Laboratory-based measures of executive function and daily living skills in young children with Down syndrome: informing future interventions.

BACKGROUND: Adaptive behaviour refers to the practical skills necessary for independence and is considered a high-priority intervention target for children with neurogenetic conditions associated with intellectual disability, like Down syndrome (DS). Daily living skills (DLS) are a critical aspect of adaptive behaviour, but they have received little intervention attention, possibly because they involve a wide variety of skills across many settings. The present study aimed to advance DLS intervention science by examining the concurrent and longitudinal association between DLS performances and a cognitive skillset hypothesised to support DLS skill acquisition, executive function (EF). METHODS: Participants were 71 children with DS between the ages of 2.5 and 8.7 years (M = 5.23 years; standard deviation = 1.65) who completed a battery of adapted EF tasks and a primary caregiver who completed the Vineland Adaptive Behavior Scales 3rd Edition Parent/Caregiver Comprehensive Report Form. A subset of caregivers also provided 6- and 12-month follow-up adaptive behaviour information. RESULTS: Results demonstrated a positive association between EF task performance and DLS standard scores and v-scores both concurrently and longitudinally. CONCLUSIONS: The findings have implications for potential future intervention approaches that aim to strengthen DLS performances by advancing EF skills in this population.

A three-years follow-up of extra intensive support for individuals with intellectual disability and severe challenging behaviour in the Netherlands.

Adults with Intellectual Disability who show severe challenging behaviour need intensive individual support. If intensive support proves to be insufficient, extra intensive support can be provided in the Netherlands, which is characterized by more time for individual care. The present study evaluates the impact of extra intensive support over time. Client characteristics of adults receiving intensive support (IS, N=70) or extra intensive support (IS+, N=35) are compared and the impact of provided support on challenging behaviour (Developmental Behaviour Checklist-Adults), adaptive behaviour (Vineland II), and Quality of Life (San Martin Scale) is evaluated over a three years period. Compared to adults receiving intensive support, those receiving extra intensive support initially showed higher intensity of challenging behaviour, higher number of mental health diagnoses and stronger focus on goals to reduce challenging behaviour. Over time, intensity of challenging behaviour decreased in adults receiving extra intensive support, although Quality of Life and adaptive functioning did not improve. Results show that the indications for receiving extra intensive support are clear and that the extra support is effective over time. It is concluded that extra individual support is serving those who need this support.

Occupational performance patterns in children with paediatric acute-onset neuropsychiatric syndrome.

INTRODUCTION: Paediatric acute-onset neuropsychiatric syndrome (PANS) is a neuroimmune condition characterised by episodic exacerbations and remissions. This study investigates the impact of PANS on children's occupational performance during these phases, using the Vineland Adaptive Behaviour Scale (VABS). Understanding these variations is crucial for developing tailored interventions and managing the condition effectively. METHODS: A two-period bidirectional case-crossover design was employed to assess occupational performance in children with PANS at exacerbation (T-E) and remission (T-R) phases. Parents of children aged 4.6-13.1 years with PANS were recruited globally, with data collected via online surveys and monthly updates. The VABS evaluated occupational performance, with analysis using linear mixed models and Least Squares Means (LSMeans) for accurate representation. RESULTS: Data from 27 participants showed significantly lower scores in the Adaptive Behaviour Composite and socialisation domain of the VABS at T-E compared with T-R, indicating a decline in occupational performance and social skills during exacerbations. During remission, participants demonstrated typical performance across all domains. CONCLUSION: The study highlights a marked decline in occupational performance and socialisation during PANS exacerbations, with improvements to typical levels during remission. These findings emphasise the need for targeted interventions to address occupational and social challenges in children with PANS during exacerbations, underscoring the episodic nature of the condition and the risk of misinterpreting behaviours if assessed during exacerbations. Multidisciplinary allied health support may benefit children with PANS. CONSUMER AND COMMUNITY INVOLVEMENT: Although there was no direct consumer and community involvement, two researchers on our team have family members with PANS. Their personal experiences provided vital insights into the challenges faced by children with PANS, deeply influencing our study's design, focus, and interpretation, ensuring it reflects the realities of those affected. PLAIN LANGUAGE SUMMARY: Our study looked at how PANS affects the things children do in their daily lives. We asked parents of children ages 4.6-13.1 years old to complete surveys measuring this at two times: once during a symptom flare-up and once during a period of wellness. Our results showed that during symptom flare-ups, children's social skills and general ability to do daily tasks were much lower compared with periods of wellness. When children were well, their abilities were similar to those of typically developing children of the same age. This highlights the need for occupational therapy during flare-ups to help improve the lives of children with PANS.

