Irreversible visual loss and posterior uveitis as the initial manifestation of Behcet’s Disease

Radha Annamalai, Ansu Ann John, M. Muthayya
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Abstract

We report a rare case of rapid onset, severe visual loss in a young male patient with Behcet’s disease who had ocular disease as an initial manifestation. Vision was perception of light with inaccurate projection in both eyes. Anterior segment showed no inflammation. Fundus examination revealed vitritis, total optic atrophy, branch retinal vein occlusion and cellophane maculopathy. Visual evoked potential showed delayed P 100 responses in the left eye suggestive of left anterior visual pathway conduction defect. Systemic examination showed no active symptom complex. There was no response to oral corticosteroids or immunosuppressive drugs. Though rare in the Indian population, Behcet’s disease can be severe, progress rapidly and is less responsive to conventional treatment as in our patient. This case is presented for its rarity, as ocular features were the initial and only manifestation. Despite immediate therapy with corticosteroids and immunosuppressives, continued inflammation resulted in visual loss.
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不可逆性视力丧失和后葡萄膜炎是白塞氏病的最初表现
我们报告一个罕见的快速发作,严重的视力丧失在一个年轻的男性患者与白塞病谁有眼部疾病为初始表现。视觉是对光的感知,双眼投射不准确。前段未见炎症。眼底检查显示玻璃体炎、全视神经萎缩、视网膜分支静脉阻塞及玻璃膜黄斑病变。视觉诱发电位显示左眼p100反应延迟,提示左前视通路传导缺陷。全身检查未见活跃症状复合体。口服皮质类固醇或免疫抑制药物无反应。虽然在印度人群中罕见,但白塞氏病可能很严重,进展迅速,对传统治疗的反应较差,就像我们的病人一样。本病例因其罕见而被提出,因为眼部特征是最初和唯一的表现。尽管立即用皮质类固醇和免疫抑制剂治疗,持续的炎症导致视力丧失。
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