Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome
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 affec...
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| Format: | Article |
| Language: | English |
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Korean Child Neurology Society
2023-07-01
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| Series: | Annals of Child Neurology |
| Subjects: | |
| Online Access: | http://www.annchildneurol.org/upload/pdf/acn-2023-00031.pdf |
| _version_ | 1797692847521005568 |
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| author | Joon Won Kang Sookyong Koh |
| author_facet | Joon Won Kang Sookyong Koh |
| author_sort | Joon Won Kang |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-12T02:33:31Z |
| format | Article |
| id | doaj.art-1e8eafe92b5441398c33fc93afd58023 |
| institution | Directory Open Access Journal |
| issn | 2635-909X 2635-9103 |
| language | English |
| last_indexed | 2024-03-12T02:33:31Z |
| publishDate | 2023-07-01 |
| publisher | Korean Child Neurology Society |
| record_format | Article |
| series | Annals of Child Neurology |
| spelling | doaj.art-1e8eafe92b5441398c33fc93afd580232023-09-05T02:05:43ZengKorean Child Neurology SocietyAnnals of Child Neurology2635-909X2635-91032023-07-0131315116010.26815/acn.2023.000311325Interleukin-1 in Febrile Infection-Related Epilepsy SyndromeJoon Won Kang0Sookyong Koh1 Department of Pediatrics & Medical Science, Brain Research Institute, Chungnam National University College of Medicine, Daejeon, Korea Department of Pediatrics, Children’s Hospital & Medical Center, University of Nebraska, Omaha, NE, USAFebrile 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.http://www.annchildneurol.org/upload/pdf/acn-2023-00031.pdfdrug resistant epilepsyfeverinterleukin-1betainterleukin 1 receptor antagonist proteininflammasomes |
| spellingShingle | Joon Won Kang Sookyong Koh Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome Annals of Child Neurology drug resistant epilepsy fever interleukin-1beta interleukin 1 receptor antagonist protein inflammasomes |
| title | Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome |
| title_full | Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome |
| title_fullStr | Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome |
| title_full_unstemmed | Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome |
| title_short | Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome |
| title_sort | interleukin 1 in febrile infection related epilepsy syndrome |
| topic | drug resistant epilepsy fever interleukin-1beta interleukin 1 receptor antagonist protein inflammasomes |
| url | http://www.annchildneurol.org/upload/pdf/acn-2023-00031.pdf |
| work_keys_str_mv | AT joonwonkang interleukin1infebrileinfectionrelatedepilepsysyndrome AT sookyongkoh interleukin1infebrileinfectionrelatedepilepsysyndrome |