Relationships between sensory reactivity and occupational performance in children with paediatric acute-onset neuropsychiatric syndrome (PANS).

INTRODUCTION: Paediatric acute-onset neuropsychiatric syndrome (PANS) is a neurodevelopmental disorder affecting children's performance in a fluctuating manner. This study investigates the relationship between sensory reactivity and occupational performance in children with PANS during exacerbation phases. Understanding these relationships is crucial for developing tailored interventions and managing the condition effectively. METHODS: This study is part of a larger project in which a two-period bidirectional case-crossover design was employed to assess sensory reactivity and occupational performance in children with PANS. The current study used data from exacerbation phases only. Parents of children aged 4.6 to 13.1 years with PANS were recruited globally, and data were collected via online surveys. The Vineland Adaptive Behaviour Scale (VABS) evaluated occupational performance, and the Sensory Processing Measure (SPM) assessed sensory reactivity. Analysis utilised Pearson correlation to determine the relationships between sensory reactivity and occupational performance domains. RESULTS: Data from 60 participants who completed the SPM and 21 who completed the VABS showed moderate to strong negative correlations between the SPM total and several domain scores (vision, hearing, body awareness, balance, and motion) and the VABS Communication and Daily Living Skills scores, indicating that increased sensory reactivity is associated with decreased occupational performance during exacerbations. No significant correlation was found between sensory reactivity and socialisation skills. CONCLUSION: Findings highlight the significant relationship between sensory reactivity and occupational performance during PANS exacerbations, particularly in communication and daily living skills domains. Further research is needed to examine factors influencing socialisation skills and to assess the effectiveness of sensory interventions in improving occupational performance. CONSUMER AND COMMUNITY INVOLVEMENT STATEMENT: Although there was no direct consumer and community involvement, two researchers on our team have family members with PANS. Their personal experiences provided vital insights into the challenges faced by children with PANS, deeply influencing our study's design, focus, and interpretation, ensuring it reflects the realities of those affected. PLAIN LANGUAGE SUMMARY: We examined the relationship between children's daily activities and their sensory reactivity during PANS symptom flare-ups. Parents of children aged 4.6 to 13.1 years completed surveys measuring sensory reactivity and their children's performance of daily tasks. Our findings showed that during symptom flare-ups, children experienced significant difficulties with communication and daily living skills. These challenges were directly related to heightened sensory reactivity during flare-ups. This highlights the importance of providing targeted occupational therapy during these times to help children manage their symptoms and improve their daily functioning.

Editorial Perspective: What can we learn from hunter-gatherers about children's mental health? An evolutionary perspective.

Humans lived as hunter-gatherers for more than 95% of our evolutionary history, thus studying contemporary hunter-gatherer populations offers insight into the conditions children may be psychologically adapted to. Here, we contrast hunter-gatherer childhoods with those of WEIRD (Western Educated Industrialised Rich Democratic) societies and consider the implications for children's mental health. Hunter-gatherer infants receive continuous physical contact and more sensitive and responsive caregiving than is typical of WEIRD societies, due to the extensive involvement of alloparents (non-parental caregivers) who generally provide 40-50% of their care. Alongside positive attachment outcomes, alloparenting likely reduces the harms of family adversity and risk of abuse/neglect. From late infancy, hunter-gatherers spend their time in mixed-age 'playgroups' where they learn via active play and exploration without adult supervision. This contrasts with WEIRD norms surrounding the need for adult supervision of children, as well as with the passive teacher-led classrooms, which could potentially lead to suboptimal learning outcomes and pose difficulties to children with ADHD. Based on this preliminary comparison, we consider practical solutions to potential harms arising from discordance between what children are adapted to and exposed to. These include infant massage and babywearing; increased sibling and extra-familial involvement in childcare; and educational adjustments.

