Abstract

Background and Purpose: Adult moyamoya disease (MMD) has a different clinical presentation compared as pediatric MMD. The present study was conducted to elucidate the correlation of angiographic characteristics and stroke presentation in adult MMD patients. Methods: We conducted a retrospective analysis of radiologic features of 315 adult patients with definite moyamoya disease which was proved by digital subtraction angiography according to the guidelines of the Research Committee on Spontaneous Occlusion of the Circle of Willis (MMD) of the Ministry of Health and Welfare in 1997. Comparison of angiographic stage and stroke patterns for 630 hemispheres was performed according to stroke presentation. Results: The mean age of diagnosis was 41.5 ( ± 11.7) years. Male to female ratio was 1 to 1.85. One hundred eighty five patients (57.8%) experienced the acute stroke at diagnosis. There was stroke episode prior to diagnosis in 139 patients (43.4%). Among 63 patients (19.7%) who had not experienced any stroke, there was TIA in 44 (13.7%) and no symptoms in 19 (5.9%). Angiographic stage was 50 in 1 (7.8%), 170 in 2 (26.6%), 221 in 3 (35.5%), 129 in 4 (20.2%), 61 in 5 (9.5%) and 9 in 6 (1.4%). Stroke rates were significantly different according to angiographic stage (P=0.041). The occlusion of middle cerebral artery was a significant association with ipsilateral acute stroke (P=0.01). The occlusion of anterior cerebral artery (ACA), middle cerebral artery (MCA), fetal-type posterior cerebral artery was significantly associated with stroke type (P=0.001, 0.002, 0.018). Cerebral blood flow and cerebral vascular reactivity were also strongly associated with ipsilateral acute stroke (P<0.001). Conclusions: In adult moyamoya disease, angiographic stage, MCA occlusion and hemodynamic status are associating factors with stroke development. Occlusion patterns of major arteries are different according to stroke type.

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