Preliminary psychometric properties of an inhibition task in young children with Down syndrome.

Background: Executive function (EF) skills are important treatment targets for people with Down syndrome (DS); however, few EF measures have been evaluated for use with young children in this population. Methods: The present study evaluated preliminary psychometric properties of a measure of the EF component of inhibition. Participants were 73 children with DS between 2.5 and 8.67 years old who completed an adapted ability to delay task using a desirable toy. Results: Across two separate trials, latencies to touch the toys were significantly correlated. Latencies increased overall with chronological and mental age, with caveats for the youngest and oldest participants. Conclusion: Findings suggest that an adapted prohibition task is an appropriate method of measuring inhibition for children with DS between 4 and 7 years old, though many children in this chronological age range are at early stages of acquiring this skill set.

Longitudinal Predictors of Neurodevelopmental Outcomes in Children with Down Syndrome.

This study examined longitudinal predictors of neurodevelopmental outcomes in children with Down syndrome (DS). Participants were assessed at Wave 1 during infancy on measures of looking behavior and caregivers provided infant sensory ratings. At Wave 2, child-age participants completed a developmental assessment and caregivers provided ratings of executive function, ADHD symptoms, and autism symptoms. Longer looking durations and greater sensory dysregulation during infancy were predictive of higher ADHD symptom ratings and other neurodevelopmental outcomes during childhood. The findings suggest that early indicators of neurodevelopmental dysregulation may be detectable during infancy in DS.

Psychometric evaluation of a working memory assessment measure in young children with Down syndrome.

BACKGROUND: Working memory involves the temporary storage and manipulation of information and is frequently an area of challenge for individuals with Down syndrome (DS). Despite the potential benefits of intervention, laboratory assessments of working memory that could capture intervention effects have not undergone rigorous evaluation for use with young children with DS. It is critical to evaluate assessments of working memory in young children with DS to ensure the reliable and accurate measurement of performance. AIM: This study evaluated an adapted laboratory measure of working memory for young children with DS 2-8 years old. METHOD: A self-ordered pointing task, the Garage Game, was administered to 78 children with DS (mean = 5.17 years; SD = 1.49). Adaptations were made to the task to minimize potential DS phenotype-related language and motor confounds. RESULTS: Results indicate that the measure is feasible, scalable, and developmentally sensitive, with minimal floor and practice effects for this population within this chronological and developmental age range. CONCLUSION: These findings demonstrate that the Garage Game is promising for use in studies of early working memory and treatment trials that aim to support the development of this critical dimension of executive functioning for children with DS.

Profiles of Caregiver-Reported Executive Function in Children with Down Syndrome.

Children with Down syndrome (DS) are at risk for challenges with aspects of executive function (EF). The current study explores whether heterogeneity in EF profiles can be detected within a sample of children with DS. Participants were 69 children with DS, ages 3-10 years (M = 6.23, SD = 1.91). T-scores from a caregiver-report measure of executive function were modeled using latent profile analysis, and auxiliary analyses examined the association between demographic and biomedical factors and probability of profile membership. The two-profile solution was the best fit for the sample, with a profile that involved elevated scores in working memory only ("Working Memory Only" profile; 43% of sample) and a "Multi-Domain" profile that involved elevated scores in planning, inhibition, and working memory (57%). The presence of congenital heart defects was associated with a higher probability of assignment to the Multi-Domain profile. Findings from this study contribute to the characterization of heterogeneous outcomes associated with DS.

Defining Expressive Language Benchmarks for Children with Down Syndrome.

Establishing expressive language benchmarks (ELBs) for children with Down syndrome (DS), as developed by Tager-Flusberg et al. for children with autism, is critically needed to inform the development of novel treatments, identify individualized treatment targets, and promote accurate monitoring of progress. In the present study, we assessed ELB assignments in three language domains (phonology, vocabulary, and grammar) for 53 young children with DS (CA range: 2.50-7.99 years) using standardized assessments. The participants were classified into one of four ELB levels (preverbal, first words, word combinations, and sentences) in each language domain. Associations with additional measures of language, chronological age, nonverbal cognition, and verbal short-term memory were considered. Analyses of individual ELB profiles indicated substantial variability across the three language domains, with six different patterns of variation across domains emerging. At the same time, the ELB categories were significantly associated with independent language measures and broader developmental domains. Moreover, ELB changes were observed in a small sample of children with DS reassessed 18-24 months after the initial visit. Results from the present study suggest the procedures outlined by Tager-Flusberg et al. for defining ELBs are a potentially useful tool for describing the language abilities of children with DS.

Patterns and predictors of adaptive skills in 2- to 7-year-old children with Down syndrome.

