Susac’s Syndrome – a case report
DOI:
https://doi.org/10.34631/sporl.2096Keywords:
Susac’s Syndrome, encephalopathy, Sensorineural hearing loss, retinal artery occlusion, audiometryAbstract
Susac’s Syndrome (SS) is an occlusive immune-mediated microangiopathy that preferentially affects cerebral, retinal and cochlear arterioles. Classically, it is associated with the triad of visual defects, encephalopathy and sensorineural hearing loss (SNH). Diagnosis is based on clinical features and findings from fluorescein angiography (FA), brain magnetic resonance imaging (CE-MRI) and audiometry. Treatment is based on the use of high-dose corticosteroid therapy and must be started as soon as possible to prevent permanent sequelae. The clinical case presented is that of a 34-year-old woman with multiple visits to the Emergency Department due to persistent headaches, psychomotor slowing and focal neurological deficits lasting 2 months. From the study carried out, multiple hyperintense lesions were found on T2 CE-MRI involving the corpus callosum, areas of retinal ischemia and hyperfluorescence on FA and bilateral moderate SNH at high frequencies on the audiogram. The diagnosis of SS was assumed and immunosuppressive therapy was started, maintaining clinical, imaging and audiometric surveillance.
References
Pereira S, Vieira B, Maio T, Moreira J, Sampaio F. Susac’s syndrome: an updated review. Neuroophthalmology. 2020 May 1;44(6):355-360. doi: 10.1080/01658107.2020.1748062.
Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T. et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013 Jun;9(6):307-16. doi: 10.1038/nrneurol.2013.82.
Kleffner I, Dörr J, Ringelstein M, Gross CC, Böckenfeld Y, Schwindt W. et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016 Dec;87(12):1287-1295. doi: 10.1136/jnnp-2016-314295.
García-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014 Apr-May;13(4-5):347-50. doi: 10.1016/j.autrev.2014.01.038.
Rennebohm RM, Asdaghi N, Srivastava S, Gertner E. Guidelines for treatment of Susac syndrome – an update. Int J Stroke. 2020 Jul;15(5):484-494. doi: 10.1177/1747493017751737.
Marrodan M, Fiol MP, Correale J. Susac syndrome: challenges in the diagnosis and treatment. Brain. 2022 Apr 29;145(3):858-871. doi: 10.1093/brain/awab476.
Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac’s syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007 Jun 15;257(1-2):270-2. doi: 10.1016/j.jns.2007.01.036.
Roeser MM, Driscoll CLW, Shallop JK, Gifford RH, Kasperbauer JL, Gluth MB. Susac syndrome - a report of cochlear implantation and review of otologic manifestations in twenty-three patients. Otol Neurotol. 2009 Jan;30(1):34-40. doi: 10.1097/mao.0b013e31818b6ac2.
David C, Sacré K, Papo T. Actualités dans le syndrome de Susac. Rev Med Interne. [Internet]. 2022 Jan [cited 2023 Oct 22];43(1):26–30. Available from: https://www.sciencedirect.com/science/article/abs/pii/S024886632100463X
Susac JO. Susac’s Syndrome. AJNR Am J Neuroradiol. [Internet]. 2004 Mar 1 [cited 2023 Oct 22];25(3):351–2. Available from: https://www.ajnr.org/content/25/3/351
Patel VA, Dunklebarger M, Zacharia TT, Isildak H. Otologic manifestations of Susac syndrome. Acta Otorhinolaryngol Ital. 2018 Dec;38(6):544-553. doi: 10.14639/0392-100X-2166.
Al-Hasan Y, Hoskin JL, Wolf JC, Kamala S. Susac syndrome and pregnancy. Case Rep Neurol Med. 2020 Dec 23:2020:6049126. doi: 10.1155/2020/6049126.
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