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Background: Children with intellectual disability are at greater risk of developing anxiety than the general population. Limited research has examined the challenges associated with recognising and responding to anxiety in children with intellectual disability, and its perceived impact. Aim: This study aimed to explore anxiety in children with intellectual disability, from the child and parent perspective to better understand how parents and children recognise and respond to anxiety. Method and Procedures: Six children with an intellectual disability (age range: 12-17, four boys) and their mothers participated in a semi-structured interview online. Interviews were transcribed verbatim and interpreted using thematic analysis. Outcome and Results: Mothers elaborated on the difficulties with recognising signs of anxiety due to the impact of the child's primary diagnosis and the overlap of symptoms with co-occurring conditions. Mothers and children discussed the 'contagious' effect of anxiety within the household and how this influenced mothers' approaches to managing their child's anxiety. They reported that anxiety limited the meaningful activities in which children and families could engage. Conclusions and Implications: These findings highlight the importance of supporting mothers to recognise their children's anxiety and to assist them with strategies on how best to respond and cope. These findings have implications for future research, and practitioners working in this field.

Research has linked prenatal alcohol and tobacco use with Attention Deficit Hyperactivity Disorder (ADHD), and variably with Autism Spectrum Disorder (ASD). Lactational use has been scantly considered. This study examined whether it may alter ADHD or ASD risk. Participants were 5107 infants recruited in 2004 and assessed longitudinally for the Growing Up in Australia Study. Logistic regression did not find any associations between maternal alcohol and tobacco use while breastfeeding and ADHD or ASD diagnosis at ages 6-7 or 10-11 years. Alcohol and tobacco use during lactation may not increase ADHD or ASD risk. Abstaining from alcohol and tobacco, however, may still be the safest option. Analyses were limited by lack of alcohol timing and retrospective variables that future research should address.

Research has linked prenatal alcohol and tobacco use with Attention Deficit Hyperactivity Disorder (ADHD), and variably with Autism Spectrum Disorder (ASD). Lactational use has been scantly considered. This study examined whether it may alter ADHD or ASD risk. Participants were 5107 infants recruited in 2004 and assessed longitudinally for the Growing Up in Australia Study. Logistic regression did not find any associations between maternal alcohol and tobacco use while breastfeeding and ADHD or ASD diagnosis at ages 6-7 or 10-11 years. Alcohol and tobacco use during lactation may not increase ADHD or ASD risk. Abstaining from alcohol and tobacco, however, may still be the safest option. Analyses were limited by lack of alcohol timing and retrospective variables that future research should address., Author(s): Louisa Gibson [sup.1] , Melanie Porter [sup.1] Author Affiliations: (1) grid.1004.5, 0000 0001 2158 5405, Department of Psychology, Faculty of Medicine, Health and Human Sciences, Macquarie University, , Balaclava [...]

While there is increasing use of standardised tools to assist in determining decision-making capacity in adults, there is limited literature evaluating these tools in children/adolescents. The current PRISMA guided systematic review aimed to evaluate standardised capacity assessment tools used in the child/adolescent population, and to examine the relationship between these capacity assessment tools and cognition. Inclusion criteria comprised: (1) validation of a new/existing tool to assess capacity or competence; (2) participants < 18 years; (3) involved validation on normative or non-normative populations; (4) published in English in a peer review journal. Ten studies were included. The majority applied a version of the Macarthur Competence Assessment Tool. Findings suggest capacity assessment tools demonstrate sound psychometric properties, but research into capacity assessment in children is still in its infancy. Limitations include the lack of diagnostic gold standard of capacity and the inconsistent and minimal incorporation of cognitive functioning into standardised capacity assessment. [ABSTRACT FROM AUTHOR]

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms 'Williams syndrome' or "Williams-Beuren syndrome" combined with "adaptive function*", "adaptive behavio*", "independ*" and "autonomy". Selection criteria included English language articles, theses and book chapters, participants with a diagnosis of Williams syndrome and inclusion of a standardised assessment of adaptive functioning. Twenty-two published articles and two PhD. dissertations (one subsequently published as a poster presentation) met the selection criteria for inclusion in the review. Fifteen investigated adaptive functioning in children and adolescents aged up to 19 years of age, five investigated adaptive functioning in adults and four included participants across a wider age range and included both children and adults. Along with identifying methodological issues, the review addressed the following areas: overall level of adaptive functioning in WS, domain strengths and weaknesses, evidence of heterogeneity, relationship to intellectual ability, changes with chronological age, relationship with maladaptive behaviour, gender differences and the potential influence of environmental factors which may be related to adaptive functioning in WS.

