Electrophysiological Abnormalities in Angelman Syndrome Correlate With Symptom Severity.

ABSTRACT:

Background

Angelman syndrome (AS) is a rare neurodevelopmental disorder caused by the absence of functional UBE3A in neurons. Excess low-frequency oscillations as measured with electroencephalography (EEG) have been identified as a characteristic finding, but the relationship of this EEG finding to the symptomatology of AS and its significance in the pathophysiology of AS remain unknown.

Methods

We used correlations and machine learning to investigate the cross-sectional and longitudinal relationship between EEG spectral power and motor, cognitive, and language skills (Bayley Scales of Infant and Toddler Development, Third Edition); adaptive behavior (Vineland Adaptive Behavior Scales, Second Edition); AS-specific symptoms (AS Clinical Severity Scale); and the age of epileps

SUBMITTER: Hipp JF 

PROVIDER: S-EPMC8622755 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature