Dramatic response to delayed treatment with tocilizumab in new-onset refractory status epilepticus.

Abstract

New-Onset Refractory Status Epilepticus (NORSE) is a severe condition characterized by seizures that are refractory to conventional treatments and in which a clearly recognizable cause cannot be found, posing significant diagnostic and therapeutic challenges. However, recent evidence suggests an underlying immunopathogenic mechanism. We present two clinical cases of NORSE that showed an impressive electroclinical response to tocilizumab, an interleukin-6 receptor antagonist, despite it was administered several weeks after the onset of the symptoms. These findings suggest the role of a suspected aberrant immune response not only in the pathogenesis, but also in the maintenance of this pathological condition, highlighting the importance of considering immunomodulatory interventions in refractory epilepsy cases and raising potential questions regarding the most ideal timing and sequence of therapeutic strategies. In conclusion, tocilizumab may represent a promising therapeutic strategy for NORSE, even if administered late in the course of this pathological condition. Further research is needed to elucidate the possible underlying immunopathogenic mechanisms and to establish the efficacy and safety of immunomodulatory interventions in NORSE.