These findings suggest that some aspects of the conversational discourse difficulties attributed to fragile X syndrome may be a function of the high rate of comorbidity between fragile X and autism, whereas some difficulties may be characteristic of fragile X syndrome.
Boys with fragile X syndrome with (n = 49) and without (n = 33) characteristics of autism spectrum disorder, boys with Down syndrome (39), and typically developing boys (n = 41) were compared on standardized measures of receptive vocabulary, expressive vocabulary, and speech administered annually over 4 years. Three major findings emerged. Boys with fragile X without autism spectrum disorder did not differ from the younger typically developing boys in receptive and expressive vocabulary and speech production when compared at similar levels of nonverbal cognitive skills. Boys with fragile X without autism spectrum disorder and typically developing boys had higher receptive vocabulary and speech production than did boys with Down syndrome. There were mixed patterns of results for the boys with fragile X and characteristics of autism spectrum disorder.
Verbal perseveration is a frequently reported language characteristic of males with Fragile X syndrome and may be a defining feature or hallmark of the syndrome. We compared the verbal perseveration of boys with Fragile X syndrome with (n = 29) and without (n = 30) autism spectrum disorder, boys with Down syndrome (n = 27), and typically developing boys (n = 25) at similar nonverbal mental ages. During a social interaction, boys with both Fragile X syndrome and autism spectrum disorder produced significantly more topic perseveration than all other groups. In social interaction as compared to narration, boys with Fragile X syndrome (regardless of autism status) produced significantly more topic perseveration. These findings suggest that autism status, as well as language sampling context, affect perseveration in boys with Fragile X syndrome.
There is little research on the functional assessment and treatment of anxiety and related problem behavior in children with autism spectrum disorder (ASD), particularly those with intellectual and developmental disability (IDD). In a recent study, we evaluated a multimethod strategy for assessing anxiety in children with ASD and IDD (Am J Intellect Dev Disabil 118:419-434, 2013). In the present study, we developed treatments for the anxiety and associated problem behavior in these same children. A multiple baseline design was used to evaluate the effectiveness of a multicomponent intervention package, incorporating individualized strategies from Positive Behavior Support and Cognitive Behavioral Therapy. During intervention, all three participants showed substantial decreases in anxiety and problem behavior and significant increases in respiratory sinus arrhythmia in the situations that had previously been identified as anxiety-provoking.
Despite the increased risk for anxiety disorders in children with autism spectrum disorders (ASD), there is a lack of research on the assessment and treatment of anxiety in this population, particularly for those with an intellectual disability (ID). The present study evaluated a multimethod strategy for the assessment of anxiety and problem behavior in three children with ASD and ID. Anxiety was operationally defined using: (1) behavioral data from anxious behaviors, (2) affective/contextual data from parent-report and observer ratings of overall anxiety, and (3) physiological data (heart rate [HR] and respiratory sinus arrhythmia [RSA]). A functional assessment of problem behavior during high- and low-anxiety conditions was conducted. Higher levels of problem behavior and HR and lower RSA were found in the high-anxiety than in the low-anxiety conditions.
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