Movement Disorders (revue)

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Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry

Identifieur interne : 002515 ( PascalFrancis/Curation ); précédent : 002514; suivant : 002516

Presentation, 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] ; Hakan 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 :

RBID : Pascal:10-0512942

Descripteurs français

English descriptors

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.
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C01 01    ENG  @0 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.
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Pascal:10-0512942

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<sZ>22 aut.</sZ>
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<inist:fA14 i1="12">
<s1>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</s1>
<s3>SWE</s3>
<sZ>23 aut.</sZ>
<sZ>24 aut.</sZ>
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<country>Suède</country>
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<inist:fA14 i1="12">
<s1>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</s1>
<s3>SWE</s3>
<sZ>23 aut.</sZ>
<sZ>24 aut.</sZ>
</inist:fA14>
<country>Suède</country>
</affiliation>
</author>
<author>
<name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name>
<affiliation wicri:level="1">
<inist:fA14 i1="13">
<s1>Department of Neurology, Philipps-University Marburg</s1>
<s3>DEU</s3>
<sZ>25 aut.</sZ>
<sZ>26 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
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<author>
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<affiliation wicri:level="1">
<inist:fA14 i1="13">
<s1>Department of Neurology, Philipps-University Marburg</s1>
<s3>DEU</s3>
<sZ>25 aut.</sZ>
<sZ>26 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
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<author>
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<affiliation wicri:level="1">
<inist:fA14 i1="14">
<s1>Istituto Carlo Besta</s1>
<s2>Milano</s2>
<s3>ITA</s3>
<sZ>27 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
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<author>
<name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name>
<affiliation wicri:level="1">
<inist:fA14 i1="14">
<s1>Istituto Carlo Besta</s1>
<s2>Milano</s2>
<s3>ITA</s3>
<sZ>27 aut.</sZ>
<sZ>28 aut.</sZ>
</inist:fA14>
<country>Italie</country>
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<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">
<inist:fA14 i1="15">
<s1>Department of Neurological Sciences, University Federico II</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>29 aut.</sZ>
<sZ>30 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
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<author>
<name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name>
<affiliation wicri:level="1">
<inist:fA14 i1="15">
<s1>Department of Neurological Sciences, University Federico II</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>29 aut.</sZ>
<sZ>30 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
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<author>
<name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name>
<affiliation wicri:level="1">
<inist:fA14 i1="16">
<s1>Department of Neurology, Rabin Medical Center</s1>
<s2>Petach-Tiqva</s2>
<s3>ISR</s3>
<sZ>31 aut.</sZ>
</inist:fA14>
<country>Israël</country>
</affiliation>
</author>
<author>
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<affiliation wicri:level="1">
<inist:fA14 i1="17">
<s1>Department of Neurological Sciences, La Sapienza University</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>32 aut.</sZ>
<sZ>33 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author>
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<inist:fA14 i1="17">
<s1>Department of Neurological Sciences, La Sapienza University</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>32 aut.</sZ>
<sZ>33 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author>
<name sortKey="Gonzalez Mandly, Antonio" sort="Gonzalez Mandly, Antonio" uniqKey="Gonzalez Mandly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name>
<affiliation wicri:level="1">
<inist:fA14 i1="18">
<s1>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>34 aut.</sZ>
<sZ>35 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
</affiliation>
</author>
<author>
<name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name>
<affiliation wicri:level="1">
<inist:fA14 i1="18">
<s1>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>34 aut.</sZ>
<sZ>35 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
</affiliation>
</author>
<author>
<name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name>
<affiliation wicri:level="1">
<inist:fA14 i1="19">
<s1>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</s1>
<s3>ISR</s3>
<sZ>36 aut.</sZ>
<sZ>37 aut.</sZ>
</inist:fA14>
<country>Israël</country>
</affiliation>
</author>
<author>
<name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name>
<affiliation wicri:level="1">
<inist:fA14 i1="19">
<s1>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</s1>
<s3>ISR</s3>
<sZ>36 aut.</sZ>
<sZ>37 aut.</sZ>
</inist:fA14>
<country>Israël</country>
</affiliation>
</author>
<author>
<name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name>
<affiliation wicri:level="1">
<inist:fA14 i1="20">
