Bogousslavsky J, Gaio J M, Caplan L R, Regli F, Hommel M, Hedges T R, Ferrazzini M, Pollak P
Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
J Neurol Neurosurg Psychiatry. 1989 Jan;52(1):43-6. doi: 10.1136/jnnp.52.1.43.
Three young women (aged 18 years, 19 years and 19 years) who developed progressive neuropsychic and neurologic disturbances with hearing loss and multifocal retinal artery branch occlusions are reported. This retinocochleocerebral syndrome has been reported previously only in 12 young North American women. Its pathogenesis is unknown, but an atypical viral infection of the vessel walls has been suggested. Abnormalities of T lymphocytes subsets in blood in one of the patients suggested an immunological dysfunction, but all other tests, including immunological reactions on brain and skin biopsies, were negative or non-specific. Steroids and immunosuppressive agents have been advocated on an empirical basis, but the second patient showed a substantial recovery without any therapy and the third gradually deteriorated despite azathioprine, cyclophosphamide, prednisone and plasma exchanges. This retinocochleocerebral syndrome probably corresponds to an arteriolopathy of unknown nature.
报告了三名年轻女性（年龄分别为18岁、19岁和19岁），她们出现了进行性神经精神和神经系统障碍，伴有听力丧失和多灶性视网膜动脉分支阻塞。这种视网膜耳蜗脑综合征此前仅在12名北美年轻女性中报道过。其发病机制尚不清楚，但有人提出可能是血管壁的非典型病毒感染。其中一名患者血液中T淋巴细胞亚群异常提示存在免疫功能障碍，但所有其他检查，包括脑和皮肤活检的免疫反应，均为阴性或非特异性。基于经验，有人主张使用类固醇和免疫抑制剂，但第二名患者未经任何治疗就有明显恢复，而第三名患者尽管使用了硫唑嘌呤、环磷酰胺、泼尼松和血浆置换，病情仍逐渐恶化。这种视网膜耳蜗脑综合征可能对应于一种性质不明的小动脉病。
