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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References
Aubert-Cohen F, Klein I, Alexandra JF, Bodaghi B, Doan S, Fardeay CM, Lavallée P, Peitte JC, Le Hoang P, Papo T.Long-term outcome in Susac syndrome. Medicine 2007; 86: 93–102.
Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29: 313–6.
Magro CM. Susac syndrome – an autoimmune endotheliopathy. 1st Susac Symposium. Ohio State University, Columbus OH, April 2005.
Waldman J, Knight D. Antiendothelial cell antibodies in Susac’s syndrome. 1st Susac Symposium. Ohio State University, Columbus OH, April 2005.
Bucciarelli S, Cervera R, Martinez M, Latorre X, Font J. Susac’s syndrome or catastrophic antiphospholipid syndrome. Lupus 2004; 13: 607–8.
Susac JO. Susac syndrome: The triad of microangiopathy of the brain and retina with hearing loss in young men. Neurology 1994; 44: 591–3.
O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR. Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophtalmology 1998; 105: 1038–44.
Papo T, Biousse V, Le Hoang P, Fardeu C, N’Guyen N, Houn DL, Aumaitre O, Bousser MG, Godeau P, Piette JC. Susac syndrome. Medicine (Baltimore) 1998; 77: 3–11.
Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchineti CF, Bartleson JD, Parisi JE, Kasperbauer JL, Rodriguez M. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998; 77: 12–40.
Egan RA, Ha Nguyen T, Gass JD, Rizzo JF III, Tivman J, Susac JO.Retinal arterial wall plaques in Susac syndrome. Am J Ophthalmol 2003; 135: 483–6.
Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, Gean AD, Galetta SL, Fox RJ, Costello FE, Lee AG, Clark J, Layzer RB, Daroff RB.MRI findings in Susac's syndrome. Neurology 2003; 61: 1783–1787.
White ML, Zhang Y, Smoker WR.Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. Am J Neuroradiol 2004; 25: 706–13.
Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac’s syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci 2007; 257: 270–2.
Gross M, Eliashar R.Update on Susac’s syndrome. Curr Opin Neurol 2005; 18: 311–4.
Lansberg MG, Thies VN, O’Brien MW, Ali JO, de Crespigny AJ, Tong DC, Moseley ME, Albers GW. Evolution of apparent diffusion coefficient, diffusion-weighted and T2-weighted signal intensity of acute stroke. Am J Neuroradiol 2001; 22: 537–44.
Xu MS, Tan CB, Umapathi T, Lim CC. Susac syndrome: Serial diffusion-weighted MR imaging. Magn Reson Imaging 2004; 22: 1295–8.
Recchia FM, Brown GC. Systemic disorders associated with retinal vascular occlusion. Curr Opin Ophthalmol 2000; 11: 462–7.
Susac JO.Susac’s syndrome. Am J Neuroradiol 2004; 25: 351–2.
Rennebohm RM, Susac JO.Treatment of Susac’s syndrome. J Neurol Sci 2007; 257: 215–20.
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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
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