey="Kim H" first="Hee T." last="Kim">Hee T. Kim</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Hooker, Juzar" sort="Hooker, Juzar" uniqKey="Hooker J" first="Juzar" last="Hooker">Juzar Hooker</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P." last="Quinn">Niall P. Quinn</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Cardozo, Adriana" sort="Cardozo, Adriana" uniqKey="Cardozo A" first="Adriana" last="Cardozo">Adriana Cardozo</name><affiliation><mods:affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</mods:affiliation></affiliation></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name><affiliation><mods:affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</mods:affiliation></affiliation></author><author><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name><affiliation><mods:affiliation>University of Bonn, Department of Neurology, Germany</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>University of Bonn, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey=" Stergaard, Karen" sort=" Stergaard, Karen" uniqKey=" Stergaard K" first="Karen" last=" Stergaard">Karen Stergaard</name><affiliation><mods:affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</mods:affiliation></affiliation></author><author><name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name><affiliation><mods:affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</mods:affiliation></affiliation></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Eggert, Karla M" sort="Eggert, Karla M" uniqKey="Eggert K" first="Karla M." last="Eggert">Karla M. Eggert</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name><affiliation><mods:affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</mods:affiliation></affiliation></author><author><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:4F6EEEBBEF9136A65A1579FC89C1E049885C1002</idno><date when="2006" year="2006">2006</date><idno type="doi">10.1002/mds.20678</idno><idno type="url">https://api.istex.fr/document/4F6EEEBBEF9136A65A1579FC89C1E049885C1002/fulltext/pdf</idno><idno type="wicri:Area/Main/Corpus">000707</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)</title><author><name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K." last="Wenning">Gregor K. Wenning</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Stampfer Ountchev, Michaela" sort="Stampfer Ountchev, Michaela" uniqKey="Stampfer Ountchev M" first="Michaela" last="Stampfer-Kountchev">Michaela Stampfer-Kountchev</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Scherfler, Christoph" sort="Scherfler, Christoph" uniqKey="Scherfler C" first="Christoph" last="Scherfler">Christoph Scherfler</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Sawires, Martin" sort="Sawires, Martin" uniqKey="Sawires M" first="Martin" last="Sawires">Martin Sawires</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Frick, Carolin" sort="Frick, Carolin" uniqKey="Frick C" first="Carolin" last="Frick">Carolin Frick</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Ndayisaba, Jean Ierre" sort="Ndayisaba, Jean Ierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Ulmer, Hanno" sort="Ulmer, Hanno" uniqKey="Ulmer H" first="Hanno" last="Ulmer">Hanno Ulmer</name><affiliation><mods:affiliation>Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, Austria</mods:affiliation></affiliation></author><author><name sortKey="Pellecchia, Maria T" sort="Pellecchia, Maria T" uniqKey="Pellecchia M" first="Maria T." last="Pellecchia">Maria T. Pellecchia</name><affiliation><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><affiliation><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation></affiliation></author><author><name sortKey="Kim, Hee T" sort="Kim, Hee T" uniqKey="Kim H" first="Hee T." last="Kim">Hee T. Kim</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Hooker, Juzar" sort="Hooker, Juzar" uniqKey="Hooker J" first="Juzar" last="Hooker">Juzar Hooker</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P." last="Quinn">Niall P. Quinn</name><affiliation><mods:affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation></affiliation></author><author><name sortKey="Cardozo, Adriana" sort="Cardozo, Adriana" uniqKey="Cardozo A" first="Adriana" last="Cardozo">Adriana Cardozo</name><affiliation><mods:affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</mods:affiliation></affiliation></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name><affiliation><mods:affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</mods:affiliation></affiliation></author><author><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name><affiliation><mods:affiliation>University of Bonn, Department of Neurology, Germany</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>University of Bonn, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey=" Stergaard, Karen" sort=" Stergaard, Karen" uniqKey=" Stergaard K" first="Karen" last=" Stergaard">Karen Stergaard</name><affiliation><mods:affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</mods:affiliation></affiliation></author><author><name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name><affiliation><mods:affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</mods:affiliation></affiliation></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Eggert, Karla M" sort="Eggert, Karla M" uniqKey="Eggert K" first="Karla M." last="Eggert">Karla M. Eggert</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><affiliation><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</mods:affiliation></affiliation></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name><affiliation><mods:affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</mods:affiliation></affiliation></author><author><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><affiliation><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</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="2006-02">2006-02</date><biblScope