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The block design task (BDT) is a visuospatial measure that individuals with Williams syndrome (WS) perform poorly on. However, it is unclear what underlies their impaired performance. This study investigated whether poorer performance is a result of visuospatial difficulties, executive function (EF) difficulties, atypical looking strategies, or a combination of these. Eleven individuals with WS participated alongside mental age (MA)- and chronological age (CA)-matched control groups. Eye movements were recorded while they took the BDT. Dwell times and visits to areas of interest in WS differed from CA, but not MA, groups. Findings suggest that BDT abilities of individuals with WS are delayed, but not atypical. Delays result from visuospatial and attention-switching difficulties rather than atypical looking strategies.

Educational applications (apps) are ubiquitous within children's learning environments and emerging evidence has demonstrated their efficacy. However, it remains unclear what the active ingredients (ie, mechanisms), or combination of ingredients, of successful maths apps are. The current study developed a new, open-access, three-step framework for assessing the educational value of maths apps, comprised of type of app, mathematical content and app design features. When applied to a selection of available maths apps previously evaluated with children in the first 3 years of school (the final sample included 23 apps), results showed that practice-based apps were the most common app type tested (n = 15). Basic number skills, such as number representation and relationships, were the most common area of mathematics targeted by apps (n = 21). A follow-up qualitative comparative analysis showed observed learning outcomes with maths apps were enhanced when apps combined the following: a scaffolded and personalised learning journey (programmatic levelling) and explanations of why answers were right or wrong (explanatory feedback), as well as praise, such as ‘Great job!’ (motivational feedback). This novel evidence stresses the significance of feedback and levelling design features that teaching practitioners and other stakeholders should consider when deciding which apps to use with young children. Directions for future research are discussed.

Neuromyths are commonly held misconceptions about the brain believed by both the general public and educators. While much research has investigated the prevalence of myths about the typically developing brain, less attention has been devoted to the pervasiveness of neuromyths about neurodevelopmental disorders, which have the potential to exacerbate stigma. This preregistered study investigated to what extent neuromyths about neurodevelopmental disorders (namely dyslexia, attention deficit hyperactivity disorder, autism spectrum disorders, and syndrome) are endorsed by two groups: the general public and those working in education. In an online survey, 366 members of the general public and 203 individuals working in education rated similar numbers of myths to be true, but more about neurodevelopmental disorders than general neuromyths. As the frequency of access to brain information emerged as a protective factor against endorsing myths in both populations, we argue that this problem may be addressed via provision of neuroeducational resources.

Young people with neurodevelopmental conditions (NDCs) have been reported to experience increased levels of anxiety during the COVID-19 pandemic. In our study, we document how young people with Down Syndrome (DS; N=557) and Williams syndrome (WS, N=247) experienced the first wave of the COVID-19 pandemic across the world. Using multilevel linear mixed regressions, we studied (a) parental reported anxiety of young people with DS and WS, (b) these young people’s specific concerns, and (c) their use and efficacy of emotion regulation (ER) strategies. Predictors of anxiety, such as the age of the individual with NDC, type of condition, and time, were investigated. Young people with WS experienced higher levels of anxiety compared to those with DS and the older the people with NDC were, the more anxiety they experienced. In terms of concerns, group effects indicated that young people with WS scored higher for most of the concerns. Higher financial concerns were found in males compared to females, whereas most of the concerns increased with age except for concerns about loss of institutional support and family conflict. Finally, significant group effects were found and indicated a more frequent use of a variety of adaptive and maladaptive ER strategies in young people with WS. We only identified group differences in ER efficacy for two strategies: isolation/withdrawal was more efficient for young people with DS, while repetitive behaviours were more efficient for young people with WS. Our results indicate that young people with WS are likely to exhibit higher levels of anxiety, but also of concerns depending on their age and gender. Similarly, young people with WS use a variety of ER strategies more frequently but these strategies are not necessarily more efficient for them. We discuss the impact of these findings in relation to anxiety identification and support across young people with NDCs.

