The development of metaphorical language comprehension in typical development and in Williams syndrome

The domain of figurative language comprehension was used to probe the developmental relation between language and cognition in typically developing individuals and individuals with Williams syndrome. Extending the work of Vosniadou and Ortony, the emergence of nonliteral similarity and category knowledge was investigated in 117 typically developing children between 4 and 12 years of age, 19 typically developing adults, 15 children with Williams syndrome between 5 and 12 years of age, and 8 adults with Williams syndrome. Participants were required to complete similarity and categorization statements by selecting one of two words (e.g., either “The sun is like ___” or “The sun is the same kind of thing as ___”) with word pairs formed from items that were literally, perceptually, or functionally similar to the target word or else anomalous (e.g., moon, orange, oven, or chair, respectively). Results indicated that individuals with Williams syndrome may access different, less abstract knowledge in figurative language comparisons despite the relatively strong verbal abilities found in this disorder

How do individuals with Williams syndrome learn a route in a real-world environment?

Individuals with Williams syndrome (WS) show a specific deficit in visuo-spatial abilities. This finding, however, is mainly based on performance on small-scale laboratory-based tasks. This study investigated large-scale route learning in individuals with WS and two matched control groups (moderate learning difficulty group [MLD], typically developing group [TD]). In a non-labelling and a labelling (verbal information provided along the route) condition, participants were guided along one of two unfamiliar 1 km routes with 20 junctions, and then retraced the route themselves (two trials). The WS participants performed less well than the other groups, but given verbal information and repeated experience they learnt nearly all of the turns along the route. The extent of improvement in route knowledge (correct turns) in WS was comparable to that of the control groups. Relational knowledge (correctly identifying spatial relationships between landmarks), compared to the TD group, remained poor for both the WS and MLD groups. Assessment of the relationship between performance on the large-scale route learning task to that on three small-scale tasks (maze learning, perspective taking, map use) showed no relationship for the TD controls, and only a few non-specific associations in the MLD and WS groups

An evaluation of alternative and augmentative systems of communication taught to nonverbal cerebral palsied children.

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Interpretation of ambiguous situations: evidence for a dissociation between social and physical threat in Williams syndrome

There is increasing evidence that Williams syndrome (WS) is associated with elevated anxiety that is non-social in nature, including generalised anxiety and fears. To date very little research has examined the cognitive processes associated with this anxiety. In the present research, attentional bias for non-social threatening images in WS was examined using a dot-probe paradigm. Participants were 16 individuals with WS aged between 13 and 34 years and two groups of typically developing controls matched to the WS group on chronological age and attentional control ability respectively. The WS group exhibited a significant attention bias towards threatening images. In contrast, no bias was found for group matched on attentional control and a slight bias away from threat was found in the chronological age matched group. The results are contrasted with recent findings suggesting that individuals with WS do not show an attention bias for threatening faces and discussed in relation to neuroimaging research showing elevated amygdala activation in response to threatening non-social scenes in WS

Visuo-spatial cognition in Williams syndrome: Reviewing and accounting for the strengths and weaknesses in performance

Individuals with Williams syndrome typically show relatively poor visuo-spatial abilities in comparison to stronger verbal skills. However, individuals' level of performance is not consistent across all visuo-spatial tasks. The studies assessing visuo-spatial functioning in Williams syndrome are critically reviewed, in order to provide a clear pattern of the relative difficulty of these tasks. This prompts a possible explanation of the variability in performance seen which focuses on the processing demands of some of these tasks. Individuals with Williams syndrome show an atypical processing style on tests of construction, which does not affect tests of perception

Mending the Levee: How Supernaturally Anchored Conceptions of the Person Impact on Trauma Perception and Healing among Children (Cases from Madagascar and Nepal)

When dealing with children and youth who experience distressing events, psychosocial diagnostics and healing programmes principally resort to biomedical models. Children are often viewed as individualised ‘victims’ suffering from trauma and ‘in need’ of outside help. Highlighting case studies from Madagascar and Nepal, this article argues that the biomedical approach to trauma would be strengthened by a concomitant analysis of social networks, including the perceived relations with the supernatural. The various tandems of family and kin relationships, the living and the dead, constitute not only a social ‘levee’ breached by distressing events, but also the locus around which social relations are rebuilt

A cross-syndrome study of the differential effects of sleep on declarative memory consolidation in children with neurodevelopmental disorders

Sleep plays an active role in memory consolidation. Because children with Down syndrome (DS) and Williams syndrome (WS) experience significant problems with sleep and also with learning, we predicted that sleep-dependent memory consolidation would be impaired in these children when compared to typically developing (TD) children. This is the first study to provide a cross-syndrome comparison of sleep-dependent learning in school-aged children. Children with DS (n = 20) and WS (n = 22) and TD children (n = 33) were trained on the novel Animal Names task where they were taught pseudo-words as the personal names of ten farm and domestic animals, e.g. Basco the cat, with the aid of animal picture flashcards. They were retested following counterbalanced retention intervals of wake and sleep. Overall, TD children remembered significantly more words than both the DS and WS groups. In addition, their performance improved following night-time sleep, whereas performance over the wake retention interval remained stable, indicating an active role of sleep for memory consolidation. Task performance of children with DS did not significantly change following wake or sleep periods. However, children with DS who were initially trained in the morning continued to improve on the task at the following retests, so that performance on the final test was greater for children who had initially trained in the morning than those who trained in the evening. Children with WS improved on the task between training and the first retest, regardless of whether sleep or wake occurred during the retention interval. This suggests time-dependent rather than sleep-dependent learning in children with WS, or tiredness at the end of the first session and better performance once refreshed at the start of the second session, irrespective of the time of day. Contrary to expectations, sleep-dependent learning was not related to baseline level of performance. The findings have significant implications for educational strategies, and suggest that children with DS should be taught more important or difficult information in the morning when they are better able to learn, whilst children with WS should be allowed a time delay between learning phases to allow for time-dependent memory consolidation, and frequent breaks from learning so that they are refreshed and able to perform at their best

Uncommon genetic syndromes and narrative production - Case Studies with Williams, Smith-Magenis and Prader- Willi Syndromes

This study compares narrative production among three syndromes with genetic microdeletions: Williams syndrome (WS), Smith-Magenis syndrome (SMS), and Prader-Willi syndrome (PWS), characterized by intellectual disabilities and relatively spared language abilities. Our objective is to study the quality of narrative production in the context of a common intellectual disability. To elicit a narrative production, the task Frog! Where Are You was used. Then, structure, process, and content of the narrative process were analysed in the three genetic disorders:WS (n52), SMS (n52), and PWS (n52). Data show evidence of an overall low narrative quality in these syndromes, despite a high variability within different measures of narrative production. Results support the hypothesis that narrative is a highly complex cognitive process and that, in a context of intellectual disability, there is no evidence of particular ‘hypernarrativity’ in these syndromes.This research was supported by the grants FEDER –