unit="volume">21</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="179">179</biblScope><biblScope unit="page" to="186">186</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">4F6EEEBBEF9136A65A1579FC89C1E049885C1002</idno><idno type="DOI">10.1002/mds.20678</idno><idno type="ArticleID">MDS20678</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Unified MSA Rating Scale</term><term>multiple system atrophy</term><term>progression</term><term>sensitivity to change</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P < 0.0001) and UMSARS I scores by 35.6% (P < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Felix Geser MD, PhD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Gregor K. Wenning MD, PhD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Klaus Seppi MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Michaela Stampfer‐Kountchev MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Christoph Scherfler MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Martin Sawires MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Carolin Frick MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Jean‐Pierre Ndayisaba Msc</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Hanno Ulmer PhD</name><affiliations><json:string>Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, Austria</json:string></affiliations></json:item><json:item><name>Maria T. Pellecchia MD</name><affiliations><json:string>Department of Neurological Sciences, University Federico II, Naples, Italy</json:string></affiliations></json:item><json:item><name>Paolo Barone MD</name><affiliations><json:string>Department of Neurological Sciences, University Federico II, Naples, Italy</json:string></affiliations></json:item><json:item><name>Hee T. Kim MD</name><affiliations><json:string>University College London, Institute of Neurology, Queen Square, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Juzar Hooker MD</name><affiliations><json:string>University College London, Institute of Neurology, Queen Square, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Niall P. Quinn MD, FRACP</name><affiliations><json:string>University College London, Institute of Neurology, Queen Square, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Adriana Cardozo MD</name><affiliations><json:string>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</json:string></affiliations></json:item><json:item><name>Eduardo Tolosa MD</name><affiliations><json:string>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</json:string></affiliations></json:item><json:item><name>Michael Abele MD</name><affiliations><json:string>University of Bonn, Department of Neurology, Germany</json:string></affiliations></json:item><json:item><name>Thomas Klockgether MD</name><affiliations><json:string>University of Bonn, Department of Neurology, Germany</json:string></affiliations></json:item><json:item><name>Karen Østergaard MD, PhD</name><affiliations><json:string>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</json:string></affiliations></json:item><json:item><name>Erik Dupont MD</name><affiliations><json:string>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</json:string></affiliations></json:item><json:item><name>Nicole Schimke MD</name><affiliations><json:string>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</json:string></affiliations></json:item><json:item><name>Karla M. Eggert MD</name><affiliations><json:string>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</json:string></affiliations></json:item><json:item><name>Wolfgang Oertel MD</name><affiliations><json:string>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</json:string></affiliations></json:item><json:item><name>Ruth Djaldetti MD</name><affiliations><json:string>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</json:string></affiliations></json:item><json:item><name>Werner Poewe MD</name><affiliations><json:string>Clinical Department of Neurology, Innsbruck Medical University, Austria</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>multiple system atrophy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Unified MSA Rating Scale</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>progression</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>sensitivity to change</value></json:item></subject><articleId><json:string>MDS20678</json:string></articleId><language><json:string>eng</json:string></language><abstract>The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P > 0.0001) and UMSARS I scores by 35.6% (P > 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society</abstract><qualityIndicators><score>7.727</score><pdfVersion>1.3</pdfVersion><pdfPageSize>594 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>4</keywordCount><abstractCharCount>1684</abstractCharCount><pdfWordCount>4727</pdfWordCount><pdfCharCount>29898</pdfCharCount><pdfPageCount>8</pdfPageCount><abstractWordCount>252</abstractWordCount></qualityIndicators><title>Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)</title><corporate><json:item><name>the European MSA Study 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SG)</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><availability><p>WILEY</p></availability><date>2006</date></publicationStmt><notesStmt><note>5th framework program of the European Community - No. QLK6‐CT‐2000‐00661;</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)</title><author><orgName>the European MSA Study Group</orgName></author><author><persName><forename type="first">Felix</forename><surname>Geser</surname></persName><roleName type="degree">MD, PhD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Gregor K.