BACKGROUND: There is substantial variability in adaptive skills among individuals with Down syndrome. Few studies, however, have focused on the early developmental period or on the potential sources of variability in adaptive skills. This study characterizes adaptive skills in young children with Down syndrome and investigates child characteristics associated with adaptive skills. METHODS: Participants were 44 children with Down syndrome ranging in age from 2.50 to 7.99 years (M = 4.66 years, SD = 1.46). The Vineland Adaptive Behavior Scales-3 (VABS-3) Comprehensive Interview Form was used to assess adaptive behavior in the three core domains: socialization, daily living, and communication skills. Caregivers also reported on motor skills and autism spectrum disorder symptoms. Child cognitive abilities were assessed. RESULTS: Analyses comparing mean standard score performance across the three VABS-3 core domains demonstrated significant differences between all pairs of domains, resulting in a group-level pattern of socialization > daily living > communication skills. At the individual level, 10 different patterns of relative strength and weakness were identified, with only 18% of participants evidencing significant differences between adaptive skill domain standard scores corresponding to the group-level pattern of significant differences. Child characteristics (cognitive abilities, motor skills, and autism spectrum disorder symptoms) were significantly associated with VABS-3 adaptive domain standard scores. CONCLUSION: These findings underscore the importance of individualizing intervention programs focused on improving the adaptive skills of young children with Down syndrome based on consideration of the child's relative adaptive strengths and weaknesses.

Early Regulatory Skills and Social Communication Development in Infants with Down Syndrome.

Children with Down syndrome (DS) demonstrate substantial variability in communication and language outcomes. One potential source of variability in this skill area may be early regulatory function. Characterizing the early link between regulatory function and early social communication may benefit infants with DS at risk of difficulties with social communication and language skill acquisition. Forty-three infants with DS were assessed at two time points, six months apart. At Time 1, the average chronological age was 9.0 months (SD = 3.9) and caregivers completed the Infant Behavior Questionnaire-Revised (IBQ-R) to assess regulatory function. Six months later, caregivers rated infant communication at the second visit using the Communication and Symbolic Behavior Scales Infant Toddler Checklist (CSBS-ITC). Infant developmental level was assessed at both visits using the Bayley Scales of Infant and Toddler Development, Third Edition and caregivers reported on developmental history and biomedical comorbidities. Infant regulatory function at Time 1 predicted social communication outcomes at Time 2, six months later. Findings from this study suggest that elevated risk for pronounced communication challenges may be detectable as early as infancy in DS.

Sensory Processing and Maladaptive Behavior: Profiles Within the Down Syndrome Phenotype.

Aim: Sensory processing impairments are well characterized in children with neurodevelopmental disorders, particularly autism, and have been associated with maladaptive behaviors. However, little is known regarding sensory processing difficulties within Down syndrome, or how these difficulties may influence maladaptive behavior. This study aims to characterize sensory processing difficulties within the Down syndrome phenotype and determine the influence of processing difficulties on maladaptive behavior. Methods: To explore this issue, we administered the Short Sensory Profile and the Developmental Behavior Checklist to parents or primary caregivers of young children with DS (N = 49; M nonverbal mental age (NVMA) = 30.92 months (SD = 12.30); M chronological age (CA) = 67.04 (SD = 25.13). Results: Results indicated that Low Energy/Weak, Under-responsive/Seeks Sensation, and Auditory Filtering were the areas of greatest sensory regulation difficulty, and that Self-Absorbed behavior and Disruptive/Antisocial behavior were elevated areas of maladaptive behavior. Multivariate regression analyses indicated that Under-responsive/Seeks Sensation was the only sensory regulation domain significantly associated with Self-Absorbed and Disruptive/Antisocial behavior. Conclusion: Findings indicate a consistent pattern of sensory processing impairments and associations with maladaptive behavior in children with DS. Implications for interventions are discussed.

Exploratory behavior and developmental skill acquisition in infants with Down syndrome.

Infants learn about objects by exploring them. Typically developing infants actively explore objects through visual, manual, and oral modalities. Attenuated exploratory behavior has been observed in various neurodevelopmental disorders, including Down syndrome (DS), presumably limiting learning options. However, a direct link between exploration and overall developmental functioning has not been characterized. This study used a Latent Profile Analysis framework to examine within-syndrome variability in exploratory behavior in infants with DS and the developmental correlates of different exploratory behavior profiles. Participants were 45 infants with DS (CA = 9.58 months; SD = 3.62) who completed an object exploration activity and the Bayley Scales of Infant Development-III (BSID-III; Bayley, 2006). Exploration behavior was coded for the percentage of time engaged in visual, manual, and oral exploration. Results indicated that a 2-profile solution provided the best model fit for exploratory behavior, yielding profiles that represented either an Active (57.78% of the sample) or a Passive Exploratory (42.22% of the sample) profile. The Active Exploratory profile was associated with significantly higher age equivalent scores on the BSID-III Cognitive, Communication, and Motor domains than the Passive Exploratory profile. Other factors, such as sex and biomedical risk factors, were not associated with exploratory profiles. These findings offer a more nuanced understanding of early within-syndrome heterogeneity in DS, and demonstrate that impoverished early exploratory behavior may serve as an important indicator of increased risk for more pronounced developmental delays in DS.

