Caracterização de habilidades lingüísticas de crianças e adolescentes com Síndrome de Williams-Beuren

• Ano de defesa: 2010
• Autor(a) principal: Segin, Miriam
• Orientador(a): Carreiro, Luiz Renato Rodrigues
• Banca de defesa: Macedo, Elizeu Coutinho de , Navas, Ana Luiza Gomes Pinto
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Presbiteriana Mackenzie
• Programa de Pós-Graduação: Distúrbios do Desenvolvimento
• Departamento: Psicologia
• País: BR
• Palavras-chave em Português: Síndrome de Williams-Beuren; habilidade intelectual; perfil comportamental; linguagem receptiva; consciência fonológica; consciência sintática; leitura e escrita
• Palavras-chave em Inglês: Williams-Beuren Syndrome; intellectual ability; behavioral profile; receptive language; phonological awareness; syntactic awareness; read and write
• Área do conhecimento CNPq: CNPQ::CIENCIAS HUMANAS::PSICOLOGIA
• Link de acesso: http://dspace.mackenzie.br/handle/10899/22458

Williams-Beuren Syndrome (WBS) is a genetic affection determined by the microdelation of contiguous genes in 7q11.23. The genetic profile of WBS is characterized by the visualconstructive deficit that contrasts with the good performance in verbal tasks, which sustains the hypothesis of dissociation between these abilities. This grants the syndrome peculiar cognitive and behavioral frames. The objective of this research is to describe the pattern of competences in tasks of linguistic abilities in 22 children and adolescents with WBS, aged between 7and 18 (M=11,6; DP=3,7), students of the 1st to 6th grades of elementary and special schools. We used the following instruments: WISC-III (to evaluate intellectual abilities); Wisconsin (used in neuropsychological assessment of abstract reasoning and cognitive strategies); CBCL/6-18 (behavioral assessment); Token-Comp (TT) used to working memory assessment; Peabody Picture Vocabulary Test (PPVT) used to receptive language assessment; Phonological Awareness by Oral Production Test; Syntactic Awareness Test; Word-Reading Efficiency Test; Test of Naming Images by Choosing Words; Test of Naming Images by Writing. The results of WISC-III, Wisconsin and CBCL/6-18 demonstrate mild to moderate intellectual disability, difficulty in concentration and identification of patterns of change, emotional and relationship problems, lack of attention, behavior disorders, challenge and opposition and somatic complaints. The results of TT and PPVT show that the receptive vocabulary is below expected for the level of schooling and age, and is close to the data found in 4-year-old-children. In the abilities of phonological and syntactic awareness, results indicate great deficiencies, with performances equivalent to those of 3-year-old children at the Phonological Awareness by Oral Production Test and of 4-year-olds at the Syntactic Awareness Test. No progression was found according to the increase of school grade. The tests Word-Reading Efficiency, Naming Images by Choosing Words and Naming Images by Writing were taken by 4 participants that presented deficits in the reading ability with graphophonemic decoding. They make more orthographic and semantic mistakes and show low capacity of naming by writing. Thus, it was possible to verify that the sample presents deficits in receptive language and work memory, difficulties in phonological and syntactic processing tasks, which are important factors to the proper development of the capacity to read and write