Department of Ophthalmology, Sarojini Naidu Medical College, Agra, India.
Indian J Ophthalmol. 2013 Dec;61(12):772-3. doi: 10.4103/0301-4738.118446.
A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac's syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.
一名两岁半大的女性因逐渐加重的头痛和恶心到急诊室就诊。患儿表现为意识水平下降和双侧听力下降。患儿嗜睡。眼科检查发现视网膜分支动脉阻塞（BRAO）。这一发现对 Susac 综合征（SS）的诊断至关重要，SS 是一种罕见的自身免疫性疾病，其特征为视网膜、脑组织和耳蜗的内皮病。脑部磁共振成像也显示出典型特征。全面的血液检查未发现任何其他异常。患者接受了免疫抑制治疗，以防止出现这种疾病的严重后遗症。在开始治疗的 48 小时内，患儿的全身状况有了显著改善。这是印度首例也是迄今为止年龄最小的病例报告。
