Spoken word processing in Rett syndrome: Evidence from event‐related potentials

Abstract

This study examined the feasibility of using auditory event‐related potentials to evaluate spoken word processing during passive listening in girls with Rett syndrome (n = 11) and typical peers (n = 33), age 4–12 years. The typical group demonstrated the expected pattern of more negative amplitudes within 200–500 ms in response to words than nonwords at left temporal sites. In participants with Rett syndrome, word‐nonword differentiation was observed at the right temporal sites. More negative left hemisphere amplitudes in response to words were associated (at trend level) with better receptive language skills and more adaptive behavior. The results indicate that girls with Rett syndrome differentiate known words from novel nonwords, but may do so using potentially atypical neural processes. Brain‐behavior correlations support validity of the proposed neural markers of word processing, making passive listening paradigms a promising approach for assessing speech and language processing in participants with limited spoken language skills.

Detection of neurophysiological features in female R255X MeCP2 mutation mice

“…EEG and ERP recordings in individuals with RTT suggested that alterations in brain activity are associated with behavioral and cognitive deficits ( Ammanuel et al, 2015 ; Foxe et al, 2016 ; Key et al, 2019 ; LeBlanc et al, 2015 ; Roche et al, 2019 ; Stauder et al, 2006 ). Some electrophysiological features evaluated in people, such as basal EEG and ERP, have been preliminarily investigated in RTT animal models ( Goffin et al, 2012 ; Goffin et al, 2014 ; LeBlanc et al, 2015 ; Liao et al, 2012 ; Wither et al, 2012 ).…”

Evoked Potentials and EEG Analysis in Rett Syndrome and Related Developmental Encephalopathies: Towards a Biomarker for Translational Research

“…No significant name discrimination effects were noted for participants with Rett syndrome. More recently, Key et al (2019) reported more negative EPs to words vs. non-words in girls with Rett syndrome, although this effect was observed in the opposite hemisphere compared to TD controls. Within the Rett group, more typical responses were associated (at trend level) with higher scores on a behavioral measure of receptive vocabulary.…”

Auditory processing atypicalities for pure tones and complex speech sounds in Rett Syndrome – towards neuromarkers of disease progression

“…Early electrophysiological studies in RTT mostly focused on the auditory brainstem response (ABR), with findings indicating that initial subcortical stages of auditory signal processing appeared mostly unaffected (Stach et al, 1994). In contrast, AEP studies assessing later cortical stages of auditory processing have tended to show quite significant impairments (Bader et al, 1989; Stach et al, 1994; Stauder et al, 2006; Foxe et al, 2016; Peters et al, 2017; Brima et al, 2019; Key et al, 2019). For example, Stach and colleagues (Stach et al, 1994) reported that more than 50% of individuals with RTT showed atypical AEPs, although they did not precisely specify what these abnormalities were.…”

“…Recent reports by Key and colleagues suggest that there may be quite profound processing atypicalities for speech tokens. They reported abnormalities in the AEP to speech stimuli in the latency range from 200-500 ms post-stimulus when the responses to real words were compared to those to non-words (Key et al, 2019) and from 250-450 ms and 450-750 ms when AEPs to a participant’s own name were compared to those evoked by other names (Peters et al, 2017). However, the work was not designed to assess the earlier stages of cortical processing and the initial component structure of the AEP as relatively few trial repetitions were recorded (n = 32), which precludes reliable differentiation of earlier AEP components.…”