Narrative skill in boys with fragile X syndrome with and without autism spectrum disorder

Abstract

We examined recalled narratives of boys with fragile X syndrome with autism spectrum disorder (FXS-ASD; N=28) and without ASD (FXS-O; N=29), and compared them to those of boys with Down syndrome (DS; N=33) and typically developing boys (TD; N=39). Narratives were scored for mentions of macrostructural Story Grammar elements (Introduction, Relationship, Initiating Events, Internal Response, Attempts/Actions, and Ending). We found that narrative recall is predicted by short-term memory and nonverbal mental age levels in almost all groups (except TD), but not by expressive syntax or caregiver education. After adjusting for these covariates, there were no differences between the three groups with intellectual disability (ID). The FXS-ASD group, however, had significantly poorer performance than the TD group on the overall Story Grammar score, and both the FXS-O and FXS-ASD groups had lower Attempts/Actions scores than the TD group. We conclude that some form of narrative impairment may be associated with FXS, that this impairment may be shared by other forms of ID, and that the presence of ASD has a significantly detrimental effect on narrative recall.

Perseveration in the Connected Speech of Boys with Fragile X Syndrome with and Without Autism Spectrum Disorder

“…If this was the case, then the pronounced occurrence of utterance-level perseveration in the typically developing group might reflect better story-retelling skills or a greater attempt to retell the story accurately and completely. For example, one typically developing boy produced the following statement that was coded for utterance-level perseveration: “When when the driver found out he was in the water, he called he called a tow truck to pull him out of the water.” In fact, these narrative samples were previously examined for recall of story grammar elements (see Estigarribia, et al, 2011), and typically developing boys recalled more story actions than boys with Fragile X syndrome with and without ASD.…”

“…Recent studies suggest that 18–52% of males with Fragile X syndrome also meet criteria for autism on gold standard instruments, and that almost half to nearly three quarters of males (43–74%) may be on the autism spectrum (Clifford et al, 2007; Hall, Lightbody, & Reiss, 2008; Kaufmann et al, 2004; Philofsky, Hepburn, Hayes, Hagerman, & Rogers, 2004; Rogers, Wehner, & Hagerman, 2001). There is some evidence that autism in boys with Fragile X syndrome is associated with greater language and communication difficulties (e.g., Bailey, Hatton, Skinner, & Mesibov, 2001; Estigarribia et al, 2011; Roberts, Martin, et al, 2007). …”

Inferential language use by youth with Down syndrome during narration

“…In contrast, a relatively large body of research has examined the pragmatic language profile of individuals with FXS, suggesting patterns of impairment contextualized within the broader phenotype of FXS (Abbeduto et al, 2008; Estigarribia et al, 2011; Klusek, Martin, & Losh, 2014; Losh, Martin, Klusek, Hogan-Brown, & Sideris, 2012; Martin et al, 2012; Roberts et al, 2007; Simon, Keenan, Pennington, Taylor, & Hagerman, 2001; Sudhalter & Belser, 2001). Thus, as further explained below, individuals with FXS who also have intellectual disability provide an interesting comparison group for considering inferential language abilities in DS.…”

A Comparison of Pragmatic Language in Boys With Autism and Fragile X Syndrome

“…Individuals with FXS also struggle with pragmatic language, such as impaired use of communicative repair strategies (Abbeduto et al, 2008), difficulty with conversational topic maintenance (Roberts, Martin, et al, 2007; Sudhalter & Belser, 2001; Sudhalter, Cohen, Silverman, & Wolf-Schein, 1990; Wolf-Schein et al, 1987), and impaired narrative processing and formulation (Estigarribia et al, 2011; Simon, Pennington, Taylor, & Hagerman, 2001). Perseveration has been documented at length (Belser & Sudhalter, 2001; Martin et al, 2012; Roberts, Martin, et al, 2007; Sudhalter et al, 1990; Wolf-Schein et al., 1987).…”