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    ABSTRACT: Abstract This study tested the hypothesis that pragmatic (i.e., social) language impairment is linked to arousal dysregulation in autism spectrum disorder (ASD) and fragile X syndrome (FXS). Forty boys with ASD, 39 with FXS, and 27 with typical development (TD), aged 4-15 years, participated. Boys with FXS were hyperaroused compared to boys with TD but did not differ from boys with ASD. Dampened vagal tone predicted pragmatic impairment in ASD, and associations emerged between cardiac activity and receptive/expressive vocabulary across groups. Findings support autonomic dysfunction as a mechanism underlying pragmatic impairment in ASD and suggest that biophysiological profiles are shared in ASD and FXS, which has implications for understanding the role of fragile X mental retardation-1 (FMR1, the FXS gene) in the pathophysiology of ASD.
    American Journal on Intellectual and Developmental Disabilities 11/2013; 118(6):475-95. DOI:10.1352/1944.7558-118.6.475 · 2.08 Impact Factor
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    ABSTRACT: Symptoms of autism are frequent in males with fragile X syndrome (FXS), but it is not clear whether symptom profiles differ from those of nonsyndromic ASD. Using individual item scores from the Autism Diagnostic Inventory-Revised, we examined which current symptoms of autism differed in boys with FXS relative to same-aged boys diagnosed with nonsyndromic ASD. In addition, different subsamples of participants were matched on autism diagnostic status and severity of autism symptoms. Between-group comparisons revealed that boys with FXS showed significantly less impairment in Social Smiling than did age-, diagnostic-, and severity-matched boys with nonsyndromic ASD. Severity-matched boys with FXS showed more impairment in complex mannerisms than did boys with nonsyndromic ASD. Behavioral differences between FXS and nonsyndromic ASD may be of theoretical importance in understanding the causes and correlates of ASD in FXS and in developing and implementing appropriate treatments.
    Journal of Autism and Developmental Disorders 01/2014; 45(7). DOI:10.1007/s10803-013-2013-6 · 3.06 Impact Factor
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    ABSTRACT: Fragile X syndrome (FXS) is associated with a wide range of cognitive, emotional, and behavioral difficulties. Although there is considerable research on the behavioral phenotype of FXS, few empirical studies of behavioral interventions with this population have been identified. Through a hand search of 34 behavioral journals, we examined the evidence base for behavioral interventions with individuals with FXS and in light of the current state of knowledge regarding the FXS behavioral phenotype. Systematic review procedures were used to identify behavioral intervention studies that included at least one participant with FXS, extract and summarize the data on several relevant dimensions, and rate the methodological quality of the studies. Results revealed 31 intervention studies with a small number of participants with FXS. Overall, results suggest a behavioral approach to intervention with individuals with FXS shows promise. Future research focused on individuals with FXS will be necessary to continue to examine differences in response to intervention and interventions that specifically address phenotypic characteristics. Copyright © 2014 Elsevier Ltd. All rights reserved.
    Research in Developmental Disabilities 01/2015; 38C:223-241. DOI:10.1016/j.ridd.2014.12.011 · 3.40 Impact Factor