Profiles and development of adaptive behavior in children with Down syndrome

Dykens, Elisabeth M.; Hodapp, Robert M.; Evans, David W.

doi:10.3104/reprints.293

Cited by 125 publications

(132 citation statements)

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“…These findings are consistent with what is observed in other populations of children with genetic syndromes [e.g., Dykens et al, 1994;Carter et al, 1998;Mervis et al, 2002]. The pattern of adaptive behavior strengths and weaknesses in this study matched what was hypothesized, reflecting the domain of motor skills as a relative weakness for children with AS.…”

Section: Discussionsupporting

confidence: 92%

Cognitive and adaptive behavior profiles of children with Angelman syndrome

Peters

Goddard‐Finegold

Beaudet

et al. 2004

American J of Med Genetics Pt A

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Angelman syndrome (AS) is a neurodevelopmental disorder caused by maternal deficiency of the UBE3A gene that encodes E6-AP ubiquitin-protein ligase. Expression of the UBE3A gene from the maternal chromosome is essential to prevent AS. AS is characterized by severe mental retardation, ataxia, and a defined behavioral pattern characterized mainly by happy/sociable disposition. This study used the Bayley Scales of Infant Development and the Vineland Adaptive Behavior Scales to examine the cognitive abilities and adaptive behavior of children (n = 20) with the four known molecular classes of AS, including patterns of strengths and weaknesses across adaptive behavior domains, and the relationship between adaptive behavior and overall cognitive abilities. Cognitive skills fell within the severe to profound range of mental deficiency. Differences in cognitive skills according to genetic subtype only partially supported previous research and suggest that there is overlap in abilities across genetic subtypes of AS. Adaptive behavior skills were also significantly delayed, with participants demonstrating a significant strength in socialization, and a weakness in motor skills. Strong, positive correlations emerge between cognitive ability scores and adaptive behaviors scores. These results provide further delineation of a cognitive/behavioral phenotype in AS.

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Section: Discussionsupporting

confidence: 92%

Cognitive and adaptive behavior profiles of children with Angelman syndrome

Peters

Goddard‐Finegold

Beaudet

et al. 2004

American J of Med Genetics Pt A

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“…Adaptive behavior is typically measured in terms of one' s ability to communicate and socialize with others, navigate the daily environment with tasks such as dressing oneself, and use coping mechanisms. As an example, on the Vineland Adaptive Behavior Scales, a common measure of adaptive behavior, children with Down syndrome tend to score lower in communication than in socialization, daily living, or motor skills, 4 whereas children with autism or PraderWilli syndrome show a strength in daily living skills and a weakness in socialization skills. 5 Fragile X syndrome, the phenotypic result of a mutation in the FMR1 gene, is the leading known inherited cause of intellectual disability and the leading known single-gene risk factor for autism spectrum disorder, affecting 1 in 4000 boys and 1 in 8000 girls.…”

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confidence: 99%

Longitudinal Profiles of Adaptive Behavior in Fragile X Syndrome

Klaiman

Quintin

et al. 2014

Pediatrics

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WHAT'S KNOWN ON THIS SUBJECT: To date, studies of adaptive behavior in fragile X syndrome have focused on particular age points, either longitudinally or cross-sectionally across a broad age spectrum. Studies have shown variable patterns in adaptive behavior among people with fragile X syndrome. WHAT THIS STUDY ADDS:This study fills a critical gap in knowledge about the profile of adaptive behavior across childhood, adolescence, and young adulthood in fragile X syndrome. This study is the first to incorporate longitudinal data from an age-matched typically developing group. abstract OBJECTIVE: To examine longitudinally the adaptive behavior patterns in fragile X syndrome.METHOD: Caregivers of 275 children and adolescents with fragile X syndrome and 225 typically developing children and adolescents (2-18 years) were interviewed with the Vineland Adaptive Behavior Scales every 2 to 4 years as part of a prospective longitudinal study. RESULTS:Standard scores of adaptive behavior in people with fragile X syndrome are marked by a significant decline over time in all domains for males and in communication for females. Socialization skills are a relative strength as compared with the other domains for males with fragile X syndrome. Females with fragile X syndrome did not show a discernible pattern of developmental strengths and weaknesses.CONCLUSIONS: This is the first large-scale longitudinal study to show that the acquisition of adaptive behavior slows as individuals with fragile X syndrome age. It is imperative to ensure that assessments of adaptive behavior skills are part of intervention programs focusing on childhood and adolescence in this condition. Pediatrics 2014;134:315-324 AUTHORS:

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“…The expressive language and working memory deficits are more marked than comprehension skills, nonverbal visual problem solving skills, daily living, or social skills (Dykens, Hodapp & Evans, 1994).…”

Section: The Developmental Emergence Of Speech and Language Skillsmentioning

confidence: 91%