Pasqualini Nicholas, Falcicchio Giovanni, Vogrig Alberto, Pellicciari Roberta, Defazio Giovanni, Gelormini Domenico, Liviero Marilena Casartelli, Gottin Leonardo, Bonetti Bruno, Zivelonghi Cecilia, Ferlisi Monica, Zanoni Tiziano
Division of Neurology B, University of Verona, Verona University Hospital, Verona, Italy.
DiBraiN Department, University of Bari 'Aldo Moro', Piazza G. Cesare, 11, Bari, 70124, Italy.
Neurol Sci. 2025 Jul;46(7):3281-3285. doi: 10.1007/s10072-025-08108-8. Epub 2025 Mar 18.
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.
新发难治性癫痫持续状态(NORSE)是一种严重病症,其特征为癫痫发作对传统治疗无效,且找不到明确可识别的病因,这带来了重大的诊断和治疗挑战。然而,最近的证据表明存在潜在的免疫致病机制。我们展示了两例NORSE临床病例,尽管在症状出现数周后才给予白细胞介素-6受体拮抗剂托珠单抗,但仍显示出令人印象深刻的电临床反应。这些发现表明,疑似异常免疫反应不仅在发病机制中起作用,而且在维持这种病理状态中也起作用,凸显了在难治性癫痫病例中考虑免疫调节干预的重要性,并引发了关于治疗策略最理想时机和顺序的潜在问题。总之,托珠单抗可能是NORSE的一种有前景的治疗策略,即使在这种病理状态后期使用。需要进一步研究以阐明可能的潜在免疫致病机制,并确定免疫调节干预在NORSE中的疗效和安全性。