La maladie de Parkinson en France (serveur d'exploration)

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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 : 001A14 ( Istex/Corpus ); précédent : 001A13; suivant : 001A15

Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry

Auteurs : Martin Köllensperger ; Felix Geser ; Jean-Pierre Ndayisaba ; Sylvia Boesch ; Klaus Seppi ; Karen Ostergaard ; Erik Dupont ; A. Cardozo ; Eduardo Tolosa ; Michael Abele ; Thomas Klockgether ; Farid Yekhlef ; Francois Tison ; Christine Daniels ; Günther Deuschl ; Miguel Coelho ; Cristina Sampaio ; Maria Bozi ; Niall Quinn ; Anette Schrag ; Chris J. Mathias ; Clare Fowler ; Christer F. Nilsson ; H Kan Widner ; Nicole Schimke ; Wolfgang Oertel ; Francesca Del Sorbo ; Alberto Albanese ; Maria Teresa Pellecchia ; Paolo Barone ; Ruth Djaldetti ; Carlo Colosimo ; Giuseppe Meco ; Antonio Gonzalez-Mandly ; Jose Berciano ; Tanya Gurevich ; Nir Giladi ; Monique Galitzky ; Olivier Rascol ; Christoph Kamm ; Thomas Gasser ; Uwe Siebert ; Werner Poewe ; Gregor K. Wenning

Source :

RBID : ISTEX:5C54E966FF32FA5C841F8E2BA415B68309A14FF2

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. © 2010 Movement Disorder Society

