A Multimethod Analysis of Pragmatic Skills in Children and Adolescents With Fragile X Syndrome, Autism Spectrum Disorder, and Down Syndrome

Abstract

Purpose Pragmatic language skills are often impaired above and beyond general language delays in individuals with neurodevelopmental disabilities. This study used a multimethod approach to language sample analysis to characterize syndrome- and sex-specific profiles across different neurodevelopmental disabilities and to examine the congruency of 2 analysis techniques. Method Pragmatic skills of young males and females with fragile X syndrome with autism spectrum disorder (FXS-ASD, n = 61) and without autism spectrum disorder (FXS-O, n = 40), Down syndrome (DS, n = 42), and typical development (TD, n = 37) and males with idiopathic autism spectrum disorder only (ASD-O, n = 29) were compared using variables obtained from a detailed hand-coding system contrasted with similar variables obtained automatically from the language analysis program Systematic Analysis of Language Transcripts (SALT). Results Noncontingent language and perseveration were characteristic of the pragmatic profiles of boys and girls with FXS-ASD and boys with ASD-O. Boys with ASD-O also initiated turns less often and were more nonresponsive than other groups, and girls with FXS-ASD were more nonresponsive than their male counterparts. Hand-coding and SALT methods were largely convergent with some exceptions. Conclusion Results suggest both similarities and differences in the pragmatic profiles observed across different neurodevelopmental disabilities, including idiopathic and FXS-associated cases of ASD, as well as an important sex difference in FXS-ASD. These findings and congruency between the 2 language sample analysis techniques together have important implications for assessment and intervention efforts.

The Phenotypic Profile Associated With the FMR1 Premutation in Women: An Investigation of Clinical-Behavioral, Social-Cognitive, and Executive Abilities

“…Pragmatic language differences have been repeatedly observed among PM carriers (27,57,(100)(101)(102), and were evident in this study as well, with additional patterns noted across the types of pragmatic language violations occurring more frequently in the PM group, who tended to display a more dominant conversational style (e.g., overly detailed, tangential language) than controls. Differences in pragmatic language have consistently emerged as a phenotypic marker associated with the PM in females (27,(100)(101)(102), and among individuals with FXS, particularly those who meet criteria for ASD (62,(103)(104)(105)(106)(107)(108)(109) and may be of clinical importance. For instance, prior work has shown that pragmatic language violations among mothers of children with FXS were associated with reduced selfreported quality of life for mothers (102), as well as elevated ASD symptoms and weaker expressive and receptive language in their children (27,100).…”

Expressive language abilities of boys with idiopathic autism spectrum disorder and boys with fragile X syndrome + autism spectrum disorder: Cross-context comparisons

“…Such comparisons can inform our understanding of how ASD impacts language, and whether uniquely tailored language interventions may be needed for these clinical groups (Abbeduto & Murphy, 2004). However, studies comparing the expressive language profiles of the two groups have been limited, and results have been inconsistent, partially as a reflection of different assessment methods being utilized (Haebig & Sterling, 2017; Klusek et al., 2014b; Martin et al., 2018; Sterling, 2018; Thurman et al., 2017). Thus, the purpose of the present study was to compare the expressive language profiles of boys with FXS + ASD and boys with idiopathic ASD, and to examine whether a similar linguistic profile emerged across two different language sampling contexts that are commonly used in the literature (i.e.…”

“…More recently, an increasing number of studies have begun to use other assessment tools, such as the Autism Diagnostic Observation Schedule (ADOS, ADOS-2; Lord et al., 1999, 2012), to examine the language abilities of children with FXS and ASD (e.g. Estigarribia, et al., 2011; Klusek et al., 2014b; Martin et al., 2018; Park et al., 2012; Roberts et al., 2007a). Indeed, Tager-Flusberg and expert colleagues (2009) noted the value of using multiple language samples in the assessment process, and recommended the ADOS as a way to collect natural language samples in children with ASD.…”

“…However, the boys with FXS þ ASD and idiopathic ASD did not differ on a standardized measure of grammar. Similarly, after controlling for nonverbal mental age, receptive vocabulary, and expressive vocabulary and grammar, others have found that boys with FXS þ ASD and idiopathic ASD do no differ in terms of the overall frequency (Martin et al, 2018) or rating of perseverative language (Klusek et al, 2014b). Interestingly, Martin et al (2018) found that boys with FXS þ ASD and ASD had a higher frequency of perseveration than boys with FXS-Only but not boys with TD.…”

Language Development From Early Childhood to Adolescence in Youths With Fragile X Syndrome

“…Most males and many females with FXS have impaired communication (Abbeduto et al, 2016). Impairments have been reported in the areas of speech intelligibility (Barnes et al, 2009), vocabulary (Roberts et al, 2007;Sudhalter & Belser, 2001), morphology and syntax (Komesidou et al, 2017;Martin et al, 2013), and pragmatics (Martin et al, 2018). In addition, individuals with FXS have impaired cognition, and there is a relationship between the amount of impairments in cognition and communication (Abbeduto et al, 2007;Oakes et al, 2013).…”