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Aspects psychologiques, cognitifs et comportementaux d’enfants présentant un syndrome de Prader-Willi : étude transversale et étude longitudinale

Abstract : Our aim was to describe the psychologic, cognitive and behavioral characteristics of children with Prader-Willi syndrom (PWS). The cohort includes 36 children from 2.5 to 15 years regularly followed in the French reference centre for PWS. Three prospective studies are presented, two transversal comparative studies and one longitudinal. The first study describes, in the entire cohort, the multidisciplinary care and the scolarisation of the children, their cognitive abilities and their adaptative behaviors as well as the known behavioral which are problematic issues in adolescents and adults with PWS. We compared the data between children aged less than 5 years and children aged more than 6 years. Our results pointed out differences with the previous results already published in retrospective studies. The second transversal study describes the capacities of the 27 children aged more than 6 years to match, to label and to infer emotional states. We then compared the results of these PWS patients with results obtained from control children matched for sex and for developmental age on one hand, and for chronological age on the other hand. The results of this innovative study show that children with PWS have heterogeneous abilities for the identification of the emotions, with an effect of the nature of the emotion and of the task. Finally, the last study describes the results of a 4 years longitudinal study of 10 children in terms of phenotypic evolution during the transition from early childhood to childhood. The evolution of their cognitive abilities and of their adaptative behaviors is detailed, showing the emergence of some behavioral troubles.
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Mélanie Glattard. Aspects psychologiques, cognitifs et comportementaux d’enfants présentant un syndrome de Prader-Willi : étude transversale et étude longitudinale. Psychologie. Université Toulouse le Mirail - Toulouse II, 2012. Français. ⟨NNT : 2012TOU20042⟩. ⟨tel-00718614⟩

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