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BackgroundIntellectual and developmental disabilities (IDDs) are associated with both cognitive challenges and difficulties in conceptual, social, and practical areas of living, commonly referred to as adaptive behavior (DSM-5). Although cross-sectional associations between intelligence or cognition and adaptive behavior have been reported in IDD populations, no study to date has examined whether developmental changes in cognition contribute to or track with changes in adaptive behavior. The present study sought to examine associations of longitudinal developmental change in domains of cognition (NIH Toolbox Cognition Battery, NIHTB-CB) and adaptive behavior domains (Vineland Adaptive Behavior Scales-3; VABS-3) including Socialization, Communication, and Daily Living Skills (DLS) over a two year period in a large sample of children, adolescents and young adults with IDD.MethodsThree groups were recruited, including those with fragile X syndrome, Down syndrome, and other/idiopathic intellectual disability. Eligible participants (n = 263) included those who were between 6 and 26 years (mage = 15.52, sd = 5.17) at Visit 1, and who had a diagnosis of, or suspected intellectual disability (ID), including borderline ID, with a mental age of at least 3.0 years. Participants were given cognitive and adaptive behavior assessments at two time points over a two year period (m = 2.45 years, range = 1.27 to 5.56 years). In order to examine the association of developmental change between cognitive and adaptive behavior domains, bivariate latent change score (BLCS) models were fit to compare change in the three cognitive domains measured by the NIHTB-CB (Fluid Cognition, Crystallized Cognition, Total Cognition) and the three adaptive behavior domains measured by the VABS-3 (Communication, DLS, and Socialization).ResultsOver a two year period, change in cognition (both Crystallized and Total Composites) was significantly and positively associated with change in daily living skills. Also, baseline cognition level predicted growth in adaptive behavior, however baseline adaptive behavior did not predict growth in cognition in any model.ConclusionsThe present study demonstrated that developmental changes in cognition and adaptive behavior are associated in children and young adults with IDD, indicating the potential for cross-domain effects of intervention. Notably, improvements in DLS emerged as a primary area of adaptive behavior that positively related to improvements in cognition. This work provides evidence for the clinical, "real life" meaningfulness of changes in cognition detected by the NIHTB-CB in IDD, and provides empirical support for the NIHTB-CB as a fit-for-purpose performance-based outcome measure for this population.

The NIH Toolbox Cognition Battery (NIHTB-CB) was developed for epidemiological and longitudinal studies across a wide age span. Such a tool may be useful for intervention trials in conditions characterized by intellectual disability (ID), such as Williams syndrome (WS). Three NIHTB-CB tasks, including two executive functioning (Flanker, Dimensional Change Card Sort) and one episodic memory (Picture Sequence Memory) task, were given to 47 individuals with WS, ages 4 to 50, to evaluate feasibility (i.e., proportion of valid administrations) in this population. Findings indicated that NIHTB-CB tests showed good feasibility. Flanker and DCCS age-corrected scores were negatively correlated with age and showed floor effects, indicating these scores may not be useful for quantifying performance on these NIHTB-CB tests in ID.

Testing cognitive skill development is important for diagnostic, prognostic, and monitoring purposes, especially for young children and individuals with neurodevelopmental disorders. Developmental tests have been created for infants and toddlers, while traditional IQ tests are often employed beginning in the later preschool period. However, IQ tests rely on developmental skills that are rapidly changing during early childhood. Here, we introduce the idea of prerequisite skills in developmental domains, which are discrete skills required for, but not explicitly tested by, traditional IQ tests. Focusing on general cognition, particularly among children with a chronological or mental age under 4 years, may fail to capture important nuances in skill development. New skill-based assessments are needed in general, and in particular for categorization, which is foundational to higher-order cognitive skills. Novel measures quantifying categorization skills would provide a more sensitive measure of development for young children and older individuals with low developmental levels.

The toddler and preschool years are a time of significant development in both expressive and receptive communication abilities. However, little is known about the neurobiological underpinnings of language development during this period, likely due to difficulties acquiring functional neuroimaging data. Functional near-infrared spectroscopy (fNIRS) is a motion-tolerant neuroimaging technique that assesses cortical brain activity and can be used in very young children. Here, we use fNIRS during perception of communicative and noncommunicative speech and gestures in typically developing 2- and 3-year-olds (Study 1, n=15, n=12 respectively) and in a sample of 2-year-olds with both fNIRS data collected at age 2 and language outcome data at age 3 (Study 2, n=18). In Study 1, 2- and 3-year-olds differentiated between communicative and noncommunicative stimuli as well as between speech and gestures in the left lateral frontal region. However, 2-year-olds showed different patterns of activation from 3-year-olds in right medial frontal regions. In Study 2, which included two toddlers identified with early language delays along with 16 typically developing toddlers, neural differentiation of communicative stimuli in the right medial frontal region at age 2 predicted receptive language at age 3. Specifically, after accounting for variance related to verbal ability at age 2, increased neural activation for communicative gestures (vs. both communicative speech and noncommunicative gestures) at age 2 predicted higher receptive language scores at age 3. These results are discussed in the context of the underlying mechanisms of toddler language development and use of fNIRS in prediction of language outcomes.

