The Social Responsiveness Scale (SRS-2) in school-age children with Down syndrome at low risk for autism spectrum disorder

Abstract

Background and aims Little is known about how autism spectrum disorder (ASD) symptoms present in individuals with Down syndrome (DS). Some behaviors may be symptomatic of comorbid ASD or more broadly representative of the DS phenotype. A prior research study documented elevated ASD-like symptoms in adolescents and young adults with DS without comorbid ASD, using a common ASD risk screening tool—the Social Responsiveness Scale (SRS). The current study applied a similar approach to younger children with DS using the SRS-2. The primary aim was to document patterns of ASD-like symptoms in children with DS at low risk of comorbid ASD to distinguish the symptoms that may be present across DS in general. Methods SRS-2 standard scores were analyzed in a sample of 40 children with DS, 6–11 years old, who were considered to be at low risk for ASD based on the Social Communication Questionnaire (SCQ) screener. Other developmental characteristics (i.e., age, nonverbal IQ, expressive language), social skills, and problem behaviors were also examined across the sample. Results SRS-2 scores were significantly elevated in this sample compared to the normative population sample. A pattern of ASD-like symptomatology was observed across SRS-2 subdomains. These findings were similar to the findings of the prior study. However, nuanced differences were observed across the two samples that may represent developmental differences across different ages in this population. Conclusions Replicating and extending a prior study's findings, certain ASD-like behaviors may occur in individuals with DS who are at low risk for comorbid ASD. Implications: Understanding the pattern of ASD-like behaviors that occur in children with DS who are at low risk for comorbid ASD will help clinicians in screening and identification efforts. In particular, it will lead to better specification of the behaviors or symptoms that are not characteristic of the DS phenotype and thus are red flags for comorbid ASD in this population.

Psychometric Evaluation of Social Cognition and Behavior Measures in Children and Adolescents with Down Syndrome

“…Further, there were minimal differences between raw scores and T-scores on the SRS-2, and the use of T-scores is appropriate for this measure of social behavior. High feasibility of the SRS-2 reinforces the suitability of this tool for the measurement of social behavior in individuals with DS [5,19]. Feasibility was below a priori criterion for the NEPSY-II Affect Recognition and floor effects were observed for both raw and standard scores and most problematic for standard scores.…”

“…Recent studies show that approximately 15-18% of children with DS also have an ASD diagnosis, which is markedly higher than the 1% reported in the general population [6,7]. Children with DS, with and without co-occurring ASD, experience social challenges that impede interactions with peers [5,8]. These social difficulties lead to greater potential for social isolation that, in turn, impacts mental health outcomes for this population [9], making social cognition and other social skills potential targets for intervention.…”

“…Individuals with Down syndrome (DS) are commonly described as socially engaged [1] and as having relatively strong nonverbal social functioning in early childhood [2]. Nevertheless, individuals with DS also experience challenges with core aspects of social relatedness including social cognition, expressive language, and social interaction [3][4][5]. Social challenges are further evident in rates of co-occurring autism spectrum disorder (ASD) in DS.…”

“…Affect and emotion recognition is also an area of challenge in DS in comparison with children with typical development matched on cognitive or receptive language level [16,17]. Studies using the Social Responsiveness Scale, Second Edition (SRS-2) [18] describe social communication and interactions in individuals with DS at low risk for ASD and show that these individuals have relative strengths in social motivation and challenges with social cognition, communication, and awareness [5,19]. For a review of social cognition development in DS, see [20].…”

“…However, standardized clinical assessments have yet to be evaluated to assess social cognition in DS. Another measure used to assess social cognition, among other social behaviors, is the SRS-2, and previous work supports its utility in 6-to 21-year-olds with DS [5,19]. A benefit of using the SRS-2 is that the parent reports on the child's typical social behavior without the child having to do more intensive in-person assessments.…”