Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review

Abstract

Susac syndrome (SS) is a rare microangiopathy that involves arterioles of the brain, retina, and cochlea. Diagnosis is extremely difficult because of the rarity of the disease and because the signs and symptoms often occur at different times. Multidisciplinary approaches and multimodal images are mandatory for diagnosis and prompt therapy. In this report, we describe a case of SS and the application of multimodal retinal imaging to evaluate the ophthalmologic changes and to confirm diagnosis. Early diagnosis and therapy based on the associations of steroids and immunosuppressants are necessary to limit the sequelae of the disease.

Keywords: fluorescein angiography; multimodal imaging; optical coherence tomography angiography; retinal branch artery occlusion; susac syndrome.

Figure 1

Magnetic resonance of the brain: the red arrows show various rotund and ellipsoid focal lesions involving the corpus callosum, periventricular and subcortical white matter, deep grey matter (basal ganglia and thalami), and cerebellum.

Figure 2

Fluorescein angiography examination exhibiting branch retinal artery occlusion in the left eye.

Figure 3

Color fundus photography revealing a glass plaque (white arrow) as yellowish lipid sediments at the mid-segment of the retinal arterioles in the left eye.

Figure 4

Foveal avascular zone enlargement in optical coherence tomography angiography images of the superficial and deep layers (A,B) right eye; (C,D) left eye).