• Ling Zhi Heng, Claire Bailey, Richard Lee, Andrew Dick, and Adam Ross.
• Bristol Eye Hospital, Bristol Royal Infirmary NHS Trust, Department of Medical Retina and Uveitis, Bristol Eye Hospital, Bristol, United Kingdom; National Institute for Health Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital, University College London Institute of Ophthalmology, London, United Kingdom.
• Surv Ophthalmol. 2019 Jul 1; 64 (4): 477-485.

AbstractSusac 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, and optical coherence tomography 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.Copyright © 2019 Elsevier Inc. All rights reserved.

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