Performance and Ratings Based Measures of Executive Function in School-Aged Children with Down Syndrome.

We examined performance-based laboratory tasks and ratings-based assessments of executive function (EF) in school-aged children with Down syndrome and mental-age matched peers along with adaptive functioning. METHODS: A battery of assessments including EF laboratory tasks was collected. RESULTS: The DS group performed both working memory/inhibition and planning laboratory tasks with significant challenges. Moderate correlations were evident only between some laboratory tasks and ratings-based EF domains. However, ratings-based EF better predicted adaptive function than performance on an EF laboratory-based task. CONCLUSIONS: Findings underscore the need to address early and targeted EF intervention in children with DS.

Impact of maladaptive behavior on school function in Down syndrome.

BACKGROUND/AIMS: Individuals with Down syndrome (DS) are at an increased risk for certain maladaptive behaviors. This study characterized maladaptive behavior in school-aged children with DS and examined the extent to which maladaptive behaviors are associated with school function. METHODS AND PROCEDURES: Participants were 24 students with DS [mean nonverbal mental age (NVMA)=43.83months; mean chronological age (CA)=77.58months] who completed the Leiter Scales of Performance- Revised (Leiter-R; Roid & Miller, 1997). Their teachers completed the Behavior Assessment Scales for Children Version 2 (Reynolds & Kamphaus, 2004), and the School Function Assessment (Coster et al., 1998), measures of maladaptive and adaptive behavior as observed in the school setting. RESULTS: Findings reveal a maladaptive behavior profile of elevated areas including Aggression, Attention Problems, and Somatization. When examining the association between maladaptive behavior and school function, multivariate regression results indicated a significant association between Aggression and Compliance, and Attention Problems and Task Completion. CONCLUSIONS AND IMPLICATIONS: Results underscore the importance of developing training for educators regarding the potential impact of maladaptive behavior on school function for students with Down syndrome.

Everyday executive functions in Down syndrome from early childhood to young adulthood: evidence for both unique and shared characteristics compared to youth with sex chromosome trisomy (XXX and XXY).

Executive functions (EF) are thought to be impaired in Down syndrome (DS) and sex chromosome trisomy (Klinefelter and Trisomy X syndromes; +1X). However, the syndromic specificity and developmental trajectories associated with EF difficulties in these groups are poorly understood. The current investigation (a) compared everyday EF difficulties in youth with DS, +1X, and typical development (TD); and (b) examined relations between age and EF difficulties in these two groups and a TD control group cross-sectionally. Study 1 investigated the syndromic specificity of EF profiles on the Behavior Rating Inventory of Executive Function (BRIEF) in DS (n = 30), +1X (n = 30), and a TD group (n = 30), ages 5-18 years. Study 2 examined age effects on EF in the same cross-sectional sample of participants included in Study 1. Study 3 sought to replicate Study 2's findings for DS by examining age-EF relations in a large independent sample of youth with DS (n = 85) and TD (n = 43), ages 4-24 years. Study 1 found evidence for both unique and shared EF impairments for the DS and +1X groups. Most notably, youth with +1X had relatively uniform EF impairments on the BRIEF scales, while the DS group showed an uneven BRIEF profile with relative strengths and weaknesses. Studies 2 and 3 provided support for fairly similar age-EF relations in the DS and TD groups. In contrast, for the +1X group, findings were mixed; 6 BRIEF scales showed similar age-EF relations to the TD group and 2 showed greater EF difficulties at older ages for +1X. These findings will be discussed within the context of efforts to identify syndrome specific cognitive-behavioral profiles for youth with different genetic syndromes in order to inform basic science investigations into the etiology of EF difficulties in these groups and to develop treatment approaches that are tailored to the needs of these groups.

Object-related generativity in children with Down syndrome.

Children with Down syndrome (DS) show challenges in some aspects of goal-directed behavior when compared to developmentally matched children (Daunhauer et al., 2014; Lee et al., 2011), particularly in the area of goal-directed action on objects (Fidler et al., 2005a,b). In this study, we examined one aspect of goal-directed action on objects, object-related generativity, in school-aged children with DS (n = 52), a developmentally matched group of children with intellectual disability, but not Down syndrome (DD; n = 21), and a group of chronologically younger, but developmentally matched typically developing children (TD; n = 34). We administered the Leiter-R, the Oral and Written Language Scales (OWLS), and an Object-Related Generativity Task, which involved 2 min of unstructured play with a variety of objects that have divergent usages. Children with DS generated significantly fewer instances of initiating actions on new objects than children in both comparison groups, were less likely to produce novel functional action on any object (new or familiar) than TD children, and they showed fewer instances of novel functional object engagement with new objects overall than TD children. Frequency of acts on new objects in DS was associated with Leiter-R Form Completion and Repeated Patterns Raw Scores and OWLS Listening Comprehension Raw Scores. These findings contribute to the growing knowledge base regarding goal-directed behavior and self-regulation in individuals with Down syndrome. Implications for education and intervention are discussed.

