Abstract
Sotos syndrome is a congenital overgrowth syndrome associated with intellectual disability. This study investigated communicative abilities of children with Sotos syndrome (n = 31), using the Children’s Communication Checklist, second edition. A cross-syndrome approach was used to establish the specificity of these abilities. Children with Williams syndrome (n = 34) were used as a comparison group. In both groups, the majority of participants had communicative impairment. Children with Sotos syndrome had an uneven pragmatic language profile and greater impairment with social relations, compared with restricted interests. Overall, children with Sotos syndrome had difficulties with both language structure and pragmatic language and a specific profile of relative difficulty with using nonverbal communication, using context-appropriate language and understanding peer relationships.
Highlights
Sotos syndrome is a congenital overgrowth syndrome with an estimated incidence of approximately 1 in 14,000 (Tatton-Brown and Rahman 2004)
Recent research has investigated the phenotype using larger and more representative samples and this has established that Sotos syndrome is associated with a high prevalence of behavioural symptomatology associated with autism spectrum disorder (ASD), as well as a cognitive profile of relative strength with verbal ability but relative weakness with nonverbal reasoning ability
The analysis identified no effect of subscale; F(3, 90) = 2.16, p = .099, ηρ2 = .067, indicating that children with Sotos syndrome had similar ability in all four language structure skills assessed by the CCC-2
Summary
Sotos syndrome is a congenital overgrowth syndrome with an estimated incidence of approximately 1 in 14,000 (Tatton-Brown and Rahman 2004). Recent research has investigated the phenotype using larger and more representative samples and this has established that Sotos syndrome is associated with a high prevalence of behavioural symptomatology associated with autism spectrum disorder (ASD), as well as a cognitive profile of relative strength with verbal ability but relative weakness with nonverbal reasoning ability (Lane et al 2017, 2018; Sheth et al 2015). Children with Down syndrome typically display greater difficulty with language structure skills, compared with pragmatic language skills, whilst children with WS have greater difficulty with pragmatic language (Hoffmann et al 2013; Smith et al 2017) This demonstrates the specificity of language profiles associated with different conditions. It is important to establish whether individuals with Sotos syndrome display a consistent and characteristic profile of communication impairment in relation to structural and pragmatic aspects of language and the extent to which these linguistic abilities are related
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