Url:
DOI: 10.1002/mds.23192

Links to Exploration step

ISTEX:5C54E966FF32FA5C841F8E2BA415B68309A14FF2

Le document en format XML

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<name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
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<name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name>
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<name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A." last="Cardozo">A. Cardozo</name>
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<name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name>
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<name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
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<name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name>
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<name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name>
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<name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name>
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<mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation>
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<name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name>
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<mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation>
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<name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name>
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<mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation>
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<name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name>
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<name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name>
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<mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation>
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<name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name>
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<mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation>
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<name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name>
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<mods:affiliation>Department of Clinical Neurosciences, Royal Free and University College Medical School, London, United Kingdom</mods:affiliation>
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<name sortKey="Mathias, Chris J" sort="Mathias, Chris J" uniqKey="Mathias C" first="Chris J." last="Mathias">Chris J. Mathias</name>
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<mods:affiliation>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United Kingdom</mods:affiliation>
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<name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name>
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<mods:affiliation>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom</mods:affiliation>
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<name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F." last="Nilsson">Christer F. Nilsson</name>
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<mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation>
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<name sortKey="Widner, H Kan" sort="Widner, H Kan" uniqKey="Widner H" first="H Kan" last="Widner">H Kan Widner</name>
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<mods:affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</mods:affiliation>
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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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<mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation>
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<name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name>
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<mods:affiliation>Department of Neurology, Philipps‐University Marburg, Germany</mods:affiliation>
</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>
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<mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation>
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<name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name>
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<mods:affiliation>Istituto Carlo Besta, Milano, Italy</mods:affiliation>
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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>
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<mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation>
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</author>
<author>
<name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name>
<affiliation>
<mods:affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name>
<affiliation>
<mods:affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name>
<affiliation>
<mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name>
<affiliation>
<mods:affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</mods:affiliation>
</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>
<mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name>
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<mods:affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name>
<affiliation>
<mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name>
<affiliation>
<mods:affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name>
<affiliation>
<mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name>
<affiliation>
<mods:affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name>
<affiliation>
<mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name>
<affiliation>
<mods:affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name>
<affiliation>
<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>
</affiliation>
<affiliation>
<mods:affiliation>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name>
<affiliation>
<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K." last="Wenning">Gregor K. Wenning</name>
<affiliation>
<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
<affiliation>
<mods:affiliation>Department of Neurology, Parkinson Disease and Movement Disorders Centre, Medical University, Anichstrasse 35, Innsbruck 6020, Austria</mods:affiliation>
</affiliation>
</author>
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<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>
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<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name>
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<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ndayisaba, Jean Ierre" sort="Ndayisaba, Jean Ierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name>
<affiliation>
<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
<affiliation>
<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name>
<affiliation>
<mods:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name>
<affiliation>
<mods:affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name>
<affiliation>
<mods:affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A." last="Cardozo">A. Cardozo</name>
<affiliation>
<mods:affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name>
<affiliation>
<mods:affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name>
<affiliation>
<mods:affiliation>Department of Neurology, University of Bonn, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<affiliation>
<mods:affiliation>Department of Neurology, University of Bonn, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name>
<affiliation>
<mods:affiliation>Service de Neurologie, CHU de Bordeaux, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name>
<affiliation>
<mods:affiliation>Service de Neurologie, CHU de Bordeaux, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name>
<affiliation>
<mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name>
<affiliation>
<mods:affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name>
<affiliation>
<mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name>
<affiliation>
<mods:affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name>
<affiliation>
<mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name>
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<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>
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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</abstract>
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<name>S Gilman</name>
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<name>P Low</name>
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<name>N Quinn</name>
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<last>362</last>
<first>359</first>
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</host>
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<forename type="first">Giuseppe</forename>
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<forename type="first">Monique</forename>
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<forename type="first">Olivier</forename>
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<forename type="first">Christoph</forename>
<surname>Kamm</surname>
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<affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</affiliation>
