Anna J. Esbensen

Background: Identifying flexible and rigorous methods to administer assessments in Down syndrome research is critical for limiting participation burden. Tablet assessments have the potential to be presented remotely, but consistency between researcher and caregiver administrations is unknown. Method: Participants were 24 children with Down syndrome aged 10–17 years and their caregivers. Children completed two sessions: one administered by their caregiver and one administered by research staff. Tablet measures were selected from the NIH Toolbox and the Cambridge Neuropsychological Test Automated Battery (CANTAB). Results: There were no significant differences between child performance on researcher‐administered compared to caregiver‐administered NIH Toolbox Flanker, CANTAB RTI and CANTAB PAL measures. Floor effects impacted additional measures with high agreement (NIH Toolbox Pattern Comparison). Conclusions: Task completion did not differ across administrators, demonstrating evidence for parents' ability to facilitate assessments. Inconsistent agreement statistics warrant future work in larger samples and additional understanding of child tablet assessment performance.

Background: The Cat/Dog Stroop task is a modification of the original Stroop task as a measure of cognitive inhibition frequently used in studies of individuals with Down syndrome (DS). There is great heterogeneity in scoring mechanisms for this task and similar Stroop tasks, potentially impacting convergence of and interpretation of study findings. We review scoring mechanisms used with the Stroop task in the general population and those that are used among individuals with DS to evaluate the strengths and weaknesses of these scoring mechanisms in this population. Method: We reviewed the literature to identify current scoring mechanisms used for the original Stroop task and for the Cat/Dog Stroop task among individuals with DS. Using a sample of 146 children and adults with DS ages 6–29 years who completed the Cat/Dog Stroop, we independently modelled the identified scoring mechanisms. Scoring mechanisms were assessed for skew, kurtosis and normality as assessed through the Anderson–Darling test with the full sample and by age bands. Results: Eight distinct scoring mechanisms were identified that could be used with the conditions present in the Cat/Dog Stroop task. Six of these eight scoring mechanisms had been used in studies of individuals with DS, with the remaining two mechanisms not yet applied to Cat/Dog Stroop in individuals with DS. Of these scoring mechanisms, one mechanism consistently emerged across stratified age groups as approximating normality for the Cat/Dog Stroop task based on skewness, kurtosis and tests of normality in a sample of individuals with DS. Discussion: For consistency in how the Cat/Dog Stroop task is scored for children and adults with DS, we recommend that the number of correct responses in the incongruent condition should be divided by completion time in the incongruent condition. Additionally, for adults, incongruent completion time minus the congruent completion time or incongruent completion time divided by congruent completion time may also be appropriate.

Challenging behaviors, including noncompliance, aggression, hyperactivity, and impulsivity, are common among individuals with Fragile X Syndrome (FXS) and Down Syndrome (DS). To identify treatment needs specific to these populations, we conducted focus groups with caregivers and educators and used their input to adapt an evidence-based caregiver training program originally designed for caregivers of autistic children (i.e., The Research Units in Behavioral Intervention; RUBI). We then completed a feasibility trial in which five families of children with FXS and four families of children with DS completed a nine-session caregiver training program targeting behavioral principles, syndrome-specific information, and visual supports tailored to the unique needs of FXS or DS (adapted version of RUBI for non-autism developmental disabilities; RUBI-DD). The program demonstrated strong acceptability, with high caregiver satisfaction, 100% retention, and 100% session attendance. Across the combined sample, caregiver reports indicated significant improvements in irritability/aggression (F_(2,15.14) = 4.42, p = 0.03), lethargy/social withdrawal (F_(2,14.47) = 3.97, p = 0.04), stereotypies (F_(2,15.29) = 4.45, p = 0.03), hyperactivity (F_(2,15.14) = 6.51, p = 0.009), social inflexibility (F_(2,15.43) = 6.33, p = 0.01), demand-based noncompliance (F_(2,15.41) = 4.95, p = 0.02), and the impact of behavior on the family (F_(2,15.07) = 4.23, p = 0.04) following participation in RUBI-DD. Caregivers of children with FXS reported significant reductions in lethargy/social withdrawal (F_(2,8.000) = 6.256, p = 0.023) and hyperactivity (F_(2,8.000) = 12.497, p = 0.003) immediately post-treatment and upon 12-week follow-up (g = 1.153, p = 0.044, and g = 1.178, p = 0.003, respectively). Among families of children with DS, caregivers reported reductions in irritability and aggression (F_(2,5.047) = 14.073, p = 0.009) and improvements in the impact on the family (F_(2,6.000) = 5.489, p = 0.044) immediately post-treatment and at follow-up (g = 1.643, p = 0.016, and g = 0.448, p = 0.045, respectively). These findings support the feasibility, acceptability, and preliminary efficacy of RUBI-DD for children with FXS or DS.

Introduction: The Anxiety, Depression and Mood Scale (ADAMS), a mental health screening tool developed for individuals with intellectual disabilities, has yet to be evaluated in adults with Down syndrome. We included the ADAMS in a Dementia Protocol.Method: We reviewed the charts of 71 adults with Down syndrome seen in a specialty clinic and collected ADAMS data from our quality improvement project. We evaluated the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of the ADAMS at five cutoff points to identify optimal screening thresholds for adults with Down syndrome.Results: Cutoff points set at two points below the 75th percentile scores of the original ADAMS publication presented optimal sensitivity of 81.82%, specificity of 82.93%, PPV of 72.00% and NPV of 89.47%.Conclusion: For adults with Down syndrome, we suggest alternate thresholds, generally two points below the original ADAMS thresholds, to adequately capture mental health concerns.

This study explored how caregiver-reported executive functioning domains, assessed by the BRIEF2 at baseline, predicted behavioral challenges reported by caregivers using the CBCL six months later. The sample included 94 youth with Down syndrome, aged 6 to 18 years. Results of hierarchical regression analyses revealed that, after controlling for the effect of IQ, working memory significantly predicted inattentive behaviors, inhibition significantly predicted rule-breaking behaviors, and both inhibition and emotional control significantly predicted aggressive and externalizing behaviors. After controlling for baseline behaviors, only working memory persisted as a significant predictor of inattentive behaviors measured six months later. The findings are discussed by referring to the relevant theories and research and providing clinical implications and recommendations for further studies.

Background: The connection between sleep problems and behavioural challenges in individuals with Down syndrome (DS) has been a central focus of research across different age groups. However, uncertainty remains regarding this association, particularly when relying solely on daily‐based assessments. This study aimed to examine the relationship between sleep problems and behavioural challenges at both overall (macro) and daily (micro) levels among youth with DS.Methods: Sleep disturbances were measured in a sample of 65 youth with DS aged 6–17 years using the Children's Sleep Habits Questionnaire (CSHQ) and actigraph watches assessing sleep efficiency, sleep duration and wake after sleep onset. Behavioural challenges were evaluated through externalising and internalising subscales of the Child Behavior Checklist (CBCL) and of the Scales of Independent Behavior, Revised (SIB‐R).Results: The findings demonstrated that over a period of time, sleep problems are significantly associated with both externalising and internalising behaviours as measured by CSHQ and CBCL, even after accounting for the effects of IQ and SIB‐R Broad Independence. No significant correlations were observed on a daily basis over seven consecutive days, as measured by actigraphy and both externalising and internalising indices of SIB‐R.Conclusions: The results highlight the complexity of the sleep–behaviour relationship in DS, indicating that while chronic sleep issues impact long‐term behaviours, nightly variations do not predict immediate behavioural changes.

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.