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Case Reports
. 2020 Dec 15:7:2329048X20979253.
doi: 10.1177/2329048X20979253. eCollection 2020 Jan-Dec.

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

Coral M Stredny  1   2 , Siobhan Case  3 , Arnold J Sansevere  2 , MaryBeth Son  3 , Lauren Henderson  3 , Mark P Gorman  1
Affiliations
Case Reports

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

Coral M Stredny et al. Child Neurol Open. .

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.

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Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Initial EEG revealed delta-beta complexes (A). Electrographic seizures onset predominantly in the right temporal (B) and left temporal (C) regions.
Figure 2.
Figure 2.
Serial MRIs on day 7 (A-C) and 16 (D-F) showed progressive T2 (C, F)/FLAIR (A, D) hyperintensities with diffusion restriction (B, E) in the right hippocampus and generalized atrophy.
Figure 3.
Figure 3.
Seizure burden displayed as approximate seizures/day and daily seizure time (minutes/day) is summarized. Notably, seizure burden on days 19-22 was nearly continuous at times and challenging to quantify, and thus represented as 500 seizures and seizure minutes/day (A). Administration of immunotherapy, ketogenic diet, (A) continuous infusions, and maintenance anti-seizure medications is highlighted (B). There was a decrease in approximate seizures/day and daily seizure time (minutes/day) within 3 days of tocilizumab and 9 days of KD initiation (A). Decreased seizure burden allowed for weaning of continuous infusions (B).
See this image and copyright information in PMC

References

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