Susac's syndrome: First from India and youngest in the world
A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child was lethargic, and had reduced level of consciousness with hypoacusis. Laboratory studies were negative for antinuclear antibodies, hepatitis, Epstein-Barr polymerase chain reaction, cardiolipin antibodies, and herpes infection. Cerebro Spinal Fluid (CSF) analysis was negative for oligoclonal bands and nonreactive for the Venereal Disease Research Laboratory (VDRL) test. However, patient's level of consciousness and age did not allow measurement of visual acuity or hearing loss. Color fundus and red free photography was performed, which showed inferotemporal retinal arteriolar branch occlusion in the right eye [Figs. and ]. Optical coherence tomography of right macula showed no significant change. Magnetic resonance imaging (MRI) revealed multiple foci of increased FLAIR T2 signal intensity in the periventricular white matter and corpus callosum [] and T1 image showed lesions with decreased intensity in corpus callosum []. She was treated with intravenous prednisolone one gram/kilogram body weight for 3 days. The response to steroid was remarkable. The symptoms of encephalopathy subsided within 48 h. She was started on oral steroid (prednisolone) 10 mg/day for 10 days, which was tapered gradually. Repeat MRI, 4 weeks later, showed almost complete disappearance of the foci [Figs. and ]. Child did not return for subsequent follow-up visits.