Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry
Identifieur interne : 000399 ( Istex/Curation ); précédent : 000398; suivant : 000400Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry
Auteurs : Martin Köllensperger [Autriche] ; Felix Geser [Autriche] ; Jean-Pierre Ndayisaba [Autriche] ; Sylvia Boesch [Autriche] ; Klaus Seppi [Autriche] ; Karen Ostergaard [Danemark] ; Erik Dupont [Danemark] ; A. Cardozo [Espagne] ; Eduardo Tolosa [Espagne] ; Michael Abele [Allemagne] ; Thomas Klockgether [Allemagne] ; Farid Yekhlef [France] ; Francois Tison [France] ; Christine Daniels [Allemagne] ; Günther Deuschl [Allemagne] ; Miguel Coelho [Portugal] ; Cristina Sampaio [Portugal] ; Maria Bozi [Royaume-Uni] ; Niall Quinn [Royaume-Uni] ; Anette Schrag [Royaume-Uni] ; Chris J. Mathias [Royaume-Uni] ; Clare Fowler [Royaume-Uni] ; Christer F. Nilsson [Suède] ; H Kan Widner [Suède] ; Nicole Schimke [Allemagne] ; Wolfgang Oertel [Allemagne] ; Francesca Del Sorbo [Italie] ; Alberto Albanese [Italie] ; Maria Teresa Pellecchia [Italie] ; Paolo Barone [Italie] ; Ruth Djaldetti [Israël] ; Carlo Colosimo [Italie] ; Giuseppe Meco [Italie] ; Antonio Gonzalez-Mandly [Espagne] ; Jose Berciano [Espagne] ; Tanya Gurevich [Israël] ; Nir Giladi [Israël] ; Monique Galitzky [France] ; Olivier Rascol [France] ; Christoph Kamm [Allemagne] ; Thomas Gasser [Allemagne] ; Uwe Siebert [Autriche, États-Unis] ; Werner Poewe [Autriche] ; Gregor K. Wenning [Autriche]Source :
- Movement Disorders [ 0885-3185 ] ; 2010-11-15.
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Abstract
Multiple system atrophy (MSA) is a Parkinson's Disease (PD)‐like α‐synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four‐hundred thirty‐seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA‐P) and 32% as cerebellar type (MSA‐C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas. © 2010 Movement Disorder Society
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DOI: 10.1002/mds.23192
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Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea></affiliation></author><author><name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name><affiliation wicri:level="1"><mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation><country xml:lang="fr">Portugal</country><wicri:regionArea>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea></affiliation></author><author><name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name><affiliation wicri:level="1"><mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London</wicri:regionArea></affiliation></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name><affiliation wicri:level="1"><mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London</wicri:regionArea></affiliation></author><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Neurosciences, Royal Free and University College Medical School, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurosciences, Royal Free and University College Medical School, London</wicri:regionArea></affiliation></author><author><name sortKey="Mathias, Chris J" sort="Mathias, Chris J" uniqKey="Mathias C" first="Chris J." last="Mathias">Chris J. Mathias</name><affiliation wicri:level="1"><mods:affiliation>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London</wicri:regionArea></affiliation></author><author><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name><affiliation wicri:level="1"><mods:affiliation>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London</wicri:regionArea></affiliation></author><author><name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F." last="Nilsson">Christer F. Nilsson</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea></affiliation></author><author><name sortKey="Widner, H Kan" sort="Widner, H Kan" uniqKey="Widner H" first="H Kan" last="Widner">H Kan Widner</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea></affiliation></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea></affiliation></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea></affiliation></author><author><name sortKey="Del Sorbo, Francesca" sort="Del Sorbo, Francesca" uniqKey="Del Sorbo F" first="Francesca" last="Del Sorbo">Francesca Del Sorbo</name><affiliation wicri:level="1"><mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea></affiliation></author><author><name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name><affiliation wicri:level="1"><mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea></affiliation></author><author><name sortKey="Pellecchia, Maria Teresa" sort="Pellecchia, Maria Teresa" uniqKey="Pellecchia M" first="Maria Teresa" last="Pellecchia">Maria Teresa Pellecchia</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea></affiliation></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea></affiliation></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Rabin Medical Center, Petach‐Tiqva</wicri:regionArea></affiliation></author><author><name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea></affiliation></author><author><name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea></affiliation></author><author><name sortKey="Gonzalez Andly, Antonio" sort="Gonzalez Andly, Antonio" uniqKey="Gonzalez Andly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name><affiliation wicri:level="1"><mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea></affiliation></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name><affiliation wicri:level="1"><mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea></affiliation></author><author><name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea></affiliation></author><author><name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea></affiliation></author><author><name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name><affiliation wicri:level="1"><mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea></affiliation></author><author><name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name><affiliation wicri:level="1"><mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea></affiliation></author><author><name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea></affiliation></author><author><name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea></affiliation></author><author><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name><affiliation wicri:level="1"><mods:affiliation>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T., Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T.</wicri:regionArea></affiliation><affiliation wicri:level="2"><mods:affiliation>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</mods:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Massachusetts</region></placeName><wicri:cityArea>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston</wicri:cityArea></affiliation></author><author><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></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 wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:5C54E966FF32FA5C841F8E2BA415B68309A14FF2</idno><date when="2010" year="2010">2010</date><idno type="doi">10.1002/mds.23192</idno><idno type="url">https://api.istex.fr/document/5C54E966FF32FA5C841F8E2BA415B68309A14FF2/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">000399</idno><idno type="wicri:Area/Istex/Curation">000399</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry</title><author><name sortKey="Kollensperger, Martin" sort="Kollensperger, Martin" uniqKey="Kollensperger M" first="Martin" last="Köllensperger">Martin Köllensperger</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author><author><name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></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 wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author><author><name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author><author><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Department of Neurology, Aarhus University Hospital, Aarhus</wicri:regionArea></affiliation></author><author><name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</mods:affiliation><country