Social affiliation motives modulate spontaneous learning in Williams syndrome but not in autism

BACKGROUND: Children with autism spectrum disorder (ASD) and those with Williams syndrome (WS) have difficulties with learning, though the nature of these remains unclear. METHODS: In this study, we used novel eye-tracking and behavioral paradigms to measure how 36 preschoolers with ASD and 21 age- and IQ-matched peers with WS attend to and learn novel behaviors (1) from the outcomes of their own actions (non-social learning), (2) through imitation of others’ actions (social learning), and across situations in which imitative learning served either an instrumental function or fulfilled social affiliation motives. RESULTS: The two groups demonstrated similar abilities to learn from the consequences of their own actions and to imitate new actions that were instrumental to the achievement of a tangible goal. Children with WS, unlike those with ASD, increased their attention and imitative learning performance when the model acted in a socially engaging manner. CONCLUSIONS: Learning abnormalities in ASD appear to be linked to the social rather than instrumental dimensions of learning

Characterising the profile of everyday executive functioning and relation to IQ in adults with Williams syndrome : is the BRIEF adult version a valid rating scale?

Although there is evidence of a distinct profile of executive dysfunction in Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, the utility of informant reports of everyday executive function (EF) impairments and their relation to intelligence is not yet clear. Here we aimed to evaluate the functional impact of executive dysfunction in adults with WS and to establish the validity of child and adult versions of the most commonly used rating scale for EF assessment, the Behaviour Rating Inventory of Executive Function (BRIEF). We were also interested in whether distinct components of everyday EF relate to intelligence in WS. Parent report child (BRIEF-C) and adult (BRIEF-A) ratings were collected on 20 adults with WS (aged 18.5 to 53 years), with a mean IQ of 60.95 (SD = 17.67). Neuropsychological measures of EF included: The Shape School Test (Espy, 2007); select subdomains of EF from the Woodcock-Johnson III Tests of Cognitive Abilities, Australian Adaptation (WJ III COG); and select subdomains from the Vineland Adaptive Behaviour Scales, Second Edition—Parent Survey (Vineland-II). Results showed that the BRIEF-A, but not the BRIEF-C, was the most highly correlated with neuropsychological measures of EF, suggesting that it was a valid measure of the profile of EF impairments in adults with WS. The profile of everyday EF dysfunction revealed relative impairments in monitoring, working memory, planning and organisation in WS. In addition, both neuropsychological and rating scale measures showed an association between the shifting component of EF and intelligence. These findings indicate that the BRIEF-A is a valid measure of the multidimensional nature of real-world impairments in EF, and highlight its utility as a less labor intensive and low-cost screening tool for measuring specific EF impairments that could become the focus of targeted intervention in adults with WS.18 page(s

Do active video games improve motor function in people with developmental disabilities? A meta-analysis of randomized controlled trials

Objective To conduct a meta-analysis to examine the effectiveness of active video games (AVGs) interventions on motor function in people with developmental disabilities. Data Sources An electronic search of 7 databases (PubMed, EbscoHost, Informit, Scopus, ScienceDirect, Proquest, PsychInfo) was conducted for randomized controlled trials (RCTs) evaluating AVGs to improve motor function in people with developmental disability, published through to May 2018. Study Selection Only articles in a peer-reviewed journal in English were selected and screened by 2 independent reviewers for RCTs that compared AVGs to conventional therapy. Twelve RCTs involving 370 people with developmental disabilities met the inclusion criteria for quantitative analysis. Data Extraction Two independent reviewers assessed risk of bias and study quality using the Egger’s R, grading of recommendation, assessment, development and evaluation, and Template for Intervention Description and Replication checklists. Data Synthesis Three meta-analyses revealed a large effect size for AVGs to improve gross motor skills (Hedges’ g=0.833, 95% confidence interval [95% CI]=0.247-1.420), small to medium effects for balance (g=0.458, 95% CI=0.023-0.948), and a small, nonsignificant effect for functional mobility (g=0.425, 95% CI= −0.03 to 0.881). Training frequency (ie, number of sessions per week) moderated the effect of AVGs on motor function in people with developmental disabilities. Conclusion We conclude that AVGs show task-specific effectiveness for gross motor skills but the effects are moderated by training intensity. However, because of the low number of trials, diverse diagnoses, variable dosage, and multiple outcome measures of the included trials, these results need to be interpreted with caution

Brief Report: The Impact of Sensory Hypersensitivity and Intolerance of Uncertainty on Anxiety in Williams Syndrome

This study explored the interrelationship between intolerance of uncertainty, sensory hyper-sensitivity and anxiety in Williams syndrome (WS). Thirty-two parents or guardians of individuals with WS (Mage = 24.76 years, SD = 7.55) were included. Associations between anxiety, intolerance of uncertainty, sensory hyper-sensitivity, and ASD symptoms were assessed. Linear regression analysis revealed that intolerance of uncertainty and sensory hyper-sensitivity were unique independent predictors of anxiety, while social communication score was not. There was evidence of a mediating effect of sensory hyper-sensitivity on the relationship between intolerance of uncertainty and anxiety. These findings bear strong resemblance to the pattern seen in ASD and emphasize the need for development of anxiety interventions that attempt to reduce negative beliefs about unpredictable situations in WS