Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry.
Identifieur interne : 002E79 ( Ncbi/Curation ); précédent : 002E78; suivant : 002E80Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry.
Auteurs : Martin Köllensperger [Autriche] ; 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 [Autriche] ; Gregor K. WenningSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2010.
English descriptors
- KwdEn :
- Age of Onset, Antiparkinson Agents (therapeutic use), Cerebellar Ataxia (diagnosis), Cerebellar Ataxia (physiopathology), Europe, Female, Humans, Hypotension, Orthostatic (diagnosis), Hypotension, Orthostatic (physiopathology), Levodopa (therapeutic use), Male, Middle Aged, Multiple System Atrophy (diagnosis), Multiple System Atrophy (physiopathology), Multiple System Atrophy (therapy), Parkinsonian Disorders (diagnosis), Parkinsonian Disorders (drug therapy), Parkinsonian Disorders (physiopathology), Registries, Shy-Drager Syndrome (diagnosis), Shy-Drager Syndrome (physiopathology).
- MESH :
- chemical , therapeutic use : Antiparkinson Agents, Levodopa.
- geographic : Europe.
- diagnosis : Cerebellar Ataxia, Hypotension, Orthostatic, Multiple System Atrophy, Parkinsonian Disorders, Shy-Drager Syndrome.
- drug therapy : Parkinsonian Disorders.
- physiopathology : Cerebellar Ataxia, Hypotension, Orthostatic, Multiple System Atrophy, Parkinsonian Disorders, Shy-Drager Syndrome.
- therapy : Multiple System Atrophy.
- Age of Onset, Female, Humans, Male, Middle Aged, Registries.
Abstract
Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like α-synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four-hundred thirty-seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA-P) and 32% as cerebellar type (MSA-C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas.
DOI: 10.1002/mds.23192
PubMed: 20922810
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Mathias</name></author><author><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name></author><author><name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F" last="Nilsson">Christer F. 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Wenning</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2010">2010</date><idno type="doi">10.1002/mds.23192</idno><idno type="RBID">pubmed:20922810</idno><idno type="pmid">20922810</idno><idno type="wicri:Area/PubMed/Corpus">001538</idno><idno type="wicri:Area/PubMed/Curation">001538</idno><idno type="wicri:Area/PubMed/Checkpoint">001691</idno><idno type="wicri:Area/Ncbi/Merge">002E79</idno><idno type="wicri:Area/Ncbi/Curation">002E79</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry.</title><author><name sortKey="Kollensperger, Martin" sort="Kollensperger, Martin" uniqKey="Kollensperger M" first="Martin" last="Köllensperger">Martin Köllensperger</name><affiliation wicri:level="1"><nlm:affiliation>Clinical Department of Neurology, Innsbruck Medical University, Austria.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Clinical Department of Neurology, Innsbruck Medical University</wicri:regionArea><wicri:noRegion>Innsbruck Medical University</wicri:noRegion></affiliation></author><author><name sortKey="Geser, Felix" sort="Geser, Felix" uniqKey="Geser F" first="Felix" last="Geser">Felix Geser</name></author><author><name sortKey="Ndayisaba, Jean Pierre" sort="Ndayisaba, Jean Pierre" uniqKey="Ndayisaba J" first="Jean-Pierre" last="Ndayisaba">Jean-Pierre Ndayisaba</name></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name></author><author><name sortKey="Seppi, Klaus" sort="Seppi, Klaus" uniqKey="Seppi K" first="Klaus" last="Seppi">Klaus Seppi</name></author><author><name sortKey="Ostergaard, Karen" sort="Ostergaard, Karen" uniqKey="Ostergaard K" first="Karen" last="Ostergaard">Karen Ostergaard</name></author><author><name sortKey="Dupont, Erik" sort="Dupont, Erik" uniqKey="Dupont E" first="Erik" last="Dupont">Erik Dupont</name></author><author><name sortKey="Cardozo, A" sort="Cardozo, A" uniqKey="Cardozo A" first="A" last="Cardozo">A. Cardozo</name></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name></author><author><name sortKey="Abele, Michael" sort="Abele, Michael" uniqKey="Abele M" first="Michael" last="Abele">Michael Abele</name></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name></author><author><name sortKey="Yekhlef, Farid" sort="Yekhlef, Farid" uniqKey="Yekhlef F" first="Farid" last="Yekhlef">Farid Yekhlef</name></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="Francois" last="Tison">Francois Tison</name></author><author><name sortKey="Daniels, Christine" sort="Daniels, Christine" uniqKey="Daniels C" first="Christine" last="Daniels">Christine Daniels</name></author><author><name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Günther" last="Deuschl">Günther Deuschl</name></author><author><name sortKey="Coelho, Miguel" sort="Coelho, Miguel" uniqKey="Coelho M" first="Miguel" last="Coelho">Miguel Coelho</name></author><author><name sortKey="Sampaio, Cristina" sort="Sampaio, Cristina" uniqKey="Sampaio C" first="Cristina" last="Sampaio">Cristina Sampaio</name></author><author><name sortKey="Bozi, Maria" sort="Bozi, Maria" uniqKey="Bozi M" first="Maria" last="Bozi">Maria Bozi</name></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name></author><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name></author><author><name sortKey="Mathias, Chris J" sort="Mathias, Chris J" uniqKey="Mathias C" first="Chris J" last="Mathias">Chris J. Mathias</name></author><author><name