A case of Susac syndrome with cervical spinal cord involvement on MRI

Abstract

We describe a typical case presentation of Susac syndrome with a novel MRI finding of cervical spinal cord involvement. A 25-year-old, 14-week gestation white woman presented with two episodes of encephalopathy, responsive to steroids, with abnormal brain magnetic resonance imaging (MRI) concerning for Susac syndrome. Further studies confirmed the clinical triad of encephalopathy, branch retinal artery occlusions and hearing loss pathognomonic for Susac syndrome. Cervical spine MRI demonstrated two focal areas of high-signal abnormality at C2 and C3. We provide a brief review of Susac syndrome, data regarding pregnancy in this rare syndrome, and discuss how this unique observation may assist in the medical management of such cases and contribute to the understanding of the underlying pathophysiology.

Keywords: Autoimmune disorder; Encephalopathy; Endotheliopathy; Microangiopathy; Spinal cord MRI; Susac syndrome.