目的:分析发育性和/或癫痫性脑病伴睡眠中尖峰波激活(DEE/EE-SWAS)患者的临床电特征,并研究不同疗法对癫痫发作控制的疗效。脑电图(EEG)改善睡眠期间癫痫持续状态(ESES),和认知结果。
方法:在治疗后3个月接受至少一次EEG随访的DEE/EE-SWAS患者。分析患者的人口统计学和临床特征。使用逻辑回归模型评估影响结果的变量。
结果:总计,包括87例患者(47例男性)。ESES识别的中位年龄为81.0个月(IQR64.0,96.0)。46例患者在ESES识别之前被诊断为自限性局灶性癫痫(SeLFEs)。24患有发育性和癫痫性脑病,在睡眠中具有尖峰和波激活(DEE-SWAS),17和其他癫痫。类固醇,苯二氮卓类药物,和抗癫痫药物(ASM)是ESES的初始治疗选择。与其他患者相比,在ESES识别时具有结构性病因或缓慢EEG背景的患者对治疗的反应较小。然而,在逻辑回归模型中,只有EEG背景缓慢的儿童有较低的应答几率.有临床或脑电图反应的儿童表现出认知改善。
结论:类固醇,苯二氮卓类药物,和ASM是DEE/EE-SWAS患者的有效治疗方法。在ESES识别时具有结构性病因或缓慢脑电图背景的儿童可能具有不良的长期预后。癫痫发作减少和脑电图改善的功效与认知改善有关。
OBJECTIVE: To analyze the electroclinical features of patients with developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (DEE/EE-SWAS) and study the efficacy of different therapies on seizure control, electroencephalogram (EEG) improvements of electrical status epilepticus during sleep (
ESES), and cognition outcomes.
METHODS: Patients with DEE/EE-SWAS who underwent at least one follow-up EEG 3 months after therapy were retrospectively enrolled. The demographic and clinical characteristics of the patients were analyzed. Variables that influenced the outcomes were evaluated using logistic regression models.
RESULTS: In total, 87 patients (47 males) were included. The median age at
ESES recognition was 81.0 months (IQR 64.0, 96.0). Forty-six patients were diagnosed with self-limited focal epilepsies (SeLFEs) before
ESES recognition, 24 with developmental and epileptic encephalopathies with spike-and-wave activation in sleep (DEE-SWAS), and 17 with other epilepsies. Steroids, benzodiazepines, and antiseizure medications (ASMs) were the initial treatment options for ESES. Patients with structural etiologies or slow EEG backgrounds at the time of
ESES recognition were less likely to respond to treatment than other patients. However, only children with slow EEG backgrounds had lower odds of response in logistic regression models. Children with clinical or EEG response showed improvements in cognition.
CONCLUSIONS: Steroids, benzodiazepines, and ASMs are effective treatments for patients with DEE/EE-SWAS. Children with structural etiologies or slow EEG backgrounds at the time of
ESES recognition may have a poor long-term prognosis. The efficacy of seizure reduction and EEG improvement is associated with cognitive improvement.