'Playing a guessing game': Recognising and responding to anxiety in children with intellectual disability.

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.

Cognitive interventions for children with acquired brain injury: A systematic review.

This systematic review identified empirically supported evidence of effective cognitive intervention for children with Acquired Brain Injury (ABI) and included clinical practice guidelines and recommendations for intervention of attention, memory and executive functioning. Databases included: PsycARTICLES; MEDLINE; PubMed; PsycINFO; PSYCHextra; Ovid; PsychBite; CINAHL and EMBASE. Abstracts and full text articles were reviewed by two independent authors. Articles reporting on a cognitive intervention for children aged 4-19 years with a primary diagnosis of ABI were included. 25 articles were identified by both reviewers (1 00% inter-rater agreement), with the last search conducted in June 201 9. Articles were assigned to one of four categories of primary intervention: (1) Attention and Memory; (2) Executive Functioning; (3) Attention, Memory, and Executive Functioning or (4) Multi-Model Comprehensive Combined Approaches. Articles were critically appraised and level of evidence was determined according to established quality methodology criteria. Of the 25 articles evaluated, nine articles were rated Class 1, eight Class 11, and nine Class 111. One practice standard and one practice option was provided. Key suggestions included using more homogeneous samples in terms of age and injury characteristics (e.g., nature and severity of ABI, age at ABI) and incorporating long-term monitoring of outcome. Interventionalists must consider the dynamic nature of brain and cognitive development and the changing environmental needs of children.

Extending the positive bias in Williams syndrome: The influence of biographical information on attention allocation.

There is evidence that individuals with Williams syndrome (WS) show an attention bias toward positive social-perceptual (happy) faces. Research has not yet considered whether this attention bias extends beyond social-perceptual stimuli to perceptually neutral stimuli that are paired with positive (trustworthy) biographical information. Fourteen participants with WS (mean age = 21 years, 1 month) learned to associate perceptually neutral faces with trustworthy (positive), neutral, or untrustworthy (negative) biographical information, before completing a dot-probe task where the same biographical faces were presented. The performance of the WS group was compared to two typically developing control groups, individually matched to the WS individuals on chronological age and mental age, respectively. No between-group bias toward untrustworthy characters was observed. The WS group displayed a selective attention bias toward trustworthy characters compared to both control groups (who did not show such a bias). Results support previous findings that indicate WS individuals show a preference for positive social-perceptual stimuli (happy faces) at the neurological, physiological, and attentional levels. The current findings extend this work to include a "top-down" positive bias. The implications of a positive bias that extends beyond social-perceptual stimuli (or "bottom-up" processes) in this syndrome are discussed.

Attention to faces in social context in children with neurofibromatosis type 1.

AIM: To examine visual attention to faces within social scenes in children with neurofibromatosis type 1 (NF1) and typically developing peers. METHOD: Using eye-tracking technology we investigated the time taken to fixate on a face and the percentage of time spent attending to faces relative to the rest of the screen within social scenes in 24 children with NF1 (17 females, seven males; mean age 10y 4mo [SD 1y 9mo]). Results were compared with those of 24 age-matched typically developing controls (11 females, 13 males; mean age 10y 3mo [SD 2y]). RESULTS: There was no significant between-group differences in time taken to initially fixate on a face (p=0.617); however, children with NF1 spent less time attending to faces within scenes than controls (p=0.048). Decreased attention to faces was associated with elevated autism traits in children with NF1. INTERPRETATION: Children with NF1 spend less time attending to faces than typically developing children when presented in social scenes. Our findings contribute to a growing body of literature suggesting that abnormal face processing is a key aspect of the social-cognitive phenotype of NF1 and appears to be related to autism spectrum disorder traits. Clinicians should consider the impact of reduced attention to faces when designing and implementing treatment programmes for social dysfunction in this population. WHAT THIS PAPER ADDS: Children with neurofibromatosis type 1 (NF1) demonstrated atypical gaze behaviour when attending to faces. NF1 gaze behaviour was characterized by normal initial fixation on faces but shorter face dwell time. Decreased attention to faces was associated with elevated autism traits in the sample with NF1.

Intellectual and academic outcomes after pediatric liver transplantation: Relationship with transplant-related factors.

As survival rates for pediatric liver transplant continue to increase, research attention is turning toward long-term functional consequences, with particular interest in whether medical and transplant-related factors are implicated in neurocognitive outcomes. The relative importance of different factors is unclear, due to a lack of methodological uniformity, inclusion of differing primary diagnoses, varying transplant policies, and organ availability in different jurisdictions. This cross-sectional, single-site study sought to address various methodological limitations in the literature and the paucity of studies conducted outside of North America and Western Europe by examining the intellectual and academic outcomes of Australian pediatric liver transplant recipients (N = 40). Participants displayed significantly poorer intellectual and mathematical abilities compared with the normative population. Greater time on the transplant waitlist was a significant predictor of poorer verbal intelligence, working memory, mathematical abilities, and reading but only when considering the subgroup of children with biliary atresia. These findings support reducing the time children wait for a transplant as a priority.