Transmission trends of the global COVID-19 pandemic with combined effects of adaptive behaviours and vaccination.

We developed a mechanism model which allows for simulating the novel coronavirus (COVID-19) transmission dynamics with the combined effects of human adaptive behaviours and vaccination, aiming at predicting the end time of COVID-19 infection in global scale. Based on the surveillance information (reported cases and vaccination data) between 22 January 2020 and 18 July 2022, we validated the model by Markov Chain Monte Carlo (MCMC) fitting method. We found that (1) if without adaptive behaviours, the epidemic could sweep the world in 2022 and 2023, causing 3.098 billion of human infections, which is 5.39 times of current number; (2) 645 million people could be avoided from infection due to vaccination; and (3) in current scenarios of protective behaviours and vaccination, infection cases would increase slowly, levelling off around 2023, and it would end completely in June 2025, causing 1.024 billion infections, with 12.5 million death. Our findings suggest that vaccination and the collective protection behaviour remain the key determinants against the global process of COVID-19 transmission.

Approach-avoidance behavioural patterns towards an affective voice.

Facial expressions are evolutionarily acquired social signals that are processed rapidly by the receiver when deciding to either approach or avoid the person sending the signal. The predominance of the approach or avoidance responses has been examined for various facial expressions. Similar to facial expressions, the approach-avoidance response to affective voice may have been acquired evolutionarily. However, studies examining approach-avoidance to an affective voice are limited. This study used the approach-avoidance task framework developed for facial expressions to determine the dominant behavioural responses to affective voices that indicate either happiness or anger. The results show that the approach response was dominant for the happy voice, while the avoidance response was dominant for the angry voice. This tendency was observed even without the presence of any facial stimuli. These results suggest that an affective voice may be an evolutionarily acquired approach-avoidance-inducing social signal, similar to facial expressions.

Associations between executive functions, intelligence and adaptive behaviour in children and adolescents with mild intellectual disability.

Background: The purpose of this study was to explore the role of everyday executive functions in relation to intelligence and adaptive behaviour in children and adolescents with mild intellectual disability. Method: A group of children and adolescents, previously diagnosed with mild intellectual disability were assessed according to intelligence, everyday executive functions and adaptive behaviour. The association between everyday executive functions and intelligence was examined, and it was explored whether intelligence or everyday executive functions would best predict adaptive behaviour. Results: Adaptive behaviour was significantly predicted by executive functions, but not by intelligence. Nor was intelligence significantly related to everyday executive functions in this group. Conclusions: Although fundamental in diagnosing intellectual disability, intelligence cannot predict adaptive behaviour. Assessing everyday executive functions and adaptive behaviour, as well as acknowledging the strong association between them, provides valuable information in the process of optimizing developmental support for children and adolescents with mild intellectual disability.

Post-error slowing: Large scale study in an online learning environment for practising mathematics and language.

The ability to monitor and adjust our performance is crucial for adaptive behaviour, a key component of human cognitive control. One widely studied metric of this behaviour is post-error slowing (PES), the finding that humans tend to slow down their performance after making an error. This study is a first attempt at generalizing the effect of PES to an online adaptive learning environment where children practise mathematics and language skills. This population was of particular interest since the major development of error processing occurs during childhood. Eight million response patterns were collected from 150,000 users aged 5 to 13 years old for 6 months, across 23 different learning activities. PES could be observed in most learning activities and greater PES was associated with greater post-error accuracy. PES also varied as a function of several variables. At the task level, PES was greater when there was less time pressure, when errors were slower, and in learning activities focusing on mathematical rather than language skills. At the individual level, students who chose the most difficult level to practise and had higher skill ability also showed greater PES. Finally, non-linear developmental differences in error processing were found, where the PES magnitude increased from 6 to 9-years-old and decreased from 9 to 13. This study shows that PES underlies adaptive behaviour in an educational context for primary school students.

Executive functions and adaptive behaviour in individuals with Down syndrome.

