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Journal of Neurodevelopmental Disorders
Published in: Journal of Neurodevelopmental Disorders 1/2017

Open Access 01-12-2017 | Research

A resting EEG study of neocortical hyperexcitability and altered functional connectivity in fragile X syndrome

Authors: Jun Wang, Lauren E. Ethridge, Matthew W. Mosconi, Stormi P. White, Devin K. Binder, Ernest V. Pedapati, Craig A. Erickson, Matthew J. Byerly, John A. Sweeney

Published in: Journal of Neurodevelopmental Disorders | Issue 1/2017

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Abstract

Background

Cortical hyperexcitability due to abnormal fast-spiking inhibitory interneuron function has been documented in fmr1 KO mice, a mouse model of the fragile X syndrome which is the most common single gene cause of autism and intellectual disability.

Methods

We collected resting state dense-array electroencephalography data from 21 fragile X syndrome (FXS) patients and 21 age-matched healthy participants.

Results

FXS patients exhibited greater gamma frequency band power, which was correlated with social and sensory processing difficulties. Second, FXS patients showed increased spatial spreading of phase-synchronized high frequency neural activity in the gamma band. Third, we observed increased negative theta-to-gamma but decreased alpha-to-gamma band amplitude coupling, and the level of increased theta power was inversely related to the level of resting gamma power in FXS.

Conclusions

Increased theta band power and coupling from frontal sources may represent a mechanism providing compensatory inhibition of high-frequency gamma band activity, potentially contributing to the widely varying level of neurophysiological and behavioral abnormalities and treatment response seen in full-mutation FXS patients. These findings extend preclinical observations and provide new mechanistic insights into brain alterations and their variability across FXS patients. Electrophysiological measures may provide useful translational biomarkers for advancing drug development and individualizing treatments for neurodevelopmental disorders with associated neuronal hyperexcitability.
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Metadata
Title
A resting EEG study of neocortical hyperexcitability and altered functional connectivity in fragile X syndrome
Authors
Jun Wang
Lauren E. Ethridge
Matthew W. Mosconi
Stormi P. White
Devin K. Binder
Ernest V. Pedapati
Craig A. Erickson
Matthew J. Byerly
John A. Sweeney
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Journal of Neurodevelopmental Disorders / Issue 1/2017
Print ISSN: 1866-1947
Electronic ISSN: 1866-1955
DOI
https://doi.org/10.1186/s11689-017-9191-z

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