[Neuropathological background of spinocerebellar degeneration--with special reference to autonomic nervous system lesions in olivopontocerebellar atrophy, Shy-Drager syndrome and multiple system atrophy].
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. The materials were 13 cases registered in our 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 found 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 brain-stem and cerebellum were systematically affected in both the sporadic and the hereditary cases. It was particularly remarked that the fastigial nucleus, tractus and nucleus solitarius, which have been 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 finding 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 emphasized that the actual neuropathological status of our 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. Finally it was remarked that the hereditary cases were different from the sporadic cases in that there occurred far less severe involvement of the locus coeruleus and intermediolateral column, and primary degeneration of the substantia nigra different obviously from SND.
D000328:Adult; D000368:Aged; D001284:Atrophy / Q000473:pathology; D001341:Autonomic Nervous System / Q000473:pathology*; D001342:Autonomic Nervous System Diseases / Q000473:pathology*; D002490:Central Nervous System / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology*; D005260:Female; D006801:Humans; D007024:Hypotension, Orthostatic; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration; D012791:Shy-Drager Syndrome / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology*