Search

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.

Background Individuals with Down syndrome (DS) demonstrate difficulties with aspects of executive function (EF) and adaptive behaviour across the lifespan. There is a current lack of information regarding how these difficulties relate to employment outcomes in adulthood. This study evaluated the adaptive behaviour and EF profiles of individuals with DS during early adulthood and the association between these areas of functioning and employment status. Methods Parents or caregivers of primarily young adults with DS (n = 31; mean chronological age = 25.9 years; SD = 5.92) completed the Vineland Adaptive Behavior Scales - Second Edition the Behavior Rating Inventory of Executive Function - Adult version and a demographic questionnaire that requested information regarding adult employment status. Results Findings indicated a distinct pattern of relative strengths and challenges in adaptive behaviour and EF. In addition, the EF sub-domain of working memory was a significant predictor of employment status. Conclusions Specific aspects of the DS cognitive profile may have an important influence on employment status in primarily young adults with DS. Implications for interventions are discussed.

Background This study investigated sensory processing in a sample of Spanish children with autism spectrum disorder (ASD). Specifically, the study aimed to explore (1) the prevalence and distribution of atypical sensory processing patterns, (2) the relationship between adaptive and maladaptive behaviour with atypical sensory processing and (3) the possible relationship between sensory subtype and maternal stress. Methods The short sensory profile 2 (Dunn 2014) and the vineland adaptive behavior scale (Sparrow et al. 1984) were administered to examine the sensory processing difficulties and maladaptive behaviours of 45 children with ASD aged 3 to 14; their mothers also completed the parenting stress index-short form (Abidin 1995). Results Atypical sensory features were found in 86.7% of the children; avoider and sensor being the two most common patterns. No significant relationship was found between atypical sensory processing and adaptive behaviour. However, the analysis showed a strong relationship between sensory processing and maladaptive behaviour. Both maladaptive behaviour and sensory processing difficulties correlated significantly with maternal stress although maternal stress was predicted only by the sensory variable, and in particular by the avoider pattern. Conclusions The findings suggest that sensory features in ASD may be driving the high prevalence of parental stress in carers. They also suggest that the effect on parental stress that has been attributed traditionally to maladaptive behaviours may be driven by sensory difficulties. The implications of these findings are discussed in relation to the development of interventions and the need to explore contextual and cultural variables as possible sources of variability.

Background The purpose of the study was to describe sensorimotor profile and visual perceptual performance in school-aged (6–12 years) children with Williams syndrome (WS). The impacts of sensorimotor and visual perception on participation in WS were examined as well to guide research and evidence-based practices. Methods A total of 38 children with WS aged 6 to 12 years were evaluated with measures of motor performance (Bruininks–Oseretsky of Motor Proficiency—Second Edition), sensory processing (Sensory Profile), visual perceptual abilities (Test of Visual Perception Skills—Third Edition) and activity participation (Vineland Adaptive Behavior Scale, School Function Assessment). Results Children with WS performed significantly less well on all sensorimotor and visual perceptual measures, and 71% of children scored in the impaired range on six or more (one third of ) out of 18 measures. They had weaker fine motor skills than gross motor skills. Sensory modulation was the most impaired among the sensory processing functions. Visual perceptions were all moderately impaired. All the sensorimotor measures and visual perceptual functions correlated to the cognitive functions (IQ) and linked to activity participation measures in WS. Conclusions Our findings characterised the profiles of body functions (sensorimotor functions and visual organisation) of children with DS, and revealed their correlations with activity participation. Interventions focused on improving body functions are needed while stressing the acquisition of functional skills that increase participation in age-appropriate activities.

Background Williams syndrome (WS) is associated with a distinct cognitive-behavioural phenotype including mild to moderate intellectual disability, visual-spatial deficits, hypersociability, inattention and anxiety. Researchers typically characterise samples of individuals with WS by their intellectual functioning and adaptive behaviour. Because of the low prevalence of the syndrome, researchers often include participants with WS across a broad age range throughout childhood and adulthood and assume participants demonstrate consistent cognitive development across ages. Indeed, IQ scores are generally stable for children and adolescents with WS, although there are significant individual differences. It is less clear whether this pattern of stable intellectual ability persists into adulthood. Furthermore, while adaptive behaviour is an important indicator of an individual's ability to apply their conceptual skills to everyday functioning, conflicting findings on the trajectories of adaptive behaviour in adolescents and adults with WS have been reported. The current study examined longitudinal profiles of cognitive and adaptive functioning in adolescents and adults with WS. Method To examine cognitive functioning, participants included 52 individuals with WS (51.9% men) who were assessed with the Kaufman Brief Intelligence Test, 2nd edition (KBIT-2) between two and seven times. At their first assessment, participants had a mean age of 25.4 years (SD = 8.4), ranging in age from 14.2 to 48.9 years. To assess adaptive behaviour, participants included a subset of 28 individuals with WS whose parents completed the Vineland Adaptive Behavior Scale, 2nd edition (VABS-II) between two and five times. At their initial administration, participants ranged from 17.1–40.2 years of age, with a mean age of 26.5 years (SD = 7.3). A series of multilevel models were used to examine changes in KBIT-2 Composite IQ, Verbal IQ and Nonverbal IQ standard scores over time, as well as the Adaptive Behavior Composite, and the Communication, Daily Living Skills and Socialization subdomains of the VABS-II. Results Consistent with the WS cognitive profile, IQ scores were significantly lower than the general population IQ score of 100, and there was significant variability in individual IQ scores and slopes. KBIT-2 IQ scores were generally stable across adolescents and adults with WS. Adaptive behaviour scores were significantly lower than the population mean score of 100, and there was significant variability in individuals' adaptive behaviour scores but not trajectories. However, in contrast to the findings with the KBIT-2, VABS-II scores were observed to significantly decrease over time. Conclusion Findings suggest that while intellectual functioning remains stable, adaptive functioning does not remain stable across adolescence and adulthood in individuals with WS. Implications for the relation between cognitive and adaptive functioning across development are discussed, with a focus on how this relates to specific aspects of the WS phenotype.

Background While behavioural abnormalities are fundamental features of Rett syndrome (RTT), few studies have examined the RTT behavioural phenotype. Most of these reports have focused on autistic features, linked to the early regressive phase of the disorder, and few studies have applied standardised behavioural measures. We used a battery of standardised measures of behaviour and functioning to test the following hypotheses: (1) autistic behaviour is prominent throughout childhood in RTT; (2) autistic features are more salient in individuals with milder presentation; (3) severity of autistic behaviour is associated with a wider range of behavioural problems; and (4) specific MECP2 mutations are linked to more severe autistic behaviour. Methods Eighty MECP2 mutation-positive girls with RTT (aged 1.6–14.9 years) were administered: (1) the Screen for Social Interaction (SSI), a measure of autistic behaviour suited for individuals with severe communication and motor impairment; (2) the Rett Syndrome Behaviour Questionnaire (RSBQ), covering a wide range of abnormal behaviours in RTT; (3) the Vineland Adaptive Behavior Scales (VABS); and (4) a modified version of the Rett Syndrome Severity Scale (RSSS). Regression analyses examined the predictive value of age and RSSS on autistic behaviour and other behavioural abnormalities. T-tests further characterised the behavioural phenotype of individual MECP2 mutations. Results While age had no significant effect on SSI or RSBQ total scores in RTT, VABS Socialization and Composite scores decreased over time. Clinical severity (i.e. RSSS) also increased with age. Surprisingly, SSI performance was not related to either RSSS or VABS Composite scores. Autistic behaviour was weakly linked with the RSBQ Hand behaviour factor scores, but not with the RSBQ Fear/Anxiety factor. Clinical (neurological) severity did not predict RSBQ scores, as evidenced by the analysis of individual MECP2 mutations (e.g. p.R106W, p.R270X and p.R294X). Conclusions Our data suggest that in RTT, autistic behaviour persists after the period of regression. It also demonstrated that neurological and behavioural impairments, including autistic features, are relatively independent of one another. Consistent with previous reports of the RTT phenotype, individual MECP2 mutations demonstrate complex associations with autistic features. Evidence of persistent autistic behaviour throughout childhood, and of a link between hand function and social skills, has important implications not only for research on the RTT behavioural phenotype, but also for the clinical management of the disorder.

Background The latter stages of dementia in individuals with Down syndrome are well documented; however, earlier cognitive and behavioural changes have only recently been described. Holland et al. suggested such early signs of dementia in this population are behavioural and are similar to those seen in frontotemporal dementia, but there is, as yet, no evidence to determine whether such behavioural changes are associated with a declines in specific cognitive functions, including those associated with the frontal lobes. Methods A longitudinal design of three time points across 16 months was used across 30 adults with Down syndrome aged 30 years and over. Measures of cognition (Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities), receptive language (British Picture Vocabulary Scales), adaptive behaviour (Vineland Adaptive Behavior Scales), behavioural excesses and behavioural deficits (Assessment for Adults with Developmental Disabilities) and measures of executive functioning were completed at each time point. Using a data-driven method, cognitive deterioration was determined using the Reliable Change Index on performance on the Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities across the duration of the study. Performance on the remaining measures were then compared between those with (n = 10) and those without (n = 20) cognitive deterioration. Results Only individuals with cognitive deterioration showed decreases on measures of executive function and significant changes in behaviour across the duration of the study, which was not solely due to declines in memory. There were no changes between the groups on levels of adaptive behaviour. Conclusions Even in the early stages of cognitive deterioration, specific behavioural changes can be identified that are not present in those without cognitive deterioration. The differing effects of cognitive deterioration on behavioural excesses and deficits are discussed in relation to potentially differing underlying neuropathological causes.

Background This study was conducted to examine the impact of a I-year intervention for children with intellectual disabilities (ID) in Vietnam. Method Subjects were 30 preschool-aged children with ID (ages 3 to 6 years). Sixteen were assigned to an intervention group and I4 to a control group. Based on the Portage Curriculum (CESA 5 2003), the intervention trained parents to work with their children through modelling and coaching by teachers during weekly home visits. Results Comparison of pre-, mid- and post-intervention assessments of the children based on the Vineland Adaptive Behavior Scales (Sparrow et al. I984a) indicated that the intervention was promising: children in the intervention group improved significantly in most domains of adaptive behaviours, and also performed significantly better than the control group in the areas of personal care and motor skills. Conclusions The results from the Vietnam programme are discussed in terms of its implications and strategies for developing programmes for children with disabilities in developing countries. Correspondence: Jin Y. Shin, Department of Psychology, Hofstra University, Hempstead, New York, USA (e-mail: Jin.Y.Shin@ hofstra.edu).

Background Little is known about the prevalence of intellectual disability (ID) and/or cognitive impairment (CI) among accused persons in the Magistrates (Local) Courts, the personal, health and mental health characteristics of this cohort, and their service provision needs in the community. The study aimed to determine the prevalence of dual diagnoses of ID and/or CI and psychiatric disorder in a sample of accused persons appearing before four Magistrates Courts. Accused persons with ID and/or CI may not be identified in the Magistrates Court as having a disability and therefore may be unable to access the legal safeguards which exist for their protection within the criminal justice system and/or may fail to receive appropriate community health and welfare services. Method The sample was drawn from accused persons aged over 18 years appearing before four Magistrates Courts in metropolitan and urban areas of a large city. Participants were assessed using the Kaufman Brief Intelligence Test, Second Edition (KBIT‐2), Vineland Adaptive Behavior Scales, Second Edition (VABS2) and the Psychiatric Assessment Schedules for Adults with Developmental Disabilities Checklist. Results On the KBIT‐2, 10% of participants achieved a standard score (SS) below 70 (mild ID range) and a further 20% were in the 70–79 (borderline) range. The VABS2 results indicated that 12% of participants had SS below 70 and a further 9% were in the 70–79 (borderline) range. The prevalence of mental illness in the group with intellectual deficits was 46%, compared with a prevalence of 36% for those without intellectual deficits. Conclusions People with ID and/or CI were found to be over‐represented in the Magistrates Court. Furthermore, results highlight the unmet mental health needs of this cohort in the criminal justice system. The results of the study have implications for the planning of services and diversionary options to facilitate better management of defendants with ID and/or CI with mental health needs.

Background The interrelationship between adaptive functioning, behaviour problems and level of special education was studied in 186 children with IQs ranging from 61 to 70. The objective was to increase the insight into the contribution of adaptive functioning and general and autistic behaviour problems to the level of education in children with intellectual disability (ID).Methods Children from two levels of special education in the Netherlands were compared with respect to adaptive functioning [Vineland Adaptive Behavior Scales (VABS)], general behaviour problems [Child Behavior Checklist (CBCL)] and autistic behaviour problems [Autism Behavior Checklist (ABC)]. The effect of behaviour problems on adaptive functioning, and the causal relationships between behaviour problems, adaptive functioning and level of education were investigated.Results Children in schools for mild learning problems had higher VABS scores, and lower CBCL and ABC scores. The ABC had a significant effect on the total age equivalent of the VABS in schools for severe learning problems, the CBCL in schools for mild learning problems. A direct effect of the ABC and CBCL total scores on the VABS age equivalent was found, together with a direct effect of the VABS age equivalent on level of education and therefore an indirect effect of ABC and CBCL on level of education.Conclusions In the children with the highest level of mild ID, adaptive functioning seems to be the most important factor that directly influences the level of education that a child attends. Autistic and general behaviour problems directly influence the level of adaptive functioning. Especially, autistic problems seem to have such a restrictive effect on the level of adaptive functioning that children do not reach the level of education that would be expected based on IQ. Clinical implications are discussed.

Background Recent theories of stress and coping in parents of children with intellectual disabilities (ID) emphasize the importance of cognitive appraisals in influencing parents’ levels of stress and their adaptations to difficulties presented by the children. This study investigated the relationships between parental cognitions, child characteristics, family support and parenting stress. The aspects of cognitions studied were: parenting self-esteem (including efficacy and satisfaction) and parental locus of control. Methods The group studied consisted of  mothers of children with ID. The Vineland Adaptive Behavior Scales and Maladaptive Behavior Domain were administered by interview. Mothers also completed four questionnaires: the Family Support Scale, the Parenting Sense of Competence Scale, a shortened form of the Parental Locus of Control Scale and the Parenting Stress Index (Short Form). Results Data were analysed using Pearson’s correlation coefficients, partial correlations and a regression analysis. The results indicated that most of the variance in parenting stress was explained by parental locus of control, parenting satisfaction and child behaviour difficulties. Whilst there was also a strong correlation between family support and parenting stress, this was mediated by parental locus of control. Conclusions The results demonstrate the potential importance of parental cognitions in influencing parental stress levels. It is argued that these results have implications for clinical interventions for promoting parents’ coping strategies in managing children with ID and behavioural difficulties.