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<persName>
<forename type="first">Thomas</forename>
<surname>Gasser</surname>
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<affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</affiliation>
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<persName>
<forename type="first">Uwe</forename>
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<forename type="first">Werner</forename>
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<forename type="first">Gregor K.</forename>
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<title level="j">Movement Disorders</title>
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<p>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</p>
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<givenNames>Christer F.</givenNames>
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<givenNames>Håkan</givenNames>
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<givenNames>Nicole</givenNames>
<familyName>Schimke</familyName>
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<personName>
<givenNames>Wolfgang</givenNames>
<familyName>Oertel</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Francesca</givenNames>
<familyName>del Sorbo</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Alberto</givenNames>
<familyName>Albanese</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Maria Teresa</givenNames>
<familyName>Pellecchia</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Paolo</givenNames>
<familyName>Barone</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Ruth</givenNames>
<familyName>Djaldetti</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Carlo</givenNames>
<familyName>Colosimo</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Giuseppe</givenNames>
<familyName>Meco</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Antonio</givenNames>
<familyName>Gonzalez‐Mandly</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Jose</givenNames>
<familyName>Berciano</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Tanya</givenNames>
<familyName>Gurevich</familyName>
<degrees>MD</degrees>
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<creator xml:id="au37" creatorRole="author" affiliationRef="#af19">
<personName>
<givenNames>Nir</givenNames>
<familyName>Giladi</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Monique</givenNames>
<familyName>Galitzky</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Olivier</givenNames>
<familyName>Rascol</familyName>
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<creator xml:id="au40" creatorRole="author" affiliationRef="#af21">
<personName>
<givenNames>Christoph</givenNames>
<familyName>Kamm</familyName>
<degrees>MD</degrees>
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<creator xml:id="au41" creatorRole="author" affiliationRef="#af21">
<personName>
<givenNames>Thomas</givenNames>
<familyName>Gasser</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Uwe</givenNames>
<familyName>Siebert</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Werner</givenNames>
<familyName>Poewe</familyName>
<degrees>MD</degrees>
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<personName>
<givenNames>Gregor K.</givenNames>
<familyName>Wenning</familyName>
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<email normalForm="gregor.wenning@i-med.ac.at">gregor.wenning@i‐med.ac.at</email>
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<groupName>on behalf of the EMSA‐SG</groupName>
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<unparsedAffiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</unparsedAffiliation>
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<unparsedAffiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</unparsedAffiliation>
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<unparsedAffiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</unparsedAffiliation>
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<unparsedAffiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</unparsedAffiliation>
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<unparsedAffiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</unparsedAffiliation>
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<unparsedAffiliation>Department of Clinical Neurosciences, Royal Free and University College Medical School, London, United Kingdom</unparsedAffiliation>
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<unparsedAffiliation>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United Kingdom</unparsedAffiliation>
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<unparsedAffiliation>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom</unparsedAffiliation>
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<unparsedAffiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</unparsedAffiliation>
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<unparsedAffiliation>Department of Neurology, Philipps‐University Marburg, Germany</unparsedAffiliation>
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<unparsedAffiliation>Istituto Carlo Besta, Milano, Italy</unparsedAffiliation>
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<unparsedAffiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af17" countryCode="IT" type="organization">
<unparsedAffiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</unparsedAffiliation>
</affiliation>
<affiliation xml:id="af19" countryCode="IL" type="organization">
<unparsedAffiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Department for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T., Austria</unparsedAffiliation>
</affiliation>
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<unparsedAffiliation>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</unparsedAffiliation>
</affiliation>
</affiliationGroup>
<keywordGroup xml:lang="en" type="author">
<keyword xml:id="kwd1">multiple system atrophy</keyword>
<keyword xml:id="kwd2">natural history</keyword>
<keyword xml:id="kwd3">EMSA‐SG</keyword>
</keywordGroup>
<fundingInfo>
<fundingAgency>EC</fundingAgency>
<fundingNumber>QLK6‐CT‐2000‐00661</fundingNumber>
</fundingInfo>
<abstractGroup>
<abstract type="main" xml:lang="en">
<title type="main">Abstract</title>
<p>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</p>
</abstract>
</abstractGroup>
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<noteGroup>
<note xml:id="fn2">
<p>Potential conflict of interest: Nothing to report.</p>
</note>
</noteGroup>
</header>
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<titleInfo lang="en">
<title>Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry</title>
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<titleInfo type="abbreviated" lang="en">
<title>Presentation, Diagnosis, and Management of MSA</title>
</titleInfo>
<titleInfo type="alternative" contentType="CDATA" lang="en">
<title>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 type="personal">
<namePart type="given">Martin</namePart>
<namePart type="family">Köllensperger</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Felix</namePart>
<namePart type="family">Geser</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Jean‐Pierre</namePart>
<namePart type="family">Ndayisaba</namePart>
<namePart type="termsOfAddress">MSc</namePart>
<affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Sylvia</namePart>
<namePart type="family">Boesch</namePart>
<namePart type="termsOfAddress">MD</namePart>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Klaus</namePart>
<namePart type="family">Seppi</namePart>
<namePart type="termsOfAddress">MD</namePart>
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<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Karen</namePart>
<namePart type="family">Ostergaard</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Erik</namePart>
<namePart type="family">Dupont</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Aarhus University Hospital, Aarhus, Denmark</affiliation>
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<name type="personal">
<namePart type="given">A.</namePart>
<namePart type="family">Cardozo</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Eduardo</namePart>
<namePart type="family">Tolosa</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Neurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, Spain</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Michael</namePart>
<namePart type="family">Abele</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, University of Bonn, Germany</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Thomas</namePart>
<namePart type="family">Klockgether</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, University of Bonn, Germany</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Farid</namePart>
<namePart type="family">Yekhlef</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Service de Neurologie, CHU de Bordeaux, France</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Francois</namePart>
<namePart type="family">Tison</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Service de Neurologie, CHU de Bordeaux, France</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Christine</namePart>
<namePart type="family">Daniels</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Günther</namePart>
<namePart type="family">Deuschl</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Christian‐Albrechts University of Kiel, Germany</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Miguel</namePart>
