Occlusive disease in circle Willis

Japanese: ウィリス動脈輪閉塞症 - ウィリスどうみゃくりんへいそくしょう（英語表記）occlusive disease in circle Willis

A disease in which the internal carotid artery, which circulates in the brain, is narrowed or blocked just before it branches off into other blood vessels. A thin bypass vascular network is formed to compensate for the narrowed or blocked area, and because this vascular network appears hazy during cerebral angiography, it is also called Moyamoya disease. It is a disease of unknown cause that mainly affects Japanese people, with a slightly higher incidence in women (1:1.8). It is divided into a juvenile type that develops around the age of 5 and an adult type that develops mainly in people in their 30s and 40s. In the juvenile type, sudden loss of consciousness, weakness, and convulsions occur, while in the adult type, it often develops in the form of a stroke. In some cases, paralysis and intellectual disability progress as the attacks continue. There is no fundamental treatment, but drugs to improve cerebral blood flow are administered, and in the case of the juvenile type, bypass vascular formation surgery can be effective. It was designated an intractable disease by the Ministry of Health, Labor and Welfare in 1982.

Source: Encyclopaedia Britannica Concise Encyclopedia About Encyclopaedia Britannica Concise Encyclopedia Information

Japanese:

脳内を循環する内頸動脈が，血管を分岐する直前で狭窄したり閉塞する疾患。その狭窄部，閉塞部を補うために細いバイパス血管網が形成され，その血管網が脳血管撮影の際にもやもやと見えることから，モヤモヤ病 Moyamoya diseaseとも呼ばれる。おもに日本人にみられる原因不明の疾患で，1対 1.8の割合で女性にやや多い。5歳前後に発病する若年型と，30～40歳代を中心に発病する成年型に分けられ，若年型では突発性の意識障害や脱力，けいれんなどが起り，成年型では脳卒中発作の形で発症することが多い。発作を繰返すうちに，麻痺や知能障害が進行していく例もある。根本的治療法はないが，脳血流改善薬などが投与されるほか，若年型の場合にはバイパス血管の形成手術が効果をみせることもある。 1982年に厚生省から難病の指定を受けた。

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