Abstract
We report a series of four patients with Susac’s syndrome, which is characterized by the triad of visual loss due to branch retinal artery occlusions, sensorineural hearing loss due to cochlear involvement, and encephalopathy due to cerebral microangiopathy. However, as we describe in this series, the clinical triad may not be apparent for years, resulting in delays in diagnosis. We also report the variable cerebrospinal fluid and brain magnetic resonance imaging findings, and treatment using a combination of steroids and intravenous immunoglobulin, followed by mycophenolate mofetil.
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Susac JO (1994) Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 44(4):591–593
Susac JO, Egan RA, Rennebohm RM, Lubow M (2007) Susac’s syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci
Rennebohm RM, Susac JO (2007) Treatment of Susac’s syndrome. J Neurol Sci 257:215–220
Fox RJ, Costello F, Judkins AR, Galetta SL, Maguire AM, Leonard B et al (2006) Treatment of Susac syndrome with gamma globulin and corticosteroids. J Neurol Sci 251(1–2):17–22
Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B et al (2000) Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp Ophthalmol 28(5):373–381
Gross M, Eliashar R (2005) Update on Susac’s syndrome. Curr Opin Neurol 18(3):311–314
Susac JO (2004) Susac’s syndrome. AJNR Am J Neuroradiol 25(3):351–352
Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB (1985) A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 35(8):1113–1121
Notis CM, Kitei RA, Cafferty MS, Odel JG, Mitchell JP (1995) Microangiopathy of brain, retina, and inner ear. J Neuroophthalmol 15(1):1–8
Aubart-Cohen F, Klein I, Alexandra JF, Bodaghi B, Doan S, Fardeau C et al (2007) Long-term outcome in Susac syndrome. Medicine (Baltimore) 86(2):93–102
O’Halloran HS, Pearson PA, Lee WB, Susac JO, Berger JR (1998) Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 105(6):1038–1044
Papo T, Biousse V, Lehoang P, Fardeau C, N’Guyen N, Huong DL et al (1998) Susac syndrome. Medicine (Baltimore) 77(1):3–11
Petty GW, Engel AG, Younge BR, Duffy J, Yanagihara T, Lucchinetti CF et al (1998) Retinocochleocerebral vasculopathy. Medicine (Baltimore) 77(1):12–40
Do TH, Fisch C, Evoy F (2004) Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol 25(3):382–388
Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N et al (2003) MRI findings in Susac’s syndrome. Neurology 61(12):1783–1787
Petty GW, Matteson EL, Younge BR, McDonald TJ, Wood CP (2001) Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc 76(9):958–960
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Pawate, S., Agarwal, A., Moses, H. et al. The spectrum of Susac’s syndrome. Neurol Sci 30, 59–64 (2009). https://doi.org/10.1007/s10072-008-0004-8
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DOI: https://doi.org/10.1007/s10072-008-0004-8