2021
DOI: 10.1111/jir.12860
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Cognitive profile of young children with Williams syndrome

Abstract: Background There is very little research on the cognitive profile of young children with Williams syndrome (WS). Method The present study utilised the Differential Ability Scales – Second Edition to examine the early cognitive abilities of 22 young children with WS (aged 3.98 to 7.70 years, 10 male and 12 female participants). Results Overall, IQ ranged from 38 (severely impaired) to 81.00 (low average). Consistent with Mervis et al. who looked at an older sample, over half (59.08%) of our young WS sample show… Show more

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Cited by 12 publications
(6 citation statements)
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“…Neurodevelopmental characteristics include a cognitive profile characterised by an almost universal developmental delay (i.e., most individuals diagnosed with WS present intellectual disability or borderline IQ, although some individuals may present severe intellectual disability or, to the contrary, average intellectual ability; Martens et al, 2008; Mervis & Greiner de Magalhães, 2022; Mervis & John, 2010; Morris et al, 2020), and a phenotypic pattern of relative peaks and valleys based on the overall intellectual ability. Among the weaknesses, visuospatial construction skills stand out (e.g., handwriting and block construction), whilst, among the strengths, linguistic (e.g., phonological processing, vocabulary breadth or verbal short-term memory) and nonverbal reasoning skills are noticeable (Mervis & Greiner de Magalhães, 2022; Mervis & John, 2010; Miezah et al, 2021).…”
Section: Introductionmentioning
confidence: 99%
“…Neurodevelopmental characteristics include a cognitive profile characterised by an almost universal developmental delay (i.e., most individuals diagnosed with WS present intellectual disability or borderline IQ, although some individuals may present severe intellectual disability or, to the contrary, average intellectual ability; Martens et al, 2008; Mervis & Greiner de Magalhães, 2022; Mervis & John, 2010; Morris et al, 2020), and a phenotypic pattern of relative peaks and valleys based on the overall intellectual ability. Among the weaknesses, visuospatial construction skills stand out (e.g., handwriting and block construction), whilst, among the strengths, linguistic (e.g., phonological processing, vocabulary breadth or verbal short-term memory) and nonverbal reasoning skills are noticeable (Mervis & Greiner de Magalhães, 2022; Mervis & John, 2010; Miezah et al, 2021).…”
Section: Introductionmentioning
confidence: 99%
“…The latter has been an area of specific interest, in light of the sociability of people with WS and their remarkably increased interest in, and gaze preference for, faces 17 , 19 22 , even in infancy 20 . Considerable further research has clearly and consistently corroborated the presence of visuospatial construction deficits as a core cognitive feature of WS 13 , 18 , 23 27 . However, reports of face processing capacities have been more nuanced, with some documenting largely intact abilities in people with WS 18 , 28 32 , while others have identified altered performance 26 , 33 35 , often varying as a function of specific task demands and stimulus types.…”
Section: Introductionmentioning
confidence: 94%
“…The WS physical phenotype includes distinctive and easily recognizable facial features, cardiovascular anomalies, endocrine-metabolic alterations, hoarse voice, and sound sensitivities (hyperacusis, odynacusis, auditory allodynia, and auditory fascinations) (Kozel et al, 2021). Individuals with WS show a specific neurocognitive profile characterized by a complex pattern of strengths and weaknesses (Karmiloff-Smith et al, 2003b;Vicari et al, 2004;Mervis and John, 2010;Hocking et al, 2015;Wuang and Tsai, 2017;Miezah et al, 2021) and they may show mild-to-moderate intellectual disability (Bellugi et al, 2000;Mervis et al, 2000). In general, deficits in visuospatial construction skills constitute a specific weakness (Mervis et al, 2000;Brown et al, 2003;Farran and Jarrold, 2003;Van der Geest et al, 2005;Porter and Coltheart, 2006), whereas auditory processing and face recognition are strengths in the WS profile (D'Souza et al, 2015;Miezah et al, 2021).…”
Section: Introductionmentioning
confidence: 99%