高度の腎病変に伴った視神経萎縮ならびに脊髄の亜急性連合変性症の1剖検例

AN AUTOPSY CASE OF OPTIC ATROPHY AND SUBACUTE COMBINED DEGENERATION OF THE SPINAL CORD WITH NEPHROPATHY

はじめに

　脊髄の亜急性連合変性症（Subacute combined de—generation of the spinal cord）は一般には悪性貧血に伴って認められるとされている^1）。しかるに最近われわれは臨床的に視力低下，難聴，嗄声，両下肢の運動感覚障害などの神経症状と，高血圧，蛋白尿，PSP排泄不良などの腎障害の所見とがみられ，剖検で高度の腎病変と視神経のびまん性脱髄ならびに脊髄の高度の連合性変性が認められた症例を経験した。本例のような高度の腎病変に伴った視神経萎縮ならびに脊髄の亜急性連合変性症の剖検例の報告はまだみられないので，本例の臨床経過および剖検所見を報告し，その神経系の病変の成因について若干の考察を試みた。

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.