Multiple system atrophy: a review of 203 pathologically proven cases.
Identifieur interne : 004C69 ( Ncbi/Checkpoint ); précédent : 004C68; suivant : 004C70Multiple system atrophy: a review of 203 pathologically proven cases.
Auteurs : G K Wenning [Royaume-Uni] ; F. Tison ; Y. Ben Shlomo ; S E Daniel ; N P QuinnSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1997.
English descriptors
- KwdEn :
- Aged, Brain (pathology), Corpus Striatum (pathology), Female, Humans, Male, Middle Aged, Nerve Degeneration (physiology), Neurologic Examination, Olivopontocerebellar Atrophies (diagnosis), Olivopontocerebellar Atrophies (pathology), Parkinson Disease, Secondary (diagnosis), Parkinson Disease, Secondary (pathology), Shy-Drager Syndrome (diagnosis), Shy-Drager Syndrome (pathology), Spinal Cord (pathology), Substantia Nigra (pathology).
- MESH :
- diagnosis : Olivopontocerebellar Atrophies, Parkinson Disease, Secondary, Shy-Drager Syndrome.
- pathology : Brain, Corpus Striatum, Olivopontocerebellar Atrophies, Parkinson Disease, Secondary, Shy-Drager Syndrome, Spinal Cord, Substantia Nigra.
- physiology : Nerve Degeneration.
- Aged, Female, Humans, Male, Middle Aged, Neurologic Examination.
Abstract
We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.
DOI: 10.1002/mds.870120203
PubMed: 9087971
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Multiple system atrophy: a review of 203 pathologically proven cases.</title><author><name sortKey="Wenning, G K" sort="Wenning, G K" uniqKey="Wenning G" first="G K" last="Wenning">G K Wenning</name><affiliation wicri:level="2"><nlm:affiliation>University Department of Clinical Neurology, London, England.</nlm:affiliation><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Department of Clinical Neurology, London</wicri:cityArea></affiliation></author><author><name sortKey="Tison, F" sort="Tison, F" uniqKey="Tison F" first="F" last="Tison">F. Tison</name></author><author><name sortKey="Ben Shlomo, Y" sort="Ben Shlomo, Y" uniqKey="Ben Shlomo Y" first="Y" last="Ben Shlomo">Y. Ben Shlomo</name></author><author><name sortKey="Daniel, S E" sort="Daniel, S E" uniqKey="Daniel S" first="S E" last="Daniel">S E Daniel</name></author><author><name sortKey="Quinn, N P" sort="Quinn, N P" uniqKey="Quinn N" first="N P" last="Quinn">N P Quinn</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1997">1997</date><idno type="RBID">pubmed:9087971</idno><idno type="pmid">9087971</idno><idno type="doi">10.1002/mds.870120203</idno><idno type="wicri:Area/PubMed/Corpus">004713</idno><idno type="wicri:Area/PubMed/Curation">004713</idno><idno type="wicri:Area/PubMed/Checkpoint">004617</idno><idno type="wicri:Area/Ncbi/Merge">004C69</idno><idno type="wicri:Area/Ncbi/Curation">004C69</idno><idno type="wicri:Area/Ncbi/Checkpoint">004C69</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Multiple system atrophy: a review of 203 pathologically proven cases.</title><author><name sortKey="Wenning, G K" sort="Wenning, G K" uniqKey="Wenning G" first="G K" last="Wenning">G K Wenning</name><affiliation wicri:level="2"><nlm:affiliation>University Department of Clinical Neurology, London, England.</nlm:affiliation><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Department of Clinical Neurology, London</wicri:cityArea></affiliation></author><author><name sortKey="Tison, F" sort="Tison, F" uniqKey="Tison F" first="F" last="Tison">F. Tison</name></author><author><name sortKey="Ben Shlomo, Y" sort="Ben Shlomo, Y" uniqKey="Ben Shlomo Y" first="Y" last="Ben Shlomo">Y. Ben Shlomo</name></author><author><name sortKey="Daniel, S E" sort="Daniel, S E" uniqKey="Daniel S" first="S E" last="Daniel">S E Daniel</name></author><author><name sortKey="Quinn, N P" sort="Quinn, N P" uniqKey="Quinn N" first="N P" last="Quinn">N P Quinn</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="1997" type="published">1997</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Aged</term><term>Brain (pathology)</term><term>Corpus Striatum (pathology)</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Nerve Degeneration (physiology)</term><term>Neurologic Examination</term><term>Olivopontocerebellar Atrophies (diagnosis)</term><term>Olivopontocerebellar Atrophies (pathology)</term><term>Parkinson Disease, Secondary (diagnosis)</term><term>Parkinson Disease, Secondary (pathology)</term><term>Shy-Drager Syndrome (diagnosis)</term><term>Shy-Drager Syndrome (pathology)</term><term>Spinal Cord (pathology)</term><term>Substantia Nigra (pathology)</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Olivopontocerebellar Atrophies</term><term>Parkinson Disease, Secondary</term><term>Shy-Drager Syndrome</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term><term>Corpus Striatum</term><term>Olivopontocerebellar Atrophies</term><term>Parkinson Disease, Secondary</term><term>Shy-Drager Syndrome</term><term>Spinal Cord</term><term>Substantia Nigra</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Nerve Degeneration</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Neurologic Examination</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li></region></list><tree><noCountry><name sortKey="Ben Shlomo, Y" sort="Ben Shlomo, Y" uniqKey="Ben Shlomo Y" first="Y" last="Ben Shlomo">Y. Ben Shlomo</name><name sortKey="Daniel, S E" sort="Daniel, S E" uniqKey="Daniel S" first="S E" last="Daniel">S E Daniel</name><name sortKey="Quinn, N P" sort="Quinn, N P" uniqKey="Quinn N" first="N P" last="Quinn">N P Quinn</name><name sortKey="Tison, F" sort="Tison, F" uniqKey="Tison F" first="F" last="Tison">F. Tison</name></noCountry><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Wenning, G K" sort="Wenning, G K" uniqKey="Wenning G" first="G K" last="Wenning">G K Wenning</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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