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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.

Background: Studies of early caregiver-mediated interventions targeting social communication of young autistic children have yielded variable child outcomes. This study examined the effects of combining two caregiver-mediated interventions on caregiver strategy use and child social communication and language outcomes. Method: This was a multisite parallel randomized controlled trial. Participants included 120 caregivers and their autistic children between 24 and 36 months of age. Dyads were randomly assigned to receive a hybrid intervention that combined Enhanced Milieu Teaching (EMT) and Joint Attention, Symbolic Play, Engagement, and Regulation (JASPER) or to a behavior management control condition, each delivered over 6 months. Caregivers in the JASP-EMT group received twice-weekly, in-home, and hour-long sessions. Outcomes were measured at baseline, the end of intervention (T1), and 6 months later (T2) and included a naturalistic language sample procedure, standardized measures, and caregiver report measures. This trial was registered at clinicaltrials.gov(NCT02595697). Results: Child outcomes did not differ between conditions at T1 or T2 for child primary (social communication) or secondary (language, play, and autism symptoms) outcomes. Relative to control group caregivers, intervention group care-givers demonstrated significantly higher use of JASP-EMT strategies at T1 andT2, with the exception of two strategies (Responsiveness and Matched Responsiveness), which were used significantly more by control group caregivers. Neither autism severity nor baseline caregiver responsiveness moderated out-comes. Post hoc analyses revealed significant correlations between specific strategies and all child outcomes. Conclusions: Twice-weekly caregiver-mediated intervention that taught care-givers of autistic children to use social communication support strategies did not yield significant child outcomes. Future studies should examine possible sources for the lack of main effects including unexpected differences in linguistic features of caregiver input, changes in control group caregiver behavior, and insufficient intervention dosage.

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.

Speeded Matching (SpM) is a new processing speed match-to-sample test within the NIH Toolbox Cognitive Battery. It was designed to developmentally extend feasibility to younger children or individuals with intellectual or developmental disabilities (IDD). SpM reduces cognitive demands to tapping an identical match as opposed to judging and indicating whether two stimuli are identical. In this study, we piloted SpM among 148 participants with fragile X syndrome, Down syndrome, or other intellectual disabilities (chronological age mean = 17.8 years, sd = 5.4; nonverbal mental age mean = 65 months, sd = 19.4). SpM had a high feasibility (96%) and internal consistency (rxx = 0.98). It converged well with other measures of processing speed, fluid cognition, and nonverbal mental age and diverged appropriately from crystallized cognitive skills. The correlation between nonverbal mental age and SpM in the IDD sample was not significantly different than the correlation between chronological age and SpM in a separate sample of 118 neurotypical children (age mean = 3.9 years sd = 0.8). This study provides initial evidence for the reliability and validity of the new SpM task, which may be appropriate as an outcome measure of processing speed for future clinical trials. It is more feasible than tasks designed for adults; it is brief, easy to administer, and engaging for young children and older individuals with lower mental ages associated with IDD.

Prior work proposed a shortened version of the Social Responsiveness Scale (SRS), a commonly used quantitative measure of social communication traits. We used data from 3031 participants (including 190 ASD cases) from the Environmental Influences on Child Health Outcomes (ECHO) Program to compare distributional properties and criterion validity of 16-item "short" to 65-item "full" SRS scores. Results demonstrated highly overlapping distributions of short and full scores. Both scores separated case from non-case individuals by approximately two standard deviations. ASD prediction was nearly identical for short and full scores (area under the curve values of 0.87, 0.86 respectively). Findings support comparability of shortened and full scores, suggesting opportunities to increase efficiency. Future work should confirm additional psychometric properties of short scores.

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.

BackgroundConcerns have been raised that scores on standard measures of autism spectrum disorder (ASD) symptoms may differ as a function of sex. However, these findings are hindered by small female samples studied thus far. The current study evaluated if, after accounting for age, IQ, and language level, sex affects ASD severity estimates from diagnostic measures among children with ASD.MethodsData were obtained from eight sources comprising 27 sites. Linear mixed-effects models, including a random effect for site, were fit for 10 outcomes (Autism Diagnostic Observation Schedule [ADOS] domain-level calibrated severity scores, Autism Diagnostic Interview-Revised [ADI-R] raw scores by age-based algorithm, and raw scores from the two indices on the Social Responsiveness Scale [SRS]). Sex was added to the models after controlling for age, NVIQ, and an indicator for language level.ResultsSex significantly improved model fit for half of the outcomes, but least square mean differences were generally negligible (effect sizes [ES]

AbstractObjective: Approximately 50% of children with autism exhibit severe tantrums, defiance, and/or aggression. We propose that the Disruptive Behavior Diagnostic Observation Schedule (DB-DOS)-a standardized clinical observation modeled after, and complementary to, the Autism Diagnostic Observation Schedule (ADOS)-could enhance earlier identification of disruptive behavior (DB) in autism populations and inform treatment planning. Methods: We adapted the DB-DOS for children with autism based on expert input and preliminary feasibility testing to accommodate varying cognitive and social communication capacities and increase the likelihood of observing DB in this population. Thereafter, we concurrently administered the modified DB-DOS and the ADOS to 12 children with autism aged 36 to 50 months. Results: Overall, children exhibited greater DB, especially behavioral regulation challenges, during the DB-DOS than during the ADOS. Conclusion: The use of a developmentally sensitive standardized observation tool that presses for DB to complement standardized observations such as the ADOS shows promise for enabling more precise research on targeted DB interventions. Such a tool holds promise as a reliable and efficient method of identifying comorbid DB disorders in the autism population.