Williams syndrome (WS) is a rare genetic syndrome. As with all rare syndromes, obtaining adequately powered sample sizes is a challenge. Here we present legacy data from seven UK labs, enabling the characterisation of cross-sectional and longitudinal developmental trajectories of verbal and non-verbal development in the largest sample of individuals with WS to-date. In Study 1, we report cross-sectional data from between N=102 and N=209 children and adults with WS on measures of verbal and non-verbal ability. In Study 2, we report longitudinal data from N=17 to N=54 children and adults with WS who had been tested on at least three timepoints on these measures. Data support the WS characteristic cognitive profile of stronger verbal than non-verbal ability, and shallow developmental progression for both domains. Both cross-sectional and longitudinal data demonstrate steeper rates of development in the child participants than the adolescent and adults in our sample. Cross-sectional data indicate steeper development in verbal than non-verbal ability, and that individual differences in the discrepancy between verbal and non-verbal ability are largely accounted for by level of intellectual functioning. A diverging developmental discrepancy between verbal and non-verbal ability, whilst marginal, is not mirrored statistically in the longitudinal data. Cross-sectional and longitudinal data are discussed with reference to validating cross-sectional developmental patterns using longitudinal data and the importance of individual differences in understanding developmental progression.

BACKGROUND: This study examines school exclusion and truancy in relation to both conduct and emotional problems. It considers these mental health problems both as predictors and as outcomes of school exclusion and truancy.METHOD: The sample included 15,236 individuals from the Millennium Cohort Study, a UK longitudinal birth cohort study. Using subscales of the Strengths and Difficulties Questionnaire, conduct and emotional problems were assessed through childhood to adolescence (age 3, 5, 7, 11, 14 and 17 years), and reports of school exclusion and truancy were collected at age 11 and 14. RESULTS: Conduct problems during childhood were associated with subsequent exclusion and truancy in early adolescence in both males and females, but childhood emotional symptoms showed no such association. Prior exclusion was associated with an increase in conduct problems at age 14 for both genders, and for males it was also associated with an increase in emotional symptoms both at age 14 and 17. Prior truancy was associated with an increase in conduct problems at age 14 for both genders, and for females also at age 17, and it was associated with increased emotional symptoms at age 14 and 17 for both genders.CONCLUSION: Overall results indicate a bidirectional association between conduct problems and school exclusion and truancy. For emotional symptoms, the association is unidirectional as these symptoms do not lead to exclusions or truancy. However, an increase in emotional symptoms may be a consequence of exclusion and truancy.

This study examined individual differences as well as the development of sensory processing difficulties in children with Williams syndrome (WS) using a cross-sectional (Experiment 1) and longitudinal design (Experiment 2). In Experiment 1, a clustering approach of sensory processing scores suggested two groups. Experiment 2 showed that the clusters identified in Experiment 1 were not stable across development, especially for those with high sensory impairments at either time point. Yet, most children experienced high impairments in sensory registration at both time points, suggesting impaired registration is a core phenotype of sensory processing in children with WS across development. Possible mechanisms, limitations and implications are discussed.

Although they have the potential to excel, twice-exceptional (2e) students of mathematics do not usually have this opportunity as their special educational abilities, and special needs, are often m...

Aims Previous international research has shown that autistic people have difficulty accessing dental care due to sensory processing, anxiety and communication issues. However, it is not known whether autistic adults in the United Kingdom are experiencing similar dental care barriers. The current study investigated autistic adults' dental experiences in the United Kingdom. Methods and results A mixed methods survey was given to self-selecting autistic (n = 37) and non-autistic adults (n = 43) to compare whether autistic individuals had more negative experiences than non-autistic individuals. Closed questions asked about anxiety, patient-practitioner communication, satisfaction and the dental environment. Open questions asked about autism-specific dental challenges, what works well at the dentist, and improving autistic individuals' experiences. Responses were analyzed using thematic analysis. Overall, results demonstrated that autistic adults in the United Kingdom have more negative dental experiences than non-autistic adults. These predominantly related to interactions with dental practitioners, a challenging sensory environment, anxiety, pain, and disclosure. Participants recommended sensory environment adaptations, ways to increase preparedness, longer appointments, and individual accommodations. Conclusion This study offers best-practice strategies for working with autistic patients and highlights the importance of dentists working with autistic individuals to maximize the quality of care and outcomes for this underserved population.

