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Published on: May 16, 2019
Interleukin-6 Blockade With Tocilizumab in Anakinra-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES)
Coral M Stredny1,2, Siobhan Case3, Arnold J Sansevere2
1Department of Neurology, Boston Children's Hospital, Neuroimmunology Program, Harvard Medical School, Boston, MA, USA.
Febrile infection-related epilepsy syndrome (FIRES) in children can cause severe epilepsy. This case study shows a potential treatment approach using the ketogenic diet and tocilizumab, an IL-6 inhibitor, after anakinra failed.
Area of Science:
- Neuroscience
- Immunology
- Pediatrics
Background:
- Febrile infection-related epilepsy syndrome (FIRES) is a rare, severe neurological disorder characterized by new-onset refractory status epilepticus in previously healthy children.
- FIRES is linked to significant cognitive impairment and chronic epilepsy, posing a major clinical challenge.
- Innate immune system dysfunction, particularly involving pro-inflammatory cytokines like IL-1β and IL-6, is a suspected key factor in FIRES pathogenesis.
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