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      Pragmatic skills in people with Williams syndrome: the perception of families

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          Abstract

          Background

          One of the most challenging linguistic areas in people with Williams Syndrome throughout their evolutionary stage is the development of pragmatic skills. The research conducted so far highlights specific problems concerning adaptation to the linguistic context and interlocutors, language comprehension, as well as other aspects interfering with verbal communication. However, until now, most scientific evidence has been based on personal assessments of this group. In a complementary manner, the goal of this study was to discover the level of pragmatic skills of people with Williams Syndrome from the point of view of the families. The sample consisted of 34 families belonging to the Williams Syndrome Association of Spain. The assessment instrument was the pragmatic awareness questionnaire, which includes 26 items related to different aspects that are part of the pragmatic area on a Likert-type scale.

          Results

          The results indicate that, families consider there to be a regular to low level in all the areas assessed. The strong points seem to lie in the paralinguistic aspects, while the weakest factors are those related to the understanding of figurative language .

          Conclusions

          Therefore, it is necessary to continue insisting on the importance of language intervention in this group throughout its development to improve its level of linguistic competence.

          Supplementary Information

          The online version contains supplementary material available at 10.1186/s13023-024-03016-0.

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          Supravalvular aortic stenosis.

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            A componential view of theory of mind: evidence from Williams syndrome.

            In this paper we argue that there are two distinct components of a theory of mind: a social-cognitive and a social-perceptual component. Evidence for this proposal is presented from various sources, including studies of children with Williams syndrome, a rare genetic neurodevelopmental disorder. Earlier work has demonstrated that people with Williams syndrome appear to be spared in the social-perceptual component of a theory of mind. In this paper we present evidence that they are not spared in the social-cognitive component of theory of mind. Three experiments with young children with Williams syndrome were conducted. In each experiment the children with Williams syndrome were compared to age-, IQ-, and language-matched children with Prader-Willi syndrome, and children with non-specific mental retardation. The experiments used different measures of theory of mind ability, including false belief (Experiment 1), explanation of action (Experiment 2), and recognition of emotional expressions (Experiment 3). In none of these experiments did the children with Williams syndrome evidence superior performance compared to the control groups. The results from this and other studies on Williams syndrome support the view that the social-cognitive and social-perceptual components of a theory of mind are dissociable. In Williams syndrome only the latter components, which are linked to distinct neurobiological substrates, are spared.
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              Neurobiology of social behavior abnormalities in autism and Williams syndrome.

              Social behavior is a basic behavior mediated by multiple brain regions and neural circuits, and is crucial for the survival and development of animals and humans. Two neuropsychiatric disorders that have prominent social behavior abnormalities are autism spectrum disorders (ASD), which is characterized mainly by hyposociability, and Williams syndrome (WS), whose subjects exhibit hypersociability. Here we review the unique properties of social behavior in ASD and WS, and discuss the major theories in social behavior in the context of these disorders. We conclude with a discussion of the research questions needing further exploration to enhance our understanding of social behavior abnormalities.
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                Author and article information

                Contributors
                esmora01@ucm.es
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central (London )
                1750-1172
                1 March 2024
                1 March 2024
                2024
                : 19
                : 95
                Affiliations
                Faculty of Psychology, University Complutense of Madrid, Campus de Somosaguas, ( https://ror.org/02p0gd045) 28223 Pozuelo de Alarcón, Spain
                Author information
                http://orcid.org/0000-0001-5486-9455
                Article
                3016
                10.1186/s13023-024-03016-0
                10908058
                38429809
                e79e12d0-a410-44ce-98c1-77cf6e54546c
                © The Author(s) 2024

                Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 13 September 2022
                : 11 January 2024
                Categories
                Research
                Custom metadata
                © Institut National de la Santé et de la Recherche Médicale (INSERM) 2024

                Infectious disease & Microbiology
                pragmatics,williams syndrome,language,parents,family perception
                Infectious disease & Microbiology
                pragmatics, williams syndrome, language, parents, family perception

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