Susac Syndrome and Pregnancy

Abstract

Susac syndrome (SuS) is a rare poorly characterised disorder that affects the brain, retina, and cochlea. Here, we present a case of a 31-year-old pregnant female with a new diagnosis of SuS that was successfully managed to 36 weeks of gestation with minimal disease burden to both the mother and newborn. She was treated initially using intravenous methylprednisolone followed by oral prednisone, and intravenous immunoglobulin (IVIg). We stress the importance of a multidisciplinary approach, involving both neurology and maternal-fetal medicine, and provide guidance in navigating the various options for immunosuppressive therapy during pregnancy.

Figure 1

(a) and (b) Axial MRI brain without contrast demonstrating patchy and confluent FLAIR hyperintensity throughout the white matter, markedly abnormal for age (arrows). (c) Coronal MRI brain FLAIR sequence demonstrating a lesion in the corpus callosum (arrowhead), performed prior to her presentation to our facility.

Figure 2

Audiogram results demonstrating low-frequency sensorineural hearing loss in the right ear (dotted line) with her left ear (solid line) unaffected.