A case of Susac’s syndrome: bilateral retinal vessel occlusion in the presence of autoimmune inflammatory endotheliopathy

Authors

  • N. A. Ulianova SI «The Filatov Institute of Eye Diseases and Tissue Therapy of the NAMS of Ukraine»; Odesa (Ukraine)
  • O. V. Zborovska SI «The Filatov Institute of Eye Diseases and Tissue Therapy of the NAMS of Ukraine»; Odesa (Ukraine)
  • O. D. Shulga Volyn Regional Clinical Hospital; Lutsk (Ukraine), Lesya Ukrainka Volyn National University; Lutsk (Ukraine)

DOI:

https://doi.org/10.31288/oftalmolzh202266569

Keywords:

Susac’s syndrome, autoimmune inflammatory endotheliopayhy, retinal artery occlusion

Abstract

The paper presents a case with a classic triad (bilateral inferotemporal branch retinal artery occlusion (BRAO), multifocal callosal micro-infarcts, and sensorineural hearing loss) of Susac’s syndrome in a female patient following COVID-19 infection. We report clinical examination data, particularly, eye examination data and neurological status with brain magnetic resonance imaging (MRI) results. Special attention was given to the diagnostic value of optical coherence tomography (OCT) in Susac’s syndrome, which allows detecting characteristic focal atrophic changes in the inner retina with a preserved structure of the photoreceptor layer. Identification of the typical neurological, ocular or otological symptoms should raise suspicion for the syndrome, which is critical for early administration of the systemic steroid therapy.

References

1.Kalisa P, Constantinides G, Bodson-Halleux M, De Laey JJ. Small infarctions of cochlear, retinal and encephalic tissue (SICRET syndrome). Bull Soc Belge Ophtalmol. 2001; 282:5-12.

2.Szilasiová J, Klímová E. Susac syndrome: retinocochleocerebral vasculopathy. Croat Med J. 2004; 45(3):338-43.

3.Yazan Al-Hasan, Justin L. Hoskin, John C. Wolf, Kamala Saha. Susac Syndrome and Pregnancy. Case Reports in Neurological Medicine [Internet]. 2020, Article ID 6049126, 6 pages. https://doi.org/10.1155/2020/6049126

4.Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T. et al. Characteristics of Susac syndrome: a review of all reported cases. Nature Reviews Neurology. 2013; 9:307-316. https://doi.org/10.1038/nrneurol.2013.82

5.Vodopivec I, Prasad S. Short follow-up bias confounds estimates of the "typical" clinical course of Susac syndrome. Journal of Neuro-Ophthalmology. 2017; 37(2):149-153. https://doi.org/10.1097/WNO.0000000000000472

6.Sauma J, Rivera D, Wu A, Donate-Lopez J, Gallego-Pinazo R, Chilov M, Wu M, Wu L. Susac's syndrome: an update. British Journal of Ophthalmology.2020;104(9):1190-1195. https://doi.org/10.1136/bjophthalmol-2019-315597

7.Redler Y, Chwalisz BK. Neuro-ophthalmic manifestations of Susac syndrome. Curr Opin Ophthalmol. 2020; 31(6):495-502. https://doi.org/10.1097/ICU.0000000000000713

8.Venditti L, Rousseau A, Ancelet C, Papo T, Denier C. Susac syndrome following COVID-19 infection. Acta Neurol Belg.2021; 121:807-809. https://doi.org/10.1007/s13760-020-01554-5

9.Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor Riri S. et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis Medicine (Baltimore). 2016 Oct; 95(43): e5223. https://doi.org/10.1097/MD.0000000000005223

10.Kleffner I, Duning T, Lohmann H, et al. A brief review of Susac syndrome. J Neurol Sci. 2012; 322(1-2):35-40. https://doi.org/10.1016/j.jns.2012.05.021

11.Hantson P., Di Fazio V., Del Mar Ramirez Fernandez M. et al. Susac-Like Syndrome in a Chronic Cocaine Abuser: Could Levamisole Play a Role? J Med Toxicol. 2015; Vol. 11(1):124-128. https://doi.org/10.1007/s13181-014-0422-3

12.Susac JO. Susac's Syndrome. Am J Neuroradiol. 2004; 25:351-352. https://doi.org/10.1097/00129492-200407000-00012

13.Pereira S, Vieira B, Maio T, Moreira J, Sampaio F. Susac's Syndrome: An Updated Review. Neuroophthalmology. 2020 May 1; 44(6):355-360. https://doi.org/10.1080/01658107.2020.1748062

14.Azevedo AGB, Lima LH, Muller L, et al. Anatomical and functional correlation in Susac syndrome: multimodal imaging assessment. Int J Retina Vitreous. 2017; 3:39. https://doi.org/10.1186/s40942-017-0092-9

Published

2025-08-27

How to Cite

1.
Ulianova NA, Zborovska OV, Shulga OD. A case of Susac’s syndrome: bilateral retinal vessel occlusion in the presence of autoimmune inflammatory endotheliopathy. J.ophthalmol. (Ukraine) [Internet]. 2025 Aug. 27 [cited 2025 Aug. 28];(6):65-9. Available from: https://ua.ozhurnal.com/index.php/files/article/view/322