脑三叉神经血管瘤病属神经皮肤综合征,本病较少见,现将两年来我科治疗的4例报告如下: 例1,男,5岁。生后3个月开始抽风:双眼上吊,双侧肢体强直痉挛,左侧重,持续3～4分,当地医院服鲁米那和苯妥英钠20余天发作减轻。患儿系第三胎足月顺产,生后无窒息,母孕期正常。智能发育迟缓,口齿不清,走路跛行。同胞健在,智力正常,家族中无类似患者。查体:体温正常,神清,反应迟钝,头围44.5cm,右额和右侧面部有红葡萄酒色血管痣,心肺腹(-),肌张力正常,左侧上、下肢肌力Ⅳ级,腱反射亢进,左手握力差,动作笨掘,左巴氏征(±),右侧肢体正常。眼底右视乳头边缘清、色苍白、血管细,视神 Brain trigeminal hemangioma disease is a neurodermatosis syndrome, the disease is rare, now two years our department treated 4 cases reported as follows: Example 1, male, 5 years old. 3 months after birth, ventilation: eyes hanging, bilateral limbs tonic spasm, left heavy for 3 to 4 minutes, the local hospital service romine and phenytoin sodium more than 20 days attack reduced. Pediatric third trimester full-term follow-up, postnatal asphyxia, normal during pregnancy. Intelligent retardation, slurred speech, walking limp. My fellow citizens are healthy, mentally normal and have no similar family members. Physical examination: normal body temperature, clear, unresponsive, head circumference 44.5cm, right frontal and right side of the face with red wine color vessels nevus, cardiolipin (-), normal muscle tone, left upper and lower limb muscle strength Ⅳ, Tendon hyperreflexia, poor left hand grip, stupid movements, left Pakistan’s sign (±), right limbs normal. Right eye edge of the nipple clear, pale, thin blood vessels, depending on God