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Susac's syndrome (SS) is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear. SS responds well to immunosuppressive therapies when treatment is prompt, aggressive, and sustained. Striking similarities exist between SS and dermatomyositis (DM), regarding immunopathogenesis, natural history, and treatment(More)
Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal(More)
BACKGROUND Susac syndrome (SS) is a self-limited syndrome, presumably autoimmune, consisting of a clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. All three elements of the triad may not be present or recognized, and MR imaging is often necessary to establish the diagnosis. OBJECTIVE To determine the spectrum of(More)
Susac's Syndrome (SS) consists of the clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss (HL). It is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear (cochlea and semicircular canals). The age range extends from 7 to 72 years, but young women (20-40) are most(More)
Susac syndrome (SS) is the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and hearing loss. Migraines may herald and accompany encephalopathy. Little is known about pathogenesis. Based on light microscopic findings in brain biopsy material analogous to anti-endothelial cell antibody (AECA)-mediated microvascular injury, we postulated(More)
INTRODUCTION Susac Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusion, and hearing loss. It is an autoimmune endotheliopathy that primarily affects young women. Two funduscopic findings, Gass plaques (GP) and arteriolar wall hyperfluorescence (AWH), have recently been described and are not only useful in making the SS(More)
OBJECTIVE This study reports five new cases of microangiopathy of the brain, retina, and cochlea (Susac syndrome) and reviews the world's literature. DESIGN Five cases were systematically studied by the authors. The cases in the literature were identified through Medline searches for Susac syndrome; microangiopathy of the brain, retina, or ear; and(More)