The current paper examined the impact of the transition from primary to secondary school on anxiety for children with Neurodevelopmental Disorders (NDDs), specifically for autistic children, children with Down Syndrome (DS) and Williams Syndrome (WS). Previous research has highlighted the impact such educational changes can have upon autistic children, but there is only limited research for children with DS and none for those with WS. Hence, this study aimed to better understand whether school transitions have a unique or similar impact on anxiety through a cross-syndrome comparison. Sixty-one parents as well as their autistic children, children with DS and WS completed an online survey at two time-points, which included questions on adjustment and psychopathology, maladaptive behaviours, and other open-ended measures about their child’s skills as well as their experiences of the transition from primary to secondary school. Children themselves completed a short interview as well as a set of cognitive abilities tasks. Both children and parents of all three groups expressed concerns about bullying and adjustment to new environments during transition from primary to secondary school. Although wide variability was found within the autism, DS and WS groups, no significant differences were revealed in overall levels of parent-reported anxiety before and after the transition for any of the groups. However, different factors, including maladaptive behaviour, social problems and peer problems predicted anxiety during pre- and post-school transition for the three groups. This first cross-syndrome comparison on the effect of transition from primary to secondary school on anxiety highlighted the importance of individual variability when examining the transition outcomes of children with NDDs. Additionally, it identified clusters of overlap in terms of parent-report and child-report experiences of transition and unique predictors that need to be considered when planning transition support for autistic children, children with DS and WS. Future research should investigate the role of protective factors both at an individual and school level to inform the development of evidence-based intervention that support successful transition to secondary education.

The present study explored the effects of the pandemic on individuals with Down Syndrome (DS; n = 67) compared to other groups with Special Education Needs and Disabilities (SEND; n = 48) and their Typically Developing Siblings (TDS; n = 56). In total, 115 caregivers reported on their own anxiety and worries and of their children. Anxiety levels for individuals with DS appeared to be lower compared to other SEND populations and to TDS. In terms of worries, individuals with DS worried more about social-related worries but worried less about family-related aspects compared to the other groups. In sum, individuals with DS might show less anxiety but still worried more about specific aspects related to the impact of COVID-19 pandemic on their lives. Supplementary Information The online version contains supplementary material available at 10.1007/s10803-022-05450-0.

Portraying the voices of children with complex genetic neurodevelopmental disorders about their health, care and education needs in their statutory documents is a challenging task. This study examined the ways by which the perspectives of children diagnosed with Down Syndrome (DS) and Williams Syndrome (WS) are portrayed in their statutory documents, namely the Education Health and Care plans, in England. Using the International Classification of Functioning Disability and Health for Children and Youth, we analysed the content of Section A of the Education Health and Care plans of 52 children and young people with WS and DS, between 5 and 26 years of age. A minority of statutory documents (7.7 %) explicitly reported the children's voices, and many failed to document how the children's voices were accessed. Only a few specific or evidence-based tools to access their voices were reported. Most statutory documents portrayed parental rather than children's voices concerning aspects of their health, care, and education. This study highlights the need to establish the use of evidence-based tools for ascertaining the voices of children with complex neurodevelopmental disorders and including them in decision making about their health, care and education needs.

It has been reported that approximate number sense (ANS) task performance is impaired in individuals with Williams syndrome (WS) and Down syndrome (DS). Research with infants has suggested this impairment is caused by sticky fixation in WS and sustained attention deficits for those with DS. This study examined looking patterns of older children and adults with WS (n = 24) and DS (n = 23) during an ANS task compared to typically developing controls matched for chronological age and those matched for mental age. Results showed that, although there were no group differences, looking patterns changed with chronological age for both the WS and DS groups. Looking behaviour related to ANS performance only in the WS group. Implications for interventions are discussed.

Williams Syndrome (WS) is a rare neurodevelopmental disorder associated with a specific cognitive profile of strengths and impairments. It has been argued that studying cognitive development of this disorder would not only allow improved knowledge of WS but also provide insight into alternative pathways in development. However, due to the rarity and nature of the disorder, there are a number of challenges to collect longitudinal data. This letter describes a new multi-lab based approach to examine development in WS longitudinally and discusses some of the challenges and solutions that need to be taken into account when putting together either previously obtained or newly collected data from different labs.

