结节性硬化症为少见的神经系畸形疾病。1862年Von Recklinghausen首先报导本病。1808年Bourneville发现一例癫痫伴先天性痴呆的患儿,脑内有许多“硬结”,认为癲痫与痴呆均为硬结所引起,命名为“结节性硬化症”,故本病亦名Bourneville氏病。1908年Vogt首次指出皮脂腺瘤与本病的关系,为本病的临床诊 Tuberous sclerosis is a rare neurological deformity disease. Von Recklinghausen first reported the disease in 1862. Bourneville discovered a case of epilepsy with congenital dementia in 1808, there are many brain “induration”, that epilepsy and dementia are caused by induration, named “tuberous sclerosis”, so the disease is also known as Bourneville’s disease. Vogt first pointed out in 1908 the relationship between sebaceous adenoma and the disease, the clinical diagnosis of the disease