Treatment of Cryptogenic New-onset Refractory Status Epilepticus (C-NORSE) with Tocilizumab

Intern Med. 2024 Dec 15;63(24):3377-3382. doi: 10.2169/internalmedicine.3392-23. Epub 2024 Apr 23.

Abstract

A 35-year-old woman with no prior history of epilepsy developed status epilepticus (SE), which was highly resistant to multiple antiseizure medications and sedatives. The etiology of SE was not identified despite extensive investigation, and the patient was diagnosed with cryptogenic new-onset refractory status epilepticus (C-NORSE). Although first-line immunotherapies such as high-dose corticosteroids and plasma exchange were ineffective, the patient manifested a resolution of SE after the administration of tocilizumab, which inhibits interleukin-6. Non-antibody-mediated inflammation has been hypothesized to be a probable pathophysiology of C-NORSE in recent studies, and tocilizumab may be a plausible second-line treatment.

Keywords: autoantibodies; autoimmune encephalitis; encephalitis; epilepsy; interleukin-6 (IL-6); seizure.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Humanized* / therapeutic use
  • Anticonvulsants / therapeutic use
  • Female
  • Humans
  • Interleukin-6 / antagonists & inhibitors
  • Interleukin-6 / blood
  • Status Epilepticus* / diagnosis
  • Status Epilepticus* / drug therapy
  • Status Epilepticus* / etiology
  • Treatment Outcome

Substances

  • tocilizumab
  • Antibodies, Monoclonal, Humanized
  • Anticonvulsants
  • Interleukin-6