Electroencephalogram characteristics of 26 children with febrile infection-related epilepsy syndrome
ZHANG Yi, DING Chang-Hong, LI Hua, WANG Xiao-Hui, CHEN Chun-Hong, FANG Fang
Department of Neurology, Neurological Center, Beijing Children's Hospital, Capital Medical University/National Center for Children's Health, Beijing 100045, China
Abstract Objective To investigate the electroencephalogram (EEG) characteristics and progression of febrile infection-related epilepsy syndrome (FIRES) in children, aiming to enhance diagnosis and treatment approaches. Methods A retrospective analysis was conducted on 26 children with FIRES between May 2017 and December 2021. Results All 26 children (100%) presented with fever at the onset, followed by frequent convulsions that rapidly progressed into convulsive status. Ventilator support was required for 22 cases (85%). During the acute phase, EEG features demonstrated the disappearance of background activity and physiological sleep cycles in all children. Diffuse slow waves and multifocal slow spike slow waves were observed as abnormal waves during the interictal period. A characteristic pattern of focal low amplitude fast wave initiation was detected in all children during seizure episodes. In the chronic phase, the background EEG activity gradually recovered, and the presence of abnormal waves was relatively limited. The characteristic pattern of focal slow wave rhythm initiation was evident during seizure episodes. Additionally, extreme δ brushes were observed in four cases (15%). Conclusions These findings suggest that EEG manifestations in children with FIRES exhibit distinctive patterns during the acute and chronic stages, providing significant value for early diagnosis and clinical staging. Extreme δ brushes may be one of the distinctive markers of children with FIRES.
ZHANG Yi,DING Chang-Hong,LI Hua et al. Electroencephalogram characteristics of 26 children with febrile infection-related epilepsy syndrome[J]. CJCP, 2023, 25(9): 923-928.
ZHANG Yi,DING Chang-Hong,LI Hua et al. Electroencephalogram characteristics of 26 children with febrile infection-related epilepsy syndrome[J]. CJCP, 2023, 25(9): 923-928.
Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions[J]. Epilepsia, 2018, 59(4): 739-744. PMID: 29399791. DOI: 10.1111/epi.14016.
Gofton TE, Gaspard N, Hocker SE, et al. New onset refractory status epilepticus research: what is on the horizon?[J]. Neurology, 2019, 92(17): 802-810. PMID: 30894443. DOI: 10.1212/WNL.0000000000007322.
Terrone G, Frigerio F, Balosso S, et al. Inflammation and reactive oxygen species in status epilepticus: biomarkers and implications for therapy[J]. Epilepsy Behav, 2019, 101(Pt B): 106275. PMID: 31171434. DOI: 10.1016/j.yebeh.2019.04.028.
Hon KL, Leung AKC, Torres AR. Febrile infection-related epilepsy syndrome (FIRES): an overview of treatment and recent patents[J]. Recent Pat Inflamm Allergy Drug Discov, 2018, 12(2): 128-135. PMID: 29745347. DOI: 10.2174/1872213X12666180508122450.
Farias-Moeller R, Bartolini L, Staso K, et al. Early ictal and interictal patterns in FIRES: the sparks before the blaze[J]. Epilepsia, 2017, 58(8): 1340-1348. PMID: 28555777. DOI: 10.1111/epi.13801.
Gaspard N, Hirsch LJ, Sculier C, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives[J]. Epilepsia, 2018, 59(4): 745-752. PMID: 29476535. DOI: 10.1111/epi.14022.
van Baalen A, Vezzani A, H?usler M, et al. Febrile infection-related epilepsy syndrome: clinical review and hypotheses of epileptogenesis[J]. Neuropediatrics, 2017, 48(1): 5-18. PMID: 27919115. DOI: 10.1055/s-0036-1597271.
Kessi M, Liu F, Zhan Y, et al. Efficacy of different treatment modalities for acute and chronic phases of the febrile infection-related epilepsy syndrome: a systematic review[J]. Seizure, 2020, 79: 61-68. PMID: 32417686. DOI: 10.1016/j.seizure.2020.04.015.
Rachfalska N, Pietruszewski J, Paprocka J. Dramatic course of paediatric cryptogenic febrile infection-related epilepsy syndrome with unusual chronic phase presentation: a case report with literature study[J]. Brain Sci, 2021, 11(8): 1030. PMID: 34439649. PMCID: PMC8392460. DOI: 10.3390/brainsci11081030.
Pavone P, Corsello G, Raucci U, et al. Febrile infection-related epilepsy syndrome (FIRES): a severe encephalopathy with status epilepticus. Literature review and presentation of two new cases[J]. Ital J Pediatr, 2022, 48(1): 199. PMID: 36527084. PMCID: PMC9756623. DOI: 10.1186/s13052-022-01389-1.
Fox K, Wells ME, Tennison M, et al. Febrile infection-related epilepsy syndrome (FIRES): a literature review and case study[J]. Neurodiagn J, 2017, 57(3): 224-233. PMID: 28898171. DOI: 10.1080/21646821.2017.1355181.
Culleton S, Talenti G, Kaliakatsos M, et al. The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES): a literature review[J]. Epilepsia, 2019, 60(4): 585-592. PMID: 30854647. DOI: 10.1111/epi.14684.