Sensory processing profiles and autistic symptoms as predictive factors in autism spectrum disorder and Williams syndrome.

BACKGROUND: Unusual sensory responses were included in the diagnostic criteria for autism spectrum disorder (ASD), yet they are also common among individuals with other neurodevelopmental disorders, including Williams syndrome (WS). Cross-syndrome comparisons of sensory atypicalities and the evaluation of their syndrome specificity however have rarely been undertaken. We aimed to (1) examine and compare the sensory profiles in ASD and WS groups and (2) investigate whether autistic symptoms, including sensory processing scores, can predict a group membership. METHODS: Parents of 26 children with ASD and intellectual disability, 30 parents of children with ASD (no intellectual disability) and 26 with WS aged between 4 and 16 years were recruited. Parents completed the Sensory Profile to provide information about their children's sensory experiences and the Social Responsiveness Scale - Second Edition (SRS-2) to assess the degree of social impairment in their children. RESULTS: No significant differences were found in sensory processing scores between the three groups. Binary logistic regression analyses were undertaken with sensory quadrants and SRS-2 total score as factors. Models significantly predicted group membership, with Low Registration, Sensory Sensitivity and SRS-2 total score being significant predictors. CONCLUSIONS: The findings suggest that high rates of sensory atypicalities are a common neurodevelopmental characteristic that do not reliably distinguish between WS and ASD groups. Low Registration and Sensory Sensitivity-related behaviours might, however, be more specific to ASD. Further work is needed to explore what behaviours within sensory profiles can discriminate between neurodevelopmental disorders and should be included in diagnostic classifications.

Parent insights into atypicalities of social approach behaviour in Williams syndrome.

BACKGROUND: Individuals with Williams syndrome have been reported to show high levels of social interest and a desire to interact with others irrespective of their familiarity. This high social motivation, when combined with reduced intellectual capacity and a profile of atypical social behaviour, is important in terms of social vulnerability of individuals with the disorder. Therefore, social approach to unfamiliar people and the role of this behaviour within the Williams syndrome (WS) social phenotype warrant further research to inform social skills' intervention design. METHODS: The current study used parent interviews (n = 21) to probe aspects of social behaviour and interactions with strangers, as well as the impact of such behaviour on the family. Using thematic analysis, it was possible to explore themes that emerged from the interviews, offering qualitatively rich insight into the variability of social approach behaviour in WS. RESULTS: Thematic analysis confirmed a significant desire to interact with strangers as well as a lack of awareness of appropriate social boundaries. However, parental reports about their child's social approach behaviour varied considerably. The within-syndrome variability of the sample was emphasised in parental reports of their child's personality characteristics (e.g. levels of impulsiveness), as well as the level of parental supervision employed. CONCLUSIONS: These in-depth parent insights can help target the needs of individuals with WS and emphasise that an individual approach to intervention will be essential because of the heterogeneity of the WS social profile.

Exploring the prevalence and phenomenology of repetitive behaviours and abnormal sensory processing in children with Williams Syndrome.

BACKGROUND: A small amount of research with individuals who have Williams Syndrome (WS) suggests that children with the condition may be vulnerable to sensory processing abnormalities and present with repetitive and restricted behaviours. METHODS: Parents of 21 children with WS aged 6-15 years completed a semi-structured interview designed to elicit the form, frequency, impact and developmental course of a range of sensory processing abnormalities and repetitive behaviours. RESULTS: Findings indicate that sensory processing difficulties are predominantly characterised by hypersensitivities, particularly in relation to vestibular, auditory, gustatory and proprioceptive functioning. Parents also reported the presence of a range of restricted and repetitive behaviours, which were often associated with their child's sensory symptoms. CONCLUSIONS: This study makes a significant contribution to our understanding of sensory functioning and repetitive behaviours in WS. It also highlights the need for a multidisciplinary assessment of the difficulties experienced by children with the disorder.

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.

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

BACKGROUND: 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.

'Too withdrawn' or 'too friendly': considering social vulnerability in two neuro-developmental disorders.

In some neuro-developmental disorders, the combined effect of intellectual disability and atypicalities of social cognition may put individuals at increased vulnerability in their social environment. The neuro-developmental disorders Williams syndrome, characterised by 'hypersociability', and autism spectrum disorders, characterised by 'social withdrawal', are at two extremes of atypical social functioning in humans. In this article, we use Williams syndrome and autism spectrum disorders as exemplars to demonstrate how atypicalities of social cognition may contribute to social vulnerability in these populations. The lives of individuals with both these disorders are marred by an increased risk of social isolation, bullying, unsteady relationships, employment difficulties and abuse. While different behavioural interventions have been tried to improve social functioning in these populations, there has been great variability in their success. Finally, we discuss different issues regarding social independence of these individuals; including employment, safety and decision making.

The Impact of COVID-19 on Anxiety and Worries for Families of Individuals with Special Education Needs and Disabilities in the UK.

COVID-19 has affected people across the world. The current study examined anxiety and worries during the first UK national lockdown in March 2020. Parents (n = 402) reported on their own anxiety and worries as well as that of their son/daughter with Special Education Needs and Disabilities (SEND) and typically developing (TD) child (n = 186) at three time points. Although both groups showed increased anxiety across the three time points, levels of anxiety in the SEND group, but not the TD siblings, were predicted by awareness about COVID-19. In addition, worries differed between the groups showing that COVID-19 impacts the wellbeing of those with SEND differently to that of their TD siblings.

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.