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AN AUTOPSY CASE OF THE ABNORMAL NETWORK OF THE INTRACRANIAL ARTERY-CONGENITAL DYSPLASIA OF THE CEREBRAL ARTERY Akira Mabuchi 1 , Harushi Tanabe 1 , Yukimura Fujikawa 2 , Keiichi Fujimoto 2 , Katsuji Nakata 2 1Department of Surgery, Osaka Medical College 2Department of Pathology, Osaka Medical College pp.1759-1765
Published Date 1973/12/1
DOI https://doi.org/10.11477/mf.1406203448
  • Abstract
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In this paper, a case of the abnormal vascularity of the intracranial arteries (so called Moya Moya disease) was reported. The patient, 16 years old Japanese boy, had frequently complained of head-ache and disturbance of consciousness since his childhood. He had precipitious onset of severe headache and lost consciousness at March 8, 1971, and the physical examination proved the subarach-noideal hemorrhage. He died without recovering his consciousness 14 months after. The cerebral angiography revealed the characteristic configula-tion of the intracranial arteries such as ; narrowing of the bilateral internal carotis arteries at the base of cranium and marked winding of small arterial branches without normal distribution of the cerebral arteries. The post morten examination showed the dysplasia of the cerebral arteries characterized by the following findings ; 1) the large arteries at the base of the brain, except for the A. basilaris and Aa. vertebrales, were extremly small, 2) they branched abruptly to numerous bush-like smallarteries, thus it was unable to trace their course to the periphery, 3) the wide spread softening of the entire brain. Histology revealed ; 1) a- or hypoplasia of the media of arteries in the brain, non of which was found in the other organs, 2) discontinuity of the internal elastic lamellae in some arteries, 3) marked edematous thickening or fibrous thickening of the inner wall of the arteries without inflammatory cell reaction, which resulted in marked stenosis of the vessel lumen, 4) marked winding and thickening of the elastic fiber in manyarteries, and 5) a recent and old ischemic necrosis of the brain. It was considered that lacking or hypoplasia of the medial layer was of congenital (dysplasia) and was the essential vascular lesion of the present case. The other vascular lesions such as edema and fibrosis of the intimal layer, winding and proliferation of the elastic fiber must be a result of insufficient reduction of arterial blood pressure.


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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