Neural synchronization deficits linked to cortical hyper-excitability and auditory hypersensitivity in fragile X syndrome
dc.contributor.author | Ethridge, Lauren E. | |
dc.contributor.author | White, Stormi P. | |
dc.contributor.author | Mosconi, Matthew W. | |
dc.contributor.author | Wang, Jun | |
dc.contributor.author | Pedapati, Ernest V. | |
dc.contributor.author | Erickson, Craig A. | |
dc.date.accessioned | 2018-11-13T19:21:43Z | |
dc.date.available | 2018-11-13T19:21:43Z | |
dc.date.issued | 2017-06-07 | |
dc.identifier.citation | Ethridge, L. E., White, S. P., Mosconi, M. W., Wang, J., Pedapati, E. V., Erickson, C. A., ... & Sweeney, J. A. (2017). Neural synchronization deficits linked to cortical hyper-excitability and auditory hypersensitivity in fragile X syndrome. Molecular autism, 8(1), 22. | en_US |
dc.identifier.uri | http://hdl.handle.net/1808/27316 | |
dc.description.abstract | Background Studies in the fmr1 KO mouse demonstrate hyper-excitability and increased high-frequency neuronal activity in sensory cortex. These abnormalities may contribute to prominent and distressing sensory hypersensitivities in patients with fragile X syndrome (FXS). The current study investigated functional properties of auditory cortex using a sensory entrainment task in FXS.Methods EEG recordings were obtained from 17 adolescents and adults with FXS and 17 age- and sex-matched healthy controls. Participants heard an auditory chirp stimulus generated using a 1000-Hz tone that was amplitude modulated by a sinusoid linearly increasing in frequency from 0–100 Hz over 2 s. ResultsSingle trial time-frequency analyses revealed decreased gamma band phase-locking to the chirp stimulus in FXS, which was strongly coupled with broadband increases in gamma power. Abnormalities in gamma phase-locking and power were also associated with theta-gamma amplitude-amplitude coupling during the pre-stimulus period and with parent reports of heightened sensory sensitivities and social communication deficits.Conclusions This represents the first demonstration of neural entrainment alterations in FXS patients and suggests that fast-spiking interneurons regulating synchronous high-frequency neural activity have reduced functionality. This reduced ability to synchronize high-frequency neural activity was related to the total power of background gamma band activity. These observations extend findings from fmr1 KO models of FXS, characterize a core pathophysiological aspect of FXS, and may provide a translational biomarker strategy for evaluating promising therapeutics. | en_US |
dc.publisher | BMC | en_US |
dc.rights | © The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. | en_US |
dc.rights.uri | https://creativecommons.org/licenses/by/4.0/ | en_US |
dc.title | Neural synchronization deficits linked to cortical hyper-excitability and auditory hypersensitivity in fragile X syndrome | en_US |
dc.type | Article | en_US |
kusw.kuauthor | Mosconi, Mathew W. | |
kusw.kudepartment | Applied Behavioral Science | en_US |
dc.identifier.doi | 10.1186/s13229-017-0140-1 | en_US |
kusw.oaversion | Scholarly/refereed, publisher version | en_US |
kusw.oapolicy | This item meets KU Open Access policy criteria. | en_US |
dc.rights.accessrights | openAccess | en_US |
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