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A review and update on the ophthalmic implications of Susac syndrome

Heng, LZ; Bailey, C; Lee, R; Dick, AD; Ross, A; (2019) A review and update on the ophthalmic implications of Susac syndrome. Survey of Ophthalmology , 64 (4) pp. 477-485. 10.1016/j.survophthal.2019.01.007. Green open access

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Dick_Update on Susac's Syndrome.pdf - Accepted Version

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Abstract

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.

Type: Article
Title: A review and update on the ophthalmic implications of Susac syndrome
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.survophthal.2019.01.007
Publisher version: https://doi.org/10.1016/j.survophthal.2019.01.007
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Branch Retinal Artery Occlusion, Fluorescein angiography, OCT angiography, Susac’s Syndrome, Treatment
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10068133
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