Purpose: To examine which cognitive, environmental, and speech-language variables predict expressive syntax in boys with fragile X syndrome (FXS), boys with Down syndrome (DS), and typically developing (TD) boys, and whether predictive relationships differed by group.
Method: We obtained Index of Productive Syntax ( Scarborough, 1990) scores for 18 boys with FXS only, 20 boys with both FXS and an autism spectrum disorder, 27 boys with DS, and 25 younger TD boys of similar nonverbal mental age. Predictors included group (diagnosis), nonverbal cognition, phonological working memory (PWM), maternal education, speech intelligibility, and expressive vocabulary. The research questions were addressed via hierarchical linear regression.
Results: Diagnostic group, nonverbal cognition, and PWM predicted 56% of the variance in syntactic ability, with approximately three-fourths of the predicted variance explained by group membership alone. The other factors did not contribute any additional significant variance in this final model. There was no evidence that predictor effects differed by group.
Conclusions: Nonverbal cognition and PWM have an effect on expressive syntax beyond that of diagnostic group. These effects are estimated to be the same in boys with FXS, boys with DS, and TD boys. Explanations for residual variance and the relative role of different predictors are discussed.
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http://dx.doi.org/10.1044/1092-4388(2012/10-0153) | DOI Listing |
J Speech Lang Hear Res
January 2025
UC Davis MIND Institute, University of California, Sacramento, CA.
Purpose: The current study addresses a gap in the literature regarding syntactic development of adolescent boys with fragile X syndrome (FXS) and Down syndrome (DS). Specifically, we ask whether syntactic skills plateau or continue to change during adolescence for these groups and whether the profile of syntactic change differs between boys with FXS and those with DS.
Method: Participants were 38 boys with FXS (with and without autism) and 20 boys with DS between the ages of 10 and 16 years, as well as 33 boys who were neurotypical between the ages of 3 and 8 years at study entry.
Neurosci Biobehav Rev
July 2024
Institute for Biomedical Research and Innovation (IRIB), National Research Council (CNR), Via Paolo Gaifami 18, Catania 95126, Italy. Electronic address:
Fragile X messenger ribonucleoprotein 1 (FMRP) is a widely expressed RNA binding protein involved in several steps of mRNA metabolism. Mutations in the FMR1 gene encoding FMRP are responsible for fragile X syndrome (FXS), a leading genetic cause of intellectual disability and autism spectrum disorder, and fragile X-associated tremor-ataxia syndrome (FXTAS), a neurodegenerative disorder in aging men. Although FMRP is mainly expressed in neurons, it is also present in glial cells and its deficiency or altered expression can affect functions of glial cells with implications for the pathophysiology of brain disorders.
View Article and Find Full Text PDFJ Neurodev Disord
May 2024
Graduate Neuroscience Program, University of California, Riverside, USA.
Background: Autism spectrum disorder (ASD) is currently diagnosed in approximately 1 in 44 children in the United States, based on a wide array of symptoms, including sensory dysfunction and abnormal language development. Boys are diagnosed ~ 3.8 times more frequently than girls.
View Article and Find Full Text PDFGenes (Basel)
March 2024
Medical Investigation of Neurodevelopmental Disorders (MIND) Institute, University of California Davis, Sacramento, CA 95616, USA.
Fragile X syndrome (FXS) is the leading inherited cause of intellectual disability (ID) and single gene cause of autism. Although most patients with FXS and the full mutation (FM) have complete methylation of the fragile X messenger ribonucleoprotein 1 () gene, some have mosaicism in methylation and/or CGG repeat size, and few have completely unmethylated FM alleles. Those with a complete lack of methylation are rare, with little literature about the cognitive and behavioral phenotypes of these individuals.
View Article and Find Full Text PDFJ Speech Lang Hear Res
March 2024
MIND Institute, UC Davis Health, Sacramento, CA.
Purpose: Past research shows that parentally responsive behavior toward the child positively influences language development in both neurotypical children and children with intellectual and developmental disabilities, including those with fragile X syndrome (FXS); however, most studies have focused exclusively on the mother-child relationship. The current study examined relationships between parent behavior (i.e.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!