sortKey="Fowler, Clare" sort="Fowler, Clare" uniqKey="Fowler C" first="Clare" last="Fowler">Clare Fowler</name></author><author><name sortKey="Nilsson, Christer F" sort="Nilsson, Christer F" uniqKey="Nilsson C" first="Christer F" last="Nilsson">Christer F. Nilsson</name></author><author><name sortKey="Widner, H Kan" sort="Widner, H Kan" uniqKey="Widner H" first="H Kan" last="Widner">H Kan Widner</name></author><author><name sortKey="Schimke, Nicole" sort="Schimke, Nicole" uniqKey="Schimke N" first="Nicole" last="Schimke">Nicole Schimke</name></author><author><name sortKey="Oertel, Wolfgang" sort="Oertel, Wolfgang" uniqKey="Oertel W" first="Wolfgang" last="Oertel">Wolfgang Oertel</name></author><author><name sortKey="Del Sorbo, Francesca" sort="Del Sorbo, Francesca" uniqKey="Del Sorbo F" first="Francesca" last="Del Sorbo">Francesca Del Sorbo</name></author><author><name sortKey="Albanese, Alberto" sort="Albanese, Alberto" uniqKey="Albanese A" first="Alberto" last="Albanese">Alberto Albanese</name></author><author><name sortKey="Pellecchia, Maria Teresa" sort="Pellecchia, Maria Teresa" uniqKey="Pellecchia M" first="Maria Teresa" last="Pellecchia">Maria Teresa Pellecchia</name></author><author><name sortKey="Barone, Paolo" sort="Barone, Paolo" uniqKey="Barone P" first="Paolo" last="Barone">Paolo Barone</name></author><author><name sortKey="Djaldetti, Ruth" sort="Djaldetti, Ruth" uniqKey="Djaldetti R" first="Ruth" last="Djaldetti">Ruth Djaldetti</name></author><author><name sortKey="Colosimo, Carlo" sort="Colosimo, Carlo" uniqKey="Colosimo C" first="Carlo" last="Colosimo">Carlo Colosimo</name></author><author><name sortKey="Meco, Giuseppe" sort="Meco, Giuseppe" uniqKey="Meco G" first="Giuseppe" last="Meco">Giuseppe Meco</name></author><author><name sortKey="Gonzalez Mandly, Antonio" sort="Gonzalez Mandly, Antonio" uniqKey="Gonzalez Mandly A" first="Antonio" last="Gonzalez-Mandly">Antonio Gonzalez-Mandly</name></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="Jose" last="Berciano">Jose Berciano</name></author><author><name sortKey="Gurevich, Tanya" sort="Gurevich, Tanya" uniqKey="Gurevich T" first="Tanya" last="Gurevich">Tanya Gurevich</name></author><author><name sortKey="Giladi, Nir" sort="Giladi, Nir" uniqKey="Giladi N" first="Nir" last="Giladi">Nir Giladi</name></author><author><name sortKey="Galitzky, Monique" sort="Galitzky, Monique" uniqKey="Galitzky M" first="Monique" last="Galitzky">Monique Galitzky</name></author><author><name sortKey="Rascol, Olivier" sort="Rascol, Olivier" uniqKey="Rascol O" first="Olivier" last="Rascol">Olivier Rascol</name></author><author><name sortKey="Kamm, Christoph" sort="Kamm, Christoph" uniqKey="Kamm C" first="Christoph" last="Kamm">Christoph Kamm</name></author><author><name sortKey="Gasser, Thomas" sort="Gasser, Thomas" uniqKey="Gasser T" first="Thomas" last="Gasser">Thomas Gasser</name></author><author><name sortKey="Siebert, Uwe" sort="Siebert, Uwe" uniqKey="Siebert U" first="Uwe" last="Siebert">Uwe Siebert</name></author><author><name sortKey="Poewe, Werner" sort="Poewe, Werner" uniqKey="Poewe W" first="Werner" last="Poewe">Werner Poewe</name><affiliation><country>Autriche</country><placeName><settlement type="city">Innsbruck</settlement><region nuts="2" type="region">Tyrol (Land)</region></placeName><orgName type="university">Université de médecine d'Innsbruck</orgName></affiliation></author><author><name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K" last="Wenning">Gregor K. Wenning</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2010" type="published">2010</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Age of Onset</term><term>Antiparkinson Agents (therapeutic use)</term><term>Cerebellar Ataxia (diagnosis)</term><term>Cerebellar Ataxia (physiopathology)</term><term>Europe</term><term>Female</term><term>Humans</term><term>Hypotension, Orthostatic (diagnosis)</term><term>Hypotension, Orthostatic (physiopathology)</term><term>Levodopa (therapeutic use)</term><term>Male</term><term>Middle Aged</term><term>Multiple System Atrophy (diagnosis)</term><term>Multiple System Atrophy (physiopathology)</term><term>Multiple System Atrophy (therapy)</term><term>Parkinsonian Disorders (diagnosis)</term><term>Parkinsonian Disorders (drug therapy)</term><term>Parkinsonian Disorders (physiopathology)</term><term>Registries</term><term>Shy-Drager Syndrome (diagnosis)</term><term>Shy-Drager Syndrome (physiopathology)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Antiparkinson Agents</term><term>Levodopa</term></keywords><keywords scheme="MESH" type="geographic" xml:lang="en"><term>Europe</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cerebellar Ataxia</term><term>Hypotension, Orthostatic</term><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term><term>Shy-Drager Syndrome</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Cerebellar Ataxia</term><term>Hypotension, Orthostatic</term><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term><term>Shy-Drager Syndrome</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Multiple System Atrophy</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Age of Onset</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Registries</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like α-synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four-hundred thirty-seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA-P) and 32% as cerebellar type (MSA-C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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