Gálvez-Ortega Kimberly, Harold Roslyn, Neo Wei Siong, Hoilett Orlando S, Borosh Amanda M, Friesen-Haarer Alexa, Gombas Stephanie, Foti Dan, Kelleher Bridgette
Res Sq. 2025 Apr 3:rs.3.rs-5112015. doi: 10.21203/rs.3.rs-5112015/v1.
We describe the development and validation of PANDABox-EEG, a novel protocol for remote EEG assessment with no on-site technician, tailored for Angelman syndrome (AS). We argue that this protocol is reliable, valid, and widely acceptable for use in families affected by Angelman syndrome. AS is a rare neurogenetic condition characterized by developmental delays, sleep problems, seizures, and a happy demeanor. People with AS are frequently monitored via EEG to inform clinical care, and EEG-measured delta activity has been proposed as a reliable biomarker to monitor treatment effectiveness. Traditional EEG assessments pose logistical and financial burdens for families due to the need to travel to a medical center to complete assessments. Telehealth methods, however, offer a pathway forward. PANDABox-EEG was developed through multidisciplinary collaboration with psychologists, psychophysiologists, engineers, and special-education scholars, incorporating caregiver feedback and user-centered design principles. It pairs PANDABox, a telehealth platform for biobehavioral assessment in rare disorders, with ANT Neuro dry electrode EEG system. Twenty-eight participants (7 AS, 7 siblings, 14 caregivers) completed three 5-minute EEG sessions each over the course of a week. Caregivers were asked to provide feedback on acceptability of the design, and EEG data was quantified and assessed for metrics of reliability and validity. PANDABox-EEG demonstrated high feasibility and acceptability, with 91% of caregivers reporting strong satisfaction assessment comfort. EEG data quality was promising, with high internal consistency (split-half reliability range for children with AS: r= .96-.98) and test-retest reliability for delta power among (test-retest reliability range for children with AS: ρ = .88-.96). Finally, we successfully detected the characteristic increased delta power in AS (effect size between AS and non-AS siblings: d=1.56-2.85) and its association with age (effect size between non-AS siblings and caregivers: d=2.19-2.72). PANDABox-EEG provides a feasible, cost-effective, and reliable method for remote EEG assessment in AS. Its high caregiver satisfaction and ability to capture relevant neurophysiological markers suggest potential for broader application. With further validation, PANDABox-EEG can enhance accessibility and inclusivity, benefiting clinical management and research in AS and other clinical populations in need of frequent EEG monitoring by eliminating the need to travel.
我们描述了PANDABox-EEG的开发与验证过程，这是一种专为天使综合征（AS）设计的新型远程脑电图评估方案，无需现场技术人员。我们认为该方案可靠、有效，且在受天使综合征影响的家庭中广泛适用。AS是一种罕见的神经遗传疾病，其特征为发育迟缓、睡眠问题、癫痫发作以及愉悦的神情。AS患者常通过脑电图进行监测以指导临床护理，脑电图测量的δ波活动已被提议作为监测治疗效果的可靠生物标志物。然而，传统的脑电图评估因需要前往医疗中心完成评估，给家庭带来了后勤和经济负担。远程医疗方法则提供了一条前进的道路。PANDABox-EEG是通过与心理学家、心理生理学家、工程师和特殊教育学者的多学科合作开发的，纳入了照顾者的反馈和以用户为中心的设计原则。它将PANDABox（一种用于罕见疾病生物行为评估的远程医疗平台）与ANT Neuro干电极脑电图系统相结合。28名参与者（7名AS患者、7名兄弟姐妹、14名照顾者）在一周内每人完成了三次5分钟的脑电图检查。要求照顾者对设计的可接受性提供反馈，并对脑电图数据进行量化和评估，以确定其可靠性和有效性指标。PANDABox-EEG显示出高度的可行性和可接受性，91%的照顾者报告对评估舒适度非常满意。脑电图数据质量良好，具有较高的内部一致性（AS患儿的分半信度范围：r = 0.96 - 0.98）以及δ波功率的重测信度（AS患儿的重测信度范围：ρ = 0.88 - 0.96）。最后，我们成功检测到了AS患者特征性的δ波功率增加（AS患者与非AS兄弟姐妹之间的效应量：d = 1.56 - 2.85）及其与年龄的关联（非AS兄弟姐妹与照顾者之间的效应量：d = 2.19 - 2.72）。PANDABox-EEG为AS的远程脑电图评估提供了一种可行、经济高效且可靠的方法。其较高的照顾者满意度以及捕捉相关神经生理标志物的能力表明了其更广泛应用的潜力。通过进一步验证，PANDABox-EEG可以提高可及性和包容性，通过消除出行需求，使AS及其他需要频繁脑电图监测的临床人群的临床管理和研究受益。
