RESUMO
New onset refractory status epilepticus (NORSE) is a rare but critical condition characterized by refractory status epilepticus (RSE) in an individual without prior history of epilepsy or known structural, toxic, or metabolic cause. Postinfectious immune activation is an important cause of NORSE. Early testing for autoimmune antibodies is strongly recommended (Wickstrom et al., 2022). We report a case of NORSE triggered by Japanese encephalitis (JE) in an unvaccinated US adult traveler. Her CSF later revealed positive anti-N-methyl-d-aspartate (NMDA)-receptor antibody. The patient responded well to first line immunotherapy with favorable functional outcome. This case highlights the diagnostic and treatment challenges in this rare presentation.