波幅负荷和癫痫样放电评分系统对促肾上腺皮质激素治疗婴儿癫痫性痉挛综合征疗效的评估价值

Valuation of burden of amplitudes and epileptiform discharges score in the efficacy of adrenocorticotropic hormone therapy for infantile epileptic spasms syndrome

目的探讨波幅负荷和癫痫样放电评分(Burden of amplitudes and epileptiform discharges score,BASED)系统在评估促肾上腺皮质激素(Adrenocorticotropic hormone,ACTH)联合硫酸镁治疗婴儿癫痫性痉挛综合征(Infantile epileptic spasms syndrome,IESS)患者疗效的评估价值。方法回顾性收集2015年—2023年东莞市妇幼保健院小儿神经专科收治的符合IESS并予ACTH联合硫酸镁治疗的124例患者的临床及脑电图资料。根据ACTH治疗后14天时是否有癫痫发作分为无发作组(n=74例)和发作组(n=50例),采用BASED系统评价ACTH治疗前后两组脑电图变化与临床疗效之间的关系。脑电图分析内容包括:①异常高波幅背景波;②≥3个棘波灶的1s格;③成组的多灶性棘波;④阵发性电压衰减。结果ACTH联合硫酸镁治疗IESS控制率为59.7%(74/124),不同病因患儿控制率比较差异无统计学差异(P=0.09)。BASED评分提示IESS患儿治疗后脑电图总缓解率为57.2%。其中发作控制组脑电图缓解率为81.0%(60/74),未控制组脑电图缓解率为22.0%(11/50),发作控制组治疗后EEG缓解明显高于未控制组(P<0.001),脑电图评分与临床疗效相关(Spearman相关系数rp=0.601,P<0.001),相关关系密切。结论BASED评分与临床疗效相关,BASED评分可以为临床ACTH联合硫酸镁治疗IESS疗效评估提供量化依据。

Objective To explore the evaluation value of burden of amplitudes and epileptiform discharges score(BASED)in the efficacy of adrenocorticotropic hormone(ACTH)combined with magnesium sulfate therapy for infantile epileptic spasms syndrome(IESS).Methods Retrospective collection the clinical and EEG data of 124 patients admitted to the Dongguan Maternal and Child Health Care Hospital from 2015 to 2023,who were diagnosed with IESS and treated with ACTH combined with magnesium sulfate.According to whether there were epileptic seizures 14 days after ACTH treatment,the patients were divided into two groups:non seizure group(n=74 cases)and seizure group(n=50 cases).The BASED system was used to evaluate the relationship between changes in EEG before and after ACTH treatment and clinical efficacy in both groups.The analysis of electroencephalogram included:①abnormally high amplitude background waves,②>3 spike foci,③grouped multifocal spikes,④paroxysmal voltage attenuation.Result The control rate of ACTH combined with magnesium sulfate in the treatment of IESS was 59.7%(74/124),and there was no statistically significant difference in the control rate among children with different etiologies(P=0.09).The BASED score suggests that the overall response rate of electroencephalogram(EEG)in infants with epileptic spasm syndrome after treatment was 57.2%.The EEG remission rate in the seizure control group was 81%(60/74),while in the uncontrolled group was 22%(11/50).The EEG remission rate in the seizure control group was significantly higher than that in the uncontrolled group after treatment(P<0.001),and the EEG score was closely related to clinical efficacy(Spearman correlation coefficient rp=0.601,P<0.001).Conclusion The BASED score is related to clinical efficacy,and it can provide a quantitative basis for evaluating the efficacy of ACTH combined with magnesium sulfate in the treatment of IESS.