Olivopontocerebellar pathology in multiple system atrophy.
Identifieur interne : 004A75 ( Ncbi/Curation ); précédent : 004A74; suivant : 004A76Olivopontocerebellar pathology in multiple system atrophy.
Auteurs : G K Wenning [Royaume-Uni] ; F. Tison ; L. Elliott ; N P Quinn ; S E DanielSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1996.
English descriptors
- KwdEn :
- Adult, Aged, Brain (pathology), Cell Count, Cerebellum (pathology), Cerebral Cortex (pathology), Female, Humans, Male, Middle Aged, Nerve Degeneration (physiology), Neurons (pathology), Olivary Nucleus (pathology), Olivopontocerebellar Atrophies (pathology), Parkinson Disease (pathology), Pons (pathology), Purkinje Cells (pathology).
- MESH :
- pathology : Brain, Cerebellum, Cerebral Cortex, Neurons, Olivary Nucleus, Olivopontocerebellar Atrophies, Parkinson Disease, Pons, Purkinje Cells.
- physiology : Nerve Degeneration.
- Adult, Aged, Cell Count, Female, Humans, Male, Middle Aged.
Abstract
Olivopontocerebellar atrophy (OPCA) is widely accepted as part of the neuropathological spectrum of multiple system atrophy (MSA). The distribution of affected sites in the olivopontocerebellar (OPC) system and their interrelationship remain poorly understood due to lack of quantitative studies. To further investigate the OPC pathology in MSA, we performed a morphometric analysis of 20 MSA cases and eight healthy controls. In the MSA cases, mean neuronal cell densities were significantly reduced in (medial and dorsal) accessory and principal inferior olives, pontine nuclei, cerebellar vermis (except nodulus), and hemispheres. Inferior olives and pontine nuclei were more severely affected than cerebellar Purkinje cells in most cases. Cerebellar Purkinje cells were more severely depleted in vermis rather than in hemisphere. There was a poor topographic correlation between neuronal cell loss in inferior olives and cerebellar cortex. These results suggest a primary degeneration of olivopontine nuclei and cerebellar Purkinje cells in OPCA. Inferior olives, pontine nuclei and cerebellar cortex were all significantly more severely affected in cases with a pure or predominating cerebellar syndrome (OPCA type, n = 4) compared to those with pure or predominating parkinsonism (SND type, n = 14). However, although cerebellar signs had been noted in life in only six cases, morphometry revealed OPCA in 17 of the 20 MSA brains.
DOI: 10.1002/mds.870110207
PubMed: 8684385
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pubmed:8684385Le document en format XML
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<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="1"><nlm:affiliation>Department of Clinical Neurology, Institute of Neurology, London, England, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Clinical Neurology, Institute of Neurology, London, England</wicri:regionArea>
<wicri:noRegion>England</wicri:noRegion>
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<author><name sortKey="Tison, F" sort="Tison, F" uniqKey="Tison F" first="F" last="Tison">F. Tison</name>
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<author><name sortKey="Elliott, L" sort="Elliott, L" uniqKey="Elliott L" first="L" last="Elliott">L. Elliott</name>
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<author><name sortKey="Quinn, N P" sort="Quinn, N P" uniqKey="Quinn N" first="N P" last="Quinn">N P Quinn</name>
</author>
<author><name sortKey="Daniel, S E" sort="Daniel, S E" uniqKey="Daniel S" first="S E" last="Daniel">S E Daniel</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Olivopontocerebellar pathology in multiple system atrophy.</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="1"><nlm:affiliation>Department of Clinical Neurology, Institute of Neurology, London, England, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Clinical Neurology, Institute of Neurology, London, England</wicri:regionArea>
<wicri:noRegion>England</wicri:noRegion>
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<author><name sortKey="Tison, F" sort="Tison, F" uniqKey="Tison F" first="F" last="Tison">F. Tison</name>
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<author><name sortKey="Elliott, L" sort="Elliott, L" uniqKey="Elliott L" first="L" last="Elliott">L. Elliott</name>
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<author><name sortKey="Quinn, N P" sort="Quinn, N P" uniqKey="Quinn N" first="N P" last="Quinn">N P Quinn</name>
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<author><name sortKey="Daniel, S E" sort="Daniel, S E" uniqKey="Daniel S" first="S E" last="Daniel">S E Daniel</name>
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<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="1996" type="published">1996</date>
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<term>Aged</term>
<term>Brain (pathology)</term>
<term>Cell Count</term>
<term>Cerebellum (pathology)</term>
<term>Cerebral Cortex (pathology)</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Nerve Degeneration (physiology)</term>
<term>Neurons (pathology)</term>
<term>Olivary Nucleus (pathology)</term>
<term>Olivopontocerebellar Atrophies (pathology)</term>
<term>Parkinson Disease (pathology)</term>
<term>Pons (pathology)</term>
<term>Purkinje Cells (pathology)</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term>
<term>Cerebellum</term>
<term>Cerebral Cortex</term>
<term>Neurons</term>
<term>Olivary Nucleus</term>
<term>Olivopontocerebellar Atrophies</term>
<term>Parkinson Disease</term>
<term>Pons</term>
<term>Purkinje Cells</term>
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<keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Nerve Degeneration</term>
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<term>Aged</term>
<term>Cell Count</term>
<term>Female</term>
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<term>Male</term>
<term>Middle Aged</term>
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<front><div type="abstract" xml:lang="en">Olivopontocerebellar atrophy (OPCA) is widely accepted as part of the neuropathological spectrum of multiple system atrophy (MSA). The distribution of affected sites in the olivopontocerebellar (OPC) system and their interrelationship remain poorly understood due to lack of quantitative studies. To further investigate the OPC pathology in MSA, we performed a morphometric analysis of 20 MSA cases and eight healthy controls. In the MSA cases, mean neuronal cell densities were significantly reduced in (medial and dorsal) accessory and principal inferior olives, pontine nuclei, cerebellar vermis (except nodulus), and hemispheres. Inferior olives and pontine nuclei were more severely affected than cerebellar Purkinje cells in most cases. Cerebellar Purkinje cells were more severely depleted in vermis rather than in hemisphere. There was a poor topographic correlation between neuronal cell loss in inferior olives and cerebellar cortex. These results suggest a primary degeneration of olivopontine nuclei and cerebellar Purkinje cells in OPCA. Inferior olives, pontine nuclei and cerebellar cortex were all significantly more severely affected in cases with a pure or predominating cerebellar syndrome (OPCA type, n = 4) compared to those with pure or predominating parkinsonism (SND type, n = 14). However, although cerebellar signs had been noted in life in only six cases, morphometry revealed OPCA in 17 of the 20 MSA brains.</div>
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