<s1>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</s1>
<s3>FRA</s3>
<sZ>38 aut.</sZ>
<sZ>39 aut.</sZ>
</inist:fA14>
<country>France</country>
</affiliation>
</author>
<author>
<name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name>
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<inist:fA14 i1="20">
<s1>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</s1>
<s3>FRA</s3>
<sZ>38 aut.</sZ>
<sZ>39 aut.</sZ>
</inist:fA14>
<country>France</country>
</affiliation>
</author>
<author>
<name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name>
<affiliation wicri:level="1">
<inist:fA14 i1="21">
<s1>Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tuebingen</s1>
<s3>DEU</s3>
<sZ>40 aut.</sZ>
<sZ>41 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
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<author>
<name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name>
<affiliation wicri:level="1">
<inist:fA14 i1="21">
<s1>Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tuebingen</s1>
<s3>DEU</s3>
<sZ>40 aut.</sZ>
<sZ>41 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
</author>
<author>
<name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name>
<affiliation wicri:level="1">
<inist:fA14 i1="22">
<s1>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT-University for Health Sciences, Medical Informatics and Technology</s1>
<s2>Hall i. T.</s2>
<s3>AUT</s3>
<sZ>42 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="23">
<s1>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health</s1>
<s2>Boston, Massachusetts</s2>
<s3>USA</s3>
<sZ>42 aut.</sZ>
</inist:fA14>
<country>États-Unis</country>
</affiliation>
</author>
<author>
<name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name>
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<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
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<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">
<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
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<country>Autriche</country>
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<title xml:lang="en" level="a">Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry</title>
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<name sortKey="Kollensperger, Martin" sort="Kollensperger, Martin" uniqKey="Kollensperger M" first="Martin" last="Köllensperger">Martin Köllensperger</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
</affiliation>
</author>
<author>
<name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
</affiliation>
</author>
<author>
<name sortKey="Ndayisaba, Jean Pierre" sort="Ndayisaba, Jean Pierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
</affiliation>
</author>
<author>
<name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
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<author>
<name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name>
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<inist:fA14 i1="01">
<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>43 aut.</sZ>
<sZ>44 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
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<author>
<name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name>
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<inist:fA14 i1="02">
<s1>Department of Neurology, Aarhus University Hospital</s1>
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<s3>DNK</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>Danemark</country>
</affiliation>
</author>
<author>
<name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Department of Neurology, Aarhus University Hospital</s1>
<s2>Aarhus</s2>
<s3>DNK</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>Danemark</country>
</affiliation>
</author>
<author>
<name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A." last="Cardozo">A. Cardozo</name>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona</s1>
<s3>ESP</s3>
<sZ>8 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
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<author>
<name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name>
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<inist:fA14 i1="03">
<s1>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona</s1>
<s3>ESP</s3>
<sZ>8 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>Espagne</country>
</affiliation>
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<author>
<name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name>
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<inist:fA14 i1="04">
<s1>Department of Neurology, University of Bonn</s1>
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<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
</author>
<author>
<name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>Department of Neurology, University of Bonn</s1>
<s3>DEU</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
</author>
<author>
<name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name>
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<inist:fA14 i1="05">
<s1>Service de Neurologie, CHU de Bordeaux</s1>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
</affiliation>
</author>
<author>
<name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name>
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<inist:fA14 i1="05">
<s1>Service de Neurologie, CHU de Bordeaux</s1>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
