Olivopontocerebellar atrophy: toward a better nosological definition.
Identifieur interne : 002B38 ( PubMed/Corpus ); précédent : 002B37; suivant : 002B39Olivopontocerebellar atrophy: toward a better nosological definition.
Auteurs : José Berciano ; Sylvia Boesch ; José M. Pérez-Ramos ; Gregor K. WenningSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2006.
English descriptors
- KwdEn :
- Brain Stem (pathology), Cerebellum (pathology), Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Multiple System Atrophy (diagnosis), Multiple System Atrophy (pathology), Neurologic Examination, Olivopontocerebellar Atrophies (diagnosis), Olivopontocerebellar Atrophies (pathology), Prognosis, Spinocerebellar Ataxias (diagnosis), Spinocerebellar Ataxias (pathology), Tomography, X-Ray Computed.
- MESH :
- diagnosis : Multiple System Atrophy, Olivopontocerebellar Atrophies, Spinocerebellar Ataxias.
- pathology : Brain Stem, Cerebellum, Multiple System Atrophy, Olivopontocerebellar Atrophies, Spinocerebellar Ataxias.
- Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Neurologic Examination, Prognosis, Tomography, X-Ray Computed.
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.
DOI: 10.1002/mds.21052
PubMed: 16874757
Links to Exploration step
pubmed:16874757Le document en format XML
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Wenning</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2006">2006</date><idno type="doi">10.1002/mds.21052</idno><idno type="RBID">pubmed:16874757</idno><idno type="pmid">16874757</idno><idno type="wicri:Area/PubMed/Corpus">002B38</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Olivopontocerebellar atrophy: toward a better nosological definition.</title><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name><affiliation><nlm:affiliation>Service of Neurology, University Hospital Marqués de Valdecilla, University of Cantabria, Santander, Spain. jaberciano@humv.es</nlm:affiliation></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name></author><author><name sortKey="Perez Ramos, Jose M" sort="Perez Ramos, Jose M" uniqKey="Perez Ramos J" first="José M" last="Pérez-Ramos">José M. Pérez-Ramos</name></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="ISSN">0885-3185</idno><imprint><date when="2006" type="published">2006</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Brain Stem (pathology)</term><term>Cerebellum (pathology)</term><term>Diagnosis, Differential</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Multiple System Atrophy (diagnosis)</term><term>Multiple System Atrophy (pathology)</term><term>Neurologic Examination</term><term>Olivopontocerebellar Atrophies (diagnosis)</term><term>Olivopontocerebellar Atrophies (pathology)</term><term>Prognosis</term><term>Spinocerebellar Ataxias (diagnosis)</term><term>Spinocerebellar Ataxias (pathology)</term><term>Tomography, X-Ray Computed</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Multiple System Atrophy</term><term>Olivopontocerebellar Atrophies</term><term>Spinocerebellar Ataxias</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain Stem</term><term>Cerebellum</term><term>Multiple System Atrophy</term><term>Olivopontocerebellar Atrophies</term><term>Spinocerebellar Ataxias</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Diagnosis, Differential</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Neurologic Examination</term><term>Prognosis</term><term>Tomography, X-Ray Computed</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml: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.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">16874757</PMID><DateCreated><Year>2006</Year><Month>10</Month><Day>26</Day></DateCreated><DateCompleted><Year>2007</Year><Month>02</Month><Day>02</Day></DateCompleted><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>21</Volume><Issue>10</Issue><PubDate><Year>2006</Year><Month>Oct</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Olivopontocerebellar atrophy: toward a better nosological definition.</ArticleTitle><Pagination><MedlinePgn>1607-13</MedlinePgn></Pagination><Abstract><AbstractText>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.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Berciano</LastName><ForeName>José</ForeName><Initials>J</Initials><AffiliationInfo><Affiliation>Service of Neurology, University Hospital Marqués de Valdecilla, University of Cantabria, Santander, Spain. jaberciano@humv.es</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Boesch</LastName><ForeName>Sylvia</ForeName><Initials>S</Initials></Author><Author ValidYN="Y"><LastName>Pérez-Ramos</LastName><ForeName>José M</ForeName><Initials>JM</Initials></Author><Author ValidYN="Y"><LastName>Wenning</LastName><ForeName>Gregor K</ForeName><Initials>GK</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D003160">Comparative Study</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001933">Brain Stem</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D002531">Cerebellum</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003937">Diagnosis, Differential</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008279">Magnetic Resonance Imaging</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D019578">Multiple System Atrophy</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009460">Neurologic Examination</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009849">Olivopontocerebellar Atrophies</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D011379">Prognosis</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D020754">Spinocerebellar Ataxias</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014057">Tomography, X-Ray Computed</DescriptorName></MeshHeading></MeshHeadingList><NumberOfReferences>50</NumberOfReferences></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2006</Year><Month>7</Month><Day>29</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2007</Year><Month>2</Month><Day>3</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2006</Year><Month>7</Month><Day>29</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="doi">10.1002/mds.21052</ArticleId><ArticleId IdType="pubmed">16874757</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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