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Images in Neurology |

Branched Retinal Artery Occlusions and Susac Syndrome

Nikisha Kothari, MD1; Ajay E. Kuriyan, MD1
[+] Author Affiliations
1Bascom Palmer Eye Institute, Department of Ophthalmology, Miller School of Medicine, University of Miami, Miami, Florida
JAMA Neurol. 2016;73(7):884-885. doi:10.1001/jamaneurol.2016.0121.
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This case report describes a woman in her early 40s who presented to the emergency department with multiple scotomas in both eyes and auditory changes in her left ear.

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.

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Figure 1.
Fundus Photography and Fluorescein Angiography

Fundus imaging demonstrates retinal whitening nasally and along the inferotemporal arcade in the right eye (A [blue arrowheads]) and along the inferotemporal arcade in the left eye (B [blue arrowhead]). Fluorescein angiography demonstrates filling defects (yellow arrowhead) and hyperfluorescence of the arterial wall of the arterioles (blue arrowheads) in the right eye (C) and the left eye (D).

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Figure 2.
T1-Weighted Fluid-Attenuated Inversion Recovery (FLAIR) Sequence Magnetic Resonance Imaging (MRI)

T1-weighted FLAIR sequence MRI demonstrates 4 hyperintense foci along the corpus callosum. The hyperintensity labeled “4” represents a centrally located lesion commonly seen in Susac syndrome.

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