Susac Syndrome: Report of Four Cases and Review of the Literature

Summary: Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensori-neural hearing loss. The features of four cases of this syndrome are presented. MR imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery. Susac syndrome is a rare disease attributed to a microangiopathy involving the arterioles of the brain, retina, and cochlea. This syndrome was first described by John O. Susac in 1979 in two young women pre-senting with the classic clinical triad of subacute en-cephalopathy, retinal arteriolar branch occlusions, and sensorineural hearing loss (1). Since then, ap-proximately 60 cases have been reported in the med-ical literature (2). Surprisingly, to our knowledge no article on this syndrome has been published in the radiologic field. Rapid diagnosis is important for early therapy, and imaging is mandatory. The radiologist plays an important role when the disease is suspected and helps orient the investigation. Four cases of Susac syndrome are presented with special emphasis on imaging findings and review of the literature