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Review
. 2020 May 1;44(6):355-360.
doi: 10.1080/01658107.2020.1748062. eCollection 2020.

Susac's Syndrome: An Updated Review

Affiliations
Review

Susac's Syndrome: An Updated Review

Sara Pereira et al. Neuroophthalmology. .

Abstract

Susac's syndrome is a rare immune-mediated endotheliopathy that mainly affects young women. It is characterised by the presence of encephalopathy, retinal vaso-occlusive disease and hearing loss. Diagnosis is based on the clinical presentation, brain magnetic resonance imaging, retinal fluorescein angiography, and audiometry. Treatment consists of immunosuppressive therapy. This review focuses on recent developments in the diagnosis and management of the condition.

Keywords: Susac’s syndrome; encephalopathy; hearing loss; retinal occlusions.

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Figures

Figure 1.
Figure 1.
Axial T2-weighted magnetic resonance imaging demonstrating typical central callosal lesions (white arrows)
Figure 2.
Figure 2.
Sagittal T1-weighted magnetic resonance image demonstrating central callosal punched-out lesions (white arrows)
Figure 3.
Figure 3.
Arterial wall hyperfluorescence (black arrow) and a BRAO (white arrow) on fluorescein angiography
Figure 4.
Figure 4.
Typical arterial wall hyperfluorescence remote from a BRAO (black arrow) on fluorescein angiography

References

    1. Kleffner I, Duning T, Lohmann H, et al. A brief review of Susac syndrome. J Neurol Sci. 2012;322(1–2):35–40. doi:10.1016/j.jns.2012.05.021. - DOI - PubMed
    1. 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. doi:10.1038/nrneurol.2013.82. - DOI - PubMed
    1. Susac JO, Egan RA, Rennebohm RM, Lubow M.. Susac’s syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007;257(1–2):270–272. doi:10.1016/j.jns.2007.01.036. - DOI - PubMed
    1. Garcia-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014;13(4–5):347–350. doi:10.1016/j.autrev.2014.01.038. - DOI - PubMed
    1. 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(1–2):259–261. doi:10.1016/j.jns.2009.07.002. - DOI - PubMed

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