现报告同胞兄弟患烟雾病3例。先证者男,4岁,第3胎。因肢体瘫痪伴言语障碍反复发作并逐渐加重2年,住院3次,每次治疗后言语恢复,肢体活动有不同程度改善,但改善程度逐渐降低。病程无发热抽搐。体检:神清,失语,右上下肢肌力I级,右腹壁、提睾、膝反射消失。每次住院查脑脊液、血流变学试验、眼底均无异常。头颅CT检查4次,左额顶叶广泛低密度,边缘模糊,灰白质分界不清,CT值15-28Hu,无占位效应。每次复查,病变范围逐渐扩大,程度加重,致左额顶叶脑萎缩,脑室轻度扩大,广泛软化灶。TCD检查,右侧MCA、ICA、ACA管腔重度狭窄,左侧则完全梗阻,基底动脉血流速度异常增 Now report siblings suffering from moyamoya disease in 3 cases. The proband male, 4 years old, the third child. Due to limb paralysis with speech disorder recurrent and gradually increased 2 years, 3 times in hospital, after each treatment speech recovery, physical activity have varying degrees of improvement, but the degree of improvement gradually reduced. No fever fever convulsions. Physical examination: Shen Qing, aphasia, upper right and lower extremity muscle strength I, right abdominal wall, cremaster, knee reflex disappeared. Check each cerebrospinal fluid, hemorheology test, fundus no abnormalities. Head CT examination 4 times, the left frontal parietal extensive low density, fuzzy edge, clear gray matter boundaries, CT value 15-28Hu, no placeholder effect. Each review, the lesion gradually expanded, the degree of aggravate, left frontal parietal atrophy, mild ventricular enlargement, extensive softening. TCD examination, right MCA, ICA, ACA severe stenosis, the left is completely obstructed, basilar artery anomalies increased blood flow velocity