Kiani Hassan, Whitney Robyn, Go Cristina, Puka Klajdi, Connolly Mary B, Jones Kevin C, Smith Mary Lou, RamachandranNair Rajesh
Department of Pediatrics (Neurology), McMaster University, Hamilton, Canada.
Department of Pediatrics (Neurology), University of British Columbia, Vancouver, Canada.
Epilepsy Behav. 2025 Oct;171:110498. doi: 10.1016/j.yebeh.2025.110498. Epub 2025 May 23.
To assess the response to high-dose daily nocturnal diazepam (HDD) in children with developmental and or epileptic encephalopathy with spike-wave activation in sleep (DEE/EE-SWAS) METHODS: A prospective cohort of patients (4-12 years), newly diagnosed with DEE/EE-SWAS and initiated on the first course of HDD therapy, was followed for one year. Sleep EEG scores (SES) pre and post-HDD were evaluated every three months for 12 months to monitor treatment response. Spike-wave index (SWI) was calculated using the percentage of 1-second bins containing at least one spike. An EEG grading system based on both sleep SW index (sSWI) (Grade: 1-4) and background distribution of epileptiform discharges (Grade: 0-4) was used and summed to yield an aggregate SES (ASES) (Grade: 1-8).
Eighteen eligible children (M:F 12:6; median age, 7.6 years; range, 4.4-11.6 years) were initiated on the first course HDD (median, 0.5 mg/kg/d; range, 0.3-0.6 mg/kg/d). sSWI decreased significantly from 85.7 % (mean, SD 13.9) to 32.6 % (mean, SD 37.1) at subsequent EEG follow-up (95 % CI = -70.60, -35.62; p < 0.001). ASES decreased from 6.5 (SD 1.3) to 3.1 (SD 1.9) (95 % CI = -4.17, -2.60; p < 0.001). EEG relapse after a period of improvement after HDD initiation occurred in 10 children. Minimal response to HDD occurred in 2 children. Five patients manifested mild side effects, including altered behaviour (2), hyperactivity (2), and lethargy (1).
We demonstrate that using a structured EEG scoring system in DEE/EE-SWAS, combining the SWI and its distribution HDD safely and significantly reduces both sSWI and ASES. However, high rates of EEG relapse were recorded.
评估大剂量夜间每日服用地西泮（HDD）对伴有睡眠中棘波激活的发育性和/或癫痫性脑病儿童（DEE/EE-SWAS）的疗效。方法：对一组新诊断为DEE/EE-SWAS并开始接受首个疗程HDD治疗的患者（4至12岁）进行前瞻性队列研究，随访一年。在12个月内，每三个月评估一次HDD治疗前后的睡眠脑电图评分（SES），以监测治疗反应。棘波指数（SWI）通过计算包含至少一个棘波的1秒时间段的百分比得出。使用基于睡眠棘波指数（sSWI）（分级：1至4级）和癫痫样放电背景分布（分级：0至4级）的脑电图分级系统，并将两者相加得出综合SES（ASES）（分级：1至8级）。
18名符合条件的儿童（男∶女为12∶6；中位年龄7.6岁；范围4.4至11.6岁）开始接受首个疗程的HDD治疗（中位剂量，0.5mg/kg/d；范围，0.3至0.6mg/kg/d）。在随后的脑电图随访中，sSWI从85.7%（均值，标准差13.9）显著降至32.6%（均值，标准差37.1）（95%CI = -70.60，-35.62；p < 0.001）。ASES从6.5（标准差1.3）降至3.1（标准差1.9）（95%CI = -4.17，-2.60；p < 0.001）。10名儿童在开始HDD治疗一段时间病情改善后出现脑电图复发。2名儿童对HDD治疗反应极小。5名患者出现轻度副作用，包括行为改变（2例）、多动（2例）和嗜睡（1例）。
我们证明，在DEE/EE-SWAS中使用结构化脑电图评分系统，结合SWI及其分布，HDD能安全且显著降低sSWI和ASES。然而，脑电图复发率较高。