Children with autism spectrum disorder benefit from early, intensive interventions to improve social communication, and parent-implemented interventions are a feasible, family-centered way to increase treatment dosage. The success of such interventions is dependent on a parent's ability to implement the strategies with fidelity. However, measurement of parent strategy use varies across studies. Most studies use one of two types of observational coding measures (macro- and micro-codes). Macro-codes are known for being efficient while micro-codes are known for being precise. This study evaluates the reliability and validity of the "NDBI-Fi," a macro-code, compared to a micro-code. Parent-child interaction videos for 177 participants were used to compare these measures. Results demonstrated that the "NDBI-Fi" had strong inter-rater reliability. It also had strong convergent validity with the micro-code after intervention. In addition, the "NDBI-Fi" was sensitive to change, and it demonstrated precision comparable to the micro-code. Furthermore, a novel scoring procedure detected differences in parents who learned different intervention strategy types. However, the "NDBI-Fi" did not demonstrate strong validity before intervention, particularly when measuring responsive intervention strategies. Taken together, findings support the use of the "NDBI-Fi" as an outcome measure, and future work should focus on continued development of valid pre-intervention macro-codes.

ObjectiveTo advance the science of cognitive outcome measurement for individuals with intellectual disability (ID), we established administration guidelines and evaluated the psychometric properties of the NIH-Toolbox Cognitive Battery (NIHTB-CB) for use in clinical research.MethodsWe assessed feasibility, test-retest reliability, and convergent validity of the NIHTB-CB (measuring executive function, processing speed, memory, and language) by assessing 242 individuals with fragile X syndrome (FXS), Down syndrome (DS), and other ID, ages 6 through 25 years, with retesting completed after 1 month. To facilitate accessibility and measurement accuracy, we developed accommodations and standard assessment guidelines, documented in an e-manual. Finally, we assessed the sensitivity of the battery to expected syndrome-specific cognitive phenotypes.ResultsAbove a mental age of 5.0 years, all tests had excellent feasibility. More varied feasibility across tests was seen between mental ages of 3 and 4 years. Reliability and convergent validity ranged from moderate to strong. Each test and the Crystallized and Fluid Composite scores correlated moderately to strongly with IQ, and the Crystallized Composite had modest correlations with adaptive behavior. The NIHTB-CB showed known-groups validity by detecting expected executive function deficits in FXS and a receptive language deficit in DS.ConclusionThe NIHTB-CB is a reliable and valid test battery for children and young adults with ID with a mental age of ≈5 years and above. Adaptations for very low-functioning or younger children with ID are needed for some subtests to expand the developmental range of the battery. Studies examining sensitivity to developmental and treatment changes are now warranted.

Prior work proposed a shortened version of the Social Responsiveness Scale (SRS), a commonly used quantitative measure of social communication traits. We used data from 3031 participants (including 190 ASD cases) from the Environmental Influences on Child Health Outcomes (ECHO) Program to compare distributional properties and criterion validity of 16-item "short" to 65-item "full" SRS scores. Results demonstrated highly overlapping distributions of short and full scores. Both scores separated case from non-case individuals by approximately two standard deviations. ASD prediction was nearly identical for short and full scores (area under the curve values of 0.87, 0.86 respectively). Findings support comparability of shortened and full scores, suggesting opportunities to increase efficiency. Future work should confirm additional psychometric properties of short scores. [This article was written on behalf of program collaborators for Environmental influences on Child Health Outcomes.]

Although norm-referenced scores are essential to the identification of disability, they possess several features which affect their sensitivity to change. Norm-referenced scores often decrease over time among people with neurodevelopmental disorders who exhibit slower-than-average increases in ability. Further, the reliability of norm-referenced scores is lower at the tails of the distribution, resulting in floor effects and increased measurement error for people with neurodevelopmental disorders. In contrast, the person ability scores generated during the process of constructing a standardized test with item response theory are designed to assess change. We illustrate these limitations of norm-referenced scores, and relative advantages of ability scores, using data from studies of autism spectrum disorder and creatine transporter deficiency.

