Quantitative pathological changes in the cerebellum of multiple system atrophy

Richard A. Armstrong

Research output: Contribution to journalArticle

Abstract

Multiple system atrophy (MSA) is a rare neurodegenerative disorder associated with parkinsonism, ataxia, and autonomic dysfunction. Its pathology is primarily subcortical comprising vacuolation, neuronal loss, gliosis, and α-synuclein-immunoreactive glial cytoplasmic inclusions (GO). To quantify cerebellar pathology in MSA, the density and spatial pattern of the pathological changes were studied in α-synuclein-immunolabelled sections of the cerebellar hemisphere in 10 MSA and 10 control cases. In MSA, densities of Purkinje cells (PC) were decreased and vacuoles in the granule cell layer (GL) increased compared with controls. In six MSA cases, GCI were present in cerebellar white matter. In the molecular layer (ML) and GL of MSA, vacuoles were clustered, the clusters exhibiting a regular distribution parallel to the edge of the folia. Purkinje cells were randomly or regularly distributed with large gaps between surviving cells. Densities of glial cells and surviving neurons in the ML and surviving cells and vacuoles in the GL were negatively correlated consistent with gliosis and vacuolation in response to neuronal loss. Principal components analysis (PCA) suggested vacuole densities in the ML and vacuole density and cell losses in the GL were the main source of neuropathological variation among cases. The data suggest that: (1) cell losses and vacuolation of the GCL and loss of PC were the most significant pathological changes in the cases studied, (2) pathological changes were topographically distributed, and (3) cerebellar pathology could influence cerebral function in MSA via the cerebello-dentato-thalamic tract.

Original languageEnglish
Pages (from-to)193-202
Number of pages10
JournalFolia Neuropathologica
Volume53
Issue number3
DOIs
Publication statusPublished - Mar 2015

Fingerprint

Multiple System Atrophy
Cerebellum
Vacuoles
Purkinje Cells
Synucleins
Gliosis
Pathology
Neuroglia
Inclusion Bodies
Parkinsonian Disorders
Ataxia
Principal Component Analysis
Neurodegenerative Diseases
Cell Count
Neurons

Bibliographical note

Copyright: © 2015 Mossakowski Medical Research Centre Polish Academy of Sciences and the Polish Association of Neuropathologists. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.

Keywords

  • cerebellum
  • multiple system atrophy
  • spatial pattern
  • vacuolation
  • α-synuclein
  • alpha-synuclein

Cite this

Armstrong, Richard A. / Quantitative pathological changes in the cerebellum of multiple system atrophy. In: Folia Neuropathologica. 2015 ; Vol. 53, No. 3. pp. 193-202.
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Quantitative pathological changes in the cerebellum of multiple system atrophy. / Armstrong, Richard A.

In: Folia Neuropathologica, Vol. 53, No. 3, 03.2015, p. 193-202.

Research output: Contribution to journalArticle

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