</inist:fA14>
<country>France</country>
</affiliation>
</author>
<author>
<name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name>
<affiliation wicri:level="1">
<inist:fA14 i1="06">
<s1>Department of Neurology, Christian-Albrechts University of Kiel</s1>
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<sZ>14 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
</author>
<author>
<name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name>
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<inist:fA14 i1="06">
<s1>Department of Neurology, Christian-Albrechts University of Kiel</s1>
<s3>DEU</s3>
<sZ>14 aut.</sZ>
<sZ>15 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
</author>
<author>
<name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name>
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<inist:fA14 i1="07">
<s1>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</s1>
<s3>PRT</s3>
<sZ>16 aut.</sZ>
<sZ>17 aut.</sZ>
</inist:fA14>
<country>Portugal</country>
</affiliation>
</author>
<author>
<name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name>
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<inist:fA14 i1="07">
<s1>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</s1>
<s3>PRT</s3>
<sZ>16 aut.</sZ>
<sZ>17 aut.</sZ>
</inist:fA14>
<country>Portugal</country>
</affiliation>
</author>
<author>
<name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name>
<affiliation wicri:level="1">
<inist:fA14 i1="08">
<s1>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square</s1>
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<s3>GBR</s3>
<sZ>18 aut.</sZ>
<sZ>19 aut.</sZ>
</inist:fA14>
<country>Royaume-Uni</country>
</affiliation>
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<author>
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<country>Royaume-Uni</country>
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<s1>Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery</s1>
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<country>Royaume-Uni</country>
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<s1>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</s1>
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<sZ>24 aut.</sZ>
</inist:fA14>
<country>Suède</country>
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<s1>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</s1>
<s3>SWE</s3>
<sZ>23 aut.</sZ>
<sZ>24 aut.</sZ>
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<country>Suède</country>
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<name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name>
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<s1>Department of Neurology, Philipps-University Marburg</s1>
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<sZ>26 aut.</sZ>
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<country>Allemagne</country>
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<s1>Department of Neurology, Philipps-University Marburg</s1>
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<sZ>25 aut.</sZ>
<sZ>26 aut.</sZ>
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<country>Allemagne</country>
</affiliation>
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<name sortKey="Del Sorbo, Francesca" sort="Del Sorbo, Francesca" uniqKey="Del Sorbo F" first="Francesca" last="Del Sorbo">Francesca Del Sorbo</name>
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<s1>Istituto Carlo Besta</s1>
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<sZ>27 aut.</sZ>
<sZ>28 aut.</sZ>
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<country>Italie</country>
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<name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name>
<affiliation wicri:level="1">
<inist:fA14 i1="14">
<s1>Istituto Carlo Besta</s1>
<s2>Milano</s2>
<s3>ITA</s3>
<sZ>27 aut.</sZ>
<sZ>28 aut.</sZ>
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<country>Italie</country>
</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">
<inist:fA14 i1="15">
<s1>Department of Neurological Sciences, University Federico II</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>29 aut.</sZ>
<sZ>30 aut.</sZ>
</inist:fA14>
<country>Italie</country>
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<author>
<name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name>
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<inist:fA14 i1="15">
<s1>Department of Neurological Sciences, University Federico II</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>29 aut.</sZ>
<sZ>30 aut.</sZ>
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<country>Italie</country>
</affiliation>
</author>
<author>
<name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name>
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<inist:fA14 i1="16">
<s1>Department of Neurology, Rabin Medical Center</s1>
<s2>Petach-Tiqva</s2>
<s3>ISR</s3>
<sZ>31 aut.</sZ>
</inist:fA14>
<country>Israël</country>
</affiliation>
</author>
<author>
<name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name>
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<s1>Department of Neurological Sciences, La Sapienza University</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>32 aut.</sZ>
<sZ>33 aut.</sZ>
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<country>Italie</country>
</affiliation>
</author>
<author>
