Susac’s syndrome (SS) is a microangiopathy of the retina, inner ear and brain manifesting as a triad of encephalopathy, hearing loss and branch retinal artery occlusion (BRAO).The pathological mechanism is thought to be an immune mediated small vessel vasculitis with some pathophysiological similarity to dermatomyositis. It has been suggested that anti-endothelial antibodies, which have been detected in serum of some of the patients possibly released due to endothelial damage, may have a role in the pathophysiology. The natural history of the disease is not well known given the rarity of SS, however it is thought that in many cases the disease course is usually self limited, however some have advised early treatment with immunosuppression to minimize permanent cognitive, audiologic and visual sequelae.

We report a case of a 33-year-old lady who presented with headache and visual changes. The initial MRI brain was unremarkable. However, within a month’s time she developed encephalopathy and hearing loss. A repeat MRI of brain revealed the characteristic multifocal supratentorial white matter lesions with involvement of corpus callosum. Fluorescein angiography of retina showed BRAO. Audiometry revealed bilateral sensorineural hearing loss. The vasculitic and thrombophilia screen were unremarkable except for a positive ANA with a titre of 1:160 (homogenous pattern).The patient had a good initial response to steroids and IVIG. Though long term immunosuppressive therapy is suggested, however the exact duration of therapy, monitoring of disease activity and gonadal function preservation with GnRH analogues remains controversial and will be discussed in this paper.