</forename><surname>Wenning</surname></persName><roleName type="degree">MD, PhD</roleName><note type="correspondence"><p>Correspondence: Clinical Department of Neurology, Innsbruck Medical University, Anichstrasse 35, A‐6020 Innsbruck, Austria</p></note><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Klaus</forename><surname>Seppi</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Michaela</forename><surname>Stampfer‐Kountchev</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Christoph</forename><surname>Scherfler</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Martin</forename><surname>Sawires</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Carolin</forename><surname>Frick</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Jean‐Pierre</forename><surname>Ndayisaba</surname></persName><roleName type="degree">Msc</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Hanno</forename><surname>Ulmer</surname></persName><roleName type="degree">PhD</roleName><affiliation>Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, Austria</affiliation></author><author><persName><forename type="first">Maria T.</forename><surname>Pellecchia</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation></author><author><persName><forename type="first">Paolo</forename><surname>Barone</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation></author><author><persName><forename type="first">Hee T.</forename><surname>Kim</surname></persName><roleName type="degree">MD</roleName><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation></author><author><persName><forename type="first">Juzar</forename><surname>Hooker</surname></persName><roleName type="degree">MD</roleName><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation></author><author><persName><forename type="first">Niall P.</forename><surname>Quinn</surname></persName><roleName type="degree">MD, FRACP</roleName><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation></author><author><persName><forename type="first">Adriana</forename><surname>Cardozo</surname></persName><roleName type="degree">MD</roleName><affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</affiliation></author><author><persName><forename type="first">Eduardo</forename><surname>Tolosa</surname></persName><roleName type="degree">MD</roleName><affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</affiliation></author><author><persName><forename type="first">Michael</forename><surname>Abele</surname></persName><roleName type="degree">MD</roleName><affiliation>University of Bonn, Department of Neurology, Germany</affiliation></author><author><persName><forename type="first">Thomas</forename><surname>Klockgether</surname></persName><roleName type="degree">MD</roleName><affiliation>University of Bonn, Department of Neurology, Germany</affiliation></author><author><persName><forename type="first">Karen</forename><surname>Østergaard</surname></persName><roleName type="degree">MD, PhD</roleName><affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</affiliation></author><author><persName><forename type="first">Erik</forename><surname>Dupont</surname></persName><roleName type="degree">MD</roleName><affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</affiliation></author><author><persName><forename type="first">Nicole</forename><surname>Schimke</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation></author><author><persName><forename type="first">Karla M.</forename><surname>Eggert</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation></author><author><persName><forename type="first">Wolfgang</forename><surname>Oertel</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation></author><author><persName><forename type="first">Ruth</forename><surname>Djaldetti</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</affiliation></author><author><persName><forename type="first">Werner</forename><surname>Poewe</surname></persName><roleName type="degree">MD</roleName><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation></author></analytic><monogr><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. 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Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P < 0.0001) and UMSARS I scores by 35.6% (P < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>multiple system atrophy</term></item><item><term>Unified MSA Rating Scale</term></item><item><term>progression</term></item><item><term>sensitivity to change</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="2005-03-22">Received</change><change when="2005-05-13">Registration</change><change when="2006-02">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/4F6EEEBBEF9136A65A1579FC89C1E049885C1002/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. 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Community</fundingAgency><fundingNumber>QLK6‐CT‐2000‐00661</fundingNumber></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (<i>P</i> < 0.0001) and UMSARS I scores by 35.6% (<i>P</i> < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>A full list of study members is presented in the <link href="#app1">Appendix</link>.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>MSA Progression</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA Study Group (EMSA SG)</title></titleInfo><name type="personal"><namePart type="given">Felix</namePart><namePart type="family">Geser</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Gregor K.