Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry
Identifieur interne : 000202 ( France/Analysis ); précédent : 000201; suivant : 000203Presentation, 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.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Age of Onset, Antiparkinson Agents (therapeutic use), Cerebellar Ataxia (diagnosis), Cerebellar Ataxia (physiopathology), Clinical management, Diagnosis, EMSA‐SG, Europe, Female, Humans, Hypotension, Orthostatic (diagnosis), Hypotension, Orthostatic (physiopathology), Levodopa (therapeutic use), Male, Middle Aged, Multiple System Atrophy (diagnosis), Multiple System Atrophy (physiopathology), Multiple System Atrophy (therapy), Multiple system atrophy, Nervous system diseases, Parkinsonian Disorders (diagnosis), Parkinsonian Disorders (drug therapy), Parkinsonian Disorders (physiopathology), Registries, Shy-Drager Syndrome (diagnosis), Shy-Drager Syndrome (physiopathology), multiple system atrophy, natural history.
- MESH :
- chemical , therapeutic use : Antiparkinson Agents, Levodopa.
- geographic : Europe.
- diagnosis : Cerebellar Ataxia, Hypotension, Orthostatic, Multiple System Atrophy, Parkinsonian Disorders, Shy-Drager Syndrome.
- drug therapy : Parkinsonian Disorders.
- physiopathology : Cerebellar Ataxia, Hypotension, Orthostatic, Multiple System Atrophy, Parkinsonian Disorders, Shy-Drager Syndrome.
- therapy : Multiple System Atrophy.
- Age of Onset, Female, Humans, Male, Middle Aged, Registries.
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
Url:
DOI: 10.1002/mds.23192
Affiliations:
- Allemagne, Autriche, Danemark, Espagne, France, Israël, Italie, Portugal, Royaume-Uni, Suède, États-Unis
- Angleterre, Grand Londres, Latium, Massachusetts, Midi-Pyrénées, Tyrol (Land)
- Innsbruck, Londres, Rome, Toulouse
- Université Toulouse III - Paul Sabatier, Université de Toulouse, Université de médecine d'Innsbruck
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ISTEX:5C54E966FF32FA5C841F8E2BA415B68309A14FF2Le document en format XML
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Cardozo</name><affiliation wicri:level="1"><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><wicri:noRegion>Universitat de Barcelona</wicri:noRegion></affiliation></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name><affiliation wicri:level="1"><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><wicri:noRegion>Universitat de Barcelona</wicri:noRegion></affiliation></author><author><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, University of Bonn</wicri:regionArea><wicri:noRegion>University of Bonn</wicri:noRegion><wicri:noRegion>University of Bonn</wicri:noRegion></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, University of Bonn</wicri:regionArea><wicri:noRegion>University of Bonn</wicri:noRegion><wicri:noRegion>University of Bonn</wicri:noRegion></affiliation></author><author><name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Service de Neurologie, CHU de Bordeaux</wicri:regionArea><wicri:noRegion>CHU de Bordeaux</wicri:noRegion><wicri:noRegion>CHU de Bordeaux</wicri:noRegion></affiliation></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Service de Neurologie, CHU de Bordeaux</wicri:regionArea><wicri:noRegion>CHU de Bordeaux</wicri:noRegion><wicri:noRegion>CHU de Bordeaux</wicri:noRegion></affiliation></author><author><name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Christian‐Albrechts University of Kiel</wicri:regionArea><wicri:noRegion>Christian‐Albrechts University of Kiel</wicri:noRegion><wicri:noRegion>Christian‐Albrechts University of Kiel</wicri:noRegion></affiliation></author><author><name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Christian‐Albrechts University of Kiel</wicri:regionArea><wicri:noRegion>Christian‐Albrechts University of Kiel</wicri:noRegion><wicri:noRegion>Christian‐Albrechts University of Kiel</wicri:noRegion></affiliation></author><author><name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name><affiliation wicri:level="1"><country xml:lang="fr">Portugal</country><wicri:regionArea>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea><wicri:noRegion>Centro de Neurosciencias de Lisboa</wicri:noRegion></affiliation></author><author><name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name><affiliation wicri:level="1"><country xml:lang="fr">Portugal</country><wicri:regionArea>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</wicri:regionArea><wicri:noRegion>Centro de Neurosciencias de Lisboa</wicri:noRegion></affiliation></author><author><name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name><affiliation wicri:level="3"><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><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name><affiliation wicri:level="3"><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><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurosciences, Royal Free and University College Medical School, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></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="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></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"><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea><wicri:noRegion>Lund University</wicri:noRegion></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"><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</wicri:regionArea><wicri:noRegion>Lund