Abstract
The primary goal of this study was to examine environmental and neuroendocrine factors that convey increased risk for elevated autistic behavior in boys with Fragile X syndrome (FXS). This study involves three related analyses: (1) examination of multiple dimensions of social approach behaviors and how they vary over time, (2) investigation of mean levels and modulation of salivary cortisol levels in response to social interaction, and (3) examination of the relationship of social approach and autistic behaviors to salivary cortisol. Poor social approach and elevated baseline and regulation cortisol are discernible traits that distinguish boys with FXS and ASD from boys with FXS only and from typically developing boys. In addition, blunted cortisol change is associated with increased severity of autistic behaviors only within the FXS and ASD group. Boys with FXS and ASD have distinct behavioral and neuroendocrine profiles that differentiate them from those with FXS alone and typically developing boys.
Highlights
Fragile X syndrome (FXS) is the most prevalent form of inherited intellectual disability, affecting approximately 1:4,000 males and 1:8,000 females [1]
analyses of covariance (ANCOVA) results show that both baseline and regulation cortisol were higher in boys with FXS + autism spectrum disorder (ASD) compared to both typically developing boys and boys with FXS without autism, which were not different from each other
We employed a dynamic multidimensional observational scale of social approach behavior and scales of autistic behavior in boys with FXS with and without severe autistic behavior (FXS + ASD vs. FXSonly), who were compared to typically developing boys
Summary
Fragile X syndrome (FXS) is the most prevalent form of inherited intellectual disability, affecting approximately 1:4,000 males and 1:8,000 females [1]. This single gene disorder is linked to the expansion of a CGG polymorphism in the (5′UTR) regulatory region of the FMR1 gene. Magnitude of FMRP deficit correlates with overall severity of physical and neurobehavioral phenotype [5,6,7], but not consistently with selective behavioral abnormalities such as autistic behavior [8, 9]. Most males with FXS tend to have mild to moderate intellectual disability, characterized by variable cognitive and language impairments, and associated neurobehavioral problems [2, 10]
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