Abstract
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.