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小儿神经皮肤综合征系指神经系统与皮肤异常同时出现,多为常染色体显性遗传。虽然发病率不高,因受累器官、系统不同,其表现多种多样,易被忽略或误诊,现将我们收住院的7例报道如下: 1 脑三叉神经血管瘤病4例,男性3例,女性1例,分别为2个月、19个月、8岁、10岁。4例中3例表现发作性、进行性加重的与面部痣对侧的局限性抽搐或一过性失语,时而头痛。智力低下者2例,其面部血管痣沿三叉神经Ⅰ、Ⅱ支走行的范围分布。4例均行头颅平片,未见异常钙化影。CT扫描亦未见异
Pediatric neural skin syndrome refers to the nervous system and skin abnormalities occur at the same time, mostly autosomal dominant. Although the incidence is not high, due to involvement of organs, different systems, its performance varied, easily overlooked or misdiagnosed, now we admitted to hospital in 7 cases are reported as follows: 1 cases of brain trigeminal neurovascular disease in 4 cases, 3 males, 1 female, respectively, 2 months, 19 months, 8 years old, 10 years old. Of the 4 cases, 3 showed episodic and progressive aggravating localized contractions or transient aphasia contralateral to the facial nevus, sometimes with headache. 2 cases of mental retardation, the facial vascular nevus along the trigeminal nerve Ⅰ, Ⅱ branch walking range. 4 cases were skull plain film, no abnormal calcification. CT scan no difference