Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome)
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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.