xml:lang="fr">Danemark</country><wicri:regionArea>Department of Neurology, Aarhus University Hospital, Aarhus</wicri:regionArea></affiliation></author><author><name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A." last="Cardozo">A. Cardozo</name><affiliation wicri:level="1"><mods:affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona</wicri:regionArea></affiliation></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name><affiliation wicri:level="1"><mods:affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona</wicri:regionArea></affiliation></author><author><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of Bonn, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, University of Bonn</wicri:regionArea></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of Bonn, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, University of Bonn</wicri:regionArea></affiliation></author><author><name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name><affiliation wicri:level="1"><mods:affiliation>Service de Neurologie, CHU de Bordeaux, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Service de Neurologie, CHU de Bordeaux</wicri:regionArea></affiliation></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name><affiliation wicri:level="1"><mods:affiliation>Service de Neurologie, CHU de Bordeaux, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Service de Neurologie, CHU de Bordeaux</wicri:regionArea></affiliation></author><author><name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Christian‐Albrechts University of Kiel</wicri:regionArea></affiliation></author><author><name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Christian‐Albrechts University of Kiel</wicri:regionArea></affiliation></author><author><name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name><affiliation wicri:level="1"><mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation><country xml:lang="fr">Portugal</country><wicri:regionArea>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea></affiliation></author><author><name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name><affiliation wicri:level="1"><mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation><country xml:lang="fr">Portugal</country><wicri:regionArea>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea></affiliation></author><author><name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name><affiliation wicri:level="1"><mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London</wicri:regionArea></affiliation></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name><affiliation wicri:level="1"><mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London</wicri:regionArea></affiliation></author><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Neurosciences, Royal Free and University College Medical School, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurosciences, Royal Free and University College Medical School, London</wicri:regionArea></affiliation></author><author><name sortKey="Mathias, Chris J" sort="Mathias, Chris J" uniqKey="Mathias C" first="Chris J." last="Mathias">Chris J. Mathias</name><affiliation wicri:level="1"><mods:affiliation>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London</wicri:regionArea></affiliation></author><author><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name><affiliation wicri:level="1"><mods:affiliation>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom</mods:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London</wicri:regionArea></affiliation></author><author><name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F." last="Nilsson">Christer F. Nilsson</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea></affiliation></author><author><name sortKey="Widner, H Kan" sort="Widner, H Kan" uniqKey="Widner H" first="H Kan" last="Widner">H Kan Widner</name><affiliation wicri:level="1"><mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea></affiliation></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea></affiliation></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea></affiliation></author><author><name sortKey="Del Sorbo, Francesca" sort="Del Sorbo, Francesca" uniqKey="Del Sorbo F" first="Francesca" last="Del Sorbo">Francesca Del Sorbo</name><affiliation wicri:level="1"><mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea></affiliation></author><author><name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name><affiliation wicri:level="1"><mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea></affiliation></author><author><name sortKey="Pellecchia, Maria Teresa" sort="Pellecchia, Maria Teresa" uniqKey="Pellecchia M" first="Maria Teresa" last="Pellecchia">Maria Teresa Pellecchia</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea></affiliation></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea></affiliation></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Rabin Medical Center, Petach‐Tiqva</wicri:regionArea></affiliation></author><author><name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea></affiliation></author><author><name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea></affiliation></author><author><name sortKey="Gonzalez Andly, Antonio" sort="Gonzalez Andly, Antonio" uniqKey="Gonzalez Andly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name><affiliation wicri:level="1"><mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea></affiliation></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name><affiliation wicri:level="1"><mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea></affiliation></author><author><name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea></affiliation></author><author><name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea></affiliation></author><author><name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name><affiliation wicri:level="1"><mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea></affiliation></author><author><name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name><affiliation wicri:level="1"><mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea></affiliation></author><author><name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea></affiliation></author><author><name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea></affiliation></author><author><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name><affiliation wicri:level="1"><mods:affiliation>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T., Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T.</wicri:regionArea></affiliation><affiliation wicri:level="2"><mods:affiliation>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</mods:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Massachusetts</region></placeName><wicri:cityArea>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston</wicri:cityArea></affiliation></author><author><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><affiliation wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></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 wicri:level="1"><mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-11-15">2010-11-15</date><biblScope unit="vol">25</biblScope><biblScope unit="issue">15</biblScope><biblScope unit="page" from="2604">2604</biblScope><biblScope unit="page" to="2612">2612</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">5C54E966FF32FA5C841F8E2BA415B68309A14FF2</idno><idno type="DOI">10.1002/mds.23192</idno><idno type="ArticleID">MDS23192</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>EMSA‐SG</term><term>multiple system atrophy</term><term>natural history</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Multiple system atrophy (MSA) is a Parkinson's Disease (PD)‐like α‐synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four‐hundred thirty‐seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA‐P) and 32% as cerebellar type (MSA‐C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas. © 2010 Movement Disorder Society</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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