Susac's syndrome: first from India and youngest in the world

• Published in: Indian journal of ophthalmology Vol. 61; no. 12; pp. 772 - 773
• Main Authors: Prakash, Gunjan, Jain, Shephali, Gupta, Mausam, Nathi, Tirupat
• Format: Journal Article
• Language: English
• Published: India Medknow Publications and Media Pvt. Ltd 01-12-2013; Medknow Publications & Media Pvt. Ltd; Medknow Publications & Media Pvt Ltd
• Subjects: Brain - pathology; Brief Communication; Care and treatment; Case studies; Child, Preschool; Children; Consciousness; Diagnosis; Diagnosis, Differential; Diseases; Female; Follow-Up Studies; Glucocorticoids - therapeutic use; Hearing loss; Humans; India; Magnetic Resonance Imaging - methods; Medical treatment; Nervous system diseases; NMR; Nuclear magnetic resonance; Retina - pathology; Susac Syndrome - diagnosis; Susac Syndrome - drug therapy; Tomography, Optical Coherence; India
• ISSN: 0301-4738; 1998-3689
• 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.