Relapsing Susac syndrome presenting with encephalopathy and refractory hiccups

Abstract

Susac syndrome is a rare autoimmune endotheliopathy characterized by occlusion of small arteries in brain, retina, and cochlea presenting with triad of encephalopathy, retinal vaso-occlusive disease and hearing loss. The disease can be monophasic or relapsing with variety of symptoms and the majority of the patients do not have the classic triad, especially at the initial clinical presentation. In CNS neuroinflammaory disorders, refractory hiccup is a recognized manifestation of NMOSD area postrema syndrome. To our knowledge, this is the first report of Susac syndrome presenting with encephalopathy and refractory hiccup.