COVID-19 has affected people across the world. However, it has been suggested that individuals with Special Education Needs and Disabilities (SEND) and their families might have been particularly impacted by the first national lockdown in the UK. In contrast to previous studies, the current study examined wellbeing and anxiety at different time points and included a control group matched for family situation. Parents of 402 individuals with SEND reported on their own anxiety and wellbeing as well as that of their son/daughter at three time points (before COVID-19, when COVID-19 pandemic started, and during the national lockdown). In addition, data from 186 typically developing (TD) siblings was obtained. Repeated measures ANOVAs and regression analyses showed that, although both individuals with SEND and their TD siblings showed increased anxiety across the three time points, levels of anxiety were not predicted by age, gender or health. Instead, levels of anxiety in the SEND group, but not the TD siblings, were predicted by awareness about COVID-19, diagnosis of an existing anxiety disorder as well as parental anxiety. In addition, whilst TD individuals were reported to increasingly worry about social related issues as well as family related issues, those with SEND were reported to worry about issues related to school closures. These findings show that COVID-19 impacts the wellbeing of those with SEND differently to that of their TD siblings and that school closures have a particular effect on this group. Further implications for policy impact and interventions are discussed.

There has been an increase in cognitive assessment via the Internet, especially since the coronavirus disease 2019 surged the need for remote psychological assessment. This is the first study to investigate the appropriability of conducting cognitive assessments online with children with a neurodevelopmental condition and intellectual disability, namely, Williams syndrome. This study compared Raven’s Colored Progressive Matrices (RCPM) and British Picture Vocabulary Scale (BPVS) scores from two different groups of children with WS age 10–11 years who were assessed online (n = 14) or face-to-face (RCPM n = 12; BPVS n = 24). Bayesian t-tests showed that children’s RCPM scores were similar across testing conditions, but suggested BPVS scores were higher for participants assessed online. The differences between task protocols are discussed in line with these findings, as well as the implications for neurodevelopmental research.

Manipulative artefacts are considered useful scaffolds of arithmetic during early years education, but their use is considered less important as children get older. Yet adult arithmetic performance often recruits artefacts to improve accuracy and efficiency, and so the same benefits should accrue to children beyond early years. We propose that interacting with manipulatives supports working memory by extending the mental workspace. This is a hitherto poorly explored aspect of the pedagogical benefits of manipulatives. Forty-three children aged between 7 and 9 years old were invited to assess whether interacting with artefacts supported arithmetic when under a working memory load. Interactivity and load were manipulated in a 2 × 2 repeated measures design. Children calculated the total of seven digits either with hands down—low interactivity—or moving numbered tokens—high interactivity. Additionally, in half the conditions, the children performed a second task designed to tax working memory. The children were also assessed across a battery of measures examining individual differences. As expected, performance was considerably worse under load. More sums were answered correctly in high-interactivity conditions regardless of working memory load. In the high-interactivity condition with a load, there were significant correlations between performance and both numeracy and fluid intelligence suggesting that even for more numerate children manipulatives confer benefits. The expression of arithmetic skills depends on the nature of the external resources offered by the environment. This dynamic agent–environment transaction highlights the pedagogical importance of external resources when assessing children’s arithmetic abilities.

Vision problems can lead to negative developmental outcomes. Children with Williams syndrome and Down syndrome are at higher risk of vision problems, and these are less likely to be detected due to diagnostic overshadowing and difficulty accessing eye-care. Education, Health and Care (EHC) plans are statutory documents, introduced by the Children and Families Act 2014 in England, with the intention of integrating provision across these domains. Vision issues should be reported in these plans, and recommendations made about appropriate adjustments for them. We analysed the EHC plans from 53 children with Down or Williams syndrome. Our results showed significant underreporting, especially for children with Williams syndrome, and little explanation of what adjustments should be made. We also report pockets of good practice.\ud \ud

