Objectives: 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.
目的: 探讨儿童发热感染相关性癫痫综合征（febrile infection-related epilepsy syndrome, FIRES）的脑电图特征及演变，有助于该病的诊治。方法: 回顾性分析2017年5月—2021年12月收治的26例FIRES患儿的临床资料。结果: 26例（100%）患儿病初均有发热，病程早期出现抽搐，很快演变为惊厥持续状态，22例（85%）需要呼吸机辅助通气。全部患儿急性期脑电图正常背景活动消失，发作间期为弥漫性慢波和多灶性棘慢波；发作期表现为游走性局灶性低波幅快节律起始的特征性发作模式。慢性期脑电图背景活动逐渐恢复，异常波相对局限；发作期表现为局灶性慢波节律起始的图形。4例（15%）患儿监测到极度δ刷。结论: FIRES患儿的急性期、慢性期脑电图存在特征性表现，对疾病诊断和分期有一定提示意义。极度δ刷可能是FIRES患儿特征性标志之一。.
Keywords: Child; Electroencephalogram; Epileptic encephalopathy; Extreme δ brush; Febrile infection-related epilepsy syndrome.