Induced gamma oscillations differentiate familiar and novel voices in children with MECP2 duplication and Rett syndromes.
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.
Department of Pediatrics, Vanderbilt University, Nashville, TN, USA Vanderbilt Kennedy Center for Research on Human Development, Nashville, TN, USA sarika.u.peters@vanderbilt.edu.
Sarika U
SU
Peters
Vanderbilt Kennedy Center for Research on Human Development, Nashville, TN, USA.
Reyna L
RL
Gordon
Vanderbilt Kennedy Center for Research on Human Development, Nashville, TN, USA Department of Speech and Hearing Sciences, Vanderbilt University, Nashville, TN, USA.
Alexandra P
AP
Key
D000161:Acoustic Stimulation; D001931:Brain Mapping; D002648:Child; D002675:Child, Preschool; D004192:Discrimination, Psychological; D004569:Electroencephalography; D065818:Gamma Rhythm / Q000235:genetics*; D006801:Humans; D007398:Interpersonal Relations; D008297:Male; D038901:X-Linked Intellectual Disability / Q000503:physiopathology*; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics*; D010775:Photic Stimulation; D021641:Recognition, Psychology / Q000502:physiology*; D015518:Rett Syndrome / Q000235:genetics* / Q000503:physiopathology*; D013223:Statistics as Topic; D013997:Time Factors
C497392:MECP2 protein, human; D051783:Methyl-CpG-Binding Protein 2
MECP2; Rett; electroencephalography; gamma