Difficulties in processing humor have been associated with individuals with autism. The current study investigated whether humor comprehension and appreciation could be augmented in children with autism by providing contextual support suggesting that humor was to be expected. A verbally presented riddle task was used in which participants were assessed for their subjective ratings and comprehension of the materials. They were also filmed to record any smiling or laughing. Both riddles and control stimuli were presented with supporting verbal context and also without it. The results showed that (a) the greater subjective appreciation of riddles than of control stimuli was dependent on the provision of context for the participants with autism and that (b) context statistically equated these ratings of riddles between participants with autism and matched typically developing controls. However, context had no effect on comprehension or affective response. The results of the current study demonstrate that children with autism are, even in the most conservative interpretation, able to use verbal context to recognize verbal humor. This lays the foundation of possible interventions based on training sensitivity to context.

This study examined the extent to which a first language (L1) influences a second language (L2). We explored this potential influence by evaluating participant responses for semantic and syntactic word strings composed from the 1K British National Corpora word list. We investigated two different first language groups, and assessed their responses to semantic and syntactic judgement tasks in their L2 (English), alongside an English control group. The participants were L1 Japanese (n = 23), L1 Italic Indo-European (n = 25), and L1 English (n = 25). Each subject was asked to judge 60 semantic word strings, and 60 syntactic word strings. We then compared each of the three groups for accuracy and response time, with the L1 English group acting as the control. Results indicated significant differences which we suggest might relate to L1 background influences. The L1 Japanese participants responded less accurately to semantic judgements compared to L1 Italic Indo-European counterparts, while there was no significant difference between the two L2 learner groups for syntactic accuracy. The L1 Japanese participants were also slower than their L1 Indo-European counterparts for both semantic and syntactic word strings. We discuss our findings in relation to recent bilingual models of lexical development.

Humor appreciation and understanding is important for children’s social relationships. The current study examined the associations between riddle comprehension, riddle appreciation, and smiling/laughter in children from a wide age-range aged 4 to 11 years old, as well as how cognitive processing style relates to riddle comprehension. Style was distinguished between local and global language processing at the sentence level. The results showed that only children above the age of 8 years old showed a reliable relationship between humor comprehension and smiling/laughter. These findings show that laughter should not be taken as an automatic indicator of explicit understanding. In addition, higher vocabulary ability was independently associated with better humor comprehension. This demonstrates a separable role language proficiency in humor comprehension and suggests avenues for future research in atypical populations known to have difficulties in this area.

Sotos syndrome is a congenital overgrowth syndrome associated with intellectual disability. This study investigated communicative abilities of children with Sotos syndrome (n = 31), using the Children’s Communication Checklist, second edition. A cross-syndrome approach was used to establish the specificity of these abilities. Children with Williams syndrome (n = 34) were used as a comparison group. In both groups, the majority of participants had communicative impairment. Children with Sotos syndrome had an uneven pragmatic language profile and greater impairment with social relations, compared with restricted interests. Overall, children with Sotos syndrome had difficulties with both language structure and pragmatic language and a specific profile of relative difficulty with using nonverbal communication, using context-appropriate language and understanding peer relationships.\ud \ud

Background and aims Mathematical difficulties in individuals with Williams Syndrome (WS) and in individuals with Down Syndrome (DS) are well-established. Perceptual subitizing and conceptual subitizing are domain-specific precursors of mathematical achievement in typically developing (TD) population. This study employed, for the first time, eye-tracking methodology to investigate subitizing abilities in WS and DS. Methods and procedures Twenty-five participants with WS and 24 participants with DS were compared to a younger group of TD children (n = 25) matched for mental age. Participants were asked to enumerate one to six dots arranged either in a dice or a random pattern. Outcomes and results Accuracy rates and analyses of reaction time showed no significant differences between the clinical groups (WS and DS) and the control group, suggesting that all participants used the same processes to perform the enumeration task in the different experimental conditions. Analyses of the eye movements showed that both individuals with WS and individuals with DS were using inefficient scanning strategies when counting. Moreover, analyses of the eye movements showed significantly shorter fixation duration in participants with DS compared to the control group in all the experimental conditions. Conclusions and implications The current study provides evidence that individuals with WS and individuals with DS perform both perceptual subitizing and conceptual subitizing. Moreover, our results suggest a fixation instability in DS group that does not affect their performance when subitizing but might explain their low accuracy rates when counting. Findings are discussed in relation to previous studies and the impact for intervention programmes to improve counting and symbolic mathematical abilities in these populations.

