Spatial representation and attention in toddlers with Williams syndrome and Down syndrome

The nature of the spatial representations that underlie simple visually guided actions early in life was investigated in toddlers with Williams syndrome (WS), Down syndrome (DS), and healthy chronological age- and mental age-matched controls, through the use of a "double-step" saccade paradigm. The experiment tested the hypothesis that, compared to typically developing infants and toddlers, and toddlers with DS, those with WS display a deficit in using spatial representations to guide actions. Levels of sustained attention were also measured within these groups, to establish whether differences in levels of engagement influenced performance on the double-step saccade task. The results showed that toddlers with WS were unable to combine extra-retinal information with retinal information to the same extent as the other groups, and displayed evidence of other deficits in saccade planning, suggesting a greater reliance on sub-cortical mechanisms than the other populations. Results also indicated that their exploration of the visual environment is less developed. The sustained attention task revealed shorter and fewer periods of sustained attention in toddlers with DS, but not those with WS, suggesting that WS performance on the double-step saccade task is not explained by poorer engagement. The findings are also discussed in relation to a possible attention disengagement deficit in WS toddlers. Our study highlights the importance of studying genetic disorders early in development. (C) 2002 Elsevier Science Ltd. All rights reserved.

Do children with Williams syndrome really have good vocabulary knowledge? Methods for comparing cognitive and linguistic abilities in developmental disorders

The comparison of cognitive and linguistic skills in individuals with developmental disorders is fraught with methodological and psychometric difficulties. In this paper, we illustrate some of these issues by comparing the receptive vocabulary knowledge and non-verbal reasoning abilities of 41 children with Williams syndrome, a genetic disorder in which language abilities are often claimed to be relatively strong. Data from this group were compared with data from typically developing children, children with Down syndrome, and children with non-specific learning difficulties using a number of approaches including comparison of age-equivalent scores, matching, analysis of covariance, and regression-based standardization. Across these analyses children with Williams syndrome consistently demonstrated relatively good receptive vocabulary knowledge, although this effect appeared strongest in the oldest children.

Prosodie et Syndrome de Williams : une étude inter-langue

L’objectif de l’étude est de mieux cerner les particularités acoustiques de la prosodie d’enfants porteurs du SW de langue maternelle anglaise et de langue maternelle française. Des productions spontanées ont été recueillies à l’aide de la tâche de narration « Grenouille, où es-tu ? » (Mayer, 1969). La prosodie des enfants SW est comparée à celle de deux groupes typiques, un de même âge chronologique et un de même âge développemental que le groupe avec SW La population se compose de 1. 7 enfants SW de langue française âgés entre 6 ans et 13 ans 7 mois, appariés à deux groupes typiques et 2. 13 enfants SW de langue anglaise âgés entre 6 ans et 13 ans 11 mois, appariés à deux groupes typiques. Notre analyse porte sur un paramètre acoustique de la prosodie des enfants avec SW : la Variation de la Fréquence Fondamentale. Les résultats sont discutés sur les points suivants : 1. les différences et similitudes entre les profils prosodiques des enfants avec SW et des enfants typiques de même âge chronologique ou développemental, en langue anglaise et en langue française, 2. les différences entre les enfants avec SWde langue maternelle anglaise et les enfants avec SWde langue maternelle française et 3. le développement prosodique observé chez les enfants avec SW. (For English abstract see "Additional Information")

Pitch range and vowel duration in the speech of children with Williams syndrome

This paper reports the pitch range and vowel duration data from a group of children with Williams syndrome (WS) in comparison with a group of typically developing children matched for chronological age (CA) and a group matched for receptive language abilities (LA). It is found that the speech of the WS group has a greater pitch range and that vowels tend to be longer in duration than in the speech of the typically developing children. These findings are in line with the impressionistic results reported by Reilly, Klima and Bellugi [17].