Profiles of everyday executive functioning in young children with down syndrome.

We investigated executive functioning (EF) in children with Down syndrome (DS; n  =  25) and typically developing (TD) children matched for mental age (MA; n  =  23) using the Behavior Rating Inventory of Executive Function-Preschool. We sought to (1) compare children with DS to a developmentally matched control group, and (2) to characterize the EF profile of children with DS. Across teacher and parent reports, significant deficits in working memory and planning were observed in the DS group. Parents, but not teachers, of children with DS also reported difficulties in inhibitory control relative to the comparison group. Results extend earlier findings regarding EF impairments in children with DS. The complementary role inhibitory control may play in this profile is discussed.

School function in students with Down syndrome.

People with Down syndrome (DS) are predisposed to specific areas of relative developmental strength and challenge, but it is unclear whether and how this profile affects participation in school and community settings. In this study we characterized the nature of school participation and performance of functional tasks in the school context for 26 elementary students with DS (mean age = 7.86 yr; standard deviation = 1.75). Students participated in assessments of cognitive status and language development. Their teachers completed the School Function Assessment (Coster, Deeney, Haltiwanger, & Haley, 1998) questionnaire and a standardized questionnaire on executive functioning (EF). Students demonstrated a pronounced pattern of assistance- and adaptation-related needs across various domains of school function. The strongest predictor of school function was EF skills, as reported by teachers (adjusted R² = .47, p = .003). Findings from this study should inform future intervention and school-related planning for elementary school students with DS.

The down syndrome behavioral phenotype: implications for practice and research in occupational therapy.

ABSTRACT Down syndrome (DS) is the most common chromosomal cause of intellectual disability. The genetic causes of DS are associated with characteristic outcomes, such as relative strengths in visual-spatial skills and relative challenges in motor planning. This profile of outcomes, called the DS behavioral phenotype, may be a critical tool for intervention planning and research in this population. In this article, aspects of the DS behavioral phenotype potentially relevant to occupational therapy practice are reviewed. Implications and challenges for etiology-informed research and practice are discussed.

Play and cognition among young children reared in an institution.

The relationship between cognitive functioning and play behaviors of children residing in an orphanage was examined. Twenty-six young children (15 boys) between 10 and 38 months of age participated. More developmentally competent play behaviors were highly related to better performance on cognitive functioning as measured by the Bayley Scales of Infant Development (r = .78, p < or = .01). The participants' play behaviors were less competent compared to previous data for children of the same chronological age living at home on four out of five levels of symbolic play (p < or = .05). However, when compared to data for children living at home with similar cognitive development, the participants demonstrated no significant differences in symbolic play (p > or =.05). The findings suggest that the relationship between thinking and playing is robust, even in an institutional environment. Additionally, practitioners assessing children from institutions who cannot engage in standardized testing may consider observing play behaviors to make an initial estimate of developmental status.

Effects of caregiver-child interactions on play occupations among young children institutionalized in Eastern Europe.

OBJECTIVE: We investigated whether children institutionalized in an orphanage would engage in more developmentally competent play with their caregivers as opposed to playing alone and whether specific qualities of caregiver-child interactions were associated with more developmentally competent play. METHOD: Twenty-six children, ages 10 to 38 months, participated in independent play sessions and in a play session with a caregiver. Interrater reliability for coding play performance was established using the weighted kappa statistic (M = .82). Twelve pediatric experts rated both child and caregiver behaviors for the interactive sessions (mean effective reliability with intraclass correlations = .89). RESULTS: The children demonstrated more developmentally competent play when interacting with a caregiver than when playing alone, t (25) = -1.88, p < .04, one-tailed. The effect size was moderate (d = .53). Longer periods of institutionalization were associated with less improvement in play performance from independent to interactive play sessions (r = -.51, p < .01). Successful engagement for the child was associated with having a caregiver who provided more structure and assistance and who was directive and encouraging (r = .82, .75, .75, and .64, respectively). CONCLUSION: Caregivers facilitated more developmentally competent participation in play with children residing in an orphanage, despite the fact that these interactions occurred in an environment vulnerable to many challenges not typical of an exclusive caregiver-child relationship. Findings are discussed in the context of environmental challenges and occupational therapy practice.