Dr Lauren Jenner

Background Relatively little is known about social cognition in people with intellectual disability (ID), and how this may support understanding of co-occurring autism. A limitation of previous research is that traditional social-cognitive tasks place a demand on domain-general cognition and language abilities. These tasks are not suitable for people with ID and lack the sensitivity to detect subtle social-cognitive processes. In autism research, eye-tracking technology has ofered an efective method of evaluating social cognition—indicating associations between visual social attention and autism characteristics. The present systematic review synthesised research which has used eye-tracking technology to study social cognition in ID. A meta-analysis was used to explore whether visual attention on socially salient regions (SSRs) of stimuli during these tasks correlated with degree of autism characteristics presented on clinical assessment tools. Method Searches were conducted using four databases, research mailing lists, and citation tracking. Following indepth screening and exclusion of studies with low methodological quality, 49 articles were included in the review. A correlational meta-analysis was run on Pearson’s r values obtained from twelve studies, reporting the relationship between visual attention on SSRs and autism characteristics. Results and conclusions Eye-tracking technology was used to measure diferent social-cognitive abilities across a range of syndromic and non-syndromic ID groups. Restricted scan paths and eye-region avoidance appeared to impact people’s ability to make explicit inferences about mental states and social cues. Readiness to attend to social stimuli also varied depending on social content and degree of familiarity. A meta-analysis using a random efects model revealed a signifcant negative correlation (r = −.28, [95% CI −.47, −.08]) between visual attention on SSRs and autism characteristics across ID groups. Together, these fndings highlight how eye-tracking can be used as an accessible tool to measure more subtle social-cognitive processes, which appear to refect variability in observable behaviour. Further research is needed to be able to explore additional covariates (e.g. ID severity, ADHD, anxiety) which may be related to visual attention on SSRs, to diferent degrees within syndromic and non-syndromic ID groups, in order to determine the specifcity of the association with autism characteristics. Keywords Eye-tracking, Social cognition, Intellectual disability, Genetic syndromes, Autism

People with intellectual disability are underrepresented and often actively excluded from autism research. A better understanding of autism requires inclusive research approaches that accurately represent the broad heterogeneity of the autistic population.

Difficulties in communication and social functioning are key diagnostic components of intellectual disability (ID), and negatively impact the wellbeing of those with ID. Communication and social difficulties are multifaceted and comprise a wide breadth of skills. Current approaches in research and intervention often do not include refined assessment of communicative and social functioning in those with ID. Yet, in-depth behavioral phenotyping in neurogenetic syndromes associated with ID indicates that aspects of communication and social functioning are dissociable and interact with one another culminating in a diverse range of unique behavioral profiles between and within these groups. Detailed assessment of such profiles contributes to refined understanding of the developmental mechanisms underlying distinct aspects of communication and social functioning, and thus the advancement of targeted and evidence-based intervention. We discuss insights gained from refined assessment in five neurogenetic syndromes with distinct profiles of social and communication skills: Angelman syndrome, Cornelia de Lange syndrome, Down syndrome, fragile X syndrome and tuberous sclerosis complex. Specifically, we compare findings and the improved knowledge derived from detailed assessments relative to broader measures that mask nuanced strengths and difficulties. We then consider how refined but practical assessment approaches may be applied in research and intervention in broader groups of people with ID with heterogeneous causes.

Previously, we have proposed that there are nine domains that warrant assessment when intervening to decrease challenging behavior and\or increase well-being in people with profound or severe intellectual disability and complex needs. These domains are: pain and discomfort, sensory sensitivity, anxiety and low mood, sleep, emotional dysregulation, cognitive difference, learned or functional behaviors, and expressive communication. In this article we: (1) identify specific challenging behaviors that might be influenced by these domains, (2) describe the relationship between these domains and the specified challenging behaviors, (3) identify assessments for each domain and (4) describe interactions between the domains. Our aim in this article is to provide practitioners with a framework for assessment and to stimulate debate about the domains that are demonstrably important when considering challenging behavior and well-being in people with profound or severe intellectual disability and complex needs.

Purpose of Review Elevated prevalence of autism characteristics is reported in genetic syndromes associated with intellectual disability. This review summarises recent evidence on the behavioural heterogeneity of autism in the following syndromes: Fragile X, Cornelia de Lange, Williams, Prader-Willi, Angelman, Down, Smith-Magenis, and tuberous sclerosis complex. Key considerations for assessment and support are discussed. Recent Findings The profile and developmental trajectory of autism-related behaviour in these syndromes indicate some degree of syndrome specificity which may interact with broader behavioural phenotypes (e.g. hypersociability), intellectual disability, and mental health (e.g. anxiety). Genetic subtype and co-occurring epilepsy within syndromes contribute to increased significance of autism characteristics. Autism-related strengths and challenges are likely to be overlooked or misunderstood using existing screening/diagnostic tools and criteria, which lack sensitivity and specificity within these populations. Summary Autism characteristics are highly heterogeneous across genetic syndromes and often distinguishable from non-syndromic autism. Autism diagnostic assessment practices in this population should be tailored to specific syndromes. Service provisions must begin to prioritise needs-led support.