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Buelens, Tom MD*; Herode, Laurent MD*; Nubourgh, Isabelle MD; Caspers, Laure MD, PhD*; Willermain, François MD, PhD*; Postelmans, Laurence MD*

Retinal Cases and Brief Reports: July 2014 - Volume 8 - Issue 3 - p 187–192
doi: 10.1097/ICB.0000000000000039
Case Report

Background: Susac syndrome is a rare disease attributed to microangiopathy involving the arterioles of the brain, retina, and cochlea. Understanding the pathogenesis is incomplete, but an immune-mediated process remains the leading hypothesis.

Methods: Report of a single case of a previously healthy 22-year-old female patient showing the complete clinical triad.

Results: Diagnosis of Susac syndrome in this patient was first questioned due to the atypical initial ophthalmologic presentation with central retinal artery occlusion. Multiple relapses occurred in the fellow eye during follow-up, showing the typical branch retinal artery occlusions, allowing definite diagnosis.

Conclusion: Susac syndrome should be considered in the differential diagnosis when facing (young) patients with central retinal artery occlusion, especially in the presence of unexplained encephalopathy and/or sensorineural hearing loss.

Report of a single case of a 22-year-old female patient with Susac syndrome presenting with central retinal artery occlusion, an atypical and often forgotten ophthalmologic finding of this disease entity. Despite immunosuppressive therapy, multiple relapses occurred in the fellow eye during follow-up, showing typical branch retinal artery occlusions.

Departments of *Ophthalmology, CHU Saint-Pierre and Brugmann, and

General Internal Medicine, CHU Saint-Pierre, Université Libre de Bruxelles, Brussels, Belgium.

Reprint requests: Tom Buelens, MD, Department of Ophthalmology,CHU Saint-Pierre and Brugmann, Université Libre de Bruxelles, 322 rue Haute, 1000 Brussels, Belgium; e-mail:

None of the authors have any financial/conflicting interests to disclose.

© 2014 by Ophthalmic Communications Society, Inc.