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Neuropathological background spinocerebellar degeneration with special reference to autonomic nervous system lesions in olivopontocerebellar atrophy shy drager syndrome and multiple system atrophy



Neuropathological background spinocerebellar degeneration with special reference to autonomic nervous system lesions in olivopontocerebellar atrophy shy drager syndrome and multiple system atrophy



Brain & Nerve 37(7): 675-685



For elucidation of actual status of olivopontocerebellar atrophy (OPCA) in nosological relation to Shy-Drager syndrome (SDS) and multiple system atrophy (MSA), neuropathological examination with morphometric survey on the intermediolateral column of the spinal cord was performed. Materials were 13 cases registered in the laboratory. Clinically, they consisted of 9 sporadic OPCA including 2 cases with prominent autonomic failure and 4 hereditary spinocerebellar degeneration. In the autonomic nervous system of CNS, degeneration of the intermediolateral column was in the sporadic cases without exception, irrespective of presence or absence of orthostatic hypotension, while the hereditary cases showed neither orthostatic hypotension nor neuronal loss in the nucleus. The autonomic centers in the brainstem and cerebellum were systematically affected in the sporadic and the hereditary cases. It was particularly remarked that the fastigial nucleus, tractus and nucleus solitarius, which were suggested to be related to cardiovascular control, was severely affected. The locus coeruleus, on the other hand, was less severely affected in the hereditary cases. The most remarkable findings was that there was no case with neuropathological change restricted only to the olivo-ponto-cerebellar (OPC) and/or autonomic nervous systems. The sporadic cases had OPC system degeneration with striato-nigral degeneration (SND) and autonomic nervous system degenerations, irrespective of differences in clinical features from case to case. It should be mephasized that the actual neuropathological status of the sporadic cases could be regarded as a multisystemic degeneration inevitably combined with OPC system degeneration, apart from whether such cases are designated as MSA or not. The hereditary cases were different from the sporadic cases in that there occurred far less severe involvement of the locus coeruleus and intermediolateral column; primary degeneration of the substantia nigra differed obviously from SND.

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Accession: 005970913

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PMID: 4063122


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