Extinguishing FIRES using tocilizumab

Abstract

AbstractFebrile infection–related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with antecedent febrile infection preceding the onset of refractory status epilepticus (RSE), without the evidence of structural, toxic, or metabolic cause. The pathophysiology of FIRES is largely unknown but has been hypothesized to be due to excessive cytokine release perpetuating the underlying seizure. Conventional immunotherapies such as steroid, IVIG, plasma exchange, and rituximab have previously been considered for the treatment of FIRES but have been disappointing. We report a successful therapeutic encounter using tocilizumab, a humanized monoclonal antibody against interleukin‐6 receptor to treat RSE in a 14‐year‐old girl with FIRES following dismal outcome using conventional immunosuppressive strategies. The overproduction of cytokines and the poor performance of immunotherapies implicate a putative role of inflammation in FIRES. Our case showed that complete termination of RSE was achieved after tocilizumab administration suggesting it may be a lifesaving drug for this catastrophic condition.