Gelwan M J, Kellen R I, Burde R M, Kupersmith M J
Department of Neurology, New York University Medical Center, NY.
J Neurol Neurosurg Psychiatry. 1988 Dec;51(12):1473-80. doi: 10.1136/jnnp.51.12.1473.
Four patients with progressive visual deterioration were found to have sarcoidosis involving the anterior visual pathway. They all developed chiasmal dysfunction and bilateral optic neuropathy, which responded to megadose corticosteroid therapy. When an attempt was made to withdraw the corticosteroids, the patients experienced a recrudescence of visual dysfunction and were subsequently unable to tolerate the corticosteroid dose levels necessary to maximise their visual potential. Each patient was treated with high-voltage radiation therapy, totalling up to 4500 rads. The beneficial response obtained was temporary, and immunosuppressive therapy with azathioprine or chlorambucil was instituted, preventing further deterioration.
4例进行性视力减退患者被发现患有累及视觉传导通路前部的结节病。他们均出现视交叉功能障碍和双侧视神经病变,大剂量皮质类固醇治疗有效。当试图停用皮质类固醇时,患者出现视力功能复发,随后无法耐受将视力潜能最大化所需的皮质类固醇剂量水平。每位患者均接受了高达4500拉德的高电压放射治疗。所获得的有益反应是暂时的,随后采用硫唑嘌呤或苯丁酸氮芥进行免疫抑制治疗,防止了病情进一步恶化。