University</wicri:noRegion></affiliation></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea><wicri:noRegion>Philipps‐University Marburg</wicri:noRegion><wicri:noRegion>Philipps‐University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps‐University Marburg</wicri:regionArea><wicri:noRegion>Philipps‐University Marburg</wicri:noRegion><wicri:noRegion>Philipps‐University Marburg</wicri:noRegion></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"><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea><wicri:noRegion>Milano</wicri:noRegion></affiliation></author><author><name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Istituto Carlo Besta, Milano</wicri:regionArea><wicri:noRegion>Milano</wicri:noRegion></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"><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea><wicri:noRegion>Naples</wicri:noRegion></affiliation></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, University Federico II, Naples</wicri:regionArea><wicri:noRegion>Naples</wicri:noRegion></affiliation></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name><affiliation wicri:level="1"><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Rabin Medical Center, Petach‐Tiqva</wicri:regionArea><wicri:noRegion>Petach‐Tiqva</wicri:noRegion></affiliation></author><author><name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name><affiliation wicri:level="3"><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></affiliation></author><author><name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name><affiliation wicri:level="3"><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Neurological Sciences, La Sapienza University, Rome</wicri:regionArea><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></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"><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea><wicri:noRegion>Santander</wicri:noRegion></affiliation></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name><affiliation wicri:level="1"><country xml:lang="fr">Espagne</country><wicri:regionArea>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander</wicri:regionArea><wicri:noRegion>Santander</wicri:noRegion></affiliation></author><author><name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name><affiliation wicri:level="1"><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea><wicri:noRegion>Movement Disorders Unit</wicri:noRegion></affiliation></author><author><name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name><affiliation wicri:level="1"><country xml:lang="fr">Israël</country><wicri:regionArea>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</wicri:regionArea><wicri:noRegion>Movement Disorders Unit</wicri:noRegion></affiliation></author><author><name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea><wicri:noRegion>Toulouse III University</wicri:noRegion><wicri:noRegion>Toulouse III University</wicri:noRegion></affiliation></author><author><name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</wicri:regionArea><wicri:noRegion>Toulouse III University</wicri:noRegion><wicri:noRegion>Toulouse III University</wicri:noRegion><placeName><settlement type="city">Toulouse</settlement><region type="region" nuts="2">Midi-Pyrénées</region></placeName><orgName type="university" n="3">Université Toulouse III - Paul Sabatier</orgName><orgName type="institution" wicri:auto="newGroup">Université de Toulouse</orgName></affiliation></author><author><name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea><wicri:noRegion>University of Tuebingen</wicri:noRegion><wicri:noRegion>University of Tuebingen</wicri:noRegion></affiliation></author><author><name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name><affiliation wicri:level="1"><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen</wicri:regionArea><wicri:noRegion>University of Tuebingen</wicri:noRegion><wicri:noRegion>University of Tuebingen</wicri:noRegion></affiliation></author><author><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name><affiliation wicri:level="1"><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><wicri:noRegion>Hall i. T.</wicri:noRegion></affiliation><affiliation wicri:level="2"><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"><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea><wicri:noRegion>Innsbruck Medical University</wicri:noRegion><placeName><settlement type="city">Innsbruck</settlement><region nuts="2" type="region">Tyrol (Land)</region></placeName><orgName type="university">Université de médecine d'Innsbruck</orgName></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"><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea><wicri:noRegion>Innsbruck Medical University</wicri:noRegion></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>Age of Onset</term><term>Antiparkinson Agents (therapeutic use)</term><term>Cerebellar Ataxia (diagnosis)</term><term>Cerebellar Ataxia (physiopathology)</term><term>Clinical management</term><term>Diagnosis</term><term>EMSA‐SG</term><term>Europe</term><term>Female</term><term>Humans</term><term>Hypotension, Orthostatic (diagnosis)</term><term>Hypotension, Orthostatic (physiopathology)</term><term>Levodopa (therapeutic use)</term><term>Male</term><term>Middle Aged</term><term>Multiple System Atrophy (diagnosis)</term><term>Multiple System Atrophy (physiopathology)</term><term>Multiple