J Korean Neurol Assoc > Volume 16(4); 1998 > Article
Journal of the Korean Neurological Association 1998;16(4): 432-438.
모야모야병의 임상 및 신경영상 소견 : 측부 순환혈관 소견을 중심으로
김남곤, 최낙천, 권오영, 박기종, 전성철, 임병훈
경상대학교 의과대학 신경과학교실, 신경과학연구소
Clinical and Neuroimaging Features of Moyamoya Disease
Nam-Gon Kim, M.D., Nack-Cheon Choi, M.D., Oh-Young Kwon, M.D., Ki-Jong Park, M.D., Sung-Chul Jeon, M.D., Byeong Hoon Lim, M.D.
Department of Neurology, Gyeongsang National University College of Medicine Gyeongsang Institute for Neuroscience, Gyeongsang National University
Abstract
Background : Moyamoya disease is a chronic cerebrovascular disorders in which stenosis of the major arteries of the circle of Willis at the base of the skull progresses to occlusion. We observed two kinds of collateral pathways from the extracranial to the intracranial arteries. Also we evaluated clinlcal and neuroimaging features of moyamoya disease to give on aid in diagnosis. Methods : We analysed 17 patients with moyamoya disease through the medical record and neuroimaging (conventional angiography and/or MR angiography) review. Six out of 17 patients were children(≤ 15 years) and the other 11 patients were adults( 15 years). There were 8 males and 9 females. Results : In our result, moyamoya disease was more common in the adult. There was bimodal age distribution, so average age of onset in children was 8-year-old and in adult 37-year-old. Common clinical features are seizure (66%), TIA (17%), and psychotic behavior (17%) in childrens and hemorrhages (73%), infarction (18%), seizures (9%) in adults. According to angiographic staging classification of Suzuki et al. (1967), our cases showed distribution of stage I (13%), stage II (8.8%), stage III (65.3%), stage IV (4.3%), stage V (4.3%), stage VI (4.3%). In collateral vessels of moyamoya disease, there were 7 cases of ethmoidal moyamoya, 3 of vault moyamoya and 1 of mixed form. It is interesting that there were five cases of unilateral moyamoya disease and one case had pseudoaneurysm. Conclusion : According to our results, we may say that seizures are common in the children and hemorrhages are in the adult. Unilateral moyamoya were mainly occurred in the adult. Ethmoidal collaterals were common among collaterals and stage III had a more cases than others in our neuroimaging data. These clinical and neuroimaging data may help interpretation and diagnosis of moyamoya disease. Key words : Moyamoya disease, Ethmoid, Vault


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