Susac Syndrome and COVID-19 Infection: A Two-Direction Therapeutic Dilemma

Abstract

Susac syndrome (SuS) is an autoimmune-mediated microvessel occlusion disease of the central nervous system, retina and inner ear. Treatment consists in the use of corticosteroids, intravenous immunoglobulin, mycophenolate mofetil, tacrolimus, rituximab and cyclophosphamide. Immunocompromised patients may be at higher risk of severe COVID-19 and, simultaneously, infections could exacerbate SuS disease activity. A 32 years-old female with extremely severe SuS, treated with a combination of mycophenolate mofetil, rituximab, cyclophosphamide and oral prednisone, developed a life-threatening COVID-19 infection. Immunosuppressants were stopped and anticoagulant therapy was instituted. Forty-one days after COVID-19 infection diagnosis, she was discharged with her neurological status unchanged. Even considering the life-threatening COVID-19 infection risk, maintaining combined immunosuppression is advised in an extremely severe SuS. Confirmation of COVID-19 infection would be the only reason to stop immunosuppressants. COVID-19 hypercoagulable state may increase thrombosis risk. Anticoagulant treatment is associated with COVID-19 lower mortality and, arguably, with a better prognosis of SuS. © 2020, Sociedade Portuguesa de Neurologia. All rights reserved.