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Susac’s Syndrome

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Retinal Vascular Disease

Part of the book series: Retina Atlas ((RA))

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Abstract

Susac’s syndrome is microangiopathy defined by a triad of encephalopathy, sensorineural hearing loss, and multiple branch retinal artery occlusions. First case reports appeared in the literature in 1973 (Weidauer and Tenner 1973; Pfaffenbach and Hollenhorst 1973) and in 1979, Dr. John O. Susac published reports of microangiopathy of the brain and retina in two young women (Susac et al. 1979). Since first reported in the 1970s, approximately 300 case reports have been published on the disease. Susac’s syndrome most commonly affects young women and was initially only thought to affect females, however, it has been reported in males also. A recent systematic case review of all reported cases found a 3.5 to 1 female to male predominance and an average age of onset between 21 and 35 years (Dörr et al. 2013).

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References

  • Aubart-Cohen F, et al. Long-term outcome in Susac syndrome. Medicine. 2007;86:93–102.

    Article  CAS  Google Scholar 

  • Bernard JT, Romero R, Agrawal K, Jager R, Rezania K. Optical coherence tomography in Susac’s syndrome. Mult Scler Relat Disord. 2014;3:110–6.

    Article  Google Scholar 

  • Boukouvala S, Jacob S, Lane M, Denniston AK, Burdon MA. Detection of branch retinal artery occlusions in Susac’s syndrome. BMC Res Notes. 2014;7:56.

    Article  Google Scholar 

  • Brandt AU, et al. Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS. PLoS One. 2012;7:e38741.

    Article  CAS  Google Scholar 

  • Demir M, Case K. 142: Susac syndrome. Radiology. 2009;250:598–602.

    Article  Google Scholar 

  • Dörr J, et al. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. Nat Rev Neurol. 2009;5:683–8.

    Article  Google Scholar 

  • Dörr J, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9:307–16.

    Article  Google Scholar 

  • Egan RA, et al. Retinal arterial wall plaques in Susac syndrome. Am J Ophthalmol. 2003;135:483–6.

    Article  Google Scholar 

  • Egan RA, Hills WL, Susac JO. Gass plaques and fluorescein leakage in Susac syndrome. J Neurol Sci. 2010;299:97–100.

    Article  Google Scholar 

  • Förl B, Krastel H, Hartmann M, Kolling G. Susac syndrome: follow-up of visual fields and multifocal electroretinogram after treatment with intravenous immunoglobulins. Neuroophthalmology. 2007;31:3–6.

    Article  Google Scholar 

  • Gass JD, Tiedeman J, Thomas MA. Idiopathic recurrent branch retinal arterial occlusion. Ophthalmology. 1986;93:1148–57.

    Article  CAS  Google Scholar 

  • Gharbiya M, et al. Indocyanine green angiographic findings for patients with systemic lupus erythematosus nephropathy. Retina. 2006;26:159–64.

    Article  Google Scholar 

  • Jarius S, Neumayer B, Wandinger KP, Hartmann M, Wildemann B. Anti-endothelial serum antibodies in a patient with Susac’s syndrome. J Neurol Sci. 2009;285:259–61.

    Article  CAS  Google Scholar 

  • London F, Pothalil D, Duprez TP, Sindic CJM. Potential benefits of early aggressive immunotherapy in Susac syndrome. Acta Neurol Belg. 2016;116:451–60.

    Article  CAS  Google Scholar 

  • Magro CM. Susac’s syndrome: an autoimmune endotheliopathy. In: 1st Susac symposium held at Ohio State University, Columbus; 2005.

    Google Scholar 

  • Magro CM, Poe JC, Lubow M, Susac JO. Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies. Am J Clin Pathol. 2011;136:903–12.

    Article  CAS  Google Scholar 

  • Mallam B, Damato EM, Scolding NJ, Bailey C. Serial retinal fluorescein angiography and immune therapy in Susac’s syndrome. J Neurol Sci. 2009;285:230–4.

    Article  Google Scholar 

  • Martinet N, et al. Fluorescein and indocyanine green angiographies in Susac syndrome. Retina. 2007;27:1238–42.

    Article  Google Scholar 

  • Matsuo T, Sato Y, Shiraga F, Shiragami C, Tsuchida Y. Choroidal abnormalities in Behçet disease observed by simultaneous indocyanine green and fluorescein angiography with scanning laser ophthalmoscopy. Ophthalmology. 1999;106:295–300.

    Article  CAS  Google Scholar 

  • Negi VS, Tripathy NK, Misra R, Nityanand S. Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension. J Rheumatol. 1998;25:462–6.

    CAS  PubMed  Google Scholar 

  • Petty GW, et al. Retinocochleocerebral vasculopathy. Medicine. 1998;77:12–40.

    Article  CAS  Google Scholar 

  • Petty GW, Matteson EL, Younge BR, McDonald TJ, Wood CP. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc. 2001;76:958–60.

    Article  CAS  Google Scholar 

  • Pfaffenbach DD, Hollenhorst RW. Microangiopathy of the retinal arterioles. JAMA. 1973;225:480–3.

    Article  CAS  Google Scholar 

  • Pope-Harman A, Ross P, Magro C, Kelsey M. C4D as a diagnostic adjunct in lung allograft biopsies. J Heart Lung Transplant. 2003;22:S103.

    Article  Google Scholar 

  • Rennebohm RM, Egan RA, Susac JO. Treatment of Susac’s syndrome. Curr Treat Options Neurol. 2008a;10:67–74.

    Article  Google Scholar 

  • Rennebohm RM, et al. Aggressive immunosuppressive treatment of Susac’s syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J. 2008b;6:3.

    Article  Google Scholar 

  • Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac’s syndrome—update. J Neurol Sci. 2010;299:86–91.

    Article  Google Scholar 

  • Saw VP, et al. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp Ophthalmol. 2000;28:373–81.

    Article  CAS  Google Scholar 

  • Spiess K, Martínez JRG. OCT angiography: assessment of retinal ischemia in Susac’s syndrome. Ophthalmic Surg Lasers Imaging Retina. 2017;48:505–8.

    Article  Google Scholar 

  • Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology. 1979;29:313–6.

    Article  CAS  Google Scholar 

  • Susac JO, et al. MRI findings in Susac’s syndrome. Neurology. 2003;61:1783–7.

    Article  CAS  Google Scholar 

  • Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac’s syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007;257:270–2.

    Article  Google Scholar 

  • Tashima K, Uyama E, Hashimoto Y, Yonehara T, Uchino M. Susac’s syndrome: beneficial effects of corticosteroid therapy in a Japanese case. Intern Med. 2001;40:135–9.

    Article  CAS  Google Scholar 

  • Vodopivec I, Prasad S. Treatment of Susac syndrome. Curr Treat Options Neurol. 2016;18:3.

    Article  Google Scholar 

  • Vodopivec I, Venna N, Rizzo JF 3rd, Prasad S. Clinical features, diagnostic findings, and treatment of Susac syndrome: a case series. J Neurol Sci. 2015;357:50–7.

    Article  Google Scholar 

  • Weidauer H, Tenner A. Bilateral acute hearing loss with transient occlusion of a branch of the retinal artery on both sides. Z Laryngol Rhinol Otol. 1973;52:121–8.

    CAS  PubMed  Google Scholar 

  • Xu MS, Tan CB, Umapathi T, Lim CCT. Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 2004;22:1295–8.

    Article  Google Scholar 

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Acknowledgments and Attributions

Figures 12.2, 12.3, and 12.4 contributed by Sumit Sharma, MD at Cole Eye Institute at the Cleveland Clinic.

Figures 12.1 and 12.5 contributed by Ramesh R Shah MD, FACS, FICS

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Authors and Affiliations

  1. Department of Ophthalmology, University of Maryland, Baltimore, MD, USA

    David Dao & Alan Sheyman

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  1. David Dao
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  2. Alan Sheyman
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Correspondence to Alan Sheyman .

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Editors and Affiliations

  1. New York Eye and Ear Infirmary of Mount Sinai, New York, NY, USA

    Alan Sheyman

  2. Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Amani A. Fawzi

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Dao, D., Sheyman, A. (2020). Susac’s Syndrome. In: Sheyman, A., Fawzi, A.A. (eds) Retinal Vascular Disease. Retina Atlas. Springer, Singapore. https://doi.org/10.1007/978-981-15-4075-2_12

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  • DOI: https://doi.org/10.1007/978-981-15-4075-2_12

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-15-4074-5

  • Online ISBN: 978-981-15-4075-2

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