Narrative skills in children with 22q11.2 deletion syndrome: a cross-syndrome comparison

Background: The ability to tell a story involves a number of language and cognitive skills. In order to tell a good story a child needs to understand cause-effect relationships, outline events and use precise vocabulary to convey ideas to aid a listener in comprehending the tale (Boudreau, 2008). Children with some genetic syndromes seem to have difficulties acquiring these skills, which has impact on their everyday social communication. Cross-syndrome research on this topic is rare. Methods: A cross-syndrome comparison of the narrative skills of Dutch speaking school-aged children with 22q11.2 deletion syndrome (22q11.2 DS) and children with Williams Syndrome is presented. These children are compared with (a) mental age matched control groups of children with an intellectual disability (ID) with unknown etiology, (b) children with ID with unknown etiology and comorbid autism spectrum disorder and (c) typically developing children. A fine-grained story analysis based upon samples of the Bus Story Test (Renfrew, 1997) and the Expression, Reception and Recall of Narrative Instrument (Bishop, 2004) was carried out. Results: The results indicate cross-syndrome and within-syndrome variability in narrative skills. The impact on everyday communication is highlighted. Conclusion: Evaluating pragmatic and more specifically narrative language skills across syndromes can contribute to the understanding of the communicative behavioral phenotype. Detailed language assessments may improve intervention targeting communication, and lead to specific parental advices for these groups of children.status: publishe