BACKGROUND: Previous research has explored executive functions (EFs) and adaptive behaviour in children and adolescents with Down syndrome (DS), but there is a paucity of research on the relationship between the two in this population. This study aims to shed light on the profile of EFs and adaptive behaviour in DS, exploring the differences by age and investigating the relationship between these two domains. METHOD: Parents/caregivers of 100 individuals with DS from 3 to 16 years old participated in the study. The sample was divided into preschoolers (3-6.11 years old) and school-age children (7-16 years old). Parents/caregivers completed either the Preschool Version of the Behaviour Rating Inventory of Executive Function (for children 2-6.11 years old) or the Second Edition of the same Inventory (for individuals 7 + years old). Adaptive behaviour was assessed with the Vineland Adaptive Behaviour Scale - Interview, Second Edition. RESULTS: Findings suggest that individuals with DS have overall difficulties, but also patterns of strength and weakness in their EFs and adaptive behaviour. The preschool-age and school-age children's EF profiles differed slightly. While both age groups showed Emotional Control as a relative strength and Working Memory as a weakness, the school-age group revealed further weaknesses in Shift and Plan/Organise. As concerns adaptive behaviour, the profiles were similar in the two age groups, with Socialisation as a strength, and Communication and Daily Living Skills as weaknesses, but with a tendency for preschoolers to obtain intermediate scores for the latter. When the relationship between EFs and adaptive behaviour was explored, Working Memory predicted Communication in the younger group, while in the older group the predictors varied, depending on the adaptive domains: Working Memory was a predictor of Communication, Inhibit of Daily Living Skills, and Inhibit and Shift of Socialisation. CONCLUSION: As well as elucidating the EF profiles and adaptive behaviour in individuals with DS by age, this study points to the role of EFs in adaptive functioning, providing important information for targeted interventions.

Executive function as measured by the Behavior Rating Inventory of Executive Function-2: children and adolescents with Williams syndrome.

BACKGROUND: Difficulties with executive functions (EF) are very common among individuals with Williams syndrome (WS). To characterise the pattern of relative strengths and weaknesses in EF for children and adolescents with WS, we considered the performance of a large sample on the parent version of the Behavior Rating Inventory of Executive Function-2 (BRIEF-2). Associations between distinct components of EF and adaptive behaviour, behaviour problems and intellectual ability were investigated. The concurrent effects of components of behaviour regulation and emotion regulation on attention problems and anxiety problems also were evaluated. METHODS: Participants were 308 6-17-year-olds with genetically confirmed classic WS deletions. Parent report of EF was measured by the BRIEF-2 questionnaire. Most participants (223/308) completed the Differential Ability Scales-II as a measure of intellectual ability. The parents of these individuals also completed the Child Behavior Checklist and the interview form of the Scales of Independent Behavior-Revised. RESULTS: As a group, the participants evidenced considerable parent-reported EF difficulty. A profile of relative strength and weakness was found at the index level, with performance on both the Behavior Regulation Index and the Emotion Regulation Index significantly better than performance on the Cognitive Regulation Index. Within each index, a statistically significant pattern of relative strength and weakness also was identified. Difficulties with behaviour regulation and emotion regulation were related to both behaviour problems and adaptive behaviour limitations. Higher inflexibility and more difficulty with self-monitoring were associated with lower overall intellectual ability. Difficulty with inhibition was uniquely associated with attention problems, and inflexibility was uniquely associated with anxiety problems. CONCLUSIONS: Executive function difficulties are highly prevalent among children and adolescents with WS and are associated with adaptive behaviour limitations, both internalising and externalising behaviour problems and more limited intellectual ability. These results highlight the importance of designing and delivering research-based interventions to improve the EF of children and adolescents with WS.

Concordance of the Adaptive Behavior Assessment System, second and third editions.

BACKGROUND: Adaptive functioning is an important area of assessment with implications for differential diagnosis, educational placement, service eligibility and criminal sentencing. While periodic normative and content updates of adaptive functioning measures are necessary to keep measures relevant, knowledge of equivalence between versions is also required if adaptive measures are to be used to track the stability of adaptive functioning skills over time. METHOD: This paper presents two studies that used between-group and within-group comparison designs to examine the equivalence of the second and third editions of the Adaptive Behavior Assessment System (ABAS) in a mixed clinical sample. In study 1, ABAS-2 scores for children assessed between 2014 and 2015 (n = 1036; mean age = 10.24, SD = 3.44) were compared with ABAS-3 scores for children assessed between 2015 and 2016 (n = 1291; mean age = 10.51, SD = 3.70). Study 2 examined a separate sample of clinically referred children (n = 572) for whom parent ratings had been obtained on both the ABAS-2 (mean age = 9.65, SD = 2.80) and ABAS-3 (mean age = 13.33, SD = 2.95) in the course of repeated assessment. RESULTS: For Study 1, while no intelligence quotient score differences were observed between the ABAS-2 group (mean Verbal Comprehension Index = 93.67, SD = 16.95) and the ABAS-3 group (mean Verbal Comprehension Index = 93.08, SD = 17.42), ABAS-2 scores were lower than ABAS-3 scores on the Conceptual, Practical, and General Adaptive Composite scales. In study 2, a similar pattern was observed (ABAS-2 < ABAS-3 on the Conceptual, Practical, and General Adaptive Composite scales), and concordance correlation coefficients ranged from 0.54 [0.49, 0.58] (Practical composite) to 0.68 [0.64, 0.72] (Conceptual composite). The Practical composite had the lowest concordance correlation coefficient value and the largest mean score difference between ABAS versions. CONCLUSIONS: The ABAS-3 scores may be higher than ABAS-2 scores in clinical populations. Knowledge of these potential discrepancies will be critical when interpreting standard score changes across ABAS versions in the course of clinical, educational and forensic assessments.

Stressed parents, happy parents. An assessment of parenting stress and family quality of life in families with a child with Phelan-McDermid syndrome.

BACKGROUND: Individuals with Phelan-McDermid syndrome (PMS) are characterised by phenotypical traits that can be experienced as challenging by their environment. This study assessed parenting stress and Family Quality of Life (FQOL) in parents of individuals with PMS and identified potential contributing variables. METHOD: Mothers (n = 14) and fathers (n = 13) of individuals with PMS (n = 14; 6 females, 8 males; age 2-37, M = 20, SD = 11.92) completed questionnaires on parenting stress, FQOL, adaptive behaviour and background characteristics. RESULTS: Mothers and fathers experienced high, similar and related levels of parenting stress and FQOL satisfaction. Parenting stress and FQOL satisfaction were inversely related. High and low ratings were retrieved for subscales measuring feelings of parental role restriction and emotional well-being, respectively. The adaptive skills of the individuals with PMS were related to fathers' parenting stress and FQOL satisfaction. CONCLUSIONS: Clinical practice is encouraged to be attentive to family dynamics and grasp opportunities to interact with these dynamics.

Concordance of the Vineland Adaptive Behavior Scales, second and third editions.

BACKGROUND: Because of its centrality in the conceptualization of intellectual disability, reliable and valid measurement of adaptive behaviour is important to both research and clinical practice. The manual of the Vineland Adaptive Behavior Scales, recently released in its third edition, provides limited reliability information obtained from a sample composed primarily of typically developing individuals. The goal of this study was to evaluate the concordance of the Vineland-3 with the Vineland-II in a sample more similar in ability level to those in which the Vineland is commonly used. METHODS: Both editions of the Vineland Interviews were conducted with a convenience sample of 106 parents/caregivers of individuals with neurodevelopmental disability, participating at two neurodevelopmental disorder research clinics. Administrations were up to 7 days apart, but most (90%) were simultaneous. The concordance correlation coefficient (CCC) (95% confidence interval) and mean differences (95% confidence interval) were calculated for domain standard scores and subdomain v-scale scores. RESULTS: Domain-level CCC ranged from 0.78 [0.70, 0.84] (Communication) to 0.86 [0.76, 0.92] (Motor). Subdomain CCC ranged from 0.71 [0.62, 0.78] (Receptive Language) to 0.91 [0.85, 0.95] (Fine Motor). Vineland-3 scores were lower than Vineland-II scores; 77% of participants had lower Adaptive Behavior Composite scores on the Vineland-3 than on the Vineland-II. For the subdomains, the magnitude of this difference depended upon the level of adaptive behaviour. For Communication, the domain with the lowest CCC, the mean difference ranged from -13.70 [-8.03, -19.35] for a Vineland-II score or 85 to a difference of -19.18 [-12.28, -26.87] for a Vineland-II score of 40. DISCUSSION: Amongst individuals with intellectual and developmental disabilities, the Vineland-3 produces lower scores than the Vineland-II, and these clinically significant differences tend to be larger for individuals with lower levels of ability. Thus, care must be taken in interpreting scores from the Vineland-3 relative to those obtained from the previous edition.