</namePart><namePart type="family">Wenning</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><description>Correspondence: Clinical Department of Neurology, Innsbruck Medical University, Anichstrasse 35, A‐6020 Innsbruck, Austria</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Klaus</namePart><namePart type="family">Seppi</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Michaela</namePart><namePart type="family">Stampfer‐Kountchev</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christoph</namePart><namePart type="family">Scherfler</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Martin</namePart><namePart type="family">Sawires</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Carolin</namePart><namePart type="family">Frick</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jean‐Pierre</namePart><namePart type="family">Ndayisaba</namePart><namePart type="termsOfAddress">Msc</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Hanno</namePart><namePart type="family">Ulmer</namePart><namePart type="termsOfAddress">PhD</namePart><affiliation>Department of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Maria T.</namePart><namePart type="family">Pellecchia</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Paolo</namePart><namePart type="family">Barone</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Hee T.</namePart><namePart type="family">Kim</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Juzar</namePart><namePart type="family">Hooker</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Niall P.</namePart><namePart type="family">Quinn</namePart><namePart type="termsOfAddress">MD, FRACP</namePart><affiliation>University College London, Institute of Neurology, Queen Square, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Adriana</namePart><namePart type="family">Cardozo</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Eduardo</namePart><namePart type="family">Tolosa</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Universitat de Barcelona, Hospital Clinic, Department of Neurology, Spain</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Michael</namePart><namePart type="family">Abele</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>University of Bonn, Department of Neurology, Germany</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>University of Bonn, Department of Neurology, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Karen</namePart><namePart type="family">Østergaard</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Erik</namePart><namePart type="family">Dupont</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Aarhus Kummunehospital, Department of Neurology, Aarhus, Denmark</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Nicole</namePart><namePart type="family">Schimke</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Karla M.</namePart><namePart type="family">Eggert</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Wolfgang</namePart><namePart type="family">Oertel</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Philipps‐University Marburg, Department of Neurology, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Ruth</namePart><namePart type="family">Djaldetti</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Werner</namePart><namePart type="family">Poewe</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="corporate"><namePart>the European MSA Study Group</namePart><description>Clinical Department of Neurology, Innsbruck Medical University, AustriaDepartment of Medical Statistics, Informatics and Health Economics, Innsbruck Medical University, AustriaDepartment of Neurological Sciences, University Federico II, Naples, ItalyUniversity College London, Institute of Neurology, Queen Square, London, United KingdomUniversitat de Barcelona, Hospital Clinic, Department of Neurology, SpainUniversity of Bonn, Department of Neurology, GermanyAarhus Kummunehospital, Department of Neurology, Aarhus, DenmarkDepartment of Neurology, Philipps‐University Marburg, Department of Neurology, GermanyDepartment of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</description></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article"></genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2006-02</dateIssued><dateCaptured encoding="w3cdtf">2005-03-22</dateCaptured><dateValid encoding="w3cdtf">2005-05-13</dateValid><copyrightDate encoding="w3cdtf">2006</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">3</extent><extent unit="tables">5</extent><extent unit="references">29</extent><extent unit="words">5398</extent></physicalDescription><abstract lang="en">The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P < 0.0001) and UMSARS I scores by 35.6% (P < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials. © 2005 Movement Disorder Society</abstract><note type="funding">5th framework program of the European Community - No. QLK6‐CT‐2000‐00661; </note><subject lang="en"><genre>Keywords</genre><topic>multiple system atrophy</topic><topic>Unified MSA Rating Scale</topic><topic>progression</topic><topic>sensitivity to change</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><genre type="Journal">journal</genre><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>2006</date><detail type="volume"><caption>vol.</caption><number>21</number></detail><detail type="issue"><caption>no.</caption><number>2</number></detail><extent unit="pages"><start>179</start><end>186</end><total>8</total></extent></part></relatedItem><identifier type="istex">4F6EEEBBEF9136A65A1579FC89C1E049885C1002</identifier><identifier type="DOI">10.1002/mds.20678</identifier><identifier type="ArticleID">MDS20678</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2005 Movement Disorder Society</accessCondition><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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