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结节性硬化,或称 Bourneville 氏病,是主要累及眼,皮肤和中枢神经的全身性综合症。该病与视网膜血管瘤病(von Hippel-lindau 氏病),先天性视网膜动—静脉畸形(Wyburn-Mason 氏综合症),视网膜海绵状血管瘤(retinal cavernous hemangioma),神经纤维瘤病(von Recklinghauson 氏病),脑面血管瘤病(Sturge-Weber 氏综合症),共济失调一毛细血管扩张综合症(Louis-Bar 氏综合症)总称为全身性错抅瘤病或母斑病(Systemic hamartomatoses 或 phakomatoses)。结节性硬化临床少见,国内眼科从1955年始见有报导,至今我们见到的国内眼科杂志中仅有6例报导。我们于1983年遇到1例,具有典型的眼,中枢神经及皮肤表现,现报告如下。
Tuberous sclerosis, or Bourneville’s disease, is a systemic syndrome that mainly affects the eyes, skin and central nervous system. The disease is associated with von Hippel-lindau’s disease, congenital retinal arteriovenous malformation (Wyburn-Mason’s syndrome), retinal cavernous hemangioma, von Recklinghauson (Sturge-Weber’s syndrome), ataxia-telangiectasia (Louis-Barr’s syndrome) are collectively referred to as Systemic hamartomatoses Or phakomatoses). Tuberous sclerosis is clinically rare, and domestic ophthalmology has been reported since 1955. Only 6 cases of domestic ophthalmic journals we have seen so far reported. We met in 1983, 1 case, with typical eye, central nervous and skin manifestations, are as follows.