脳底部異常血管網における組織計測学的検討及び本邦12剖検例の概要

CEREBROVASCULAR MOYAMOYA DISEASE:A CASE REPORT AND REVIEW OF 12 AUTOPSY CASES IN JAPAN

I．はじめに

　内頸動脈末端部に狭窄もしくは閉塞を来し，脳底部に異常血管網を伴う疾患（cerebrovascular Moyamoyadisease^19），　cerebral juxtabasal teleangiectasia^17），　cere—bral arterial rete^5），　spontaneous occulusion of thecircle of Willis^12），　cerebral basal rete mirable^15），）は，1971年5月までに396例報告されているが^11），その成因に関して血管網それ自体を先天的な血管奇型とする説と，内頸動脈閉塞に起因する側副血行路とする説とがあり，本症の予後が比較的良好で病理学的検討の機会が少ないこともあつて，その本態は未だ解明されるに至つていない。

　私達は脳内血腫で発症した47歳男子の剖検例を経験し，臨床病理学的に特に組織計測学的立場から本症の発生機序を検討したので，併せて本邦における剖検12例について文献的考察を行ない報告する。

An autopsy study for an adult Japanese male ofcerebrovascular moyamoya disease was reportedherein in detail with a review of twenty autopsycases described so far in Japan.

A 47-year-old man was admitted in comatosestate three days after the episode of stroke. Carotidangiography revealed remarkable stenosis at theterminal portion of bilateral internal carotid arterieswith abnormal vascular network at the base of thebrain. The patient died without recovery of con-ciousness on the 7th hospital day and necropsywas done.

Leptomeninges were almost normal and no gran-ular atrophy was seen in the cerebrum. Therewas an hensegg-sized intracerebral hematoma inthe right posterior lobe, which ruptured into thelateral ventricle. The anterior half of Willis' circleshowed remarkable narrowing and stenosis.

Microsection of affected vessels demonstratedmarked concentric thickening of the intima bycellural fibrous tissue with fine elastic fibrils. Theinternal elastic membrane was considerably redu-plicated and showed a tortuous appearance. Themedia was atrophic in almost all of sectioned ves-sels. The perforating vessels arising from each ofthe major cerebral arteries at the base of the brainhad widened lumina and thin muscle layer. Therewas no intimal thickening at the proximal portionof the perforating artery.

In order to estimate a degree of growth of nar-rowed major cerebral arteries, the surroundinglength of the internal elastic membrane (L) wastraced and a radius of each affected vessel (R) wascaleulated by the formula of R=L/2π. R of eachnarrowed artery was almost within normal limits.

Microscopic study in the presented case showedan arteriosclerotic stenosis of affected major cere-bral arteries with diffuse thickening of the intimaand secondary atrophy of the muscle layer.