结节性硬化症,临床罕见,现将我院所见二例报告如下。例1:男,9岁。以频频抽搐、呆傻、生活不能自理,面部红褐色丘疹就诊。患儿于生后8个月被人发现面颊部红褐色丘疹,一岁左右出现抖动、耸肩,点头样痉挛,日发作3～4次,历时1～2秒。两岁时出现四肢抽搐、颈强、嘴歪、二便失禁、呈大发作,诊为癫痫,服用苯妥英钠、苯巴比妥、抗癫灵等一年余,无好转。同时,又发现前额、背腰部皮肤鲨鱼皮样斑多处。4岁时会走,动作蠢笨,步态不稳,行为无目的性,违拗。近2年上述症状加重,不认亲人, Tuberous sclerosis, clinically rare, now see our hospital reported two cases are as follows. Example 1: Male, 9 years old. To convulsions, stay stupid, life can not take care of themselves, face reddish brown pimple treatment. Children were found 8 months after birth cheek red brown papules, one year old appeared jitter, shrugs, nodded spasms, the Japanese attack 3 to 4 times lasted 1 to 2 seconds. Two-year-old appeared limbs convulsions, neck strong, crooked mouth, second incontinence, was a major episode, diagnosed as epilepsy, taking phenytoin sodium, phenobarbital, anti epileptic more than a year, no improvement. At the same time, also found that the forehead, back and waist skin shark skin spots in many places. 4 years old will go, stupid movements, unsteady gait, behavior without purpose, contrary to the bend. The past two years the above symptoms worse, do not recognize their loved ones,