<name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name>
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<inist:fA14 i1="17">
<s1>Department of Neurological Sciences, La Sapienza University</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>32 aut.</sZ>
<sZ>33 aut.</sZ>
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<country>Italie</country>
</affiliation>
</author>
<author>
<name sortKey="Gonzalez Mandly, Antonio" sort="Gonzalez Mandly, Antonio" uniqKey="Gonzalez Mandly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name>
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<inist:fA14 i1="18">
<s1>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>34 aut.</sZ>
<sZ>35 aut.</sZ>
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<country>Espagne</country>
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<author>
<name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name>
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<inist:fA14 i1="18">
<s1>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>34 aut.</sZ>
<sZ>35 aut.</sZ>
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<country>Espagne</country>
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<name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name>
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<s1>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</s1>
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<sZ>36 aut.</sZ>
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<country>Israël</country>
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<name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name>
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<inist:fA14 i1="19">
<s1>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</s1>
<s3>ISR</s3>
<sZ>36 aut.</sZ>
<sZ>37 aut.</sZ>
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<country>Israël</country>
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<author>
<name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name>
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<s1>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</s1>
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<sZ>39 aut.</sZ>
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<country>France</country>
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<name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name>
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<s1>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</s1>
<s3>FRA</s3>
<sZ>38 aut.</sZ>
<sZ>39 aut.</sZ>
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<country>France</country>
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<author>
<name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name>
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<s1>Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tuebingen</s1>
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<country>Allemagne</country>
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<author>
<name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name>
<affiliation wicri:level="1">
<inist:fA14 i1="21">
<s1>Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tuebingen</s1>
<s3>DEU</s3>
<sZ>40 aut.</sZ>
<sZ>41 aut.</sZ>
</inist:fA14>
<country>Allemagne</country>
</affiliation>
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<author>
<name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name>
<affiliation wicri:level="1">
<inist:fA14 i1="22">
<s1>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT-University for Health Sciences, Medical Informatics and Technology</s1>
<s2>Hall i. T.</s2>
<s3>AUT</s3>
<sZ>42 aut.</sZ>
</inist:fA14>
<country>Autriche</country>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="23">
<s1>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health</s1>
<s2>Boston, Massachusetts</s2>
<s3>USA</s3>
<sZ>42 aut.</sZ>
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<country>États-Unis</country>
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<author>
<name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name>
<affiliation wicri:level="1">
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<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
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<name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K." last="Wenning">Gregor K. Wenning</name>
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<s1>Clinical Department of Neurology, Innsbruck Medical University</s1>
<s3>AUT</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
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<country>Autriche</country>
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</analytic>
<series>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint>
<date when="2010">2010</date>
</imprint>
</series>
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<seriesStmt>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
</seriesStmt>
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<profileDesc>
<textClass>
<keywords scheme="KwdEn" xml:lang="en">
<term>Clinical management</term>
<term>Diagnosis</term>
<term>Europe</term>
<term>Multiple system atrophy</term>
<term>Nervous system diseases</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr">
<term>Atrophie multisystématisée</term>
<term>Pathologie du système nerveux</term>
<term>Diagnostic</term>
<term>Conduite à tenir</term>
