CASE REPORT
Susac syndrome as a multidisciplinary diagnostic challenge
1
Chair and Department of Neurology, Medical University, Lublin, Poland
2
Department of General Ophthalmology, Medical University, Lublin, Poland
3
Department of Interventional Radiology and Neuroradiology, Medical University, Lublin, Poland
Corresponding author
Ann Agric Environ Med. 2022;29(1):157-161
KEYWORDS
TOPICS
ABSTRACT
Introduction:
Susac syndrome (SuS) is a disease manifested as the clinical triad of encephalopathy, branch retinal artery occlusion, and loss of sensory neural hearing.
Case Report:
The case is presented of a 28-year-old patient hospitalized due to visual impairment of the left eye, and whose hearing and neuropsychiatric disorders had appeared two years earlier. Magnetic resonance imaging demonstrated lesions located in the white matter and along the corpus callosum. An audiogram showed bilateral sensory neural hearing loss. Fluorescein angiography examination revealed branch retinal artery occlusion of the left eye. Based on the clinical picture and results of tests, the diagnosis of SuS was made. Despite the use of steroid and immunosuppression therapy the disease progressed.
Conclusions:
The prognosis for SuS depends on the early diagnosis and implementation of treatment. It should be underlined that in case of hearing loss or encephalopathy of unknown cause, SuS should always be excluded.
Susac syndrome (SuS) is a disease manifested as the clinical triad of encephalopathy, branch retinal artery occlusion, and loss of sensory neural hearing.
Case Report:
The case is presented of a 28-year-old patient hospitalized due to visual impairment of the left eye, and whose hearing and neuropsychiatric disorders had appeared two years earlier. Magnetic resonance imaging demonstrated lesions located in the white matter and along the corpus callosum. An audiogram showed bilateral sensory neural hearing loss. Fluorescein angiography examination revealed branch retinal artery occlusion of the left eye. Based on the clinical picture and results of tests, the diagnosis of SuS was made. Despite the use of steroid and immunosuppression therapy the disease progressed.
Conclusions:
The prognosis for SuS depends on the early diagnosis and implementation of treatment. It should be underlined that in case of hearing loss or encephalopathy of unknown cause, SuS should always be excluded.
REFERENCES (17)
1.
Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology. 1979; 29(3): 313–316. https://doi.org/10.1212/wnl.29....
2.
Nazari F, Azimi A, Abdi S. What is Susac syndrome? – A brief review of articles. Iran J Neurol. 2014; 13(4): 209–214.
3.
Gross CC, Meyer C, Bhatia U, et al. CD8+ T cell-mediated endothelio-pathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019; 18; 10(1): 5779. https://doi.org/10.1038/s41467....
4.
Pereira S, Vieira B, Maio T, et al. Susac’s Syndrome: An Updated Review. Neuroophthalmology. 2020; 44(6): 355–360. https://doi.org/10.1080/016581....
5.
Jarius S, Kleffner I, Dörr JM, et al. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation. 2014; 8; 11: 46. https://doi.org/10.1186/1742-2....
6.
Murata Y, Inada K, Negi A. Susac syndrome. Am J Ophthalmol. 2000; 129(5): 682–684. https://doi.org/10.1016/s0002-....
7.
Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1994; 44(4): 591–593. https://doi.org/10.1212/wnl.44....
8.
Dorr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013; 9(6): 307–316. https://doi.org/10.1038/nrneur....
9.
García-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014; 13(4–5): 347–350. https://doi.org/10.1016/j.autr....
10.
Hardy TA, Taylor RL, Qiu J, et al. The neuro-otology of Susac syndrome. J Neurol. 2020; 267(12): 3711–3722. https://doi.org/10.1007/s00415....
11.
Marrodan M, Correale J, Alessandro L, et al. Susac Syndrome: A differential diagnosis of white matter lesions. Mult Scler Relat Disord. 2017; 15: 42–46. https://doi.org/10.1016/j.msar....
12.
Heng LZ, Bailey C, Lee R, et al. A review and update on the ophthalmic implications of Susac syndrome. Surv Ophthalmol. 2019; 64(4): 477–485. https://doi.org/10.1016/j.surv....
13.
Ringelstein M, Albrecht P, Kleffner I, et al. Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography. Neurology. 2015; 85(7): 610–8. https://doi.org/10.1212/WNL.00....
14.
Patel VA, Dunklebarger M, Zacharia TT, et al. Otologic manifestations of Susac syndrome. Acta Otorhinolaryngol Ital. 2018; 38(6): 544–553. https://doi.org/10.14639/0392-....
15.
Vodopivec I, Venna N, Rizzo JF, et al. Clinical features, diagnostic findings, and treatment of Susac syndrome: a case series. J Neurol Sci. 2015; 357(1–2): 50–57. https://doi.org/10.1016/j.jns.....
16.
Vishnevskia-dai V, Chapman J, Sheinfeld R, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine (Baltimore). 2016; 95(43): e5223. https://doi.org/10.1097/MD.000....
17.
Egan RA. Diagnostic Criteria and Treatment Algorithm for Susac Syndrome. J Neuroophthalmol. 2019; 39(1): 60–67. https://doi.org/10.1097/WNO.00....
| eISSN: | 1898-2263 |
| ISSN: | 1232-1966 |
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