Notis C M, Kitei R A, Cafferty M S, Odel J G, Mitchell J P
Department of Ophthalmology, St. Luke's Roosevelt Hospital Center, New York, New York, USA.
J Neuroophthalmol. 1995 Mar;15(1):1-8.
Microangiopathy of brain, retina, and inner ear is a rare syndrome manifesting as arteriolar occlusions of the brain, retina, and inner ear, with resultant encephalopathy, visual, and hearing loss. Despite exhaustive laboratory examinations in these patients, no evidence of a systemic disease can be found. We treated and followed an adolescent with this disorder who initially presented with a branch retinal artery occlusion. A unique finding in this case was retinal vessel wall hyperfluorescence noted five days prior to retinal infarction. The patient developed recurrent branch artery occlusions, sensorineural hearing loss, and central nervous system infarctions despite anticoagulation and immunosuppressive treatment.
脑、视网膜和内耳微血管病是一种罕见综合征，表现为脑、视网膜和内耳的小动脉闭塞，继而导致脑病、视力和听力丧失。尽管对这些患者进行了详尽的实验室检查，但未发现系统性疾病的证据。我们治疗并随访了一名患有该疾病的青少年，其最初表现为视网膜分支动脉闭塞。该病例的一个独特发现是在视网膜梗死前五天出现视网膜血管壁高荧光。尽管进行了抗凝和免疫抑制治疗，该患者仍出现反复的分支动脉闭塞、感音神经性听力丧失和中枢神经系统梗死。
