Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Feb;30(2):145-52.
doi: 10.1177/0883073814530503. Epub 2014 Apr 27.

Induced gamma oscillations differentiate familiar and novel voices in children with MECP2 duplication and Rett syndromes

Sarika U Peters  1 , Reyna L Gordon  2 , Alexandra P Key  3
Affiliations

Induced gamma oscillations differentiate familiar and novel voices in children with MECP2 duplication and Rett syndromes

Sarika U Peters et al. J Child Neurol. 2015 Feb.

Abstract

Normal levels of the methyl CpG-binding protein 2 (MeCP2) are critical to neurologic functioning, and slight alterations result in intellectual disability and autistic features. It was hypothesized that children with MECP2 duplication (overexpression of MeCP2) and Rett syndrome (underexpression of MeCP2) would exhibit distinct electroencephalographic (EEG) indices of auditory stimulus discrimination. In this study, gamma-band oscillatory responses to familiar and novel voices were examined and related to social functioning in 17 children (3-11 years old) with MECP2 duplication (n = 12) and Rett syndrome (n = 5). Relative to the stranger's voice, gamma activity in response to the mother's voice was increased in MECP2 duplication but decreased in Rett syndrome. In MECP2 duplication, greater mother versus stranger differences in gamma activity were associated with higher social functioning. For the first time, brain responses in a passive voice discrimination paradigm show that overexpression and underexpression of MeCP2 have differential effects on cortical information processing.

Keywords: MECP2; Rett; electroencephalography; gamma.

PubMed Disclaimer

Conflict of interest statement

Declaration of Conflicting Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1
Figure 1
Differences in induced gamma (26-50 Hz) power in response to mother's voice (top) vs. stranger's voice -varied (bottom) in children with MECP2 duplication syndrome. Electrodes belonging to the significant clusters at the time point indicated in each topographic plot (right) are represented with asterisks, and overlay activity for the data used in the contrast. The scale is the percent change from each participant's average activity across conditions. Time-frequency representations (left) were generated by averaging together all of the electrodes belonging to the significant cluster, the time and frequency of which is delineated in the black box; the same power scale applies to both topographic plots and time-frequency representations.
Figure 2
Figure 2
Differences in induced gamma (26-50 Hz) power in response to mother's voice (top) vs. stranger's voice –varied (bottom) in children with Rett syndrome. See legend of Figure 1 for an explanation of plotting.
Figure 3
Figure 3
Correlation between Social Affect and induced gamma cluster sum differences in MECP2 duplication syndrome.
See this image and copyright information in PMC

References

    1. Peters SU, Hundley RJ, Wilson AK, Warren Z, Vehorn A, Carvalho CM, et al. The Behavioral Phenotype in MECP2 Duplication Syndrome: A Comparison With Idiopathic Autism. Autism Res. 2013;6(1):42–50. - PMC - PubMed
    1. Ramocki MB, Peters SU, Tavyev YJ, Zhang F, Carvalho CM, Schaaf CP, et al. Autism and other neuropsychiatric symptoms are prevalent in individuals with MeCP2 duplication syndrome. Ann Neurol. 2009;66(6):771–82. - PMC - PubMed
    1. Ramocki MB, Tavyev YJ, Peters SU. The MECP2 duplication syndrome. Am J Med Genet A. 2010;152A(5):1079–88. - PMC - PubMed
    1. Grasshoff U, Bonin M, Goehring I, Ekici A, Dufke A, Cremer K, et al. De novo MECP2 duplication in two females with random X-inactivation and moderate mental retardation. Eur J Hum Genet. 2011;19(5):507–12. - PMC - PubMed
    1. Na ES, Nelson ED, Adachi M, Autry AE, Mahgoub MA, Kavalali ET, et al. A mouse model for MeCP2 duplication syndrome: MeCP2 overexpression impairs learning and memory and synaptic transmission. J Neurosci. 2012;32(9):3109–17. - PMC - PubMed

Publication types

Substances

Supplementary concepts