Vocabulary Abilities of Children With Williams Syndrome: Strengths, Weaknesses, and Relation to Visuospatial Construction Ability

Abstract

Purpose-This project was designed to identify relative strengths and weaknesses in vocabulary ability for children with Williams syndrome (WS) and to demonstrate the importance of stringent matching criteria for cross-group comparisons.Methods-Children with WS and typically developing (TD) children completed standardized assessments of intellectual and language ability. Children with WS also completed a visuospatial construction ability assessment.Results-Study 1: Concrete and relational vocabulary standard scores were significantly lower for 5 -7-year-olds with WS than for TD children. Children with WS earned significantly higher standard scores for concrete than for relational vocabulary. When groups were stringently matched for relational vocabulary size, children with WS did not evidence a specific weakness in spatial vocabulary. Study 2: Standard scores for relational vocabulary were similar to those for visuospatial construction ability for 5 -7-year-olds with WS. Study 3: 9 -11-year-olds with WS demonstrated very limited relational vocabulary ability; relational vocabulary ability at 5 -7 years was highly correlated with later relational language ability.Conclusions-Concrete vocabulary is a relative strength for children with WS; relational vocabulary ability is very limited and is at about the level of visuospatial construction ability. Accurate determination of group comparison results depends on stringent group matching. KeywordsWilliams syndrome; intellectual disability; language acquisition; vocabulary; visuospatial construction Williams syndrome (WS) is a neurodevelopmental disorder caused by the deletion of ~25 genes on chromosome 7q11.23 (Hillier et al., 2003;Osborne, 2006). The WS deletion, which has a prevalence of 1/7500 (Strømme, Bjørnstad, & Ramstad, 2003), results in a characteristic pattern of physical characteristics, including a distinctive facial appearance, cardiovascular disease (especially supravalvar aortic stenosis), connective tissue abnormalities, and growth deficiency (see Mervis & Morris, 2007 for a review). Individuals with WS typically have mild to moderate intellectual disability, but some individuals have low average to average intellectual ability and a smaller minority have severe intellectual disability. WS is associated with a specific cognitive profile, including relative strengths in verbal short term memory and in language and severe weakness in visuospatial construction (Mervis et al., 2000). The personality profile was one of the first diagnostic clues provided for WS (Bennett et al., 1978;Cassidy & Morris, 2002) and includes overfriendliness, gregariousness, and high levels of empathy, with an undercurrent of anxiety (Klein-Tasman & Mervis, 2003). The psychiatric profile includes ADHD, specific phobia, and generalized anxiety disorder beginning in early adolescence (Leyfer et al., 2006).WS came to the attention of researchers in the United States because of the pioneering work of Ursula Bellugi (e.g., Bates, 1990;Bellugi, Marks, Bihrle, & Sabo, 1988) arguin...

Neural representation of abstract and concrete concepts: A meta‐analysis of neuroimaging studies

“…How abstract and concrete concepts are represented in the brain is relevant to understanding language function in both healthy and clinical populations [Eviatar et al, 1990;Kuperberg et al, 2008;Mervis and John, 2008]. Behavioral differences in processing between abstract and concrete concepts have been well documented and referred to as the concreteness effect: concrete words are acquired earlier, and are remembered and recognized more rapidly than abstract words [Kroll and Merves, 1986;Schwanenflugel, 1991].…”

Facilitating complex shape drawing in Williams syndrome and typical development

“…Individuals with WS typically display mild to moderate learning difficulties (average IQ between 50 and 60) and an unusual cognitive profile comprising of relatively strong linguistic ability and poor visuo-spatial performance (Ewart et al, 1993;Ferrero et al, 2007;Mervis & John, 2008;Smoot, Zhang, Klaiman, Schultz, & Pober, 2005).…”

Perceiving and acting in depth in Williams syndrome and typical development

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