Monteiro M L, Swanson R A, Coppeto J R, Cuneo R A, DeArmond S J, Prusiner S B
Neurology. 1985 Aug;35(8):1113-21. doi: 10.1212/wnl.35.8.1113.
A syndrome consisting of a subacute encephalopathy, sensorineural hearing loss, and retinal arteriolar occlusions is described in two women. Laboratory investigations did not reveal any systemic vasculitis. CT and cerebral angiography showed no abnormalities, but magnetic resonance imaging revealed small, discrete lesions in the white matter. Biopsy of cortical brain from one patient showed disseminated microinfarcts in the gray matter as well as sclerosis of small vessels. This syndrome is characterized as an occlusive vasculopathy rather than vasculitis, and should be considered in evaluations of young women presenting with encephalopathy and hearing loss.
两名女性被描述患有一种由亚急性脑病、感音神经性听力损失和视网膜小动脉闭塞组成的综合征。实验室检查未发现任何系统性血管炎。CT和脑血管造影显示无异常，但磁共振成像显示白质中有小的、散在的病变。一名患者的大脑皮质活检显示灰质中有弥漫性微梗死以及小血管硬化。该综合征的特征为闭塞性血管病而非血管炎，在对出现脑病和听力损失的年轻女性进行评估时应予以考虑。
