Multiple system atrophy of the cerebellar type: clinical state of the art.
Identifieur interne : 000508 ( Main/Exploration ); précédent : 000507; suivant : 000509Multiple system atrophy of the cerebellar type: clinical state of the art.
Auteurs : David J. Lin [États-Unis] ; Katherine L. Hermann ; Jeremy D. SchmahmannSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2014.
English descriptors
- KwdEn :
- MESH :
- chemical , metabolism : alpha-Synuclein.
- metabolism : Cerebellar Ataxia, Cerebellum, Multiple System Atrophy, Parkinsonian Disorders.
- therapy : Multiple System Atrophy, Parkinsonian Disorders.
- Animals, Disease Models, Animal, Humans.
Abstract
Multiple system atrophy (MSA) is a late-onset, sporadic neurodegenerative disorder clinically characterized by autonomic failure and either poorly levodopa-responsive parkinsonism or cerebellar ataxia. It is neuropathologically defined by widespread and abundant central nervous system α-synuclein-positive glial cytoplasmic inclusions and striatonigral and/or olivopontocerebellar neurodegeneration. There are two clinical subtypes of MSA distinguished by the predominant motor features: the parkinsonian variant (MSA-P) and the cerebellar variant (MSA-C). Despite recent progress in understanding the pathobiology of MSA, investigations into the symptomatology and natural history of the cerebellar variant of the disease have been limited. MSA-C presents a unique challenge to both clinicians and researchers alike. A key question is how to distinguish early in the disease course between MSA-C and other causes of adult-onset cerebellar ataxia. This is a particularly difficult question, because the clinical framework for conceptualizing and studying sporadic adult-onset ataxias continues to undergo flux. To date, several investigations have attempted to identify clinical features, imaging, and other biomarkers that may be predictive of MSA-C. This review presents a clinically oriented overview of our current understanding of MSA-C with a focus on evidence for distinguishing MSA-C from other sporadic, adult-onset ataxias.
DOI: 10.1002/mds.25847
PubMed: 24615754
Affiliations:
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Hermann</name></author><author><name sortKey="Schmahmann, Jeremy D" sort="Schmahmann, Jeremy D" uniqKey="Schmahmann J" first="Jeremy D" last="Schmahmann">Jeremy D. 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Lin</name><affiliation wicri:level="2"><nlm:affiliation>Ataxia Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Ataxia Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts</wicri:regionArea><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Hermann, Katherine L" sort="Hermann, Katherine L" uniqKey="Hermann K" first="Katherine L" last="Hermann">Katherine L. Hermann</name></author><author><name sortKey="Schmahmann, Jeremy D" sort="Schmahmann, Jeremy D" uniqKey="Schmahmann J" first="Jeremy D" last="Schmahmann">Jeremy D. Schmahmann</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="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Animals</term><term>Cerebellar Ataxia (metabolism)</term><term>Cerebellum (metabolism)</term><term>Disease Models, Animal</term><term>Humans</term><term>Multiple System Atrophy (metabolism)</term><term>Multiple System Atrophy (therapy)</term><term>Parkinsonian Disorders (metabolism)</term><term>Parkinsonian Disorders (therapy)</term><term>alpha-Synuclein (metabolism)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>alpha-Synuclein</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Cerebellar Ataxia</term><term>Cerebellum</term><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Multiple System Atrophy</term><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Animals</term><term>Disease Models, Animal</term><term>Humans</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Multiple system atrophy (MSA) is a late-onset, sporadic neurodegenerative disorder clinically characterized by autonomic failure and either poorly levodopa-responsive parkinsonism or cerebellar ataxia. It is neuropathologically defined by widespread and abundant central nervous system α-synuclein-positive glial cytoplasmic inclusions and striatonigral and/or olivopontocerebellar neurodegeneration. There are two clinical subtypes of MSA distinguished by the predominant motor features: the parkinsonian variant (MSA-P) and the cerebellar variant (MSA-C). Despite recent progress in understanding the pathobiology of MSA, investigations into the symptomatology and natural history of the cerebellar variant of the disease have been limited. MSA-C presents a unique challenge to both clinicians and researchers alike. A key question is how to distinguish early in the disease course between MSA-C and other causes of adult-onset cerebellar ataxia. This is a particularly difficult question, because the clinical framework for conceptualizing and studying sporadic adult-onset ataxias continues to undergo flux. To date, several investigations have attempted to identify clinical features, imaging, and other biomarkers that may be predictive of MSA-C. This review presents a clinically oriented overview of our current understanding of MSA-C with a focus on evidence for distinguishing MSA-C from other sporadic, adult-onset ataxias.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Massachusetts</li></region></list><tree><noCountry><name sortKey="Hermann, Katherine L" sort="Hermann, Katherine L" uniqKey="Hermann K" first="Katherine L" last="Hermann">Katherine L. Hermann</name><name sortKey="Schmahmann, Jeremy D" sort="Schmahmann, Jeremy D" uniqKey="Schmahmann J" first="Jeremy D" last="Schmahmann">Jeremy D. Schmahmann</name></noCountry><country name="États-Unis"><region name="Massachusetts"><name sortKey="Lin, David J" sort="Lin, David J" uniqKey="Lin D" first="David J" last="Lin">David J. 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