Respiratory sinus arrhythmia (RSA) is proposed to index cognitive and behavioral inflexibility. Broad autism phenotype (BAP) traits are prevalent in family members of children with autism spectrum disorder (ASD). The study investigated whether RSA and BAP traits in mothers of typically developing (TD) children and mothers of children with ASD influence maternal affect. It was hypothesized that these factors would interact to influence mother-child interactions. Twenty-three mother-child dyads participated in a challenging interaction while measuring mother's RSA. Results indicated that mothers of children with ASD show different RSA reactivity than mothers of TD children. Furthermore, preliminary analyses revealed RSA reactivity moderated the relationship between mothers' rigidity and maternal affect during this interaction. Implications for future research and interventions are discussed.

Respiratory sinus arrhythmia (RSA) is proposed to index cognitive and behavioral inflexibility. Broad autism phenotype (BAP) traits are prevalent in family members of children with autism spectrum disorder (ASD). The study investigated whether RSA and BAP traits in mothers of typically developing (TD) children and mothers of children with ASD influence maternal affect. It was hypothesized that these factors would interact to influence mother-child interactions. Twenty-three mother-child dyads participated in a challenging interaction while measuring mother's RSA. Results indicated that mothers of children with ASD show different RSA reactivity than mothers of TD children. Furthermore, preliminary analyses revealed RSA reactivity moderated the relationship between mothers' rigidity and maternal affect during this interaction. Implications for future research and interventions are discussed., Author(s): Emma E. Condy [sup.1] , Reina S. Factor [sup.1] [sup.2] , Deanna M. Swain [sup.1] [sup.2] , Marlene V. Strege [sup.1] , Angela Scarpa [sup.1] [sup.2] Author Affiliations: (1) [...]

In addition to social communication deficits, restricted repetitive behaviors (RRBs) are a key diagnostic feature of autism spectrum disorder (ASD). Dysfunction of the autonomic nervous system (ANS) in ASD has been posited as a mechanism of RRBs; however, most studies investigating ANS activity in ASD have focused on its relation to social functioning. This study used respiratory sinus arrhythmia (RSA) patterns to measure ANS functioning and analyze its relation to RRBs in children with and without an ASD diagnosis. Baseline RSA and RSA reactivity predicted RRB severity and exploratory analyses revealed these measures may be associated with RRB subgroups. These results are discussed in regards to the behavioral literature on RRBs and the benefits of finding biomarkers for these behaviors.

While the function of restricted repetitive behaviors (RRBs) in autism spectrum disorder (ASD) is unclear, RRBs may function as anxiety reduction strategies (Joosten et al. "J Autism Dev Disord" 39(3):521-531, 2009. Moreover, anxiety in ASD is associated with low social motivation (Swain et al. "J Autism Dev Disord," 2015. The present study examined social motivation as a mediator between anxiety and RRBs in a sample of 44 children (2-17 years old; 80% male) with ASD. The relationship between anxiety and IS, but not other RRBs, was partially mediated by social motivation. These findings suggest anxiety is linked to social motivation deficits in children with ASD, which may increase ritualized behaviors and difficulties with changes in routine. Implications are discussed for differing functions and treatment of RRB domains.

In addition to social communication deficits, restricted repetitive behaviors (RRBs) are a key diagnostic feature of autism spectrum disorder (ASD). Dysfunction of the autonomic nervous system (ANS) in ASD has been posited as a mechanism of RRBs; however, most studies investigating ANS activity in ASD have focused on its relation to social functioning. This study used respiratory sinus arrhythmia (RSA) patterns to measure ANS functioning and analyze its relation to RRBs in children with and without an ASD diagnosis. Baseline RSA and RSA reactivity predicted RRB severity and exploratory analyses revealed these measures may be associated with RRB subgroups. These results are discussed in regards to the behavioral literature on RRBs and the benefits of finding biomarkers for these behaviors., Author(s): Emma E. Condy [sup.1] , Angela Scarpa [sup.1] , Bruce H. Friedman [sup.1] Author Affiliations: (1) Department of Psychology (0436), Virginia Polytechnic Institute and State University, 0000 0001 0694 [...]

