Olivopontocerebellar atrophy: Toward a better nosological definition
Identifieur interne : 003305 ( Main/Exploration ); précédent : 003304; suivant : 003306Olivopontocerebellar atrophy: Toward a better nosological definition
Auteurs : José Berciano [Espagne] ; Sylvia Boesch [Autriche] ; José M. Pérez-Ramos [Espagne] ; Gregor K. Wenning [Autriche]Source :
- Movement Disorders [ 0885-3185 ] ; 2006-10.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Brain Stem (pathology), Cerebellum (pathology), Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Multiple System Atrophy (diagnosis), Multiple System Atrophy (pathology), Multiple system atrophy, Nervous system diseases, Neurologic Examination, Olivopontocerebellar, Olivopontocerebellar Atrophies (diagnosis), Olivopontocerebellar Atrophies (pathology), Prognosis, Spinocerebellar Ataxias (diagnosis), Spinocerebellar Ataxias (pathology), Spinocerebellar ataxia, Tomography, X-Ray Computed, multiple‐system atrophy, olivopontocerebellar atrophy, spinocerebellar ataxia.
- 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. © 2006 Movement Disorder Society
Url:
DOI: 10.1002/mds.21052
Affiliations:
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Le document en format XML
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2006-10">2006-10</date><biblScope unit="vol">21</biblScope><biblScope unit="issue">10</biblScope><biblScope unit="page" from="1607">1607</biblScope><biblScope unit="page" to="1613">1613</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">2C3E801248EEA65EC5A255D2E3C565C137C015B3</idno><idno type="DOI">10.1002/mds.21052</idno><idno type="ArticleID">MDS21052</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></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>Multiple system atrophy</term><term>Nervous system diseases</term><term>Neurologic Examination</term><term>Olivopontocerebellar</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>Spinocerebellar ataxia</term><term>Tomography, X-Ray Computed</term><term>multiple‐system atrophy</term><term>olivopontocerebellar atrophy</term><term>spinocerebellar ataxia</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><keywords scheme="Pascal" xml:lang="fr"><term>Ataxie spinocérébelleuse</term><term>Atrophie multisystématisée</term><term>Olivopontocérébelleux</term><term>Système nerveux pathologie</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></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. © 2006 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Autriche</li><li>Espagne</li></country><region><li>Tyrol (Land)</li></region><settlement><li>Innsbruck</li></settlement><orgName><li>Université de médecine d'Innsbruck</li></orgName></list><tree><country name="Espagne"><noRegion><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name></noRegion><name sortKey="Perez Amos, Jose M" sort="Perez Amos, Jose M" uniqKey="Perez Amos J" first="José M." last="Pérez-Ramos">José M. Pérez-Ramos</name></country><country name="Autriche"><region name="Tyrol (Land)"><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name></region><name sortKey="Wenning, Gregor K" sort="Wenning, Gregor K" uniqKey="Wenning G" first="Gregor K." last="Wenning">Gregor K. Wenning</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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