Abstract

HAYASHIUCHI, Ana Yaemi et al. Competências escolares e sociais em crianças e adolescentes com Síndrome de Williams. Rev. bras. educ. espec. [online]. 2012, vol.18, n.03, pp.375-390. ISSN 1413-6538.

Williams Syndrome (WS) is a genetic and neurobehavioral disease caused by a hemizygous deletion of multiples genes in the chromosome region 7q11-23. It is characterized by cognitive and behavioral changes that interfere in psychosocial adjustment. The objective of the study was to verify behavioral indicators of competence in social, educational and performance activities in a group of children and adolescents with WS and signs of inattention and hyperactivity. The sample was composed of 22 children and adolescents with WS clinical and genetic diagnoses between 7 and 18 years, average age 11.6 (standard deviation 3.7) and their mothers. The data collection instruments were the Wechsler Intelligence Scale for Children, the Child Behavior Checklist for Children and Adolescents between 6 and 18 years (CBCL/6-18) and a questionnaire that assessed signs of inattention and hyperactivity based on clinical criteria for Attention Deficit Hyperactivity Disorder of the Manual of Classification of Mental Disorders of the American Psychiatric Association. The main results showed a high number of signs of inattention and hyperactivity (90% of the group scored positively). Severe underachievement in academic skills were also observed, as opposed to positive results in socialization and sports scales. In conclusion, positive results in social areas can be inhibiting factors for the development of emotional problems such as withdrawal, loneliness and low self-esteem.

Keywords : Special Education; Social Competence; Inattention; Hyperactivity.

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