Abstract
Susac syndrome is a microangiopathy causing small infarcts in the brain, cochlea, and retina. It is characterized by the clinical triad of acute or subacute encephalopathy, sensorineural hearing loss, and retinal branch artery occlusions. This syndrome is probably an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear, but it has also been postulated that Susac syndrome can be due to a thrombotic occlusion of the small vessels of these organs.
The diagnosis is made by the presence of the triad of acute or subacute encephalopathy, sensorineural hearing loss, and retinal branch artery occlusions. When this triad is not completed, the imaging techniques can help with the diagnosis.
The treatment includes immunosuppression (with high-dose steroids and cytotoxic drugs) and anticoagulation.
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© 2008 Humana Press, a part of Springer Science+Business Media, LLC
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Ugarte, M.F., Cervera, R. (2008). Susac Syndrome. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_22
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DOI: https://doi.org/10.1007/978-1-60327-285-8_22
Publisher Name: Humana Press
Print ISBN: 978-1-60327-427-2
Online ISBN: 978-1-60327-285-8
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