The goal of these studies was to use quantitative (q)EEG techniques on data from children with Angelman syndrome (AS) using spectral power analysis, and to evaluate this as a potential biomarker and quantitative method to evaluate therapeutics. Although characteristic patterns are evident in visual inspection, using qEEG techniques has the potential to provide quantitative evidence of treatment efficacy. We first assessed spectral power from baseline EEG recordings collected from children with AS compared to age-matched neurotypical controls, which corroborated the previously reported finding of increased total power driven by elevated delta power in children with AS. We then retrospectively analyzed data collected during a clinical trial evaluating the safety and tolerability of minocycline (3 mg/kg/d) to compare pretreatment recordings from children with AS (4-12 years of age) to EEG activity at the end of treatment and following washout for EEG spectral power and epileptiform events. At baseline and during minocycline treatment, the AS subjects demonstrated increased delta power; however, following washout from minocycline treatment the AS subjects had significantly reduced EEG spectral power and epileptiform activity. Our findings support the use of qEEG analysis in evaluating AS and suggest that this technique may be useful to evaluate therapeutic efficacy in AS. Normalizing EEG power in AS therefore may become an important metric in screening therapeutics to gauge overall efficacy. As therapeutics transition from preclinical to clinical studies, it is vital to establish outcome measures that can quantitatively evaluate putative treatments for AS and neurological disorders with distinctive EEG patterns.

中文翻译：

---

Angelman 综合征的定量脑电图分析：评估治疗的候选方法

这些研究的目的是使用光谱功率分析对天使综合征 (AS) 儿童的数据使用定量 (q)EEG 技术，并将其作为评估治疗的潜在生物标志物和定量方法进行评估。虽然特征模式在视觉检查中很明显，但使用 qEEG 技术有可能提供治疗效果的定量证据。我们首先评估了从 AS 儿童收集的基线脑电图记录与年龄匹配的神经典型对照相比的频谱功率，这证实了先前报告的 AS 儿童中 delta 功率升高导致总功率增加的发现。然后，我们回顾性分析了在一项评估米诺环素 (3 mg/kg/d) 的安全性和耐受性的临床试验中收集的数据，以比较 AS 儿童（4-12 岁）的治疗前记录与治疗结束时的脑电图活动和清除 EEG 频谱功率和癫痫样事件后。在基线和米诺环素治疗期间，AS 受试者表现出增加的 delta 功率；然而，在从米诺环素治疗中清除后，AS 受试者的 EEG 频谱功率和癫痫样活动显着降低。我们的研究结果支持使用 qEEG 分析评估 AS，并表明该技术可能有助于评估 AS 的治疗效果。因此，使 AS 中的脑电图功率正常化可能成为筛选疗法中衡量整体疗效的重要指标。