Delta power robustly predicts cognitive function in Angelman syndrome.

ABSTRACT:

Objective

Angelman syndrome (AS) is a severe neurodevelopmental disorder caused by loss of function of the maternally inherited UBE3A gene in neurons. Promising disease-modifying treatments to reinstate UBE3A expression are under development and an early measure of treatment response is critical to their deployment in clinical trials. Increased delta power in EEG recordings, reflecting abnormal neuronal synchrony, occurs in AS across species and correlates with genotype. Whether delta power provides a reliable biomarker for clinical symptoms remains unknown.

Methods

We analyzed combined EEG recordings and developmental assessments in a large cohort of individuals with AS (N = 82 subjects, 133 combined EEG and cognitive assessments, 1.08-28.16 years; 32F) and evaluated delta

SUBMITTER: Ostrowski LM 

PROVIDER: S-EPMC8283185 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature