Jo Van Herwegen

Small and large number processing in infants and toddlers with Williams syndrome.

Previous studies have suggested that typically developing 6-month-old infants are able to discriminate between small and large numerosities. However, discrimination between small numerosities in young infants is only possible when variables continuous with number (e.g. area or circumference) are confounded. In contrast, large number discrimination is successful even when variables continuous with number are systematically controlled for. These findings suggest the existence of different systems underlying small and large number processing in infancy. How do these develop in atypical syndromes? Williams syndrome (WS) is a rare neurocognitive developmental disorder in which numerical cognition has been found to be impaired in older children and adults. Do impairments of number processing have their origins in infancy? Here this question is investigated by testing the small and large number discrimination abilities of infants and toddlers with WS. While infants with WS were able to discriminate between 2 and 3 elements when total area was confounded with numerosity, the same infants did not discriminate between 8 and 16 elements, when number was not confounded with continuous variables. These findings suggest that a system for tracking the features of small numbers of object (object-file representation) may be functional in WS, while large number discrimination is impaired from an early age onwards. Finally, we argue that individual differences in large number processing in infancy are more likely than small number processing to be predictive of later development of numerical cognition.

Genetic and environmental vulnerabilities in children with neurodevelopmental disorders

One might expect that children with varying genetic mutations or children raised in low socioeconomic status environments would display different deficits. Although this expectation may hold for phenotypic outcomes in older children and adults, cross-syndrome comparisons in infancy reveal many common neural and sociocognitive deficits. The challenge is to track dynamic trajectories over developmental time rather than focus on end states like in adult neuropsychological studies. We contrast the developmental and adult approaches with examples from the cognitive and social domains, and we conclude that static models of adult brain lesions cannot be used to account for the dynamics of change in genetic and environmentally induced disorders in children.

Comprehension of metaphor and metonymy in children with Williams syndrome

BACKGROUND Figurative language, such as metaphor and metonymy, is very common in daily language use. Its underlying cognitive processes are sometimes viewed as lying at the interface of language and thought. Williams syndrome, which is a rare genetic developmental disorder, provides an opportunity to study this interface because individuals with this disorder have relative strengths in vocabulary and syntax against a background of low general cognitive ability. Few studies have investigated metaphor comprehension in Williams syndrome and none has investigated metonymy. AIMS This is the first study to investigate metaphor and metonymy comprehension in Williams syndrome and to compare their performance with a group of typically developing children. METHODS & PROCEDURES Ten children with Williams syndrome were compared with eleven typically developing children in a novel metaphor-metonymy comprehension task. Cross-sectional trajectory analyses were used to compare the development of metaphor and metonymy using a child-friendly story picture task. Trajectories were constructed linking task performance either to chronological age or to measures of mental age (receptive vocabulary, visuospatial construction). OUTCOMES & RESULTS The performance of children with Williams syndrome was significantly poorer than the typically developing group. The comprehension of metonyms was in line with receptive vocabulary, but comprehension of metaphors fell below this level. CONCLUSIONS & IMPLICATIONS Metonyms may be part of vocabulary and treated as synonyms in Williams syndrome, while metaphor engages additional cognitive mechanisms outside language that develop atypically in this disorder. Despite earlier reports that emphasize good language skills, the Williams syndrome language system shows anomalies compared with typical development.

Item and Error Analysis on Raven's Coloured Progressive Matrices in Williams Syndrome.

Ravens Coloured Progressive Matrices (RCPM) is a standardised test that is commonly used to obtain a non-verbal reasoning score for children. As the RCPM involves the matching of a target to a pattern it is also considered to be a visuo-spatial perception task. RCPM is therefore frequently used in studies in Williams Syndrome (WS), in order to match WS participants to a control group or as a single measure to predict performance on a test-condition in developmental trajectory analyses. However, little is known about the performance of participants with WS on the RCPM. The current study compared the type of errors and the difficulty of each item for 53 participants with WS to 53 typically developing children who were individually matched on the total raw score for RCPM. Results showed that the participants with WS made the same proportion of error types and that the proportion of error types changed similarly to those of typically developing controls over development. Furthermore, the differential item difficulty between the two groups was highly similar. It is therefore argued that, although participants with WS are delayed on RCPM, their performance is not atypical which suggests that RCPM performance is supported by typical mechanisms. The RCPM is therefore a useful tool to match WS to control groups or to construct developmental trajectories.

Development of novel metaphor and metonymy comprehension in typically developing children and Williams syndrome

This study investigated the development of novel metaphor and metonymy comprehension in both typically developing (TD) children and individuals with Williams syndrome (WS). Thirty-one TD children between the ages of 3;09 and 17;01 and thirty-four individuals with WS between the ages of 7;01 and 44 years old were administered a newly developed task examining novel metaphor and metonymy comprehension, as well as a range of standardised tests that assess semantic knowledge. This age range and the background measures allowed construction of developmental trajectories to investigate whether chronological age or mental age, represented by word knowledge, relate to novel metaphor and metonymy comprehension. The results showed that comprehension of figurative language did not increase with chronological age in WS, in contrast to TD. Although there was no difference for the different types of metaphors, certain metonymy expressions were found to be easier than others in the TD group. In addition, semantic knowledge was a reliable predictor for novel metaphor and metonymy comprehension in the TD but only for metonymy in the WS group. In sum, development of novel metonymy in the WS group is only delayed while comprehension of novel metaphor is both delayed and atypical. However, future research should further investigate differences between sub-types, as well as what cognitive factors relate to novel metaphor comprehension in individuals with Williams syndrome.

