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Quantitative pathological changes in the cerebellum of multiple system atrophy
1Dr Richard A. Armstrong, Vision Sciences, Aston University, Birmingham B4 7ET, UK, phone: 0121-204-4102, fax: 0121-204-4048,
Folia Neuropathologica
|October 8, 2015
Summary
Multiple system atrophy (MSA) causes cerebellar pathology, including Purkinje cell loss and vacuolation. These changes are topographically distributed and may impact brain function.
Area of Science:
- Neuroscience
- Neuropathology
Background:
- Multiple system atrophy (MSA) is a rare neurodegenerative disorder.
- MSA is characterized by parkinsonism, ataxia, and autonomic dysfunction.
- Pathology includes neuronal loss, gliosis, and α-synuclein-immunoreactive glial cytoplasmic inclusions (GCI).
Purpose of the Study:
- To quantify cerebellar pathology in MSA.
- To analyze the density and spatial pattern of pathological changes.
- To investigate the relationship between pathology and neuronal loss.
Main Methods:
- Studied α-synuclein-immunolabelled cerebellar sections from 10 MSA and 10 control cases.
- Quantified densities of Purkinje cells (PC), vacuoles, and glial cells.
- Utilized Principal Components Analysis (PCA) to identify sources of neuropathological variation.
Main Results:
- MSA cases showed decreased PC densities and increased vacuolation in the granule cell layer (GL) compared to controls.
- GCI were present in cerebellar white matter in six MSA cases.
- Vacuoles were clustered in the molecular layer (ML) and GL, with regular distribution.
- Purkinje cells exhibited random or regular distribution with large gaps.
- Negative correlations observed between glial/neuronal densities and vacuole densities, indicating gliosis and vacuolation response to neuronal loss.
Conclusions:
- Cell loss and vacuolation in the GCL, and PC loss, are significant pathological changes in MSA cerebellums.
- Pathological changes exhibit topographic distribution.
- Cerebellar pathology may influence cerebral function in MSA via the cerebello-dentato-thalamic tract.
