Susac J O, Hardman J M, Selhorst J B
Neurology. 1979 Mar;29(3):313-6. doi: 10.1212/wnl.29.3.313.
Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 months of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a "healed" angitis. Both patients seemed to respond to steroid therapy.
两名女性(分别为26岁和40岁)患上了一种罕见的微血管病,累及大脑和视网膜。精神症状最初掩盖了这种进行性神经疾病的亚急性特征。多灶性视网膜分支动脉阻塞的眼底镜检查结果为血管病变提供了临床证据。实验室数据未显示包括系统性红斑狼疮(SLE)和梅毒在内的已知血管炎的证据。脑血管造影显示较年轻患者存在血管炎。老年患者(在接受3个月类固醇治疗后)的脑活检显示软脑膜和皮质小血管硬化,与“愈合”的血管炎相符。两名患者似乎对类固醇治疗均有反应。