System Atrophy (therapy)</term><term>Multiple system atrophy</term><term>Nervous system diseases</term><term>Parkinsonian Disorders (diagnosis)</term><term>Parkinsonian Disorders (drug therapy)</term><term>Parkinsonian Disorders (physiopathology)</term><term>Registries</term><term>Shy-Drager Syndrome (diagnosis)</term><term>Shy-Drager Syndrome (physiopathology)</term><term>multiple system atrophy</term><term>natural history</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Antiparkinson Agents</term><term>Levodopa</term></keywords><keywords scheme="MESH" type="geographic" xml:lang="en"><term>Europe</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cerebellar Ataxia</term><term>Hypotension, Orthostatic</term><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term><term>Shy-Drager Syndrome</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Cerebellar Ataxia</term><term>Hypotension, Orthostatic</term><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term><term>Shy-Drager Syndrome</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Multiple System Atrophy</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Age of Onset</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Registries</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Atrophie multisystématisée</term><term>Conduite à tenir</term><term>Diagnostic</term><term>Europe</term><term>Pathologie du système nerveux</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><affiliations><list><country><li>Allemagne</li><li>Autriche</li><li>Danemark</li><li>Espagne</li><li>France</li><li>Israël</li><li>Italie</li><li>Portugal</li><li>Royaume-Uni</li><li>Suède</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Grand Londres</li><li>Latium</li><li>Massachusetts</li><li>Midi-Pyrénées</li><li>Tyrol (Land)</li></region><settlement><li>Innsbruck</li><li>Londres</li><li>Rome</li><li>Toulouse</li></settlement><orgName><li>Université Toulouse III - Paul Sabatier</li><li>Université de Toulouse</li><li>Université de médecine d'Innsbruck</li></orgName></list><tree><country name="Autriche"><noRegion><name sortKey="Kollensperger, Martin" sort="Kollensperger, Martin" uniqKey="Kollensperger M" first="Martin" last="Köllensperger">Martin Köllensperger</name></noRegion><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name><name sortKey="Ndayisaba, Jean Ierre" sort="Ndayisaba, Jean Ierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name><name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K." last="Wenning">Gregor K. Wenning</name></country><country name="Danemark"><noRegion><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name></noRegion><name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name></country><country name="Espagne"><noRegion><name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A." last="Cardozo">A. Cardozo</name></noRegion><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name><name sortKey="Gonzalez Andly, Antonio" sort="Gonzalez Andly, Antonio" uniqKey="Gonzalez Andly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name></country><country name="Allemagne"><noRegion><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name></noRegion><name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name><name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name><name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name><name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name></country><country name="France"><noRegion><name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name></noRegion><name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name><name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name></country><country name="Portugal"><noRegion><name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name></noRegion><name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name></country><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name></region><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name><name sortKey="Mathias, Chris J" sort="Mathias, Chris J" uniqKey="Mathias C" first="Chris J." last="Mathias">Chris J. Mathias</name><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name></country><country name="Suède"><noRegion><name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F." last="Nilsson">Christer F. Nilsson</name></noRegion><name sortKey="Widner, H Kan" sort="Widner, H Kan" uniqKey="Widner H" first="H Kan" last="Widner">H Kan Widner</name></country><country name="Italie"><noRegion><name sortKey="Del Sorbo, Francesca" sort="Del Sorbo, Francesca" uniqKey="Del Sorbo F" first="Francesca" last="Del Sorbo">Francesca Del Sorbo</name></noRegion><name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name><name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name><name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name><name sortKey="Pellecchia, Maria Teresa" sort="Pellecchia, Maria Teresa" uniqKey="Pellecchia M" first="Maria Teresa" last="Pellecchia">Maria Teresa Pellecchia</name></country><country name="Israël"><noRegion><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name></noRegion><name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name><name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name></country><country name="États-Unis"><region name="Massachusetts"><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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