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Molecular Autism
Published in: Molecular Autism 1/2018

Open Access 01-12-2018 | Research

Abnormal coherence and sleep composition in children with Angelman syndrome: a retrospective EEG study

Authors: Hanna den Bakker, Michael S. Sidorov, Zheng Fan, David J. Lee, Lynne M. Bird, Catherine J. Chu, Benjamin D. Philpot

Published in: Molecular Autism | Issue 1/2018

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Abstract

Background

Angelman syndrome (AS) is a neurodevelopmental disorder characterized by intellectual disability, speech and motor impairments, epilepsy, abnormal sleep, and phenotypic overlap with autism. Individuals with AS display characteristic EEG patterns including high-amplitude rhythmic delta waves. Here, we sought to quantitatively explore EEG architecture in AS beyond known spectral power phenotypes. We were motivated by studies of functional connectivity and sleep spindles in autism to study these EEG readouts in children with AS.

Methods

We analyzed retrospective wake and sleep EEGs from children with AS (age 4–11) and age-matched neurotypical controls. We assessed long-range and short-range functional connectivity by measuring coherence across multiple frequencies during wake and sleep. We quantified sleep spindles using automated and manual approaches.

Results

During wakefulness, children with AS showed enhanced long-range EEG coherence across a wide range of frequencies. During sleep, children with AS showed increased long-range EEG coherence specifically in the gamma band. EEGs from children with AS contained fewer sleep spindles, and these spindles were shorter in duration than their neurotypical counterparts.

Conclusions

We demonstrate two quantitative readouts of dysregulated sleep composition in children with AS—gamma coherence and spindles—and describe how functional connectivity patterns may be disrupted during wakefulness. Quantitative EEG phenotypes have potential as biomarkers and readouts of target engagement for future clinical trials and provide clues into how neural circuits are dysregulated in children with AS.
Appendix
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Metadata
Title
Abnormal coherence and sleep composition in children with Angelman syndrome: a retrospective EEG study
Authors
Hanna den Bakker
Michael S. Sidorov
Zheng Fan
David J. Lee
Lynne M. Bird
Catherine J. Chu
Benjamin D. Philpot
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Molecular Autism / Issue 1/2018
Electronic ISSN: 2040-2392
DOI
https://doi.org/10.1186/s13229-018-0214-8

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