Stranger Danger Awareness in Williams Syndrome

Background: The developmental disorder Williams syndrome (WS) is characterised by a distinctive cognitive profile and an intriguing social phenotype. Individuals with the disorder are often highly social engaging with familiar and unfamiliar people and once in an interaction they often show subtle abnormalities of social behaviour. Atypically increased approach to unfamiliar people is widely reported in the existing literature for both children and adults. Parents frequently report interactions with unfamiliar people as a major concern. Methods: In this study we aimed to evaluate ‘stranger danger’ awareness using a video vignette task with individuals who had WS. When linked to other components of the WS phenotype (e.g. reduced intellectual ability, increased social approach) an awareness of stranger danger is particularly important. Results: Qualitative and quantitative data showed that young people with WS have difficulties making judgements about whether or not to trust and engage in conversation with unfamiliar people. Qualitative data showed that individuals with WS often suggested that they would engage in an interaction with an unfamiliar person. Conclusions: The findings have substantial implications for the safety of young people with the disorder and emphasise the need for intervention regarding this behaviour

Attentional Lapse and Inhibition Control in Adults with Williams Syndrome

Research exploring cognitive processing associated with Williams Syndrome (WS) has suggested that executive functioning deficits exist across the developmental spectrum. Such executive functions include problem solving, planning, dividing attention and inhibiting responses. Within a framework of executive functions, the aim of the current study was to explore attentional lapse and inhibition skills in older adults with WS (n = 20; aged 36–61 yr) and consider the implications of deficits within this group. Participants with WS were compared to typical adults of the same chronological age and typical older adults (aged 65+ yr) to consider attentional changes seen in the ageing process. The study employed a sustained attention to response task known to assess inhibition and attentional lapse but which had not previously been used with this population. Compared to both groups of typical matches, the results indicated atypicalities of attention and inhibition in adults with WS. Specifically, compared to the typical matches, adults with WS failed to withhold a response (showing inhibition deficits), had problems re-engaging attentional control processes after making an error and showed a generalised deficit of concentration and task engagement. We conclude that further attention should be paid to the cognitive capacity of older individuals with WS in order to consider the everyday challenges faced by this group and to provide adequate intervention and support for daily living

Brief report: Exploring the relationship between repetitive behaviours and sensory processing in Williams syndrome

This study explored the relationship between sensory processing abnormalities and repetitive behaviours in children with Williams Syndrome (WS; n = 21). This is a novel investigation bringing together two clinical phenomena for the first time in this neuro-developmental disorder. Parents completed the Sensory Profile (Short Form; Dunn in The sensory profile manual. San Antonio: The Psychological Corporation, 1999) and the Repetitive Behaviour Questionnaire (Turner 1995). A significant correlation was evident between the total scores on each of these measures; suggesting that children with WS who exhibit increased sensory processing abnormalities also display a higher number of repetitive behaviours. Further exploratory analyses of subscales of the measures indicated potentially important relationships that suggest a role for arousal regulation in the relationship between sensory processing abnormalities and repetitive behaviours in WS

A cross-syndrome approach to the social phenotype of neurodevelopmental disorders: Focusing on social vulnerability and social interaction style

Background: Following Annette Karmiloff-Smith’s approach to cognitive research, this study applied a cross-syndrome approach to the social phenotype, focusing on social vulnerability (SV) and the factors that contribute to it. Aims: To (i) identify syndrome-specific differences in SV across four neurodevelopmental disorder (NDD) groups, (ii) determine the contribution of intellectual disability (ID), age or gender to SV, and (iii) explore its relationship with social interaction style (SIS). Methods and procedures: 262 parents of children: Autism (n = 29), Williams syndrome (n = 29), Attention deficit hyperactivity disorder (n = 36), Fragile X syndrome (n = 18), and Neurotypical (n = 150) reported on their child’s SV, quality of SIS and other factors (ID, age, gender). Outcomes and results: Heightened SV was not syndrome-specific. Instead it was found equally across NDD groups (and not in the neurotypical group), and independently of ID, age and gender. Different atypical SISs were also distributed across NDD groups and each were significantly related to SV, independent of the factors above and beyond neurodevelopmental diagnosis. Conclusions and implications: The findings emphasise that social phenotypes are best understood as distributed across diagnostic boundaries and offer opportunities to further test the role of varied atypical SISs in the development of heightened SV

Mapping real-world to online vulnerability in young people with developmental disorders: Illustrations from autism and Williams syndrome

The Internet poses a new kind of threat, especially for those individuals already vulnerable in society. The current paper draws on the social phenotypes associated with autism spectrum disorder (ASD) and Williams syndrome (WS) to propose that individuals with some developmental disorders face an elevated level of risk whilst online. Many individuals with ASD struggle to maintain social relations and are frequent users of screen-based technology, using the Internet to seek out social connections. Similarly, individuals with WS harbour an extreme pro-social drive to interact with others, both familiar and unfamiliar, and experience difficulties understanding the subtle nuances of social behaviour. Specific risk factors such as these are used to illustrate the case for online vulnerability in developmental disorders

