Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome

• 영문명: Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome
• 발행기관: 대한소아신경학회
• 저자명: Joon Won Kang Sookyong Koh
• 간행물 정보: 『Annals of Child Neurology(구 대한소아신경학회지)』vol.31 no.3, 151~160쪽, 전체 10쪽
• 주제분류: 의약학 > 소아과학
• 파일형태: PDF
• 발행일자: 2023.06.30

영문 초록

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.

목차

Introduction

Diagnosis of FIRES

Basic Pathogenesis of FIRES: Blood-Brain Barrier Disruption and Neuroinflammation

IL-1 and Its Relevance: IL-1, IL-1R1, IL-1Ra, and NLRP3

IL-1 and Seizures

IL-1 and FIRES

Conclusion

References

키워드

Drug resistant epilepsy Fever Interleukin-1beta Interleukin 1 receptor antagonist protein Inflammasomes