Atypical Local Interference Affects Global Processing in Children with Neurofibromatosis Type 1.

OBJECTIVES: To examine hierarchical visuospatial processing in children with neurofibromatosis type 1 (NF1), a single gene disorder associated with visuospatial impairments, attention deficits, and executive dysfunction. METHODS: We used a modified Navon paradigm consisting of a large "global" shape composed of smaller "local" shapes that were either congruent (same) or incongruent (different) to the global shape. Participants were instructed to name either the global or local shape within a block. Reaction times, interference ratios, and error rates of children with NF1 (n=30) and typically developing controls (n=24) were compared. RESULTS: Typically developing participants demonstrated the expected global processing bias evidenced by a vulnerability to global interference when naming local stimuli without a cost of congruence when naming global stimuli. NF1 participants, however, experienced significant interference from the unattended level when naming both local and global levels of the stimuli. CONCLUSIONS: Findings suggest that children with NF1 do not demonstrate the typical human bias of processing visual information from a global perspective. (JINS, 2017, 23, 446-450).

The neuropsychological profile of children with basal ganglia encephalitis: a case series.

Inflammatory basal ganglia encephalitis (BGE) is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Unlike most brain injuries, BGE is a radiologically pure basal ganglia syndrome. The current study systematically describes the neuropsychological outcomes of four paediatric cases of BGE, and thus the neuropsychological outcomes of focal basal ganglia insult in childhood. Although all patients made significant motor recoveries, all four cases displayed executive dysfunction, fine motor difficulties, and anxiety. Three out of four cases displayed attention deficits. The case who received intravenous immunoglobulin (IVIg) treatment and steroids during the acute phase of the disease had the best cognitive outcome. These findings highlight the need for detailed neuropsychological assessment and long-term follow-up.

The neuropsychological function of children with achondroplasia.

The current observational study had three specific objectives: (i) to document any neuropsychological impairment in a sample of children with achondroplasia; (ii) to explore individual variability; and (iii) to determine the functional impact of any impairments. Fourteen children aged between 6 and 15 years with a medically confirmed diagnosis of achondroplasia (FGFR 3 mutation positive) underwent a comprehensive standardized neuropsychological evaluation. On average, while generally still within normal limits, significantly lower scores compared to standardized means were identified on: Full-scale IQ, verbal IQ, working memory, arithmetic, attention, executive functioning and aspects of day-to-day emotional, social, and behavioral functioning. Clinically significant levels of impairment at a group level were identified on measures of: arithmetic, attention, and executive functioning. There was variability among the group and for most measures scores ranged from impaired to within normal limits. A high percentage of children were impaired on measures of: verbal IQ, attention and executive functioning. Results of this study suggest a need for individual neuropsychological evaluation and monitoring of children with achondroplasia and suggest verbal IQ, arithmetic, attention, and executive functioning are particularly common areas of impairment. © 2016 Wiley Periodicals, Inc.

Decision-making capacity evaluation in adult guardianship: a systematic review.

BACKGROUND: Evidence of impaired decision-making capacity is a legal requirement for adult guardianship. To understand the quality of the evidence health professionals commonly provide in reports submitted to guardianship courts, a systematic review was undertaken to appraise the design and methodological quality of the published literature on health professionals' written reports of decision-making capacity and to describe the content of these reports. METHODS: Electronic searches from 1980 to 2015 identified 1183 articles of which 11 met the inclusion criteria where each evaluated quantitatively the content of health professionals' written reports submitted to adult guardianship proceedings. Methodological quality of the selected studies was rated using a critical appraisal tool. RESULTS: Nine studies sourced files from courts in the U.S. and one from Sweden; another reported on guardianship decisions from Australia. Four studies were rated as moderately strong or strong. Strengths included the use of comparison groups and a reliable and valid instrument to code reports. The review showed a person's medical condition was often cited as evidence of impaired decision-making capacity. Cognitive, psychiatric and functional abilities were less often described, and a person's values and preferences were rarely recorded. CONCLUSIONS: It is recommended health professionals describe the process by which a person makes a particular decision (their ability to understand, appreciate, reason and communicate) in addition to providing medical information, including cognitive, psychiatric and functional abilities. This approach provides support for a professional's opinion and evidence for a court. International studies of health professionals' approach to decision-making capacity evaluation are needed.

