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…

In this study, we focus on Rubinstein-Taybi syndrome (RTS) to explore the associations between executive function deficits and repetitive behaviors. Thirty individuals with RTS completed direct assessments of inhibition, working memory and set-shifting. Informants completed repetitive behavior and executive function questionnaires. Repetitive…

Variation in the "FMR1" gene may affect aspects of cognition, such as executive function and memory. Environmental factors, such as stress, may also negatively impact cognitive functioning. Participants included 1,053 mothers of children with and without developmental disabilities. Participants completed self-report measures of executive…

People with Down syndrome (DS) commonly experience challenges with sleep, executive functioning, everyday memory, and symptoms of attention deficit hyperactivity disorder (ADHD). A path analysis was conducted to determine if executive function mediated the relationship between sleep problems and both everyday memory and ADHD symptoms. Parents of…

This study evaluated the appropriateness of scoring the Behavior Rating Inventory of Executive Function--Preschool (BRIEF-P) using age-equivalent scores generated from multiple measures of cognition and language among school-age children with Down syndrome (DS). Subscale T scores for 95 children with DS were contrasted using standard scoring on…

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,…

The block design task (BDT) is a visuospatial measure that individuals with Williams syndrome (WS) perform poorly on. However, it is unclear what underlies their impaired performance. This study investigated whether poorer performance is a result of visuospatial difficulties, executive function (EF) difficulties, atypical looking strategies, or a…

The current study evaluates the concurrent relationship between parent ratings of executive functioning and maladaptive behavior among children and adolescents with Down syndrome and then repeats this evaluation using teacher reports. Parents and teachers of 63 school-age children with Down syndrome rated the child's executive functioning…

A subset of mothers who carry the "FMR1" premutation may express a unique phenotype. The relationship between the "FMR1" phenotype and mother-child interaction in families with fragile X-associated disorders has not been well characterized, despite the importance of high-quality mother-child interaction for child development.…

Adaptive behavior is a critical metric for measuring outcomes in those with autism spectrum disorder (ASD). Executive function skills predict adaptive behavior in youth with ASD with average or higher IQ; however, no study has examined this relationship in ASD with lower IQ (IQ = 75). The current study evaluated whether executive function…

Executive function (EF) decline is a consistent early sign of Alzheimer's disease (AD) among adults with Down syndrome (DS), which means that baseline measures of EF for individuals with DS are vital to allow detection of meaningful decline. We developed a framework to extract measures of three core components of EF (memory updating, inhibitory,…

The current study evaluates the psychometric properties of the Behavior Rating Inventory of Executive Function (BRIEF) with children with Down syndrome. Caregivers of 84 children with Down syndrome rated their child's behavior with the BRIEF. Teacher ratings were obtained for 57 children. About 40% of children with Down syndrome were reported by…

Longitudinal growth modeling was utilized to examine adaptive behavior over eight years across the three time points (i.e., ages 2-10). Seventy-six parents completed the Vineland Adaptive Behavior Scales interviews of adaptive behavior. Child participants completed standardized developmental testing and an executive function task in toddlerhood…

The present investigation explored long-term relationships of behavioral symptoms of adolescents and adults with developmental disabilities with the mental health of their mothers. Fragile X premutation carrier mothers of an adolescent or adult child with fragile X syndrome (n = 95), and mothers of a grown child with autism (n = 213) were…

This longitudinal study examined pragmatic language in boys and girls with Down syndrome (DS) at up to three time points, using parent report, standardized and direct assessments. We also explored relationships among theory of mind, executive function, nonverbal mental age, receptive and expressive vocabulary, grammatical complexity, and pragmatic…