Linking social behaviour and anxiety to attention to emotional faces in Williams syndrome

The neurodevelopmental disorder Williams syndrome (WS) has been associated with a social phenotype of hypersociability, non-social anxiety and an unusual attraction to faces. The current study uses eye tracking to explore attention allocation to emotionally expressive faces. Eye gaze and behavioural measures of anxiety and social reciprocity were investigated in adolescents and adults with WS when compared to typically developing individuals of comparable verbal mental age (VMA) and chronological age (CA). Results showed significant associations between high levels of behavioural anxiety and attention allocation away from the eye regions of threatening facial expressions in WS. The results challenge early claims of a unique attraction to the eyes in WS and suggest that individual differences in anxiety may mediate the allocation of attention to faces in WS

Cognitive training as a resolution for early executive function difficulties in children with intellectual disabilities

Core executive functions (EF) such as attention, and working memory have been strongly associated with academic achievement, language development and behavioral stability. In the case of children who are vulnerable to cognitive and learning problems because of an underlying intellectual disability, EF difficulties will likely exacerbate an already compromised cognitive system. The current review examines cognitive training programs that aim to improve EF, specifically focusing on the potential of this type of intervention for children who have intellectual disabilities. We conclude that despite considerable discrepancies regarding reported intervention effects, these inconsistencies can be attributed to flaws in both program and study design. We discuss the steps needed to address these limitations and to facilitate the advancement of non-pharmaceutical interventions for children with intellectual disabilities

Looking and Thinking: How individuals with Williams syndrome make judgements about mental states

Individuals with the neuro-developmental disorder Williams syndrome (WS) are characterised by a combination of features which makes this group vulnerable socially, including mild-moderate cognitive difficulties, pro-social drive, and indiscriminate trust. The purpose of this study was to explore a key socio-communicative skill in individuals with WS, namely, mental state recognition abilities. We explored this skill in a detailed way by looking at how well individuals with WS recognise complex everyday mental states, and how they allocate their attention while making these judgements. Participants with WS were matched to two typically developing groups for comparison purposes, a verbal ability matched group and a chronological age matched group. While eye movements were recorded, participants were shown displays of eight different mental states in static and dynamic form, and they performed a forced-choice judgement on the mental state. Mental states were easier to recognise in dynamic form rather than static form. Mental state recognition ability for individuals with WS was poorer than expected by their chronological age, and at the level expected by their verbal ability. However, the pattern of mental state recognition for participants with WS varied according to mental state, and we found some interesting links between ease/difficulty recognising some mental states (worried/do not trust) and the classic behavioural profile associated with WS (high anxiety/indiscriminate trust). Furthermore, eye tracking data revealed that participants with WS allocated their attention atypically, with less time spent attending the information from the face regions. This challenges the widely held understanding of WS being associated with prolonged face and eye gaze, and indicates that there is more heterogeneity within this disorder in terms of socio-perception than previous reports would suggest

Children with autism spectrum disorder (ASD) attend typically to faces and objects presented within their picture communication systems

ackground Children with autism spectrum disorder (ASD) may require interventions for communication difficulties. One type of intervention is picture communication symbols which are proposed to improve comprehension of linguistic input for children with ASD. However, atypical attention to faces and objects is widely reported across the autism spectrum for several types of stimuli. Method In this study we used eye-tracking methodology to explore fixation duration and time taken to fixate on the object and face areas within picture communication symbols. Twenty-one children with ASD were compared with typically developing matched groups. Results Children with ASD were shown to have similar fixation patterns on face and object areas compared with typically developing matched groups. Conclusions It is proposed that children with ASD attend to the images in a manner that does not differentiate them from typically developing individuals. Therefore children with and without autism have the same opportunity to encode the available information. We discuss what this may imply for interventions using picture symbols

Social feedback enhances learning in Williams syndrome

Williams syndrome (WS) is a rare genetic condition characterized by high social interest and approach motivation as well as intellectual disability and anxiety. Despite the fact that social stimuli are believed to have an increased intrinsic reward value in WS, it is not known whether this translates to learning and decision making. Genes homozygously deleted in WS are linked to sociability in the general population, making it a potential model condition for understanding the social brain. Probabilistic reinforcement learning was studied with either social or non-social rewards for correct choices. Social feedback improved learning in individuals with Williams syndrome but not in typically developing controls or individuals with other intellectual disabilities. Computational modeling indicated that these effects on social feedback were mediated by a shift towards higher weight given to rewards relative to punishments and increased choice consistency. We conclude that reward learning in WS is characterized by high volatility and a tendency to learn how to avoid punishment rather than how to gain rewards. Social feedback can partly normalize this pattern and promote adaptive reward learning