Children with neurofibromatosis type 1 (NF1) are at considerable risk for cognitive difficulties, including visuospatial deficits and executive dysfunction. This study aimed to (1) assess the overall performance of children with NF1 on the Rey-Osterrieth Complex Figure Test (RCFT) compared to unaffected siblings and (2) examine neuropsychological predictors of RCFT performance in children with NF1. A retrospective clinical audit was performed on neuropsychological records from a multidisciplinary NF1 Clinic in Australia. We searched for children that had completed an assessment between 2000 and 2015 which included the RCFT and other neuropsychological outcomes in this study. These included the Wechsler Intelligence Scale for Children, Judgment of Line Orientation (JLO), Tower of London test, Conners ADHD Scales, and the Behavioral Rating Inventory of Executive Function (BRIEF). The study population consisted of 191 children with NF1 aged 6–16 years, and 55 unaffected siblings recruited from a separate study. Results revealed that 62% of children with NF1 performed at or below the first percentile on the RCFT copy, which was significantly worse than their unaffected siblings. Visuospatial skills, parent-rated executive abilities, ADHD symptoms, and intellectual skills all predicted poorer performance on the RCFT copy, however the best fitting multiple regression model only contained the JLO, BRIEF Metacognition Index, and chronological age. The JLO emerged as the strongest predictor of RCFT performance. This study provides evidence that visuospatial deficits are a key driver of reduced RCFT performance in NF1 and that executive skills as well as a younger age are also independent predictors of RCFT performance. [ABSTRACT FROM AUTHOR]

Evidence is emerging that individuals with Fragile X syndrome (FXS) display emotion recognition deficits, which may contribute to their significant social difficulties. The current study investigated the emotion recognition abilities, and social approachability judgments, of FXS individuals when processing emotional stimuli. Relative to chronological age- (CA-) and mental age- (MA-) matched controls, the FXS group performed significantly more poorly on the emotion recognition tasks, and displayed a bias towards detecting negative emotions. Moreover, after controlling for emotion recognition deficits, the FXS group displayed significantly reduced ratings of social approachability. These findings suggest that a social anxiety pattern, rather than poor socioemotional processing, may best explain the social avoidance observed in FXS.

Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetic disorders which present with similar cognitive-behavioral problems, but distinct social phenotypes. Despite these social differences both syndromes display poor social relations which may result from abnormal social processing. This study aimed to manipulate the location of socially salient information within scenes to investigate the visual attentional mechanisms of: capture, disengagement, and/or general engagement. Findings revealed that individuals with FXS avoid social information presented centrally, at least initially. The WS findings, on the other hand, provided some evidence that difficulties with attentional disengagement, rather than attentional capture, may play a role in the WS social phenotype. These findings are discussed in relation to the distinct social phenotypes of these two disorders.

Previous studies suggest that individuals with Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, show specific weaknesses in visual attention and response inhibition within the visuospatial domain. Here we examine the extent to which impairments in attentional control extend to the visuomotor domain using a well-validated measure of choice stepping reaction time (CSRT) in individuals with WS. We examined the interaction between executive control and visually guided stepping using a verbal fluency dual-task or Go/NoGo paradigm during CSRT performance. Relationships between dual-task and inhibitory stepping and behavioural inattention and hyperactivity were also examined. Our results showed clear dual-task costs in stepping response times when performing a concurrent cognitive task in the WS group when compared to spatial and verbal ability matched typically developing controls. Although no group differences in stepping accuracy were observed between the WS and typically developing control groups, the WS group showed progressive slowing and more variable response times across the duration of the Go/NoGo task. These results suggest dysfunction in circuits involved in top-down attentional control processes in WS. These findings provide novel evidence that core executive control deficits in WS extend to the visuomotor domain, and impact on ADHD-related inattentive symptoms. (Contains 6 figures and 4 tables.)

