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Japanese

AN AUTOPSY CASE OF OPTIC ATROPHY AND SUBACUTE COMBINED DEGENERATION OF THE SPINAL CORD WITH NEPHROPATHY Teruo Shirabe 1 , Shiro Mawatari 1 , Yoshigoro Kuroiwa 1 , Hiroshi Iwasaki 2 , Masahiro Kikuchi 2 1Department of Neurology, Faculty of Medicine, Kyushu University 2Department of Pathology, Faculty of Medicine, Kyushu University pp.83-89
Published Date 1973/1/1
DOI https://doi.org/10.11477/mf.1406203263
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A patient was a 27-year-old unmarried woman whose parents were cousins. She has had mild hear-ing impairment due to chronic otitis media from the childhood. She noticed sensory loss in her lower extremities in Jan. 1970. Next month visual im-pairment and gait disturbance appeared with pro-gression of the hearing impairment.

She was admitted to the Kyushu University Hos-pital on Nov. 6, 1970. Examination revealed hyper-tension, proteinuria, suspected butterfly rash, scle-roderma in hands, optic atrophy, hearing loss, hoarseness, weakness of lower extremities, hyper-reflexia in four extremities and sensory impairment, especially for pain and temperature, under the 10 th thoracic level on the right half, and under the 12 th thoracic level on the left. She died of hypertensive encephalopathy on April 23, 1971, fifteen months after the onset.

In general autopsy, malignant nephrosclerotic changes were found in the kidney. Heart was hyper-trophic in the left ventricle. The bone marrow was normoplastic.

Neuropathologically there were cerebral edema with petechial hemorrhage probably due to hyper-tensive encephalopathy, scattered small softenings in the right internal capsule and the brain stem, diffuse demyelination of the optic nerves, and com-bined degeneration of the spinal cord. Hyaline degeneration was found in the walls of the small arteries.

It was presumed the metabolic disorder or defi-ciency due to nephropathy might be responsible to the pathogenesis of the optic atrophy and the combined degeneration of the spinal cord.


Copyright © 1973, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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