Interleukin-6 Blockade With Tocilizumab in Anakinra-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES)

Coral M Stredny; Siobhan Case; Arnold J Sansevere; MaryBeth Son; Lauren Henderson; Mark P Gorman

doi:10.1177/2329048X20979253

Interleukin-6 Blockade With Tocilizumab in Anakinra-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES)

Child Neurol Open. 2020 Dec 15:7:2329048X20979253. doi: 10.1177/2329048X20979253. eCollection 2020 Jan-Dec.

Authors

Coral M Stredny^{1

2}, Siobhan Case³, Arnold J Sansevere², MaryBeth Son³, Lauren Henderson³, Mark P Gorman¹

Affiliations

¹ Department of Neurology, Boston Children's Hospital, Neuroimmunology Program, Harvard Medical School, Boston, MA, USA.
² Division of Epilepsy, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
³ Department of Rheumatology and Allergy/Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is characterized by new onset refractory status epilepticus in a previously healthy child that is associated with poor cognitive outcomes and chronic epilepsy. Innate immune system dysfunction is hypothesized to be a key etiologic contributor, with a potential role for immunotherapy blocking pro-inflammatory cytokines, such as interleukin-1β and interleukin-6. We present a case of FIRES refractory to anakinra, an interleukin-1 receptor antagonist, subsequently treated with the ketogenic diet and tocilizumab, an interleukin-6 receptor antagonist, temporally associated with seizure cessation and a favorable 1-year outcome.

Keywords: anakinra; antiseizure drugs; children; febrile infection-related epilepsy syndrome; ketogenic diet; neuroimmunology; status epilepticus; super refractory status epilepticus; tocilizumab.

Publication types

Case Reports