Background The current systematic review is the first to systematically explore and synthesis research to date on mathematical abilities in Williams syndrome (WS), a rare genetic disorder that results in an uneven cognitive profile. As mathematical development is complex and relies on both domain-specific and domain-general abilities, it is currently not clear what mathematical abilities have been examined in WS and also what the current gaps in this research area are. Methods and procedures A total of 27 studies across 22 publications were identified through a systematic review search process. Results Overall, all mathematical abilities, except for simple counting and subitizing abilities, were reported to be impaired but in line with overall mental-age abilities. However, the literature to date has not established the underlying causes of these mathematical difficulties in WS. Some studies suggested that mathematical abilities in WS follow an atypical developmental pathway with a greater reliance on verbal abilities than in typical development but coupled with impaired understanding of counting and knowledge of the number system more broadly. However, most included studies used different assessments of mathematical skills and there is a lack of studies that have examined more than one particular aspect of mathematical development within the same study. In addition, studies have often included large age ranges and small participant samples, despite the known large individual variability in WS. Conclusion Although we know mathematical abilities in WS are impaired, this area is under-researched and there is a lack of longitudinal studies that provide insight into the cognitive mechanisms that underpin mathematical development in WS. Therefore, there is a lack of an evidence-base to inform interventions or educational practice.

BACKGROUND: Studies in Down syndrome (DS) and Williams syndrome (WS) have suggested that mathematical abilities are impaired. However, it is unclear which domain-general or domain-specific abilities impact on mathematical development in these developmental disorders. METHOD: The current study examined the foundations of mathematical development across participants with WS (n = 24) and DS (n = 26) compared to typically developing (TD) children (n = 26) in relation to domain-general (i.e., general intelligence and visuospatial abilities) and domain-specific abilities (non-symbolic and symbolic number abilities). RESULTS: Developmental trajectories showed that mathematical abilities were delayed in line with overall mental age in DS and WS. Whilst visuospatial abilities predicted performance for DS and TD participants, this was not the case for the WS group, instead Approximate Number Sense abilities predicted mathematical development. CONCLUSIONS: These findings suggest that those with DS and WS may benefit from different mathematical intervention programmes.

Background The current study examined parents’ views about their child’s educational provision for children with Williams syndrome (WS), Down syndrome (DS), and Autism Spectrum Disorders (ASD). Aims This cross-syndrome comparison explored the specific and general difficulties that parents of children with neurodevelopmental disorders experience about their child’s educational provision. Methods and procedures Parents of children aged 4–18;11 years old, including 99 with WS, 88 with DS, and 82 with ASD completed a survey. Outcomes and results Children with DS were more likely to access mainstream settings and 1-to-1 support compared to those with WS and ASD. Parental satisfaction was lowest for those with ASD but all parents mentioned concerns about professionals’ knowledge of how to support children with special educational needs and disabilities (SEND). There were also group differences for access to specialist support but overall access to occupational therapy and mental health was low. Conclusions and implications In contrast to previous studies, educational provision and satisfaction with educational provision are syndrome-specific. These results also highlight the need for training and raising awareness about the specific needs of children with neurodevelopmental disorders. In addition, our findings suggest improved communication between parents and the school is required about the type of support children with SEND are receiving.

Williams syndrome (WS) is a rare neurodevelopmental disorder associated with physical health problems, limitations in cognitive abilities and increased risk of mental health difficulties. This profile of complex needs may make it challenging to support children with WS in schools. Surprisingly, in the current international move for inclusion, limited research exists on the educational provision and academic achievements of children with WS, including the non-existing literature on their voices and the perspectives of key stakeholders. This letter calls for additional research on the risk and protective factors associated with the educational outcomes of these children, the perspectives of the children themselves and the development of the evidence-base about the effectiveness of education intervention programs.