Item and error analysis on Raven's Coloured Progressive Matrices in Williams Syndrome

Raven's Coloured Progressive Matrices (RCPM) is a standardised test that is commonly used to obtain a non-verbal reasoning score for children. As the RCPM involves the matching of a target to a pattern it is also considered to be a visuo-spatial perception task. RCPM is therefore frequently used in studies in Williams Syndrome (WS), in order to match WS participants to a control group or as a single measure to predict performance on a test-condition in developmental trajectory analyses. However, little is known about the performance of participants with WS on the RCPM. The current study compared the type of errors and the difficulty of each item for 53 participants with WS to 53 typically developing children who were individually matched on the total raw score for RCPM. Results showed that the participants with WS made the same proportion of error types and that the proportion of error types changed similarly to those of typically developing controls over development. Furthermore, the differential item difficulty between the two groups was highly similar. It is therefore argued that, although participants with WS are delayed on RCPM, their performance is not atypical which suggests that RCPM performance is supported by typical mechanisms. The RCPM is therefore a useful tool to match WS to control groups or to construct developmental trajectories

Home literacy environment and literacy outcomes in individuals with Williams syndrome and Down syndrome

Background: The home literacy environment (HLE) has rarely been examined for individuals with neurodevelopmental disorders, including individuals with Williams syndrome and Down syndrome. Method: The current study surveyed carers of individuals with Down syndrome (n = 48) and Williams syndrome (n = 18) in the United Kingdom (UK). Results: The study reports that individuals with Down syndrome were rated higher in general reading skills and writing, while the Williams syndrome group scored higher for speaking. Yet, individuals with Down syndrome were more likely to engage in informal activities than instructional activities and the frequency of informal activities related to reading outcomes for those with Down syndrome but not Williams syndrome. Additionally, this study reports that age was not related to the HLE for both groups. Conclusion: This is the first study to report on the HLE of individuals with Williams syndrome and supports the key role of the HLE in the development of literacy skills for individuals with Williams syndrome and Down syndrome

Sensory Processing in Williams Syndrome: Individual differences and changes over time

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

40 facts about Williams Syndrome

To celebrate the 40th anniversary of the Williams Syndrome Foundation and to raise awareness of Williams syndrome, we have put together 40 research-evidenced facts about Williams syndrome. Williams Syndrome is a rare genetic condition that is caused by a deletion of genetic material on the long arm of chromosome 7. It occurs sporadically in about 1 in 18,000 people. This fact sheet provides information on the medical aspects, brain development, cognitive and behavioural profiles, education, support and intervention and career outcomes of people with Williams syndrome, all of which are based on scientific research. These facts were randomly chosen from topics that we discussed during our lab meetings. This fact sheet can be used as a resource to pass on to family members, teachers and professionals

Young children’s science learning from a touchscreen app

Many technological applications (apps) purport to help children learn academic material. Building on research in developmental and educational psychology, we developed and tested an app to teach biological and physical science content to preschool children. There were 21 children in the control condition (Mage = 50.30 months, SD = 8.61) and 21 children in the intervention condition (Mage = 53.21 months old, SD = 6.36). Children were given pre- assessments and post-assessments of their understanding of living things, inheritance, buoyancy, and balance. Half were assigned to play the app for 3 weeks or to a control condition that only completed the assessments. Based on ANCOVA and ANOVA models, children in the app condition increased in their understanding of living things and buoyancy on a near-transfer task from pre-test to post-test assessment, whereas the children in the control condition did not increase their understanding. The findings suggest that drill and tests apps focusing on science content that take account of folk theories of science can support children’s science learning

Perceptual subitizing and conceptual subitizing in Williams syndrome and Down syndrome: Insights from eye movements

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

The home learning environment of primary school children with down syndrome and those with williams syndrome

Background and aims: Research on typically developing (TD) populations has shown that the home learning environment plays a significant role in cognitive development and learning, but very little is known about the home learning environment of children with Down syndrome (DS) or children with Williams syndrome (WS). The present study examined and compared, for the first time, the home learning environment of children diagnosed with DS and children diagnosed with WS to investigate whether different cognitive profiles were reflected in their home literacy and number experiences. Methods and procedures: Quantitative and qualitative data were collected through a web-based survey from 58 parents and one foster parent of primary school children with DS (n = 35) and WS (n = 24) mostly based in the UK. The survey targeted the children’s general level of functioning and academic skills; type, format, and frequency of home learning activities; parents’ expectations for their child’s academic outcomes; parents’ attitudes towards literacy and mathematics; children’s interest towards mathematics; and the use of technology to support home learning activities. Outcomes and results: Our results showed that, overall, the home learning environment of children with DS and children with WS were similar but changed based on the child’s cognitive profile. Comparative analyses showed that parents of children with DS engaged more often in activities supporting counting than parents of children with WS, despite both groups reporting difficulties with this skill. Moreover, our results indicated that literacy-based activities occurred more often than mathematics-based activities and that the home numeracy environment was characterized by activities supporting different mathematical skills such as counting, arithmetic, and numeracy. Parents in both groups engaged with their child in both formal and informal literacy and mathematics-based activities, but informal activities occurred more often when supporting counting and number recognition skills. Conclusions and implications: The current study provides evidence that the home learning environment of children with DS and children with WS include different literacy-and mathematics-based activities and that the home learning environment changes on the basis of the child’s strengths and weaknesses. The findings are discussed in relation to previous studies and the impact on parental interventions

Twice-Exceptional Students of Mathematics in England: What Do the Teachers Know?

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 misdiagnosed or “missed” diagnosed in schools due to the teachers’ lack of knowledge. The study explored this issue using an electronic survey for primary school teachers in four local authorities in England. It was planned as a pilot study to gather insights from a small number of schools aiming to identify areas for further study and larger-scale research. When comparing responses from teachers with gifted-related training and those without, the study found some knowledge of specific types of 2e students among both groups of teachers, but no significant difference between them. This raised concerns about the effectiveness of the training, as well as identifying areas that need further and more systematic research

Studying development in Williams syndrome: Progress, prospects and challenges

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

Neuromyths About Neurodevelopmental Disorders: Misconceptions by Educators and the General Public

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