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Journal of the Korean Child Neurology Society 2007;15(2):170-178.
Published online November 30, 2007.
Clinical Features of Moyamoya Disease in Children.
Woo Sik Yang, Eun Young Oh, Geun Young Lee, Dae Chul Jeong, Young Hoon Kim, In Goo Lee, Seung Yun Chung, Hui Sung Hwang
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. pedhwang@catholic.ac.kr
Abstract
PURPOSE
Moyamoya disease, is a chronic progressive cerebrovascular occlusive disorder that can lead to permanent neurologic deficit. The aim of this study is to analyze clinical features, radiologic findings, and prognosis of Moyamoya disease comparing with previous studies. METHODS: We retrospectively reviewed on the clinical data of 26 patients and they were analyzed in terms of age, sex, clinical features, radiologic findings, treatment and prognosis. RESULTS: The male to female ratio was 1:1.6 and the mean age was 9.3 years(male 6.1 years and female 10.9 years). There was a higher occurrence rate in 11-15 year-old group, compared to the previous studies. The most common symptoms at onset were hemiparesis and hemiplegia. Brain infarction, hemorrhage and seizure were found in five, three and two patients on brain imaging respectively. Eight patients(44%) showed reduced perfusion without structural abnormalities on brain perfusion CT. The internal carotid artery was the most common site of occlusion on conventional angiography. Indirect encephalo-duro-arterio-synangiosis was performed in 76.9% of the patients. One patient(3.85%) died and twelve patients(46.15%) showed neurologic sequelae at the time of discharge. Sequelae included hemiplegia, gaze disturbance, facial palsy, and seizures. CONCLUSION: Compared with previous studies, the age at onset was higher in our patients, with a peak incidence after the age of 10. Lower rate of severe neurologic sequelae, such as hemiparesis and hemiplegia was observed in this study. Because this study was limited by the small number of patients included, further investigations such as multi-center prospective analysis is needed.
Key Words: Moyamoya disease, Children


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