Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome)
Abstract
Glial cytoplasmic inclusions (GCIs) were demonstrated by silver staining, immunocytochemistry and by electron microscopy in the central nervous system (CNS) of 11 patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome. Although their configuration in light microscope can sometimes resemble neurofibrillary tangles, their cellular localisation, measurements, ultrastructure, immunocytochemical characteristics and regional distribution all differ from these Alzheimer type changes. The majority of GCIs were localized in the white matter and appeared to be accompanied by an increase in the number of interfascicular oligodendroglial cells and pallor or loss of myelin staining. Our histological, ultrastructural and immunocytochemical findings all indicate that the cells which contain GCIs are oligodendrocytes and the inclusions themselves are composed of tubular structures. The presence of the until now unknown GCIs in all the 11 CNS, but not in age- and sex-matched control brains, indicates that GCI is a cellular change characteristic of multiple system atrophy and the three syndromes are various manifestations of the same disease.
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MSA is characterized neuropathologically by α-syn-positive glial cytoplasmic inclusions (GCIs) in oligodendroglia.
“MSA is a synucleinopathy, however, unlike PD and dementia with Lewy bodies, in which α-syn accumulates and aggregates in neurons, MSA is characterized neuropathologically by α-syn-positive glial cytoplasmic inclusions (GCIs) in oligodendroglia [1, 42, 54, 58, 59].”
From Novel oligodendroglial alpha synuclein viral vector models of multiple system atrophy: studies in rodents and nonhuman primates (2017) · cites this paper as [42]
Papp and Lantos first described accumulation of insoluble protein aggregates in oligodendrocytes, naming GCIs as the pathological hallmark of MSA.
“Using silver impregnation (Gallyas method), Papp and Lantos first described the accumulation of insoluble protein aggregates in oligodendrocytes, naming GCIs as the pathological hallmark of MSA [42].”
From Novel oligodendroglial alpha synuclein viral vector models of multiple system atrophy: studies in rodents and nonhuman primates (2017) · cites this paper as [42]
The pathological hallmark of MSA is abnormal accumulation of α-syn in the cytoplasm of oligodendrocytes, named glial cytoplasmic inclusions (GCIs).
“The pathological hallmark of MSA is the abnormal accumulation of α-syn in the cytoplasm of oligodendrocytes, named glial cytoplasmic inclusions (GCIs).271–275”
From Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023) · cites this paper as [271]
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- Authors
- Papp, M. I., Kahn, J. E., & Lantos, P. L.
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1989-12
- Volume
- 94
- Issue
- 1-3
- Pages
- 79-100
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2090048744
- Cited by
- 991
- References
- 32
- Citations by year
- 2026: 14 · 2025: 34 · 2024: 49 · 2023: 38 · 2022: 38 · 2021: 36 · 2020: 64 · 2019: 40 · 2018: 29 · 2017: 42 · 2016: 27 · 2015: 48 · 2014: 41 · 2013: 34 · 2012: 22
- FWCI
- 7.66
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- glial cytoplasmic inclusions, multiple system atrophy, striatonigral degeneration, olivopontocerebellar atrophy, Shy-Drager syndrome, oligodendrocytes, white matter, demyelination, neurofibrillary tangles, ultrastructure, immunocytochemistry, central nervous system pathology
- MeSH terms
- Adult, Aged, Aged, 80 and over, Autonomic Nervous System Diseases, Inclusion Bodies, Corpus Striatum, Female, Humans, Male, Middle Aged, Neuroglia, Olivopontocerebellar Atrophies, Shy-Drager Syndrome, Spinocerebellar Degenerations, Substantia Nigra
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