A new case of Susac syndrome and a review of the literature

We report a single case of Susac syndrome (microangiopathy of the brain, retina, and cochlea). A 26-year-old woman developed branch retinal artery occlusion in the right eye, associated with bilateral hearing loss that mostly involved low frequencies. MRI of the brain revealed small multifocal hyperintensities in the white matter of the cerebrum on T2-weighted images with gadolium enhancement. The treatment consisted of anticoagulation and antiplatelet drugs. Seventy-one cases of Susac syndrome have been reported in the literature. The Susac syndrome is more frequent in females and its etiology remains unknown. However, immune inflammatory disorders, vasospastic phenomena, and coagulopathy could be involved in its pathophysiology. Treatment options are not standardized, ranging from antithrombotic drugs to immunomodulatory therapy. The course of the disease is self-limited after an initial fluctuating active phase. The prognosis of Susac syndrome is good in most cases.