结节性硬化又名Bourneville病,临床特征为面部皮脂腺瘤,癫痫发作,智能低下,是一种少见的先天性疾病,现将本院2例报告如下。 例1 女,14岁,面部皮疹13年,反复凝视10天,于1987年8月就诊。患儿于出生后5个月时出现抽搐,双手握拳,双眼上翻,口吐白沫。每3～4天发作1次,每次约4～5分钟,不伴发热。服用大仑丁及鲁米那1个多月未见发作。此后未再服抗痫药,亦无抽搐。就诊前10天反复失神。步行中突然站立不动,双眼向左或向右凝视约1分钟。自半岁始面部出 Nodular sclerosis, also known as Bourneville disease, the clinical features of facial sebaceous tumors, seizures, mental retardation, is a rare congenital disease, now the hospital 2 cases are reported as follows. Example 1 Female, 14 years old, facial rash 13 years, repeated stare 10 days, in August 1987 treatment. Children with convulsions at 5 months after birth, both hands fist, eyes up, spit foam. Attack every 3 to 4 days 1, each about 4 to 5 minutes, without fever. Daren Daren and taking more than 1 month without a seizure. No further antiepileptic drugs since then, nor convulsions. 10 days before treatment repeated absences. Suddenly standing motionless on foot, his eyes staring left or right for about 1 minute. Since the first half of the face out