<term>Europe</term>
</keywords>
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</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.</div>
</front>
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<s1>Department of Neurology, Aarhus University Hospital</s1>
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<fA14 i1="04">
<s1>Department of Neurology, University of Bonn</s1>
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<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
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<fA14 i1="05">
<s1>Service de Neurologie, CHU de Bordeaux</s1>
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<s1>Department of Neurology, Christian-Albrechts University of Kiel</s1>
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<sZ>15 aut.</sZ>
</fA14>
<fA14 i1="07">
<s1>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa</s1>
<s3>PRT</s3>
<sZ>16 aut.</sZ>
<sZ>17 aut.</sZ>
</fA14>
<fA14 i1="08">
<s1>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>18 aut.</sZ>
<sZ>19 aut.</sZ>
</fA14>
<fA14 i1="09">
<s1>Department of Clinical Neurosciences, Royal Free and University College Medical School</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>20 aut.</sZ>
</fA14>
<fA14 i1="10">
<s1>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>21 aut.</sZ>
</fA14>
<fA14 i1="11">
<s1>Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>22 aut.</sZ>
</fA14>
<fA14 i1="12">
<s1>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University</s1>
<s3>SWE</s3>
<sZ>23 aut.</sZ>
<sZ>24 aut.</sZ>
</fA14>
<fA14 i1="13">
<s1>Department of Neurology, Philipps-University Marburg</s1>
<s3>DEU</s3>
<sZ>25 aut.</sZ>
<sZ>26 aut.</sZ>
</fA14>
<fA14 i1="14">
<s1>Istituto Carlo Besta</s1>
<s2>Milano</s2>
<s3>ITA</s3>
<sZ>27 aut.</sZ>
<sZ>28 aut.</sZ>
</fA14>
<fA14 i1="15">
<s1>Department of Neurological Sciences, University Federico II</s1>
<s2>Naples</s2>
<s3>ITA</s3>
<sZ>29 aut.</sZ>
<sZ>30 aut.</sZ>
</fA14>
<fA14 i1="16">
<s1>Department of Neurology, Rabin Medical Center</s1>
<s2>Petach-Tiqva</s2>
<s3>ISR</s3>
<sZ>31 aut.</sZ>
</fA14>
<fA14 i1="17">
<s1>Department of Neurological Sciences, La Sapienza University</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>32 aut.</sZ>
<sZ>33 aut.</sZ>
</fA14>
<fA14 i1="18">
<s1>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED</s1>
<s2>Santander</s2>
<s3>ESP</s3>
<sZ>34 aut.</sZ>
<sZ>35 aut.</sZ>
</fA14>
<fA14 i1="19">
<s1>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit</s1>
<s3>ISR</s3>
<sZ>36 aut.</sZ>
<sZ>37 aut.</sZ>
</fA14>
<fA14 i1="20">
<s1>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University</s1>
<s3>FRA</s3>
<sZ>38 aut.</sZ>
<sZ>39 aut.</sZ>
</fA14>
<fA14 i1="21">
<s1>Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tuebingen</s1>
<s3>DEU</s3>
<sZ>40 aut.</sZ>
<sZ>41 aut.</sZ>
</fA14>
<fA14 i1="22">
<s1>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT-University for Health Sciences, Medical Informatics and Technology</s1>
<s2>Hall i. T.</s2>
<s3>AUT</s3>
<sZ>42 aut.</sZ>
</fA14>
<fA14 i1="23">
<s1>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health</s1>
<s2>Boston, Massachusetts</s2>
<s3>USA</s3>
<sZ>42 aut.</sZ>
</fA14>
<fA17 i1="01" i2="1">
<s1>EMSA-SG</s1>
<s3>INC</s3>
</fA17>
<fA20>
<s1>2604-2612</s1>
</fA20>
<fA21>
<s1>2010</s1>
</fA21>
<fA23 i1="01">
<s0>ENG</s0>
</fA23>
<fA43 i1="01">
<s1>INIST</s1>
<s2>20953</s2>
<s5>354000191411680180</s5>
</fA43>
<fA44>
<s0>0000</s0>
<s1>© 2010 INIST-CNRS. All rights reserved.</s1>
</fA44>
<fA45>
<s0>35 ref.</s0>
</fA45>
<fA47 i1="01" i2="1">
<s0>10-0512942</s0>
</fA47>
<fA60>
<s1>P</s1>
</fA60>
<fA61>
<s0>A</s0>
</fA61>
<fA64 i1="01" i2="1">
<s0>Movement disorders</s0>
</fA64>
<fA66 i1="01">
<s0>USA</s0>
</fA66>
<fC01 i1="01" l="ENG">
<s0>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.</s0>
</fC01>
<fC02 i1="01" i2="X">
<s0>002B17</s0>
</fC02>
<fC02 i1="02" i2="X">
<s0>002B17F</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE">
<s0>Atrophie multisystématisée</s0>
<s2>NM</s2>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG">
<s0>Multiple system atrophy</s0>
<s2>NM</s2>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA">
<s0>Atrofia multisistematizada</s0>
<s2>NM</s2>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE">
<s0>Pathologie du système nerveux</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG">
<s0>Nervous system diseases</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA">
<s0>Sistema nervioso patología</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE">
<s0>Diagnostic</s0>
<s5>09</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG">
<s0>Diagnosis</s0>
<s5>09</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA">
<s0>Diagnóstico</s0>
<s5>09</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE">
<s0>Conduite à tenir</s0>
<s5>10</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG">
<s0>Clinical management</s0>
<s5>10</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA">
<s0>Actitud médica</s0>
<s5>10</s5>
</fC03>
<fC03 i1="05" i2="X" l="FRE">
<s0>Europe</s0>
<s2>NG</s2>
<s5>11</s5>
</fC03>
<fC03 i1="05" i2="X" l="ENG">
<s0>Europe</s0>
<s2>NG</s2>
<s5>11</s5>
</fC03>
<fC03 i1="05" i2="X" l="SPA">
<s0>Europa</s0>
<s2>NG</s2>
<s5>11</s5>
</fC03>
<fN21>
<s1>347</s1>
</fN21>
<fN44 i1="01">
<s1>OTO</s1>
</fN44>
<fN82>
<s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
</record>

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