Development, behaviour and sensory processing in Marshall-Smith syndrome and Malan syndrome: phenotype comparison in two related syndromes.

BACKGROUND: Ultrarare Marshall-Smith and Malan syndromes, caused by changes of the gene nuclear factor I X (NFIX), are characterised by intellectual disability (ID) and behavioural problems, although questions remain. Here, development and behaviour are studied and compared in a cross-sectional study, and results are presented with genetic findings. METHODS: Behavioural phenotypes are compared of eight individuals with Marshall-Smith syndrome (three male individuals) and seven with Malan syndrome (four male individuals). Long-term follow-up assessment of cognition and adaptive behaviour was possible in three individuals with Marshall-Smith syndrome. RESULTS: Marshall-Smith syndrome individuals have more severe ID, less adaptive behaviour, more impaired speech and less reciprocal interaction compared with individuals with Malan syndrome. Sensory processing difficulties occur in both syndromes. Follow-up measurement of cognition and adaptive behaviour in Marshall-Smith syndrome shows different individual learning curves over time. CONCLUSIONS: Results show significant between and within syndrome variability. Different NFIX variants underlie distinct clinical phenotypes leading to separate entities. Cognitive, adaptive and sensory impairments are common in both syndromes and increase the risk of challenging behaviour. This study highlights the value of considering behaviour within developmental and environmental context. To improve quality of life, adaptations to environment and treatment are suggested to create a better person-environment fit.

The functional impact of cognition in adults with autism spectrum disorders.

Purpose and aim: The overall aim of this study was to examine the relationship between adaptive function and cognitive factors in young adults diagnosed with autism spectrum disorder (ASD) in adult age.Methods: The study included 30 adults (age 18-30) diagnosed with ASD in adulthood. All participants were clinically referred to an adult psychiatric clinic for assessment. Adaptive functioning was measured with Adaptive Behavior Assessment System - 2nd edition (parent version). Wechsler scales of intelligence and Delis-Kaplan Executive Function System were used to measure intelligence and executive function.Results: We found considerable adaptive functioning deficits regardless of Full Scale Intelligence Quotient (FSIQ) level. FSIQ, working memory and processing speed were positively associated with adaptive functioning. No associations were found between adaptive functioning and cognitive flexibility, inhibition, word generation or shifting. Regression analysis showed that working memory and processing speed predicted 23% of the variance in adaptive functioning in this group.Conclusions: The results suggest that cognitive dysfunction could be an important area for intervention to improve adaptive functioning in ASD.

Influence of adaptive behaviour on the quality of life of adults with intellectual and developmental disabilities.

BACKGROUND: This study evaluated the role of adaptive behaviour, individual variables (age, gender and problem behaviours) and environmental variables (living arrangements, employment status and city dimension) in affecting the quality of life of individuals with IDD measured from third-party (caregiver) and individuals with IDD' perspective. METHOD: For 93 adults with an IDD diagnosis (47% males) aged 19-65 years, third-party and participants' perspective on participants' quality of life (Personal Outcome Scale), adaptive behaviour (Vineland-II scale), problem behaviours (PIMRA and DASH-II scales), and individual and environmental variables were collected. RESULTS: Adaptive behaviour was the main determinant of quality of life for individuals with IDD. The effect of adaptive behaviour was significant and relevant from both third-party and participants' perspectives. Problem behaviours had a modest negative impact on the quality of life. CONCLUSIONS: Adaptive behaviour is relevant for planning support and interventions for people with IDD to increase their quality of life.

Developmental trajectories of adaptive behavior in autism spectrum disorder: a high-risk sibling cohort.

