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Adaptive Behavior and Development of Infants and Toddlers with Williams Syndrome.

ABSTRACT: Williams syndrome (WS) is a neurodevelopmental disorder that causes deficits in adaptive behavior, difficulties eating and sleeping, cognitive delays, and delayed development. Although researchers have conducted characterizations of children and adults with WS, less is known about young children with this disorder. This study characterizes the developmental and adaptive behavior features of 16 infants and toddlers with WS aged 3 months - 5 years. Data for this project was obtained from 2007 to 2014, and includes parent report data and standardized developmental testing. Thirty-one percent (31.3%) of parents reported that their infant/toddler with WS had sleeping problems and 58.3% reported feeding difficulties. Levels of adaptive behavior were in the Mildly Delayed range as measured by the Adaptive Behavior Assessment System, Second Edition. Self-care skills such as feeding or dressing oneself were significantly weaker than skills needed to function in the community, such as recognizing his/her home or throwing away trash. The difficulty with self-care skills is hypothesized to be related to the reported difficulties with eating and sleeping. Motor skills were significantly lower than both cognitive and language skills on the Bayley Scales of Infant and Toddler Development, Third Edition. The current study highlights the need for early intervention in these young children across all areas of development, particularly in self-care skills.

SUBMITTER: Kirchner RM

PROVIDER: S-EPMC4848290 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature

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Adaptive Behavior and Development of Infants and Toddlers with Williams Syndrome.

Kirchner Rebecca M RM Martens Marilee A MA Andridge Rebecca R RR

Frontiers in psychology 20160428

Williams syndrome (WS) is a neurodevelopmental disorder that causes deficits in adaptive behavior, difficulties eating and sleeping, cognitive delays, and delayed development. Although researchers have conducted characterizations of children and adults with WS, less is known about young children with this disorder. This study characterizes the developmental and adaptive behavior features of 16 infants and toddlers with WS aged 3 months - 5 years. Data for this project was obtained from 2007 to 2 ...[more]

PMID: 27199832

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Project description:Williams syndrome is a neurodevelopmental genetic disorder characterized by a unique phenotype, including mild to moderate intellectual disability and an uneven neuropsychological profile of relative strengths and weaknesses. Language structure components (i.e., phonology, morphosyntax, and vocabulary) have been considered an area of specific ability compared to pragmatic language use. However, research on phonological development in Williams syndrome is very scarce, and it suggests atypical patterns. Therefore, the aim of the present study was to explore the profiles of late phonological development in Spanish-speaking children, adolescents, and adults with Williams syndrome, based on the analysis of five classes of processes (Syllable Structure, Substitution, Omission, Assimilation, and Addition) in spontaneous speech. The phonological profiles of seven children (aged 3-8 years), and seven adolescents and young adults (aged 14-25 years) with Williams syndrome were compared with two normative groups of typically developing (TD) children at different stages of late phonological development (aged 3 and 5 years). The frequency of phonological processes in the group of children with Williams syndrome was similar to that of 3-year-old TD children, which suggests that they would be in the first stage of late phonological development (expansion stage). The group of older individuals with Williams syndrome showed a much lower frequency of processes, similar to that of 5-year-old TD children in the last stage of phonological development (resolution stage). However, their phonological processes appeared to be persistent and independent of chronological age. Furthermore, asynchronies in quantitative and qualitative profiles (relative frequency) indicated atypical and complex trajectories in late phonological development, which cannot be described as simply delayed or protracted. Remarkable individual differences were observed, especially in the group of adolescents and adults with Williams syndrome, although the majority of cases conformed to the modal profiles of their groups. A major tendency for Omission, including final consonant deletion, may be considered atypical and specific to Williams syndrome at all ages. The results of the present study raise the need for continued and appropriate phonological assessment and treatment for people with Williams syndrome across the lifespan.

Project description:STUDY OBJECTIVES:To examine the prevalence and correlates of nighttime awakenings and to explore the association between sleep and cognitive development in a community sample of infants and toddlers. METHODS:A total of 590 healthy infants (aged 2-11 months) and 512 toddlers (aged 12-30 months) from 8 provinces of China were assessed for their sleep and cognitive development. Data on sleep duration and nighttime awakenings were collected through the Brief Infant Sleep Questionnaire. Cognitive development was assessed by trained pediatricians using the Bayley Scales of Infant Development. RESULTS:Prevalence of no nighttime awakening, and nighttime awakening(s) for 1×/night, 2×/night, and ≥ 3×/night was 6.8%, 20.2%, 33.2%, and 39.3% in infants, and was 25.8%, 34.6%, 23.8%, and 15.8% in toddlers, respectively. Nighttime awakenings were generally associated with younger age, lower maternal education level, and being currently breastfed. In addition, nighttime awakenings were associated with being boys in toddlers. After controlling for potential confounders, infants with nighttime awakenings for 2×/night were found to have significantly higher Mental Development Index (MDI) score, as compared to those without and those with more frequent nighttime awakenings. However, toddlers with nighttime awakenings for ≥ 3×/night had significantly lower MDI, as compared to those with fewer nighttime awakenings. Total sleep duration was not associated with any developmental indices in both infants and toddlers. CONCLUSIONS:Frequent nighttime awakenings are associated with poor cognitive functions in toddlers. Meanwhile, a nonlinear association between nighttime awakenings and cognitive performance was found among infants. The findings provide a developmental context for the effect of sleep on cognitive abilities in young children. Further longitudinal studies and interventional studies on the effects of parent-based sleep-focused intervention on cognitive abilities among young children are warranted.

Project description:Interstitial deletions of 7q11.23 cause the well-known Williams-Beuren Syndrome (WBS), while duplication of the same region leads to duplication 7 syndrome (Dup7). Children with WBS share a distinct neurobehavioral phenotype including mild to severe intellectual disability, severely impaired visual spatial abilities, relatively preserved verbal expressive skills, anxiety problems, enhanced social motivation (i.e., hypersociable behaviors) and socio-communicative problems. Children with Dup7 syndrome exhibit some "inverted" features when compared to those of individuals with WBS, such as reduced social motivation and impairment of expressive language. Direct comparison of WBS and Dup7 represents a unique opportunity for the neurobehavioral characterization of the 7q11.23 section. However, most of the available data come from qualitative analysis between different studies. To the best of our knowledge, there are no studies directly comparing features of two matched samples of individuals with WBS and Dup7 syndromes. In this pilot study, we compare the adaptive functioning - measured with the Vineland Adaptive Behavior Scales, Second Edition - of two relatively small samples of children with molecularly confirmed diagnosis of WBS and Dup7 matched for IQ and chronological age, with a particular attention to socialization domain and expressive subdomain. Contrary to our assumption, we have not found any significant difference on socialization domain and expressive subdomains. This pilot investigation suggests that, when matched for chronological age and cognitive level, children with WBS and Dup7 share more similarities than expected. The inverted features that emerge in clinical settings on expressive language and social motivation seem not to differently interfere with the daily abilities to communicate and socialize with meaningful others during daily lives. Differences highlighted by previous undirected comparisons could be due to general and non-specific factors such as cognitive level, which is more severely impaired in individuals with WBS than Dup7. Implications for assessment and treatment are discussed.

Dataset Information

Adaptive Behavior and Development of Infants and Toddlers with Williams Syndrome.

Publications

Adaptive Behavior and Development of Infants and Toddlers with Williams Syndrome.

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