<namePart type="family">Coelho</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Cristina</namePart>
<namePart type="family">Sampaio</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, Portugal</affiliation>
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<roleTerm type="text">author</roleTerm>
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</name>
<name type="personal">
<namePart type="given">Maria</namePart>
<namePart type="family">Bozi</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Niall</namePart>
<namePart type="family">Quinn</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Anette</namePart>
<namePart type="family">Schrag</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Department of Clinical Neurosciences, Royal Free and University College Medical School, London, United Kingdom</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Chris J.</namePart>
<namePart type="family">Mathias</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United Kingdom</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Clare</namePart>
<namePart type="family">Fowler</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Christer F.</namePart>
<namePart type="family">Nilsson</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Håkan</namePart>
<namePart type="family">Widner</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, Sweden</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Nicole</namePart>
<namePart type="family">Schimke</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Philipps‐University Marburg, Germany</affiliation>
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<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Wolfgang</namePart>
<namePart type="family">Oertel</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Philipps‐University Marburg, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Francesca</namePart>
<namePart type="family">del Sorbo</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Istituto Carlo Besta, Milano, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
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<name type="personal">
<namePart type="given">Alberto</namePart>
<namePart type="family">Albanese</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Istituto Carlo Besta, Milano, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Maria Teresa</namePart>
<namePart type="family">Pellecchia</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Paolo</namePart>
<namePart type="family">Barone</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurological Sciences, University Federico II, Naples, Italy</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Ruth</namePart>
<namePart type="family">Djaldetti</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Rabin Medical Center, Petach‐Tiqva, Israel</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Carlo</namePart>
<namePart type="family">Colosimo</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Giuseppe</namePart>
<namePart type="family">Meco</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurological Sciences, La Sapienza University, Rome, Italy</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Antonio</namePart>
<namePart type="family">Gonzalez‐Mandly</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</affiliation>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Jose</namePart>
<namePart type="family">Berciano</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Services of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, Spain</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Tanya</namePart>
<namePart type="family">Gurevich</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Nir</namePart>
<namePart type="family">Giladi</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Israel</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Monique</namePart>
<namePart type="family">Galitzky</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Olivier</namePart>
<namePart type="family">Rascol</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Laboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, France</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Christoph</namePart>
<namePart type="family">Kamm</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Thomas</namePart>
<namePart type="family">Gasser</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Germany</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Uwe</namePart>
<namePart type="family">Siebert</namePart>
<namePart type="termsOfAddress">MD</namePart>
<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</affiliation>
<affiliation>Department of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Werner</namePart>
<namePart type="family">Poewe</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Gregor K.</namePart>
<namePart type="family">Wenning</namePart>
<namePart type="termsOfAddress">MD, PhD</namePart>
<affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria</affiliation>
<affiliation>Department of Neurology, Parkinson Disease and Movement Disorders Centre, Medical University, Anichstrasse 35, Innsbruck 6020, Austria</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="corporate">
<namePart>on behalf of the EMSA‐SG</namePart>
<description>Clinical Department of Neurology, Innsbruck Medical University, AustriaDepartment of Neurology, Aarhus University Hospital, Aarhus, DenmarkNeurology Service, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic, IDIBAPS, Universitat de Barcelona, SpainDepartment of Neurology, University of Bonn, GermanyService de Neurologie, CHU de Bordeaux, FranceDepartment of Neurology, Christian‐Albrechts University of Kiel, GermanyFaculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de Lisboa, PortugalSobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United KingdomDepartment of Clinical Neurosciences, Royal Free and University College Medical School, London, United KingdomNeurovascular Medicine Unit, Imperial College London at St Mary's Hospital, London, United KingdomDepartment of Uro‐Neurology, National Hospital for Neurology and Neurosurgery, London, United KingdomDepartment of Clinical Sciences, Divisions of Neurology and Geriatric Psychiatry, Lund University, SwedenDepartment of Neurology, Philipps‐University Marburg, GermanyIstituto Carlo Besta, Milano, ItalyDepartment of Neurological Sciences, University Federico II, Naples, ItalyDepartment of Neurology, Rabin Medical Center, Petach‐Tiqva, IsraelDepartment of Neurological Sciences, La Sapienza University, Rome, ItalyServices of Neurology and Radiology, University Hospital Marques de Valdecilla, CIBERNED, Santander, SpainDepartment of Neurology, Tel Aviv Sourasky Medical Center, Movement Disorders Unit, IsraelLaboratoire de Pharmacologie, Faculte de Medecine, Toulouse III University, FranceDepartment of Neurodegenerative Diseases, Hertie‐Institute for Clinical Brain Research, University of Tuebingen, GermanyDepartment for Public Health, Medical Decision Making and Health Technology Assessment, UMIT‐University for Health Sciences, Medical Informatics and Technology, Hall i. T., AustriaDepartment of Health Policy and Management, Program in Health Decision Science, Harvard School of Public Health, Boston, Massachusetts</description>
</name>
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<publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<place>
<placeTerm type="text">Hoboken</placeTerm>
</place>
<dateIssued encoding="w3cdtf">2010-11-15</dateIssued>
<dateCaptured encoding="w3cdtf">2009-01-11</dateCaptured>
<dateValid encoding="w3cdtf">2010-03-22</dateValid>
<copyrightDate encoding="w3cdtf">2010</copyrightDate>
</originInfo>
<language>
<languageTerm type="code" authority="rfc3066">en</languageTerm>
<languageTerm type="code" authority="iso639-2b">eng</languageTerm>
</language>
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<internetMediaType>text/html</internetMediaType>
<extent unit="figures">1</extent>
<extent unit="tables">3</extent>
<extent unit="references">35</extent>
<extent unit="words">6529</extent>
</physicalDescription>
<abstract 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</abstract>
<note type="content">*Potential conflict of interest: Nothing to report.</note>
<note type="funding">EC - No. QLK6‐CT‐2000‐00661; </note>
<subject lang="en">
<genre>keywords</genre>
<topic>multiple system atrophy</topic>
<topic>natural history</topic>
<topic>EMSA‐SG</topic>
</subject>
<relatedItem type="host">
<titleInfo>
<title>Movement Disorders</title>
</titleInfo>
<titleInfo type="abbreviated">
<title>Mov. Disord.</title>
</titleInfo>
<genre type="journal">journal</genre>
<subject>
<genre>article-category</genre>
<topic>Research Article</topic>
</subject>
<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part>
<date>2010</date>
<detail type="volume">
<caption>vol.</caption>
<number>25</number>
</detail>
<detail type="issue">
<caption>no.</caption>
<number>15</number>
</detail>
<extent unit="pages">
<start>2604</start>
<end>2612</end>
<total>9</total>
</extent>
</part>
</relatedItem>
<identifier type="istex">5C54E966FF32FA5C841F8E2BA415B68309A14FF2</identifier>
<identifier type="DOI">10.1002/mds.23192</identifier>
<identifier type="ArticleID">MDS23192</identifier>
<accessCondition type="use and reproduction" contentType="copyright">Copyright © 2010 Movement Disorder Society</accessCondition>
<recordInfo>
<recordContentSource>WILEY</recordContentSource>
<recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin>
</recordInfo>
</mods>
</metadata>
<serie></serie>
</istex>
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