Xiaoyue Hu, Hongwei Tang, Jianbiao Wang, Jingbo Ma, Ying Hua
Department of Neurology, Wuxi Children's Hospital, Affiliated Children's Hospital of Jiangnan University, Wuxi, China.
Front Pediatr. 2024 Aug 9;12:1388008. doi: 10.3389/fped.2024.1388008. eCollection 2024.
This study aims to evaluate the therapeutic efficacy of high-dose corticosteroid therapy in children diagnosed with epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS), investigate associated clinical indicators influencing treatment outcomes, and establish a predictive model for recurrence.
Children diagnosed with EE-SWAS who received high-dose corticosteroid therapy were categorized into responder group and non-responder group. Data on clinical parameters, electroencephalogram (EEG) features, and serum cytokine levels were collected. Six months post-treatment, the effectively treated children were further stratified into recurrence and non-recurrence groups. Risk factors for poor outcomes following corticosteroid therapy were identified using univariate analysis. Multivariate logistic regression analysis was then employed to determine independent factors influencing the recurrence of corticosteroid therapy, which facilitated the development of a predictive model.
The study included 48 children, with 33 cases in the responder group (effective rate = 68.8%) and 15 cases in the non-responder group. The responder group exhibited an older onset age of electrical status epilepticus in sleep (ESES) and higher proportions of combined benzodiazepines (BZDs) use ( < 0.05). Among those responding to corticosteroid therapy, 11 cases experienced a recurrence (recurrence rate = 33.3%), while 22 cases did not. Significant differences were observed between the two groups concerning age of seizure onset, age of ESES onset, seizure frequency, atypical presentations, and concomitant frontal lobe discharges (all < 0.05). Concomitant frontal lobe discharges and an earlier age of seizure onset were identified as risk factors for ESES recurrence following corticosteroid therapy. The predictive model was formulated as Logit(P) = 2.35 × presence of frontal lobe discharges-0.802 × age of seizure onset + 2.457. The Area Under the Curve (AUC) of Receiver Operating Characteristics (ROC) was 0.93, with sensitivity and specificity at 100% and 77.3%, respectively.
High-dose corticosteroid therapy for EE-SWAS exhibited a high effective rate as well as a notable recurrence rate. Onset age of ESES and combined benzodiazepines usage correlated with therapeutic efficacy. Seizure onset age and the presence of frontal lobe discharges may hold predictive value for recurrence following corticosteroid therapy.
本研究旨在评估大剂量皮质类固醇激素疗法对诊断为睡眠期棘慢波激活型癫痫性脑病（EE-SWAS）患儿的治疗效果，调查影响治疗结果的相关临床指标，并建立复发预测模型。
将接受大剂量皮质类固醇激素疗法的EE-SWAS诊断患儿分为反应组和无反应组。收集临床参数、脑电图（EEG）特征及血清细胞因子水平的数据。治疗6个月后，将治疗有效的患儿进一步分为复发组和未复发组。采用单因素分析确定皮质类固醇激素治疗后不良结局的危险因素。然后采用多因素logistic回归分析确定影响皮质类固醇激素治疗复发的独立因素，从而建立预测模型。
本研究纳入48例患儿，其中反应组33例（有效率=68.8%），无反应组15例。反应组睡眠期癫痫性电持续状态（ESES）起病年龄较大，联合使用苯二氮䓬类药物（BZD）的比例较高（<0.05）。在对皮质类固醇激素治疗有反应的患儿中，11例复发（复发率=33.3%），22例未复发。两组在癫痫起病年龄、ESES起病年龄、癫痫发作频率、非典型表现及伴有额叶放电方面存在显著差异（均<0.05）。伴有额叶放电和较早的癫痫起病年龄被确定为皮质类固醇激素治疗后ESES复发的危险因素。预测模型公式为Logit(P)=2.35×额叶放电情况-0.802×癫痫起病年龄+2.457。受试者工作特征曲线（ROC）下面积（AUC）为0.93，敏感性和特异性分别为100%和77.3%。
大剂量皮质类固醇激素疗法治疗EE-SWAS有效率高，但复发率也显著。ESES起病年龄和联合使用苯二氮䓬类药物与治疗效果相关。癫痫起病年龄和额叶放电情况可能对皮质类固醇激素治疗后的复发具有预测价值。
