Remote EEG Acquisition in Angelman Syndrome using PANDABox-EEG

Abstract

Objective We describe the development and validation of PANDABox-EEG, a novel protocol for remote EEG assess‑ ment 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. 

Background 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 assess‑ ments 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. 

Methods PANDABox-EEG was developed through multidisciplinary collaboration with psychologists, psychophysi‑ ologists, 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 a dry electrode EEG system. Twenty-eight participants (7 AS, 7 siblings, 14 caregivers) completed three 5-min 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. 

Results 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). 

Conclusion 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. 

Keywords Telehealth, EEG, Angelman syndrome, Delta, Reliability, Assessment

Authors

Gálvez-Ortega, K., Harold, R., Neo, W. S., Hoilett, O. S., Borosh, A. M., Friesen-Haarer, A., Gombas, S., Foti, D., & Kelleher, B.