Background: Heterogeneity in adaptive behavior abilities among people with autism spectrum disorder (ASD) is expressed not only as uneven levels of impairment across domains, but also in the developmental trajectories of adaptive skills. We studied the question of whether, after accounting for global adaptive behavior development, we find evidence of heterogeneity in the trajectories of specific domains of adaptive behavior. Methods: A sample of 504 children with ASD was obtained by combining data from two independent natural history studies conducted in North America. We used a factor of curves model to explain growth between 36 and 138 months in Vineland Adaptive Behavior Scales, Second Edition (VABS) age equivalents as a function of domain‐specific and global growth processes. Results: The domain‐specific trajectories in all three domains (Communication, Daily Living Skills, and Socialization) reflected impairment relative to age expectations as well as slower‐than‐expected growth with age, and the parameters of these trajectories were moderately‐to‐strongly correlated across domains. The global adaptive behavior trajectory had an initial (36–41 months of age) developmental level of about 22 age‐equivalent months, and eventually slowed after initially increasing by about 6 months each year. The global trajectory accounted for the majority of variance in the domain‐level processes; however, additional variance remained (14%–38%) in the domain‐level intercepts, slopes, and quadratic processes. Conclusions: These results extend existing theoretical and empirical support for the hierarchical structure of adaptive behavior to include its development over time in clinical samples of children with ASD. A latent global trajectory may be sufficient to describe the growth of adaptive behavior in children with ASD; however, the remaining domain‐specific variability after accounting for global adaptive behavior development allows for the possibility that differential effects of intervention on specific domains may be possible and detectable. [ABSTRACT FROM AUTHOR]

The NIH Toolbox includes a cognitive battery that provides an Early Childhood Composite score for children age 3–7. However, very few studies have evaluated feasibility when it is used in the youngest segment of this age range—3-year-olds. The current study evaluated performance on the four cognitive subtests composing the early childhood composite, two of which assess executive function, in a large sample of 3-year-olds enrolled in a Vanguard pilot of the National Children's Study. Results found that in a cohort of 609 3-year-olds (mean age = 39.6 months, SD = 1.6, 53% male, 64% White, 87% Non-Hispanic) who were administered four subtests included in the Early Childhood Composite, up to approximately 30% were unable to pass practice items on the Flanker, Dimensional Change Card Sort, and Picture Sequence Memory, whereas only approximately 3% were unable to pass practice items on the Picture Vocabulary Test. Furthermore, of those that did pass practice and achieve scores on the subtests, approximately 70% and 80% performed at or below chance level on the executive function tasks (Flanker and Dimensional Change Card Sort) and Picture Sequence Memory, respectively. Ultimately, the average 3-year-old has difficulty with three of the four NIH Toolbox tasks composing the Early Childhood Composite and may not yet have developed the requisite skills. These findings indicate that changes compatible with the developmental level of preschoolers are recommended to increase the feasibility and effectiveness of the NIH Toolbox in measuring individual cognition differences in 3-year-old children. [ABSTRACT FROM AUTHOR]

Previous work has found gender differences in restricted and repetitive behaviors and interests (RRBI) for autism spectrum disorder (ASD). Compared to girls, affected boys have increased stereotyped and restricted behaviors; however much less is known about gender differences in other areas of RRBI. This study aims to identify whether specific RRBI (i.e., stereotyped, self‐injurious, compulsive, insistence on sameness, ritualistic, and restricted), as measured by item‐level data on the Repetitive Behavior Scale‐Revised (RBS‐R), can distinguish girls from boys with ASD. Participants included 615 individuals with ASD (507 boys; 82.4%), ages 3–18 years of age (M = 10.26, SD = 4.20), who agreed to share data with the National Database for Autism Research (NDAR). Multivariate analysis of variance and discriminant function analysis (DFA) were used to determine whether item‐level RBS‐R data could correctly classify cases by gender. DFA results suggest that RBS‐R items significantly differentiate gender. Strongly differentiating RBS‐R items had greater success in correctly classifying affected boys (67.90%) than girls (61.00%). Items that best‐discriminated gender were heightened stereotyped behaviors and restricted interests items in boys and compulsive, sameness, restricted, and self‐injurious behavior items in girls. This study is the first to find that girls with ASD may have increased compulsive, sameness, and restricted RRBI compared to boys. Additionally, findings support heightened self‐injurious behaviors in affected girls. Future research should disentangle whether elevated rates of RRBI in girls are central to the presentation of ASD in girls or an epiphenomenon of the high rates of co‐occurring disorders (e.g., anxiety) noted in girls. Autism Res 2019, 12: 274–283 © 2018 International Society for Autism Research, Wiley Periodicals, Inc. Lay Summary: This study is the first to examine a comprehensive measure of repetitive behavior in children with autism, with findings of increased compulsive, insistence on sameness, and self‐injurious behavior characterizing girls and increased stereotyped and restricted behavior characterizing boys. Future research should determine whether these elevated behaviors in girls are directly part of the autism presentation in girls or symptoms of co‐occurring psychopathology. It is important for autism diagnostic measures to best capture the types of repetitive behavior girls may demonstrate. [ABSTRACT FROM AUTHOR]