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

Abstract

Verbal perseveration is a frequently reported language characteristic of males with Fragile X syndrome and may be a defining feature or hallmark of the syndrome. We compared the verbal perseveration of boys with Fragile X syndrome with (n = 29) and without (n = 30) autism spectrum disorder, boys with Down syndrome (n = 27), and typically developing boys (n = 25) at similar nonverbal mental ages. During a social interaction, boys with both Fragile X syndrome and autism spectrum disorder produced significantly more topic perseveration than all other groups. In social interaction as compared to narration, boys with Fragile X syndrome (regardless of autism status) produced significantly more topic perseveration. These findings suggest that autism status, as well as language sampling context, affect perseveration in boys with Fragile X syndrome.

Pragmatic Language Features of Mothers With the FMR1 Premutation Are Associated With the Language Outcomes of Adolescents and Young Adults With Fragile X Syndrome

“…One report by Simon, Keenan, Pennington, Taylor, and Hagerman (2001) found that women with the FMR1 premutation (n = 25) did not show any impairments in component discourse processing skills, although other reports do support broader language deficits in this group, such as impaired language formulation (Sterling, Mailick, Greenberg, Warren, & Brady, 2013). In contrast to the scarcity of evidence in the FMR1 premutation, pragmatic language deficits are well documented in the fragile X full mutation (Abbeduto et al, 2006;Losh, Martin, Klusek, HoganBrown, & Sideris, 2012;Martin et al, 2012;Roberts et al, 2007) and are associated with FMR1-related variation (i.e., CGG repeat length and percentage methylation; .…”

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

“…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). While it is clear that individuals with FXS struggle with pragmatics, the impact of ASD comorbidity on such deficits is less well understood, as most investigations have not accounted for co-occurring ASD.…”

“…While it is clear that individuals with FXS struggle with pragmatics, the impact of ASD comorbidity on such deficits is less well understood, as most investigations have not accounted for co-occurring ASD. Some evidence suggests that ASD comorbidity has an added detrimental effect on pragmatic language abilities; boys with FXS and comorbid ASD exhibit more off-topic conversational turns (Roberts, Martin, et al, 2007), increased stereotyped and perseverative language (Martin et al, 2012; McDuffie et al, 2010), and perform more poorly on standardized pragmatic language assessments (Losh, Martin, Klusek, Hogan-Brown, & Sideris, 2012) than their counterparts without ASD.…”

A Multi-Method Investigation of Pragmatic Development in Individuals With Down Syndrome

“…Difficulty conveying communicative intent relative to mental age-matched controls has also been observed (Abbeduto et al, 2008). Further, individuals with DS have been reported to demonstrate increased use of stereotyped language as compared to TD controls of a similar mental age (Laws & Bishop, 2004), though at lower rates than observed in other developmental disabilities, such as FXS or ASD (Abbeduto, Brady, & Kover, 2007; Martin, G. E., Roberts, Helm-Estabrooks, Sideris, & Assal, 2012; Roberts et al, 2007). Of note, studies of pragmatic abilities in DS have historically only included boys or combined sexes without examining sex differences, despite well-documented sex differences in pragmatic abilities in typical development (Berghout, Salehi, & Leffler, 1987; Cook, Fritz, McCornack, & Visperas, 1985; Kothari, Skuse, Wakefield, & Micali, 2013; Leaper, 1991; Sigleman & Holtz, 2013).…”