Objective We describe the development of a new computer adaptive vocabulary test, Mobile Toolbox (MTB) Word Meaning, and validity evidence from 3 studies. Method Word Meaning was designed to be a multiple-choice synonym test optimized for self-administration on a personal smartphone. The items were first calibrated online in a sample of 7,525 participants to create the computer-adaptive test algorithm for the Word Meaning measure within the MTB app. In Study 1, 92 participants self-administered Word Meaning on study-provided smartphones in the lab and were administered external measures by trained examiners. In Study 2, 1,021 participants completed the external measures in the lab and Word Meaning was self-administered remotely on their personal smartphones. In Study 3, 141 participants self-administered Word Meaning remotely twice with a 2-week delay on personal iPhones. Results The final bank included 1363 items. Internal consistency was adequate to good across samples (ρxx = 0.78 to 0.81, p  < .001). Test–retest reliability was good (ICC = 0.65, p  < .001), and the mean theta score was not significantly different upon the second administration. Correlations were moderate to large with measures of similar constructs (ρ = 0.67–0.75, p  < .001) and non-significant with measures of dissimilar constructs. Scores demonstrated small to moderate correlations with age (ρ = 0.35 to 0.45, p  < .001) and education (ρ = 0.26, p  < .001). Conclusion The MTB Word Meaning measure demonstrated evidence of reliability and validity in three samples. Further validation studies in clinical samples are necessary. [ABSTRACT FROM AUTHOR]

Cognitive research with developmental samples requires improved methods that support large‐scale, diverse, and open science. This paper offers initial evidence to support the Mobile Toolbox (MTB), a self‐administered remote smartphone‐based cognitive battery, in youth populations, from a pilot sample of 99 children (Mage = 11.79 years; 36% female; 53% White, 33% Black or African American, 9% Asian, and 15% Hispanic). Completion rates (95%–99%), practice performance (96%–100%), internal consistency (0.60–0.98), and correlations with similar NIHTB measures (0.55–0.77) provide the first evidence to support the MTB in a youth sample, although there were some inconsistencies across measures. Preliminary findings provide promising evidence of the MTB in developmental populations, and further studies are encouraged. [ABSTRACT FROM AUTHOR]

Arranging Pictures is a new episodic memory test based on the NIH Toolbox (NIHTB) Picture Sequence Memory measure and optimized for self-administration on a personal smartphone within the Mobile Toolbox (MTB). We describe evidence from three distinct validation studies. In Study 1, 92 participants self-administered Arranging Pictures on study-provided smartphones in the lab and were administered external measures of similar and dissimilar constructs by trained examiners to assess validity under controlled circumstances. In Study 2, 1,021 participants completed the external measures in the lab and self-administered Arranging Pictures remotely on their personal smartphones to assess validity in real-world contexts. In Study 3, 141 participants self-administered Arranging Pictures remotely twice with a two-week delay on personal iOS smartphones to assess test-retest reliability and practice effects. Internal consistency was good across samples (ρxx =.80 to.85, p <.001). Test-retest reliability was marginal (ICC =.49, p <.001) and there were significant practice effects after a two-week delay (ΔM = 3.21 (95% CI [2.56, 3.88]). As expected, correlations with convergent measures were significant and moderate to large in magnitude (ρ =.44 to.76, p <.001), while correlations with discriminant measures were small (ρ =.23 to.27, p <.05) or nonsignificant. Scores demonstrated significant negative correlations with age (ρ = -.32 to −.21, p <.001). Mean performance was slightly higher in the iOS compared to the Android group (MiOS = 18.80, NiOS = 635; MAndroid = 17.11, NAndroid = 386; t(757.73) = 4.17, p <.001), but device type did not significantly influence the psychometric properties of the measure. Indicators of potential cheating were mixed; average scores were significantly higher in the remote samples (F(2, 850) = 11.415, p <.001), but there were not significantly more perfect scores. The MTB Arranging Pictures measure demonstrated evidence of reliability and validity when self-administered on personal device. Future research should examine the potential for cheating in remote settings and the properties of the measure in clinical samples. [ABSTRACT FROM AUTHOR]

Measurement invariance (MI) is a psychometric property of an instrument indicating the degree to which scores from an instrument are comparable across groups. In recent years, there has been a marked uptick in publications using MI in intellectual and developmental disability (IDD) samples. Our goal here is to provide an overview of why MI is important to IDD researchers and to describe some challenges to evaluating it, with an eye towards nudging our subfield into a more thoughtful and measured interpretation of studies using MI. [ABSTRACT FROM AUTHOR]

Autism spectrum disorder (ASD) and anxiety frequently co-occur. Research on the phenomenology and treatment of anxiety in ASD is expanding, but is hampered by the lack of instruments validated for this population. This study evaluated the self- and parent-reported Revised Child Anxiety and Depression Scale and the Multidimensional Anxiety Scale in Children2 among 46 youth with ASD. Internal consistency and test-retest reliability were acceptable, but inter-rater reliability was poor. Parent-child agreement was better for youth with higher IQs, less severe ASD symptoms, or more social cognitive skills. Convergent and divergent validity were acceptable. Demographic characteristics were considered as predictors of anxiety: they were unrelated to parent-report, but younger age and more severe ASD were related to increased self-reported anxiety.