Longitudinal assessment of cognition and T2-hyperintensities in NF1: an 18-year study.

The developmental course of cognitive deficits in individuals with neurofibromatosis type 1 (NF1) is unclear. The objectives of this study were to determine the natural history of cognitive function and MRI T2-hyperintesities (T2H) from childhood to adulthood and to examine whether the presence of discrete T2H in childhood can predict cognitive performance in adulthood. We present cognitive and structural neuroimaging data from 18 patients with NF1 and five sibling controls assessed prospectively across an 18-year period. Longitudinal analyses revealed a significant increase in general cognitive function in patients with NF1 over the study period. Improvements were limited to individuals with discrete T2H in childhood. Patients without lesions in childhood exhibited a stable profile. The number of T2H decreased over time, particularly discrete lesions. Lesions located within the cerebral hemispheres and deep white matter were primarily stable, whereas those located in the basal ganglia, thalamus and brainstem tended to resolve. Our results support the hypothesis that resolution of T2H is accompanied by an improvement in general cognitive performance, possibly as a result of increased efficiency within white matter tracts.

Early life parechovirus infection neuropsychological outcomes at 8 years: a cohort study.

Human parechovirus (HPeV) is a leading cause of Central Nervous System (CNS) infection in infancy. Despite this, little is known regarding the long-term neuropsychological impacts from HPeV infection. The aim of the present study was to explore the long-term neuropsychological impacts eight-year post-HPeV infection contracted during infancy. This study also aimed to investigate the differential impacts of HPeV itself compared to the effects of secondary meningitis (n = 23) or encephalitis (n = 3) associated with HPeV infection. Thirty-nine HPeV children participated in the study. Children completed performance-based measures of neuropsychological and language functioning (the Wechsler Abbreviated Scale of Intelligence, the Clinical Evaluation of Language Fundamentals - Fourth Edition, and the Test of Everyday Attention for Children). Parents completed questionnaire-based measures of emotional, behavioral, and pragmatic language functioning (the Behaviour Rating Inventory of Executive Functioning, the Child Behavior Checklist, and the Social Communication Questionnaire). Results revealed that, overall, children with HPeV were significantly more impaired on measures of selective, sustained, and divided attention compared to normative test populations. The current study incidentally found at least double the prevalence of Attention-Deficit/Hyperactivity Disorder (ADHD) in the HPeV sample than what is typical in the normal population, suggesting that HPeV infection during infancy may be a risk factor for the later development of ADHD. Additionally, the presence of secondary meningitis or encephalitis did not relate to poorer neuropsychological outcomes in the current sample. The findings of this study have important implications regarding clinical management for children following HPeV infection in infancy.

Neuropsychological factors associated with performance on the rey-osterrieth complex figure test in children with neurofibromatosis type 1.

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.

The interplay between executive control and motor functioning in Williams syndrome.

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.

Comprehension of sarcasm, metaphor and simile in Williams syndrome.

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.

Autism Spectrum Disorder in Children with an Early History of Paediatric Acquired Brain Injury.

Autism spectrum disorder (ASD) is a neurodevelopmental condition that arises from a combination of both genetic and environmental risk factors. There is a lack of research investigating whether early acquired brain injury (ABI) may be a risk factor for ASD. The current study comprehensively reviewed all hospital records at The Brain Injury Service, Kids Rehab at the Children's Hospital at Westmead (Australia) from January 2000 to January 2020. Of the approximately 528 cases, 14 children with paediatric ABI were subsequently given an ASD diagnosis (2.7%). For this ASD sample, the mean age at the time of the ABI was 1.55 years, indicating a high prevalence of early ABI in this diagnostic group. The mean age of ASD diagnosis was, on average, 5 years later than the average ASD diagnosis in the general population. Furthermore, 100% of children had at least one medical comorbidity and 73% had three or more co-occurring DSM-5 diagnoses. Although based on a small data set, results highlight early paediatric ABI as a potential risk factor for ASD and the potential for a delayed ASD diagnosis following early ABI, with comorbidities possibly masking symptoms. This study was limited by its exploratory case series design and small sample size. Nonetheless, this study highlights the need for longitudinal investigation into the efficacy of early screening for ASD symptomatology in children who have sustained an early ABI to maximise potential intervention.

Internalising and Externalising Symptoms and Their Association with the Family Environment in Young Children with Williams Syndrome: A Longitudinal Study.