BACKGROUND: Children with autism spectrum disorder (ASD) often experience impairments in adaptive behavior. METHODS: Developmental trajectories of adaptive behavior in ASD were examined in children from high-risk (siblings of children diagnosed with ASD, n = 403) and low-risk (no family history of ASD, n = 163) families. Children were assessed prospectively at 12, 18, 24, and 36 months of age using the Vineland Adaptive Behavior Scales and underwent a blind independent diagnostic assessment for ASD at 36 months of age. RESULTS: The semi-parametric group-based modeling approach using standard scores on the Adaptive Behavior Composite revealed three distinct developmental trajectories: (a) Group 1 (21.2% of sample) showed average performance at 12 months and a declining trajectory; (b) Group 2 (52.8% of the sample) showed average performance at 12 months with a slightly declining trajectory; and (c) Group 3 (26.0% of the sample) showed a higher level of adaptive behavior at 12 months and a stable trajectory. The Mullen Scales of Early Learning Early Learning Composite and the Autism Observation Scale for Infants total score at 6 and 12 months predicted trajectory membership. CONCLUSIONS: The results emphasize heterogeneous development associated with ASD and the need for interventions tailored to individual presentations.

Bladder and bowel dysfunction, adaptive behaviour and psychiatric profiles in adults affected by autism spectrum disorders.

AIMS: Bladder and bowel dysfunction (BBD) have been recognized in children affected by autism spectrum disorder (ASD), but no consistent information exist in adults with the condition. We evaluated the prevalence of BBD and the impact of psychiatric and behavioural profiles in adults affected by ASD. METHODS: Twenty-two adults and 13 children/teens with ASD and a matched group of typically developing subjects (TD) were prospectively studied. Patients and TD subjects underwent the evaluation of urinary incontinence (UI: diurnal, continuous or intermittent), nocturnal enuresis (NE), and bowel disturbances with the 3-day voiding and bowel diary. In addition, assessment of intellectual disability (ID) and psychiatric and adaptive behaviours with the Neuropsychiatric Inventory Scale (NPI) and the Vineland Adaptive Behaviour Scale 2nd Edition (Vineland-II), was performed. RESULTS: In adults, any type of incontinence was observed in 81.8% of cases, and NE and intermittent UI in 59.0% and 36.3% of patients, respectively. Faecal incontinence and constipation were detected 36.3% and in 68.1% of cases, respectively. ID was severe in 2 cases and profound in 18; NPI and Vineland-II items most affected were "Irritability/Lability," "Motor Activity," and "Agitation," and IQ-Socialization and IQ-Communication. Significant relationships were identified between intermittent UI and greater ID (P < .02) and high "anxiety" (P < .05), and between NE and high "euphoria/elevated mood" (P < .05). These results were similar to those observed in children/teens. CONCLUSIONS: Adults with ASD, and greater ID and mood disorders, present with a high prevalence of BBD. A shared pathogenetic mechanism could underlie the co-occurrence of ASD, mood disorders, and BBD.

Relationship between the Vineland Adaptive Behaviour Scales and the Wechsler Adult Intelligence Scale IV in adults with intellectual disabilities.

BACKGROUND: Diagnoses of intellectual disability (ID) are based on three criteria: significant impairments in intellectual functioning, concurrent deficits in adaptive behaviour, and both being acquired in the developmental period. Adaptive behaviour was formally incorporated into the diagnosis in 1959; the rationale being that IQ and adaptive behaviour were different constructs that independently contribute to the diagnosis. However, there are limited data on the relationship between IQ and adaptive behaviour and especially so for adults who have ID. The aim of this study was to investigate this relationship on two widely used assessment tools: the Wechsler Adult Intelligence Scale - fourth edition (WAIS-IV) and the Vineland Adaptive Behaviour Scales (VABS). METHOD: Data were extracted from the case files of 147 adults who had a formal diagnosis of ID based on the WAIS-IV and VABS. Internal consistency was computed and compared to general population data. Correlations between the WAIS-IV and the VABS were computed. RESULTS: Internal consistencies for the tests when used with adults who have ID were generally good. The correlations between the WAIS-IV and VABS composite and subdomain scores were all low and similar to those reported for the general population. CONCLUSION: These results suggest that the WAIS-IV and VABS are measuring different constructs that contribute to the diagnosis of ID. Unfortunately, by the time such data have been collected, tests are being revised. The Vineland has now been revised and is in its third edition, but no data on the relationship between IQ and the VABS were collected during test development. The WAIS-V is now in development, and so it is recommended that such work be incorporated into this process or shortly thereafter to ensure that the tools continue to access independent constructs.