Narrowing perceptual sensitivity to the native language in infancy: exogenous influences on developmental timing

The infancy literature situates the perceptual narrowing of speech sounds at around 10 months of age, but little is known about the mechanisms that influence individual differences in this developmental milestone. We hypothesized that such differences might in part be explained by characteristics of mother-child interaction. Infant sensitivity to syllables from their native tongue was compared longitudinally to sensitivity to non-native phonemes, at 6 months and again at 10 months. We replicated previous findings that at the group level, both 6- and 10- month-olds were able to discriminate contrasts in their native language, but only 6-month-olds succeeded in discriminating contrasts in the non-native language. However, when discrimination was assessed for separate groups on the basis of mother-child interaction—a ‘high contingency group’ and a ‘moderate contingency’ group—the vast majority of infants in both groups showed the expected developmental pattern by 10 months, but only infants in the ‘high contingency’ group showed early specialization for their native phonemes by failing to discriminate non-native contrasts at 6-months. The findings suggest that the quality of mother-child interaction is one of the exogenous factors influencing the timing of infant specialization for speech processing.

Youth with autism spectrum disorder comprehend lexicalized and novel primary conceptual metaphors.

Individuals with autism spectrum disorder (ASD) have difficulty comprehending metaphors. However, no study to date has examined whether or not they understand conceptual metaphors (i.e. mappings between conceptual structures), which could be the building blocks of metaphoric thinking and understanding. We investigated whether 13 participants with ASD (age 7;03–22;03) and 13 age-matched typically developing (TD) controls could comprehend lexicalized conceptual metaphors (e.g., Susan is a warm person) and novel ones (e.g., Susan is a toasty person). Individuals with ASD performed at greater than chance levels on both metaphor types, although their performance was lower than TD participants. We discuss the theoretical relevance of these findings and educational implications.

Performance on verbal and low-verbal false belief tasks : Evidence from children with Williams syndrome

UNLABELLED Previous studies that have investigated the relationship between performance on theory of mind (ToM) tasks and verbal abilities in individuals with Williams syndrome (WS) have reported contradictory findings with some showing that language abilities aid performance on ToM tasks while others have found that participants with WS fail these tasks because of their verbal demands. The current study investigated this relationship again comparing performance on a classical change-location task to two newly developed low-verbal tasks, one change-location task and one unexpected content task. Thirty children with WS (aged 5-17;01 years) and 30 typically developing (TD) children (aged between 2;10 years and 9;09 years), who were matched for vocabulary comprehension scores were included in the study. Although performance in the WS group was significantly poorer compared to the TD group on all three tasks, performance was not predicted by their receptive vocabulary or grammatical ability scores. In addition, ToM abilities in both groups depended on the cognitive demands of the task at hand. This finding shows that performance on ToM tasks in WS is not necessarily hindered by their delayed language abilities but rather by the task administered. This could potentially affect the diagnosis of developmental disorders, such as Autism Spectrum Disorders, and comparison of ToM abilities across developmental disorders. LEARNING OUTCOMES Readers of this article should be able to (1) describe the current state of theory of mind research in Williams syndrome, (2) identify which cognitive abilities might explain performance on theory of mind tasks in both typically developing children and in children with Williams syndrome, and (3) interpret the importance of task demands when assessing childrens theory of mind abilities.

Colour as an Environmental Cue when Learning a Route in a Virtual Environment: Typical and Atypical Development.

Typically developing (TD) 6-year-olds and 9-year-olds, and older children and adults with Williams syndrome (WS) navigated through brick-wall mazes in a virtual environment. Participants were shown a route through three mazes, each with 6 turns. In each maze the floor of each path section was a different colour such that colour acted as an environmental cue. The colours employed were either easy to verbalise (focal colours) or difficult to verbalise (non-focal colours). We investigated whether participants would verbally code the colour information in the focal colour condition only, and whether this facilitated route-learning. All groups could learn the routes; the WS group required more learning trials to learn the route and achieved lower memory scores than both of the TD groups. Despite this, all groups showed the same pattern of results. There was no effect of condition on the ability to learn the maze. However, when asked which colours featured in each route, higher memory scores were achieved for the focal colour (verbalisable) than the non-focal colour (non-verbalisable) condition. This suggests that, in both young children and individuals with WS, once a route has been learnt, the nature of the environmental cues within it can impact an individuals representation of that route.

Variability and standardized test profiles in typically developing children and children with Williams Syndrome

Williams Syndrome (WS) is a developmental disorder, which due to its specific cognitive profile, has been of interest to multidisciplinary research in order to study the pathways between cognition, brain, and genes. Previous studies investigating individual performance on cognitive tasks have reported large variability within the WS cognitive profile, which has encouraged the investigation of WS subgroups. The current study compared the variability in performance scores on five verbal and non-verbal standardized tests in 33 children with WS and in 33 typically developing (TD) children of a similar chronological age (CA). In contrast to previous studies, the current study did not find significant differences in variability in performance on British Picture Vocabulary scale, Test Reception of Grammar and Digit span Forward between WS and TD groups when CA was controlled for. However, there was significantly less variability in younger WS participants for performance scores on Pattern Construction compared to the TD group. In light of these results, methodological issues and the importance of taking CA into account in analyses will be discussed.