Susac syndrome: case reports and review of the literature

BACKGROUND: Susac syndrome is a rare disease of unknown pathogenesis. It consists of retinal arterial occlusion, hearing loss and encephalopathy (triad). PATIENTS: The features of two cases of this syndrome are presented. CONCLUSION: A high percentage of patients do not have the clinical triad at the time of onset of symptoms and, therefore, this disease may be under-diagnosed. Any patient with unexplained encephalopathy should thus be examined by an ophthalmologist and have an audiogram performed.