People with Williams syndrome (WS) are said to have sociable and extremely trusting personalities, approaching strangers without hesitation. This study investigated whether people with WS are less likely than controls to attribute negative intent to others when interpreting a series of ambiguous pictures. This may, at least partially, explain their hypersociability toward strangers. Twenty-seven individuals with WS and 54 typically developing controls (27 matched to WS participants on sex and chronological age and 27 matched on sex and mental age) viewed 10 ambiguous pictures, where one person in the picture may be seen as having a negative objective. Participants were asked to describe what was happening in the picture. Responses were scored for negative intention attribution (NIA). NIA was reduced in WS individuals relative to typically developing controls of the same chronological age, but was similar to typically developing controls of the same mental age. Findings are discussed in relation to possible underlying neurological and cognitive mechanisms and practical implications for understanding and teaching stranger danger to people with WS. (Contains 3 figures and 1 table.)

This study investigated emotion recognition abilities and visual scanning of emotional faces in 16 Fragile X syndrome (FXS) individuals compared to 16 chronological-age and 16 mental-age matched controls. The relationships between emotion recognition, visual scan-paths and symptoms of social anxiety, schizotypy and autism were also explored. Results indicated that, compared to both control groups, the FXS group displayed specific emotion recognition deficits for angry and neutral (but not happy or fearful) facial expressions. Despite these evident emotion recognition deficits, the visual scanning of emotional faces was found to be at developmentally appropriate levels in the FXS group. Significant relationships were also observed between visual scan-paths, emotion recognition performance and symptomology in the FXS group.

Background: Although people with Williams syndrome (WS) are often characterized as friendly and sociable with relatively good general language abilities, there is emerging evidence of pragmatic difficulties and trouble comprehending aspects of non-literal language. Aims: The main aim was to investigate the comprehension of sarcasm, metaphor and simile in WS relative to typically developing controls. A secondary aim was to examine the association between non-literal language comprehension and a range of other cognitive abilities, both in WS and in the typically developing population. Methods & Procedures: Twenty-six participants with WS were compared with 26 typically developing chronological age-matched controls (TDCA) and 26 typically developing mental age-matched controls (TDMA). Participants listened to stories in which characters made non-literal comments. They were then asked what each character meant by their comment. In order to investigate the second aim of the study, cognitive abilities were also assessed using the Woodcock-Johnson (Revised) Tests of Cognitive Ability, including expressive vocabulary, verbal working memory, perceptual integration, inferential reasoning and overall cognitive ability. Outcomes & Results: Comprehension of non-literal language in WS was significantly below TDCA levels, but was not significantly different to TDMA levels. For typically developing controls, each of the cognitive measures was strongly correlated with each of the measures of non-literal language comprehension. The same relationships were not always found for participants with WS. In particular, sarcasm comprehension in WS was not significantly correlated with any of the assessed cognitive abilities, and expressive vocabulary was not significantly correlated with any measure of non-literal comprehension. Conclusions & Implications: Comprehension of simile in WS is below TDCA levels but seems on par with their mental age level. It appears that comprehension of sarcasm and metaphors is above the cognitive capabilities and mental age level of most individuals with WS. Further, the pattern of correlations between non-literal comprehension and cognitive abilities in WS relative to controls suggests that perhaps the linguistic and cognitive systems that underpin non-literal language comprehension in typically developing individuals interact and integrate in different ways in WS.

The focus of this paper is on the assessment of the two main processes that children must acquire at the single word reading level: word recognition (lexical) and decoding (nonlexical) skills. Guided by the framework of the dual route model, this study aimed to (1) investigate the impact of item characteristics on test performance, and (2) determine to what extent widely used reading measures vary in their detection of lexical and nonlexical reading difficulties. Thirty children with reading difficulties were administered selected reading subtests from the Woodcock-Johnson III, the Wechsler Individual Achievement Test--Second Edition, the Castles and Coltheart Reading Test 2 (CC2), as well as a measure of nonverbal IQ. Both within-subjects analyses and descriptive data are presented. Results suggest that in comparison to a pure measure of irregular word reading, children with reading difficulties perform better on word identification subtests containing both regular and irregular word items. Furthermore, certain characteristics (e.g., length, similarity to real words) appear to influence the level of difficulty of nonword items and tests. The CC2 subscales identified the largest proportions of children with reading difficulties. Differences between all test scores were of statistical and clinical significance. Clinical and theoretical implications are discussed.

Williams syndrome (WS) is associated with an unusual profile of anxiety, characterised by increased rates of non-social anxiety but not social anxiety (Dodd and Porter, J Ment Health Res Intellect Disabil 2(2):89-109, "2009"). The present research examines whether this profile of anxiety is associated with an interpretation bias for ambiguous physical, but not social, situations. Sixteen participants with WS, aged 13-34 years, and two groups of typically developing controls matched to the WS group on chronological age (CA) and mental age (MA), participated. Consistent with the profile of anxiety reported in WS, the WS group were significantly more likely to interpret an ambiguous physical situation as threatening than both control groups. However, no between-group differences were found on the ambiguous social situations.

