Increasingly individuals with intellectual and developmental disabilities, including Down syndrome, are being targeted for clinical trials. However, a challenge exists in effectively evaluating the outcomes of these new pharmacological interventions. Few empirically evaluated, psychometrically sound outcome measures appropriate for use in clinical trials with individuals with Down syndrome have been identified. To address this challenge, the NIH assembled leading clinicians and scientists to review existing measures and identify those that currently are appropriate for trials; those that may be appropriate after expansion of age range addition of easier items, and/or downward extension of psychometric norms; and areas where new measures need to be developed. This paper focuses on measures in the areas of cognition and behavior.
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.
The current study describes everyday executive function (EF) profiles in young children with Down syndrome. Caregivers of children with Down syndrome (n = 26; chronological ages = 4-10 years; mental ages = 2-4 years) completed the Behavior Rating Inventory of Executive Function-Preschool (BRIEF-P; G. A. Gioia, K. A. Espy, & P. K. Isquith, 2003), a caregiver report measure of everyday/functional EF skills in multiple domains. On the BRIEF-P, elevations were noted on a global EF composite as well as the Working Memory and Plan/Organize scales in particular (relative to norms developed for typically developing children of a similar mental age). These results suggest a specific pattern ofEF weaknesses in young children with Down syndrome, consistent with the extant literature that has focused primarily on older individuals who have been tested using laboratory EF tasks.
Objective
We assessed the prevalence of autism spectrum disorders (ASD) and screening test characteristics in children with Down syndrome.
Method
Eligible children born in a defined geographic area between January 1, 1996, and December 31, 2003, were recruited through a population-based birth defects registry and community outreach, then screened with the modified checklist for autism in toddlers or social communication questionnaire, as appropriate. Screen-positive children and a random sample of screen-negative children underwent developmental evaluation.
Results
We screened 123 children (27.8% of the birth cohort). Mean age was 73.4 months (range, 31–142). Compared to screen-negative children, screen-positive children had similar sociodemo-graphic characteristics but a lower mean developmental quotient (mean difference: 11.0; 95% confidence interval: 4.8–17.3). Weighted prevalences of autistic disorder and total ASD were 6.4% (95% confidence interval [CI]: 2.6%–11.6%) and 18.2% (95% CI: 9.7%–26.8%), respectively. The estimated minimum ASD prevalence, accounting for unscreened children, is 5.1% (95% CI: 3.3%–7.4%). ASD prevalence increased with greater cognitive impairment. Screening test sensitivity was 87.5% (95% CI: 66.6%–97.7%); specificity was 49.9% (95% CI: 37.0%–61.4%).
Conclusion
The prevalence of ASD among children with Down syndrome aged 2 to 11 years is substantially higher than in the general population. The modified checklist for autism in toddlers and social communication questionnaire were highly sensitive in children with Down syndrome but could result in many false positive tests if universal screening were implemented using current algorithms. Research needs include development of specific ASD screening algorithms and improved diagnostic discrimination in children with Down syndrome. Timely identification of these co-occurring diagnoses is essential so appropriate interventions can be provided.
-Background:Though the Down syndrome behavioural phenotype has been described as involving relative strengths in visuo-spatial processing and sociability, and relative weaknesses in verbal skills and motor planning, the early emergence of this phenotypic pattern of strengths and weaknesses has not yet been fully explored. Method: In this study, we compared the performance of eighteen 2 to 3-year-olds with Down syndrome to an MA-matched comparison group of nineteen 2 to 3-year-olds with mixed developmental disabilities, and an MA-matched comparison group of 24 children with typical development on two developmental measures: the Mullen Scales of Early Learning and the Vineland Adaptive Behaviour Scales. Results: While the specificity of the Down syndrome profile was (for the most part) not yet evident, results showed that toddlers with Down syndrome in this study did show emerging areas of relative strength and weakness similar to that which has been described in older children and young adults with Down syndrome. This pattern included relatively stronger social skills, weaker expressive language, and poor motor coordination. When this pattern of strengths and weaknesses was compared to the developmental profiles of the two comparison groups, socialisation strengths differentiated the Down syndrome group from the mixed developmental disabilities group.
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