Abstract
Multisystem inflammatory syndrome in adults (MIS-A) is an extremely rare para-infectious or post-infectious complication of coronavirus disease 2019 (COVID-19) that requires prompt recognition and early treatment to avert severe morbidity and mortality. A 55-year-old woman presented to us with fever, multiple ischemic strokes, thrombocytopenia, elevated inflammatory markers, and multiorgan dysfunction a few days after COVID-19 illness. She was severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-negative at admission. MRI showed multiple posterior circulation infarctions. She required intensive treatment with intravenous methylprednisolone (IVMP), intravenous immunoglobulin (IVIg), sustained low-efficiency dialysis (SLED), and plasmapheresis for disease remission. Initially, her presentation raised concern for thrombotic thrombocytopenic purpura, however, many features raised the suspicion of a multisystem inflammatory syndrome in adults (MIS-A). Our patient had increased levels of D-dimer, fibrinogen, interleukin 6 (IL-6), and large artery thromboembolism, A positive direct Coomb's test was also more suggestive of immune-mediated hemolysis rather than traction hemolysis, which is the pathophysiology of hemolytic anemia in TTP. Furthermore, MIS-A is known to present with gastrointestinal (GI) symptoms, whereas our case reports predominantly neurological symptoms with relative GI sparing. The overall inflammatory milieu secondary to MIS-A would have contributed to the formation of immune thrombosis, which would have embolized up the vertebrobasilar tree. The MR angiogram did not show any atherosclerotic changes, ruling out an atherosclerotic etiology, which is quite common in posterior circulation infarctions. Multiple courses of immunomodulatory treatment and prolonged treatment with steroids led to disease stabilization.