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In this study a semiquantitative mapping of oligodendroglial cytoplasmic inclusions (GCIs), a feature of oligodendroglial degeneration in multiple system atrophy (MSA), was undertaken by means of a sensitive silver technique in 14 brains and 11 spinal cords of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and autonomic failure. The results show a system-bound extensive degeneration of interfascicular, perineuronal and perivascular oligodendrocytes. Oligodendroglial cytoplasmic inclusion-rich structures occur in the supra-segmental motor systems (primary motor and higher motor areas of cerebral cortex, 'pyramidal', 'extrapyramidal' and cortico-cerebellar systems), in the supraspinal autonomic systems and in their targets. In contrast, the visual and auditory pathways, olfactory structures, somatosensory systems, association and limbic cortical areas and subcortical limbic structures contain no or only a few GCIs. Comparison of the severity of oligodendroglial degeneration (GCI-density) with that of the neuronal alterations (neuronal cytoplasmic and nuclear inclusions, degenerated neuronal processes and loss of nerve cells) indicates a striking preponderance of oligodendroglial degeneration and that degeneration neither of axons, nor of neuronal cell bodies is a prerequisite of the development of GCIs. It also suggests that inclusion-bearing oligodendroglial degeneration may cause or contribute to the manifestation of clinical symptomatology in structures with GCI accumulation but without convincing neuronal alterations, i.e. cerebral motor cortical areas and reticular formation of the lower brainstem, both previously thought to be spared in MSA.

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Olfactory dysfunction is a characteristic clinical sign in Parkinson's disease (PD); it is also present in multiple system atrophy (MSA). The pathological basis of hyposmia or anosmia in PD is well known: the olfactory bulb (OB) contains numerous Lewy bodies and severe neuronal loss is present in the anterior olfactory nucleus (AON). We established that glial cytoplasmic inclusions (GCIs) are present in all the OBs from MSA cases. Their presence in the OB is diagnostic for MSA. Additionally, neuronal loss is present in the AON in MSA. These pathological changes might be responsible for the olfactory dysfunction seen in MSA.

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