Susac syndrome is also known as or subsumes Microangiopathy of the brain, retina, and cochlea; Retinocochleocerebral vasculopathy; Retinopathy, encephalopathy, deafness-associated microangiopathy; and Small infarctions of cochlear, retinal, and encephalic tissue. -ed.
Susac syndrome presents with a triad of retinal arterial occlusion, deafness, and encephalopathy. Since its recognition about 35 years ago, approximately 137 cases have been reported in the literature. In this clinical article, Dr. K K Jain, a neurology consultant in Basel, Switzerland, describes the clinical features, pathology, and diagnosis of this syndrome as well as atypical presentations. The pathological findings include microinfarcts in the territories of end arterioles of the brain, retina, and inner ear. MRI plays an important role in demonstrating the CNS lesions. Management of Susac syndrome is also discussed.