상세검색
최근 검색어 전체 삭제
다국어입력
즐겨찾기0
Annals of Child Neurology(구 대한소아신경학회지) vol.31 no.3.jpg
학술저널

Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome

Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome

DOI : 10.26815/acn.2023.00031

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.

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

로딩중