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Brain and Nerve No to Shinkei Volume 27, Issue 4 （April 1975）

Brain and Nerve 脳と神経 27巻4号（1975年4月発行）

Japanese English

原著

脳血管"Moyamoya"病の局所脳循環—¹³³Xe Clearance法と脳血管連続撮影法による検討 REGIONAL CEREBRAL BLOOD FLOW ON CEREBROVASCULAR "MOYAMOYA" DISEASE:STUDY BY ¹³³XE CLEARANCE METHOD AND CEREBRAL ANGIOGRAPHY 上村和夫 ¹ , 山口昂一 ¹ , 小嶋俊一 ¹ , 桜井芳明 ^2,4 , 伊藤善太郎 ^2,4 , 川上倖司 ³ , 沓沢尚之 ³ Kazuo Uemura ¹ , Koichi Yamaguchi ¹ , Shunichi Kojima ¹ , Yoshiharu Sakurai ^2,4 , Zentaro Ito ^2,4 , Hiroshi Kawakami ³ , Takashi Kutsuzawa ³ ¹秋田県立脳血管研究所放射線科 ²秋田県立脳血管研究所脳神経外科 ³秋田県立脳血管研究所内科 ⁴現：東北大脳研脳神経外科 ¹Division of Radiology ²Division of Neurological Surgery, and Internal Medicine ³Division of Research Institute of Brain and Blood Vessels pp.385-393

発行日 1975年4月1日 Published Date 1975/4/1

DOI https://doi.org/10.11477/mf.1406203690

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Abstract 文献概要
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I．はじめに

　いわゆる脳血管"Moyamoya"病（Willis動脈輪閉塞症）は本邦の脳神経外科医により最初に報告され，研究されて来たが^{2,9,12,15,17）}，最近諸外国からも本症と同一と考えられる特異な脳血管撮影所見を示す疾患の報告がみられるようになつた^3〜5,18）。

　従来，本疾患の脳血管撮影所見や病因論については多くの研究がみられる。一方，その多彩な脳血管撮影所見や臨床症状から脳循環障害の存在は推定出来るが，脳循環についての研究は極めて少なく^16），特に局所脳循環に関する論文は私共の知るかぎりでは未だみられない。

　私共は本疾患の局所脳循環動態を明らかにする目的で，¹³³Xe clearance法と脳血管連続撮影所見より得た結果を華に，局所脳循環と，それの脳血管撮影所見との関連について考察し興味ある知見を得たので報告する。

Cerebrovascular "Moyamoya" disease (Spontane-ous occlusion of the circle of Willis) was firstlyreported by Japanese neurosurgeon. Recently,attention has been drawn to this disease by thephysicians of other countries, but the observationof regional cerebral blood flow of the disease hasnot been reported.

We studied the regional cerebral hemodynamicson six patients with the disease by ¹³³Xe clearancemethod using 6 channel detector and serial cerebralangiography.

Materials: Three cases of juvenile patients andthree adult cases were examined. In all cases,characteristic bilateral occlusions or stenosis of thecarotid forks with prominent vascular networks inthe base of the brain were observed. Neurologicalsymptom was more prominent in the juvenile group.

Results: 1) Decrease of hemispheric CBF wasobserved in 4 out of 6 cases. Abnormal regionalcerebral perfusion was proved in the patients withdecreased hemispheric CBF. 2) Reduced responseto hypercapnea was found chiefly in the juvenilepatients. Disturbance of COs Response of the dis-ease was thought to maximal dilatation of peri-pheral vessels and increased vascular resistance ofmain trunks of the cerebral vessels. 3) The lepto-meningeal anastomoses between the PCA and theMCA were proved as important collateral patheson the disease. 4) In spite of the angiographicalvisualization of prominent vascular network in thebase of the brain and early opacification of thedraining veins (3 out of 7 cases), increase of rCBFat the corresponding region could not be detected.

It was supposed that some of the blood flow inthe abnormal vascular networks at the base of thebrain might be non-effective to the cerebral me-tabolism.