Individuals with Williams syndrome (WS) exhibit striking social behaviour that may be indicative of abnormally low social anxiety. The present research aimed to determine whether social anxiety is unusually low in WS and to replicate previous findings of increased generalised anxiety in WS using both parent and self report. Fifteen individuals with WS aged 12-28 years completed the Spence Children's Anxiety Scale (SCAS) and the Children's Automatic Thoughts Scale (CATS). Their responses were compared to clinically anxious and community comparison groups matched on mental age. The findings suggest that WS is not associated with unusually low social anxiety but that generalised anxiety symptoms and physical threat thoughts are increased in WS, relative to typically developing children.

This research aimed to comprehensively explore psychopathology in Williams syndrome (WS) across the life span and evaluate the relationship between psychopathology and age category (child or adult), gender, and cognitive ability. The parents of 50 participants with WS, ages 6-50 years, were interviewed using the Schedule for Affective Disorders and Schizophrenia for School-Age Children. The prevalence of a wide range of Axis I "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV; American Psychiatric Association, 1994) disorders was assessed. In addition to high rates of anxiety and attention-deficit/hyperactivity disorder (38% and 20%, respectively), 14% of our sample met criteria for a Depressive Disorder and 42% of participants were not experiencing any significant psychopathological difficulties. There was some evidence for different patterns of psychopathology between children and adults with WS and between males and females. These relationships were largely in keeping with those found in the typically developing population, thus supporting the validity of applying theory and treatment approaches for psychopathology in the typically developing population to WS. (Contains 3 tables.)

This study examined Theory of Mind in Williams syndrome (WS) and in normal chronological age-matched and mental age-matched control groups, using a picture sequencing task. This task assesses understanding of pretence, intention and false belief, while controlling for social-script knowledge and physical cause-and-effect reasoning. The task was selected because it is entirely non-verbal, so that the WS individuals could not rely on their good verbal skills when performing the task. Results indicated a specific deficit in understanding of false belief within the WS group. There was also evidence of heterogeneity in the WS group, with the false belief impairment restricted to only a particular subgroup of WS individuals identified originally by Porter, M., & Coltheart, M. (2005). Cognitive heterogeneity in Williams syndrome. "Developmental Neuropsychology," 27(2), 275-306.

Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetic disorders which present with similar cognitive-behavioral problems, but distinct social phenotypes. Despite these social differences both syndromes display poor social relations which may result from abnormal social processing. This study aimed to manipulate the location of socially salient information within scenes to investigate the visual attentional mechanisms of: capture, disengagement, and/or general engagement. Findings revealed that individuals with FXS avoid social information presented centrally, at least initially. The WS findings, on the other hand, provided some evidence that difficulties with attentional disengagement, rather than attentional capture, may play a role in the WS social phenotype. These findings are discussed in relation to the distinct social phenotypes of these two disorders. Keywords Fragile X syndrome * FXS * Williams syndrome * WS * Social processing * Attentional disengagement * Attentional capture * Eye-tracking, Introduction Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetically-defined neurodevelopmental disorders associated with cognitive and intellectual disability. Both syndromes present with cognitive profiles that are characterized by [...]

This study investigated emotion recognition abilities and visual scanning of emotional faces in 16 Fragile X syndrome (FXS) individuals compared to 16 chronological-age and 16 mental-age matched controls. The relationships between emotion recognition, visual scan-paths and symptoms of social anxiety, schizotypy and autism were also explored. Results indicated that, compared to both control groups, the FXS group displayed specific emotion recognition deficits for angry and neutral (but not happy or fearful) facial expressions. Despite these evident emotion recognition deficits, the visual scanning of emotional faces was found to be at developmentally appropriate levels in the FXS group. Significant relationships were also observed between visual scan-paths, emotion recognition performance and symptomology in the FXS group. Keywords Fragile X syndrome , Developmental disorders , Emotion recognition , Eye-tracking , Scan-paths, Introduction Individuals with Fragile X syndrome (FXS) display a wide range of social difficulties in everyday life such as symptoms of social anxiety, autistic asociality and idiosyncratic traits of schizotypal [...]