Immunocytochemical and ultrastructural studies of neuronal and oligodendroglial cytoplasmic inclusions in multiple system atrophy. 1. Neuronal cytoplasmic inclusions

Neuronal alterations in five cases of multiple system atrophy (MSA) were investigated histologically, immunocytochemically and ultrastructurally. Argentophilic neuronal cytoplasmic inclusions (NCIs) were observed in all cases. They were distributed, in order of decreasing frequency, in the pontine n...

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Published inActa neuropathologica Vol. 83; no. 5; p. 453
Main Authors Arima, K, Murayama, S, Mukoyama, M, Inose, T
Format Journal Article
LanguageEnglish
Published Germany 01.04.1992
Subjects
Adult
Atrophy - pathology
Cytoplasmic Granules - immunology
Cytoplasmic Granules - ultrastructure
Female
Humans
Immunohistochemistry
Intermediate Filaments - immunology
Male
Microscopy, Electron
Microtubules - immunology
Middle Aged
Neurons - immunology
Neurons - ultrastructure
Oligodendroglia - immunology
Oligodendroglia - ultrastructure
Ubiquitins - immunology
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Summary:Neuronal alterations in five cases of multiple system atrophy (MSA) were investigated histologically, immunocytochemically and ultrastructurally. Argentophilic neuronal cytoplasmic inclusions (NCIs) were observed in all cases. They were distributed, in order of decreasing frequency, in the pontine nucleus, striatum, subiculum, amygdala, hippocampus, dentate fascia, substantia nigra and inferior olivary nucleus. Anti-ubiquitin antibodies visualized many thickened neurites in the degenerating gray matter as well as NCIs. Some NCIs were also recognized by anti-phosphorylated neurofilament antibodies. Ultrastructurally, NCIs consisted of a meshwork of granule-associated filaments, the diameter ranging from 18 to 28 nm, that were mixed with neurofilaments. The granule-associated filaments were also present in the axoplasm of myelinated fibers. Our studies demonstrate widespread distribution of NCIs in the central nervous system of MSA. The same pathological process that forms the granule-associated filaments in axons may also be responsible for the formation of ubiquitin-positive thickened neurites. These axonal alterations, as well as neuronal perikaryal changes, may play an important role in the impaired neuronal function in MSA.
ISSN:0001-6322
1432-0533
DOI:10.1007/BF00310020