[An autopsy case of moyamoya disease (author's transl)].

An autopsy case of severely progressed "Moya Moya" disease was presented. A 38-year-old male was admitted because of unconsciousness. On examination, he showed spider-like extremities, suggesting Marfan's syndrome. In the past history, he experienced acute rt. hemiparesis at 6 years old and generalized convulsion, lt. hemiparesis and aphasia at 7 years old. After these episodes, he spent an uneventful life until 38 years old, when he was found unconscious in his room by the family. Lt. CAG showed severe stenosis at the siphon of lt. ICA and fine vascular networks in the basal ganglia and many transdural collateral arteries. Three days later, he died and an autopsy was performed. On histological examination, proliferation of the intima, elongated elastica interna and lost of the media were identified in the stenotic arteries. In the vessels of other organs, especially of the heart, proliferated intima and lost of the media were also noticed. From these findings, "Moya Moya" disease was thought to be one of the manifestations of the systemic vascular diseases. The autopsy cases of "Moya Moya" disease in Japan were reviewed. According to the review, most of these autopsy cases died from intracerebral bleeding attack. Recently, EC-IC bypass operations have been performed in order to improve ischemic condition of "Moya Moya" disease. According to the finding of the review, however, the financial efficiency of such by-pass operations for "Moya Moya" disease must be elucidated after long-term follow-up study.