Behavioural features of Italian infants and young adults with Williams-Beuren syndrome

C. Gagliardi, S. Martelli, A. Tavano, R. Borgatti

Research output: Contribution to journalArticle

Abstract

Background The increased interest in social interaction in Williams-Beuren syndrome (WBS) is evident from infancy onwards, together not only with increased empathy, positive interpersonal bias, but also with social disinhibition. Previous studies have described behavioural and emotional problems as being widely represented in WBS. There is limited scope for comparisons between literature data because of the variety of instruments used to assess behaviour.Method Forty-one children and young adults with WBS were enrolled and underwent general cognitive assessment. In order to compare our data with the literature, we used standardised questionnaires used in previous studies (Developmental Behaviour Checklist: DBC-P). General cognitive abilities, gender and age were included in the analysis.Results Behavioural problems were more relevant than expected according to intellectual impairment. Some features were present at any age: inattention, anxiety, disruptive behaviours. Antisocial conduct was almost absent; perseverative conduct, a poor sense of danger and, more generally, self-absorbed behaviours tended to diminish along with age and to be linked to more pronounced cognitive impairment.Conclusion As previously described for other countries, behaviour disturbances occur frequently in the Italian WBS population. Our data could support the existence of some 'intrinsic' behavioural characteristics in WBS such as inattention and anxiety, which are detectable and important at any age; both learning and social exposure to a structured context such as school could help diminish self-absorbed behaviour.

Original languageEnglish
Pages (from-to)121-131
Number of pages11
JournalJournal of Intellectual Disability Research
Volume55
Issue number2
DOIs
Publication statusPublished - Feb 2011

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Keywords

  • Anxiety
  • Behavioural phenotype
  • Development
  • Social drive
  • Williams-Beuren syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Psychiatry and Mental health
  • Rehabilitation

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