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A woman in her early 40s presented to the emergency department with multiple scotomas in both eyes and auditory changes in her left ear. On examination, her visual acuity was 20/25 in both eyes. A fundoscopic examination revealed multiple areas of retinal whitening consistent with ischemia secondary to multiple branched retinal artery occlusions (BRAOs). Fluorescein angiography revealed multiple areas of vascular filling defects and hyperfluorescence of the arterial wall of the arterioles (Figure 1A-C). Hyperfluorescence of the arterioles without associated BRAOs, as seen superonasal to the disc and inferotemporally in the left eye on a fluorescein angiographic image (Figure 1D), are classic signs of Susac syndrome. On a T1-weighted fluid-attenuated inversion recovery sequence magnetic resonance imaging scan, scattered hyperintense foci were present in the periventricular white matter and corpus callosum (Figure 2). Branched retinal artery occlusions can occur with many illnesses, but when associated with white matter lesions, hearing loss, and a lack of thromboembolic disease, they suggest the diagnosis of Susac syndrome. The patient started intravenous methylprednisolone sodium succinate, followed by oral prednisone, with improvement of her symptoms.
Kothari N, Kuriyan AE. Branched Retinal Artery Occlusions and Susac Syndrome. JAMA Neurol. 2016;73(7):884-885. doi:10.1001/jamaneurol.2016.0121