Williams Syndrome (WS) involves high rates of psychopathology across the lifespan. However, little is known about the early, longitudinal trajectory of internalising/externalising symptoms or the association between these and the family environment in WS. WS (n = 16; aged 2 years, 2 months to 9 years, 5 months) and typically developing or TD (n = 46; aged 2 years, 2 months to 11 years, 1 month) children were assessed on two occasions over 2.5 years utilising parent report questionnaires-the Child Behaviour Checklist and the Family Environment Scale. No statistically significant changes were found in CBCL/psychopathology profiles across timepoints, on average, for either WS or TD children. However, reliable change scores showed WS children had considerable variability in CBCL scores over time. Cross-sectionally, the WS group showed higher scores (reflecting more psychopathology) compared to TD controls at both time points across most CBCL subscales, with elevated overall psychopathology problems identified in 56-68% of WS children (versus 8% in TD controls). Psychopathology was not associated with sex, chronological age, or cognitive ability in WS. Conflict in the family environment was positively associated with higher Attention Problems at Time 1 in the WS group, whilst the TD group showed associations between family conflict and total psychopathology problems at both time points and between family cohesion and total psychopathology problems at Time 2. Family environment did not differ between groups, except for lower engagement in intellectual and cultural activities in WS. Findings highlight variable Internalising and Externalising Problems in young WS children over time, with greater biological than environmental contributions to psychopathology in WS.

Understanding nonliteral language abilities in children with neurofibromatosis type 1.

OBJECTIVE: Neurofibromatosis Type 1 (NF1) is a genetic syndrome that affects cognitive, behavioral, and social development. Nonliteral language (NLL) comprehension has not been examined in children with NF1. This study examined NLL comprehension in children with NF1 and associated neuropsychological correlates. METHOD: NLL comprehension was examined in children with NF1 (n = 49) and typically developing (TD) controls (n = 27) aged 4-12 years using a novel NLL task. The task assessed comprehension of sarcasm, metaphor, simile, and literal language. Cognitive (Wechsler Scales Composites or the Woodcock-Johnson Test of Cognitive Abilities Revised scaled scores) and behavioral (attention deficit hyperactivity disorder [ADHD] symptoms) correlates of NLL comprehension in children with NF1 were also examined. RESULTS: Children with NF1 demonstrated significantly poorer sarcasm comprehension than TD children and a vulnerability in metaphor comprehension. Simile and literal language comprehension were not significantly different between groups. Working memory difficulties and impulsive/hyperactive ADHD symptoms were associated with a reduced ability to identify sarcasm in NF1, while verbal comprehension, fluid reasoning, and inattentive ADHD symptoms were not. CONCLUSIONS: Results suggest children with NF1 experience challenges in understanding complex NLL comprehension, which are related to reduced working memory and increased impulsivity/hyperactivity. This study provides an initial insight into the figurative language abilities of children with NF1, which should be examined in relation to their social difficulties in future studies. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Alcohol and Tobacco use While Breastfeeding and Risk of Autism Spectrum Disorder or Attention Deficit/Hyperactivity Disorder.

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.

Neuroanatomical correlates of social approach in Williams Syndrome and down syndrome.

Individuals with Williams Syndrome (WS) or Downs Syndrome (DS) are often described as hypersociable, friendly and overly trusting of others. This hypersociability is a major concern for parents/caregivers due to the associated increased risk of exploitation and victimisation. Two brain regions - the amygdala and the orbitofrontal cortex (OFC) - have been implicated in driving this hypersociability in WS, and in the general population and have associations with emotional evaluation, threat detection and social motivation. However, there has been little neuroimaging research on this topic, especially in DS, to date. The aim of the present study was to investigate the potential neuroanatomical and neuropsychological correlates of hypersociability in WS and DS. Twelve individuals with WS (M = 22 years of age) and eleven individuals with DS (M = 26 years of age) completed a neuropsychological battery of executive functioning and social measures, including informant ratings on an ecologically measure of social approach. Clinical groups and twelve typically developing controls (M = 23 years) underwent a magnetic resonance imaging scan to investigate volumetric differences in the OFC and the amygdala. As expected, WS individuals displayed the highest overall social approach, especially in relation to need to approach strangers and drive to interact with strangers, as well as inappropriate/overfriendly behaviours. Both groups rated similarly in terms of social trust and unconditional positive regard. Emotion recognition abilities were similar across groups, with the DS group displaying some difficulties with negative emotions (especially anger). Inhibition and flexibility were similarly impaired across WS and DS. Compared to neurotypical controls, the DS group showed increased amygdala volumes bilaterally, while the WS group showed an enlarged right medial OFC. Approach ratings were significantly correlated with left amygdala and medial and left lateral OFC volumes in WS, and with these same regions bilaterally in DS. Results provide potential biological explanations for the hypersociability seen in WS and DS. Future research should focus on other potential neural correlates, as well as potential genetic and hormonal contributions to approach.