Dataset Information

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.

SUBMITTER: Hahn LJ

PROVIDER: S-EPMC5345555 | biostudies-literature | 2016 Oct

REPOSITORIES: biostudies-literature

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Publications

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

Hahn Laura J LJ Brady Nancy C NC Fleming Kandace K KK Warren Steven F SF

Journal of speech, language, and hearing research : JSLHR 20161001 5

<h4>Purpose</h4>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).<h4>Method</h4>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 f ...[more]

PMID: 27681878

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Project description:BackgroundThe lack of robust and reliable clinical biomarkers in Fragile X Syndrome (FXS), the most common inherited form of intellectual disability, has limited the successful translation of bench-to-bedside therapeutics. While numerous drugs have shown promise in reversing synaptic and behavioral phenotypes in mouse models of FXS, none have demonstrated clinical efficacy in humans. Electroencephalographic (EEG) measures have been identified as candidate biomarkers as EEG recordings of both adults with FXS and mouse models of FXS consistently exhibit alterations in resting state and task-related activity. However, the developmental timing of these EEG differences is not known as thus far EEG studies have not focused on young children with FXS. Further, understanding how EEG differences are associated with core symptoms of FXS is crucial to successful use of EEG as a biomarker, and may improve our understanding of the disorder.MethodsResting-state EEG was collected from FXS boys with full mutation of Fmr1 (2.5-7 years old, n = 11) and compared with both age-matched (n = 12) and cognitive-matched (n = 12) typically developing boys. Power spectra (including aperiodic and periodic components) were compared using non-parametric cluster-based permutation testing. Associations between 30 and 50 Hz gamma power and cognitive, language, and behavioral measures were evaluated using Pearson correlation and linear regression with age as a covariate.ResultsFXS participants showed increased power in the beta/gamma range (~ 25-50 Hz) across multiple brain regions. Both a reduction in the aperiodic (1/f) slope and increase in beta/gamma periodic activity contributed to the significant increase in high-frequency power. Increased gamma power, driven by the aperiodic component, was associated with better language ability in the FXS group. No association was observed between gamma power and parent report measures of behavioral challenges, sensory hypersensitivities, or adaptive behaviors.LimitationsThe study sample size was small, although comparable to other human studies in rare-genetic disorders. Findings are also limited to males in the age range studied.ConclusionsResting-state EEG measures from this study in young boys with FXS identified similar increases in gamma power previously reported in adults and mouse models. The observed positive association between resting state aperiodic gamma power and language development supports hypotheses that alterations in some EEG measures may reflect ongoing compensatory mechanisms.

Project description:BACKGROUNDFXLEARN, the first-ever large multisite trial of effects of disease-targeted pharmacotherapy on learning, was designed to explore a paradigm for measuring effects of mechanism-targeted treatment in fragile X syndrome (FXS). In FXLEARN, the effects of metabotropic glutamate receptor type 5 (mGluR5) negative allosteric modulator (NAM) AFQ056 on language learning were evaluated in 3- to 6-year-old children with FXS, expected to have more learning plasticity than adults, for whom prior trials of mGluR5 NAMs have failed.METHODSAfter a 4-month single-blind placebo lead-in, participants were randomized 1:1 to AFQ056 or placebo, with 2 months of dose optimization to the maximum tolerated dose, then 6 months of treatment during which a language-learning intervention was implemented for both groups. The primary outcome was a centrally scored videotaped communication measure, the Weighted Communication Scale (WCS). Secondary outcomes were objective performance-based and parent-reported cognitive and language measures.RESULTSFXLEARN enrolled 110 participants, randomized 99, and had 91 who completed the placebo-controlled period. Although both groups made language progress and there were no safety issues, the change in WCS score during the placebo-controlled period was not significantly different between the AFQ056 and placebo-treated groups, nor were there any significant between-group differences in change in any secondary measures.CONCLUSIONDespite the large body of evidence supporting use of mGluR5 NAMs in animal models of FXS, this study suggests that this mechanism of action does not translate into benefit for the human FXS population and that better strategies are needed to determine which mechanisms will translate from preclinical models to humans in genetic neurodevelopmental disorders.TRIAL REGISTRATIONClincalTrials.gov NCT02920892.FUNDING SOURCESNeuroNEXT network NIH grants U01NS096767, U24NS107200, U24NS107209, U01NS077323, U24NS107183, U24NS107168, U24NS107128, U24NS107199, U24NS107198, U24NS107166, U10NS077368, U01NS077366, U24NS107205, U01NS077179, and U01NS077352; NIH grant P50HD103526; and Novartis IIT grant AFQ056X2201T for provision of AFQ056.

Project description:Recent theories propose that domain-general deficits in prediction (i.e., the ability to anticipate upcoming information) underlie the behavioral characteristics associated with autism spectrum disorder (ASD). If these theories are correct, autistic children might be expected to demonstrate difficulties on linguistic tasks that rely on predictive processing. Previous research has largely focused on older autistic children and adolescents with average language and cognition. The present study used an eye-gaze task to evaluate predictive language processing among 3- to 4-year-old autistic children (n = 34) and 1.5- to 3-year-old, language-matched neurotypical (NT) children (n = 34). Children viewed images (e.g., a cake and a ball) and heard sentences with informative verbs (e.g., Eat the cake) or neutral verbs (e.g., Find the cake). Analyses of children's looking behaviors indicated that young autistic children, like their language-matched NT peers, engaged in predictive language processing. Regression results revealed a significant effect of diagnostic group, when statistically controlling for age differences. The NT group displayed larger difference scores between the informative and neutral verb conditions (in looks to target nouns) compared to the ASD group. Receptive language measures were predictive of looking behavior across time for both groups, such that children with stronger language skills were more efficient in making use of informative verbs to process upcoming information. Taken together, these results suggest that young autistic children can engage in predictive processing though further research is warranted to explore the developmental trajectory relative to NT development. LAY SUMMARY: This study found that 3- to 4-year-old autistic children and younger, language-matched neurotypical (NT) children both used verbs to predict upcoming nouns in sentences like "Eat the cake." For both autistic and NT children, those with stronger language skills were able to predict upcoming nouns more quickly.

Dataset Information

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

Purpose

Method

Results

Conclusion

Publications

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

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