Joint Engagement and Early Language in Young Children With Fragile X Syndrome

Abstract

Purpose: In this study, we examine joint engagement (JE) in young children with fragile X syndrome (FXS) and its relationship to language abilities and autism spectrum disorder symptomatology at 24 to 36 months (toddler period) and 59 to 68 months (child period). Method: Participants were 28 children with FXS (24 boys, four girls) and their mothers. Videotaped home observations were conducted during the toddler period and coded for JE. Language abilities were measured at both ages from a developmental assessment, a functional measure, and from a language sample. The Childhood Autism Rating Scale (Schopler, Reichler, & Renner, 1988) was completed at both ages.Results: Children with FXS spent more time in supported JE than in coordinated JE. Using a weighted JE variable, we found that children with FXS who had higher weighted JE scores also had more advanced expressive language skills at both the toddler and child periods. Weighted JE was negatively related to autism symptomatology in the toddler period. Conclusion: This study provides evidence that children with FXS who use more JE also have more advanced expressive language skills in early development. Therefore, existing early interventions that target JE behaviors may be effective for promoting language, social communication, and social interaction in this population.

Joint Attention and Early Social Developmental Cascades in Neurogenetic Disorders

“…Therefore, the presence of autism spectrum disorder will also have a cascading effect on early social development in neurogenetic disorders. For example, higher levels of autism spectrum disorder symptomatology have been consistently related to more impaired functioning in FXS (Bailey, Hatton, Skinner, & Mesibov, 2001; Hahn, Brady, Warren, & Fleming, 2015; Roberts, Mankowski, et al, 2009), and this pattern has also been observed in early social development (Flenthrope & Brady, 2010; Hahn et al, 2016). Similarly, a study examining the presence of autism spectrum disorder in DS indicates that potential early indicators include impairments in social and communication skills (Hepburn, Philofsky, Fidler, & Rogers, 2008).…”

“…Similarly, observational studies of young children with FXS suggest that joint attention and joint engagement are commensurate with developmental level (Hahn et al, 2016; Roberts et al, 2002). Also during social interactions, it seems that young children with FXS are particularly skilled at using eye gaze shifts for the purpose of sharing interests, making requests, and commenting (Roberts et al, 2002), despite the extant research on children, adolescents, and adults with FXS that reports impairments in eye gaze, especially for social purposes (Cohen et al, 1988; Hall et al, 2015; Hessl, Glaser, Dyer-Friedman, & Reiss, 2006; Murphy et al, 2007; Wolff et al, 1989).…”

“…However, while there is initial evidence that some aspects of joint attention appear to be mental-age appropriate in FXS (Hahn et al, 2016; Marschik et al, 2014; Roberts et al, 2002), there is also evidence that disruptions in gesture use exist (Flenthrope & Brady, 2010; Roberts et al, 2002). While more research is needed, the available research on joint attention and joint attention related behaviors provides insight into early social development in FXS.…”

The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes

“…Impairments in joint attention, a social referencing skill [ 47 ] that is a core precursor to social cognition and social interaction skills and behaviours [ 48 , 49 ], may also contribute to the break in performance. Whilst participants can pass the two easiest ESCogS tasks by making inferences based on the examiner’s actions, the next four tasks require participants to either respond to the examiner’s use of joint attention, by indicating which box a toy is hidden in (both Gestures tasks) or initiate joint attention by directing the examiner’s attention so that they can fulfil their role in completing a joint goal (both Cooperation tasks).…”

Characterizing the Richness of Maternal Input for Word Learning in Neurogenetic Disorders

“…Each behavior-child engagement state, child gesture, maternal gesture-is coded within one observation file in separate passes to ensure that no behaviors are missed, while allowing all behaviors to be viewed together (see Table 2 for definitions and examples). Child engagement is coded using the procedures and coding scheme of Hahn and colleagues 74 based on the work of Adamson and colleagues. 75,76 In this coding scheme, codes are based on the engagement of the child during a naturalistic interaction (free play).…”