结节性硬化症是Ｂｏｕｒｎｅｖｉｌｌｅ首先报导并加以命名，是较罕见的遗传性神经皮肤综合征。本病为常染色体显性遗传，由于多数患者临床表现不典型，从而导致误诊或漏诊。ＣＴ的出现为其诊断提供了可靠的依据，我们收集资料完整并经证实的２１例报导如下，以期提高诊断水平 Tuberous sclerosis, first reported and named by Bourneville, is a rare inherited neurodermatosis. The disease is autosomal dominant inheritance, because most patients with atypical clinical manifestations, resulting in misdiagnosis or missed diagnosis. The emergence of CT provides a reliable basis for its diagnosis, we collected data complete and confirmed 21 cases reported as follows, with a view to raising the level of diagnosis