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KMID : 1151120230310030151
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Annals of Child Neurology
2023 Volume.31 No. 3 p.151 ~ p.160
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Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome
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Kang Joon-Won
Koh Soo-Kyong
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Abstract
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Febrile infection-related epilepsy syndrome (FIRES) characteristically affects previously healthy children, who experience a sudden and explosive onset of super-refractory status epilepticus preceded by febrile infection and accompanied by fulminant neurogenic inflammation. FIRES, however, can affect individuals of all ages and is a subcategory of new-onset refractory status epilepticus. This definition of FIRES excludes febrile status epilepticus in infants. FIRES is a rare type of epileptic encephalopathy with rapidly progressive onset of seizures and a devastating prognosis, as drug-resistant epilepsy often follows without a latency period. Although the exact pathogenesis of FIRES has not been elucidated, a functional deficiency in the endogenous interleukin-1 receptor antagonist has been implicated in a genetic predisposition to FIRES. Dysregulation of the interleukin-1β?interleukin-1 receptor 1 (IL-1β?IL-1R1) signaling pathway appears to be involved in the pathogenesis of FIRES. In this review, the authors summarize the definition of FIRES, IL-1β?IL-1R1 signaling, the nucleotide-binding oligomerization domain the NLRP3 inflammasome, and IL-1 targeted therapy for FIRES.
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KEYWORD
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Drug resistant epilepsy, Fever, Interleukin-1beta, Interleukin 1 receptor antagonist protein, Inflammasomes
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