Behavioral style of young boys with fragile X syndrome

Abstract

To study the behavioral style or temperament of 45 boys, aged 47 to 88 months, with full-mutation fragile X syndrome (FXS), 102 parent ratings on the Behavioral Style Questionnaire (McDevitt and Carey 1978) were recorded. These ratings were analysed with a variety of statistical techniques. Considerable variability was evident in temperament profiles; consequently, a characteristic profile was not identified for FXS. Boys with FXS differed significantly from the reference sample on five of nine temperament dimensions. They were more active and less intense, approachable, adaptable, and persistent. No significant differences were found in distractibility, rhythmicity, mood, or sensory threshold. Only 16 of the 45 boys in the sample could be classified as easy, difficult, or slow to warm up. There was no link between severity of developmental disability and temperament ratings. This supports the theory that intelligence and temperament are separate constructs. Scores on temperament dimensions were stable over time. Our results suggest that many of the behaviors observed in boys with FXS may be related to temperament. Consequently, parent counseling and environmental modifications should be considered as first line treatment. The question of whether the behavior problems observed in boys with FXS are innate or whether they result from poorness of fit between child and environment is an important issue that needs further study.

Temperament and Sensory Features of Children with Autism

“…Based on previous characterizations of temperament in children with ASD using Thomas and Chess’ model (Bailey, et al, 2000; Bieberich & Morgan, 2004; Garon et al, 2009; Hatton, et al, 1999; Hepburn & Stone, 2006), we hypothesized that the ASD group would be more active, more withdrawn, less adaptive, less intense in their reactions, and less sensitive to environmental stimuli compared to the typically developing group. It also was expected that the ASD group would be more withdrawn on the approach dimension compared to the DD group.…”

“…Given this inherent conceptual overlap, it is not surprising that previous studies have found that the temperament profiles for children with ASD, as a group, differ from that of typically developing (TD) children (Bailey et al, 2000; Bieberich & Morgan, 2004; Garon et al, 2009; Hatton et al, 1999; Hepburn & Stone, 2006). Compared to TD comparison groups, children with ASD were more active, more withdrawn (Bailey et al, 2000; Bieberich & Morgan, 2004; Garon et al, 2009), less adaptive (Bailey et al, 2000; Hepburn & Stone, 2006), less persistent (Bailey et al, 2000), less intense in their reactions (Hatton et al, 1999), less sensitive to environmental stimuli (Hepburn & Stone, 2006), and had lower positive affect, higher negative affect, and more difficulty controlling attention and behavior (Garon et al, 2009).…”

“…Compared to TD comparison groups, children with ASD were more active, more withdrawn (Bailey et al, 2000; Bieberich & Morgan, 2004; Garon et al, 2009), less adaptive (Bailey et al, 2000; Hepburn & Stone, 2006), less persistent (Bailey et al, 2000), less intense in their reactions (Hatton et al, 1999), less sensitive to environmental stimuli (Hepburn & Stone, 2006), and had lower positive affect, higher negative affect, and more difficulty controlling attention and behavior (Garon et al, 2009). Only a few published studies have compared the temperament of ASD and developmentally delayed (DD) groups, finding that children with ASD showed less self-regulation (Bieberich & Morgan, 2004; Gomez & Baird, 2005); however, these studies used a different model of temperament (Rothbart & Derryberry, 1981) than the Thomas & Chess (1968) model adopted for the present study.…”

“…First, we aimed to determine the replicability of previous studies that found differences between children with ASD and TD on measures of temperament, and expand these findings by specifically testing differences between children with ASD and children with DD. Based on previous characterizations of temperament in children with ASD using the Thomas and Chess model of temperament (Bailey, et al, 2000; Bieberich & Morgan, 2004; Garon et al, 2009; Hatton, et al, 1999; Hepburn & Stone, 2006), we hypothesized that compared to the TD group, the ASD group would be temperamentally more active, more withdrawn, less adaptive, less intense in their reactions, and less sensitive to environmental stimuli. We expected that because social impairment is a diagnostic feature of autism, and because high levels of both sensory hypo- and hyperresponsiveness may contribute to difficulties with social engagement (Baranek et al, 2006), the ASD group will be more temperamentally withdrawn (on the approach dimension) than the DD group.…”

Early Negative Affect Predicts Anxiety, not Autism, in Preschool Boys with Fragile X Syndrome

“…Levels of temperament have been shown to remain relatively stable in FXS across early childhood (Hatton, et al). Compared to typical controls, parents have indicated elevated activity and withdrawal; as well as lower intensity, approachability, adaptability, sadness, anger, and persistence in young boys with FXS (Bailey et al 2000; Hatton et al 1999; Roberts et al 2006; Shanahan et al 2008). Evidence suggests that temperament does not differ across participants FXS+autism and FXS-only (Bailey et al 2000); however, dampened facial sadness has been associated with autistic symptoms in FXS (Shanahan et al).…”