Olivopontocerebellar atrophy: Toward a better nosological definition
Identifieur interne : 001555 ( Istex/Corpus ); précédent : 001554; suivant : 001556Olivopontocerebellar atrophy: Toward a better nosological definition
Auteurs : José Berciano ; Sylvia Boesch ; José M. Pérez-Ramos ; Gregor K. WenningSource :
- Movement Disorders [ 0885-3185 ] ; 2006-10.
English descriptors
Abstract
Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the CNS. This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. The term “OPCA” is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple‐system atrophy (MSA), and many cases of idiopathic late‐onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar‐plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society
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DOI: 10.1002/mds.21052
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ISTEX:2C3E801248EEA65EC5A255D2E3C565C137C015B3Le document en format XML
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This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. The term “OPCA” is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple‐system atrophy (MSA), and many cases of idiopathic late‐onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar‐plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>José Berciano MD</name><affiliations><json:string>Service of Neurology, University Hospital “Marqués de Valdecilla,” University of Cantabria, Santander, Spain</json:string></affiliations></json:item><json:item><name>Sylvia Boesch MD</name><affiliations><json:string>Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria</json:string></affiliations></json:item><json:item><name>José M. Pérez‐Ramos MD</name><affiliations><json:string>Service of Neurology, University Hospital “Marqués de Valdecilla,” University of Cantabria, Santander, Spain</json:string></affiliations></json:item><json:item><name>Gregor K. Wenning MD, PhD</name><affiliations><json:string>Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>spinocerebellar ataxia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>olivopontocerebellar atrophy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>multiple‐system atrophy</value></json:item></subject><language><json:string>eng</json:string></language><abstract>Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the CNS. This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. 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Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>spinocerebellar ataxia</term></item><item><term>olivopontocerebellar atrophy</term></item><item><term>multiple‐system atrophy</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Viewpoint</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2005-11-10">Received</change><change when="2006-03-28">Registration</change><change when="2006-10">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/2C3E801248EEA65EC5A255D2E3C565C137C015B3/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName><publisherLoc>Hoboken</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1531-8257</doi><issn type="print">0885-3185</issn><issn type="electronic">1531-8257</issn><idGroup><id type="product" value="MDS"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title><title type="short">Mov. 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This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. The term “OPCA” is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple‐system atrophy (MSA), and many cases of idiopathic late‐onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar‐plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Olivopontocerebellar atrophy: Toward a better nosological definition</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Olivopontocerebellar Atrophy</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Olivopontocerebellar atrophy: Toward a better nosological definition</title></titleInfo><name type="personal"><namePart type="given">José</namePart><namePart type="family">Berciano</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service of Neurology, University Hospital “Marqués de Valdecilla,” University of Cantabria, Santander, Spain</affiliation><description>Correspondence: Service of Neurology, University Hospital “Marqués de Valdecilla” (UC), 39008 Santander, Spain</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Sylvia</namePart><namePart type="family">Boesch</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">José M.</namePart><namePart type="family">Pérez‐Ramos</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service of Neurology, University Hospital “Marqués de Valdecilla,” University of Cantabria, Santander, Spain</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>Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">reviewArticle</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2006-10</dateIssued><dateCaptured encoding="w3cdtf">2005-11-10</dateCaptured><dateValid encoding="w3cdtf">2006-03-28</dateValid><copyrightDate encoding="w3cdtf">2006</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">3</extent><extent unit="tables">1</extent><extent unit="references">50</extent><extent unit="words">4320</extent></physicalDescription><abstract lang="en">Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the CNS. This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar‐plus syndrome. The term “OPCA” is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple‐system atrophy (MSA), and many cases of idiopathic late‐onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar‐plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label “OPCA” is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA. © 2006 Movement Disorder Society</abstract><note type="funding">Centro de Investigación de Enfermedades Neurológicas, Nodo HUMV/UC (ISCIII, Madrid, Spain)</note><note type="funding">IFIMAV (Fundación Marqués de Valdecilla, Santander, Spain)</note><note type="funding">Austrian Parkinson Society</note><note type="funding">European MSA Study Group</note><subject lang="en"><genre>Keywords</genre><topic>spinocerebellar ataxia</topic><topic>olivopontocerebellar atrophy</topic><topic>multiple‐system atrophy</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><subject><genre>article category</genre><topic>Viewpoint</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>2006</date><detail type="volume"><caption>vol.</caption><number>21</number></detail><detail type="issue"><caption>no.</caption><number>10</number></detail><extent unit="pages"><start>1607</start><end>1613</end><total>7</total></extent></part></relatedItem><identifier type="istex">2C3E801248EEA65EC5A255D2E3C565C137C015B3</identifier><identifier type="DOI">10.1002/mds.21052</identifier><identifier type="ArticleID">MDS21052</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2006 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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