[An autopsy case of multiple system atrophy with many Lewy bodies--striatonigral degeneration, olivo-ponto-cerebellar atrophy and autonomic nerve nucleus involvement in the spinal cord].
A 72 years old man developed slowness of the motion and orthostatic hypotension at the age of 69. Neurological examination showed slight finger tremor, rigidity of extremities, bradykinesia, and marked orthostatic hypotension. The illness progressed steadily and the patient died of pneumonia. At autopsy brain weighed 1220 g. Grossly the putamen was bilaterally shrunken, the color of the substantia nigra and locus ceruleus became pale. Base of the pons and the cerebellum were atrophic. Microscopically the most remarkable change was seen in the striato-nigral system. In the putamen, there were severe loss of small neurons and intense gliosis and brownish pigments were observed in the neuropil and within some of the astrocytes. There found neuronal loss and gliosis in the substantia nigra. A few Lewy bodies were seen in the substantia nigra. In the cerebellum there were slight loss of Purkinje cell and many torpedos were seen. There were demyelination and fibrirally gliosis in the cerebellar white matter except the hilus of dentate nucleus. The transverse pontocerebellar fibers were degenerated and fibrirally gliosis was seen there. The inferior olivary nuclei showed neuronal loss and astrocytosis. But the degeneration of the olivo-ponto-cerebellar system in this case was not so severe as the typical case of OPCA. In the spinal cord there was depletion of nerve cells in the intermediolateral nuclei and Onufrowitz nuclei. Slight neuronal loss and many spheroids were observed in the anterior horns and there was demyelination in the corticospinal tracts.(ABSTRACT TRUNCATED AT 250 WORDS)
D000368:Aged; D001284:Atrophy; D001341:Autonomic Nervous System / Q000473:pathology*; D006801:Humans; D007024:Hypotension, Orthostatic / Q000150:complications; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008297:Male; D009410:Nerve Degeneration; D009849:Olivopontocerebellar Atrophies / Q000473:pathology*; D013118:Spinal Cord Diseases / Q000473:pathology*; D013132:Spinocerebellar Degenerations / Q000473:pathology*; D013378:Substantia Nigra / Q000473:pathology