An EEG investigation of alpha and beta activity during resting states in adults with Williams syndrome.

BACKGROUND: Williams syndrome (WS) is neurodevelopmental disorder characterised by executive deficits of attention and inhibitory processing. The current study examined the neural mechanisms during resting states in adults with WS in order to investigate how this subserves the attention and inhibitory deficits associated with the syndrome. METHOD: Adopting electroencephalography (EEG) methodology, cortical electrical activity was recorded from eleven adults with WS aged 35 + years during Eyes Closed (EC) and Eyes Open (EO) resting states, and compared to that of thirteen typically developing adults matched for chronological age (CA) and ten typically developing children matched for verbal mental ability (MA). Using mixed-design analyses of variance (ANOVA), analyses focused on the full alpha (8-12.5 Hz), low-alpha (8-10 Hz), upper-alpha (10-12.5 Hz), and beta (13-29.5 Hz) bands, as these are thought to have functional significance with attentional and inhibitory processes. RESULTS: No significant difference in alpha power were found between the WS and CA groups across all analyses, however a trend for numerically lower alpha power was observed in the WS group, consistent with other developmental disorders characterised by attentional/inhibitory deficits such as Attention Deficit Hyperactivity Disorder (ADHD). In contrast, comparable beta power between the WS and CA groups during both EC/EO conditions suggests that their baseline EEG signature is commensurate with successful attentional processing, though this needs to be interpreted with caution due to the small sample size. Analyses also revealed an unusual trend for low variability in the EEG signature of the WS group, which contradicts the heterogeneity typically observed behaviourally. CONCLUSIONS: This novel finding of low variability in the EEG spectra in the WS group has been previously associated with poor behavioural performance in ADHD and is highly informative, highlighting future research needs to also consider how the role of low variability in the EEG profile of WS manifests in relation to their behavioural and cognitive profiles.

Speaker-Versus Listener-Oriented Disfluency: A Re-examination of Arguments and Assumptions from Autism Spectrum Disorder.

We re-evaluate conclusions about disfluency production in high-functioning forms of autism spectrum disorder (HFA). Previous studies examined individuals with HFA to address a theoretical question regarding speaker- and listener-oriented disfluencies. Individuals with HFA tend to be self-centric and have poor pragmatic language skills, and should be less likely to produce listener-oriented disfluency. However, previous studies did not account for individual differences variables that affect disfluency. We show that both matched and unmatched controls produce fewer repairs than individuals with HFA. For silent pauses, there was no difference between matched controls and HFA, but both groups produced more than unmatched controls. These results identify limitations in prior research and shed light on the relationship between autism spectrum disorders and disfluent speech.

An event related potential study of ihibitory and attentional control in Williams syndrome adults.

The primary aim of the current study was to employ event-related potentials (ERPs) methodology to disentangle the mechanisms related to inhibitory control in older adults with Williams syndrome (WS). Eleven older adults with WS (mean age 42), 16 typically developing adults (mean age 42) and 13 typically developing children (mean age 12) participated in the study. ERPs were recorded during a three-stimulus visual oddball task, during which participants were required to make a response to a rare target stimulus embedded in a train of frequent non-target stimuli. A task-irrelevant infrequent stimulus was also present at randomised intervals during the session. The P3a latency data response related to task-irrelevant stimulus processing was delayed in WS. In addition, the early perceptual N2 amplitude was attenuated. These data are indicative of compromised early monitoring of perceptual input, accompanied by appropriate orientation of responses to task-irrelevant stimuli. However, the P3a delay suggests inefficient evaluation of the task-irrelevant stimuli. These data are discussed in terms of deficits in the disengagement of attentional processes, and the regulation of monitoring processes required for successful inhibition.