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A review and update on the Ophthalmic implications for Susac Syndrome

Research output: Contribution to journalReview article

Original languageEnglish
JournalSurvey of Ophthalmology
Early online date28 Jan 2019
DateAccepted/In press - 17 Jan 2019
DateE-pub ahead of print (current) - 28 Jan 2019


Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindness, dementia and other neurological deficits. The plethora of imaging technology, including magnetic resonance imaging, retinal fluorescein angiography, optical coherence tomography (OCT) and OCT-angiography allows deeper and more discrete anatomical-physiological correlation of underlying pathology, early diagnosis, and imaging biomarkers for early detection of relapse during follow up. We highlight the current clinical classification of Susac syndrome, available investigations, treatment and care pathways.

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Copyright © 2019 Elsevier Inc. All rights reserved.



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    Rights statement: This is the accepted author manuscript (AAM). The final published version (version of record) is available online via Elsevier at DOI: 10.1016/j.survophthal.2019.01.007. Please refer to any applicable terms of use of the publisher.

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    Embargo ends: 29/01/20

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    Licence: CC BY-NC-ND


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