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Chapter 1.2 - Chapter

from 1 - Inflammatory Conditions

Published online by Cambridge University Press:  06 October 2022

Anita Arsovska
Affiliation:
University of Ss Cyril and Methodius
Derya Uluduz
Affiliation:
Istanbul Üniversitesi
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Summary

Susac syndrome (SS) is an occlusive arteriolar disease, leading to infarcts in the retina, cochlea and brain. The classic triad of the disease consists of subacute encephalopathy, visual disturbances and hearing loss. It is accepted as an autoimmune disease causing an endoteliopathy disturbing the microvasculature of the inner ear, retina and brain. The rarity and the clinical diversity of the disease can make cases challenging for clinicians to diagnose. There are no defined criteria for the diagnosis of SS. Diagnosis can be made based on the findings of brain magnetic resonance imaging (MRI), fundus fluorescein angiography (FFA) and audiometry. In brain MRI; small, roundish, multifocal T2-hyperintense lesions in the periventricular, subcortical and deep white matter areas with at least one of them centrally located in the corpus callosum (‘snowball’ appearance) can be observed. The callosal lesions are accepted as pathognomonic signs. There are no standardized treatment protocols. High doses of corticosteroids for 3–5 days and oral prednisolone treatment for the following 4 weeks are recommended in the acute phase. For patients who do not respond to this first line treatment, plasmapheresis or intravenous immunoglobulin can be chosen as an alternative option. If these treatment steps fail, more aggressive immunosuppression with agents of cylophosphamide and rituxumab can be considered. The usage of antiaggregant agents such as acetylsalicylic acid is also recommended for all patients with SS to decrease the risk of thrombosis

Type
Chapter
Information
Rare Causes of Stroke
A Handbook
, pp. 45 - 49
Publisher: Cambridge University Press
Print publication year: 2022

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References

Dörr, J, Krautwald, S, Wildemann, B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9(6): 307316.CrossRefGoogle ScholarPubMed
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Mateen, FJ, Zubkoc, AY, Muralidharan, R, et al. Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 2012;19(6): 800811.Google Scholar
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European Susac Consortium (EuSaC), Kleffner, I, Dörr, J, Ringelstein, M, et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016;87(12): 12871295.CrossRefGoogle ScholarPubMed
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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.009
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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.009
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.009
Available formats
×