Research reportDistinguishing response to names in Rett and MECP2 Duplication syndrome: An ERP study of auditory social information processing

- A passive auditory task differentiated between Rett-related neurogenetic disorders.
- The MECP2 Duplication group partially mirrored the responses seen in typical children.
- Better name discrimination in MECP2 Duplication correlated with adaptive behavior.
- RTT participants did not resemble the typical or MECP2 Duplication group.
- Different neural responses to social stimuli may relate to levels of MeCP2 expression.

Despite significant advances at the level of basic research, the characterization of higher-level processes in Rett and MECP2 Duplication syndrome remains understudied. In this pilot study, we assessed social-emotional information processing by testing whether children (ages 4-12 years) with Rett (n = 9) and MECP2 Duplication syndrome (n = 7) distinguished their own spoken name from other names. We hypothesized that own and familiar names would elicit more positive parietal P300 responses than unknown names, and that the groups would have different neural responses to these stimuli. The MECP2 Duplication group partially mirrored the parietal responses to own name observed in typically developing participants, and better name discrimination correlated with more adaptive behaviors. Conversely, participants with RTT did not resemble the typical group, and showed greater responses to close other names at frontal/central regions. These results may reflect the different consequences of too much (MECP2 Duplication) vs. too little (RTT) MeCP2 protein.