Treatment of Susac syndrome with gamma globulin and corticosteroids.

Susac syndrome is a rare vasculopathy characterized by visual, hearing, and cognitive dysfunction. Optimal treatment is unknown, but many patients require chemotherapy to control disease activity. We describe two patients with Susac syndrome and their response to intravenous immune globulin (IVIg) and corticosteroids. Both patients improved following acute treatment with IVIg and intravenous methylprednisolone (IVMP), and no further relapses were observed. One patient showed significant improvement in hearing and MRI lesions shortly following acute treatment. Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome.