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

Rebecca M Kirchner,Marilee A Martens,Rebecca R Andridge

doi:10.3389/fpsyg.2016.00598

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.

Highlights

Williams syndrome (WS) is a neurodevelopmental disorder caused by a deletion of approximately 26–28 genes on chromosome 7 (7q11.23; Peoples et al, 2000)
Thirty-one percent (31.3%) of parents reported that their infant/toddler had sleeping problems, 58.3% reported feeding difficulties, and 42.9% reported that their infant/toddler needed a special diet, which was most commonly the utilization of supplemental nutrition
Forty-six percent (46%) reported that their child avoided certain sounds, 46% reported that their child was attracted to certain sounds, and nearly 54% reported that their child was afraid of certain sounds

Summary

Introduction

Williams syndrome (WS) is a neurodevelopmental disorder caused by a deletion of approximately 26–28 genes on chromosome 7 (7q11.23; Peoples et al, 2000) This rare syndrome affects 1 in 7,500 to 1 in 20,000 individuals (Wang et al, 1997; Strømme et al, 2002) and is genetically confirmed via florescence in situ hybridization or microarray analysis (Lowery et al, 1995). Research has demonstrated that this oversensitivity is the most severe during early childhood, and declines as the child ages (Gothelf et al, 2006). Cardiovascular abnormalities are another common characteristic of individuals with WS, and occur in 82% of individuals (Collins et al, 2010). The most commonly occurring cardiac abnormalities are supravalvular aortic stenosis and peripheral

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