Social Escape Behaviors in Children with Fragile X Syndrome

Department of Psychiatry and Behavioral Sciences, Stanford University, Palo Alto, California, United States
Journal of Autism and Developmental Disorders (Impact Factor: 3.34). 11/2006; 36(7):935-47. DOI: 10.1007/s10803-006-0132-z
Source: PubMed


Social escape behavior is a common behavioral feature of individuals with fragile X syndrome (fraX). In this observational study, we examined the effect of antecedent social and performance demands on problem behaviors in four conditions: face-to-face interview, silent reading, oral reading and a singing task. Results showed that problem behaviors were significantly more likely to occur during the interview and singing conditions. Higher levels of salivary cortisol were predictive of higher levels of fidgeting behavior and lower levels of eye contact in male participants. There were no associations between level of FMRP expression and social escape behaviors. These data suggest that specific antecedent biological and environmental factors evoke social escape behaviors in fragile X syndrome.

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    • "Assessing the operant function of challenging behavior in young children with FXS is important as previous research indicates that challenging behaviors often appear very early in males with full mutation FXS (Bailey, Raspa, Olmstead, & Holiday, 2008; Symons, Byiers, Raspa, Bishop, & Bailey, 2010). Additionally, a growing body of research demonstrates that even the challenging behaviors associated with genetic syndromes such as FXS are sensitive to environmental antecedents and consequences (Anderson, Dancis, & Alpert, 1978; Hall et al., 2006; Hall, Oliver, & Murphy, 2001; Lesniak-Karpiak, Mazzocco, & Ross, 2003; Oliver, Murphy, Crayton, & Corbett, 1993; Sloneem, Arron, Hall, & Oliver, 2009; Symons et al., 2010; Taylor & Oliver, 2008). Using functional analysis methodology to determine the operant function of challenging behaviors in younger children with FXS will be important because it may lead to the development of effective early behavioral intervention, thus preventing the worsening of behaviors, as the child grows older. "
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    ABSTRACT: This study used experimental functional analyses to examine the operant function of challenging behaviors exhibited by 12 males (ages 27–51 months) with fragile X syndrome (FXS). Eight children met criteria for negatively reinforced challenging behavior in the form of escape from demands and/or escape from social interactions. Nine children met criteria for positively reinforced challenging behavior in the form of obtaining access to highly preferred items. Attention was identified as a maintaining consequence for three children. The functional analysis was inconclusive for one child. Results suggest that, for young males with FXS, challenging behaviors may more likely be tangibly and escape maintained than attention maintained. Our findings affirm past research suggesting a unique behavioral phenotype for this population.
    Research in developmental disabilities 07/2014; 35(7). DOI:10.1016/j.ridd.2014.03.014 · 4.41 Impact Factor
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    • "These findings are consistent with previous FXS studies, which have reported hyperarousal for social stimuli in FXS individuals compared to CA-matched controls, when using either social interactional paradigms (Belser and Sudhalter, 1995; Hall et al., 2009) or images displaying direct-gaze faces (Farzin et al., 2009, 2011). They are also commensurate with behavioural studies which report social avoidance and social anxiety in the FXS population (e.g., Cohen et al., 1991; Cordeiro et al., 2011; Hall et al., 2006; Hessl et al., 2006; Tsiouris and Brown, 2004). The fact that hyper-reactivity to direct-gaze emotional faces was found more so for negative emotional expressions (disgusted, sad and angry) is also consistent with emerging evidence of amygdala dysfunction in the FXS population (e.g., Hessl et al., 2007, 2011; Suvrathan and Chattarji, 2011). "
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    ABSTRACT: Fragile X syndrome (FXS) is characterized by hyper-reactivity, autistic tendencies and social anxiety. It has been hypothesized that the FXS social phenotype is secondary to a generalized hyper-reactivity that leads to social avoidance. No study, however, has investigated whether hyperarousal in FXS is generalized or more specific to socially salient information. We recorded skin conductance responses (SCRs) while females with FXS, as well as chronological age- (CA-) and mental age- (MA-) matched controls, viewed two sets of visual images: direct-gaze emotional faces and affectively arousing scenes. Explicit emotion recognition and subjective ratings of emotions aroused by images were also recorded. Overall, females with FXS displayed hyper-reactivity only when viewing the more socially salient stimuli (emotional faces), compared to CA-matched controls, but not MA-matched controls. Moreover, females with FXS also displayed atypical emotion recognition abilities and subjective ratings of their own emotional states. These findings suggest that any hyper-reactivity observed in FXS may be more specific to socially salient stimuli, rather than generalized.
    International journal of psychophysiology: official journal of the International Organization of Psychophysiology 01/2013; 88(1). DOI:10.1016/j.ijpsycho.2012.12.009 · 2.88 Impact Factor
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    • "An example of the utility of psychophysiological measures in early-phase clinical trials in FXS was the demonstration of improvement in prepulse inhibition in an open label, single dose trial of the mGluR5- blocker fenobam (Berry-Kravis et al. 2009). Gaze avoidance is a hallmark behavioral feature of FXS (Cohen et al. 1988; Bregman et al. 1988; Garrett et al. 2004; Farzin et al. 2009; Cohen et al. 1989), and has been physiologically linked with cortisol dysregulation (Hessl et al. 2002, 2006) and enhanced autonomic reactivity (Farzin et al. 2009; Hall et al. 2006, 2009; Belser and Sudhalter 1995). Findings from neuroimaging studies suggest atypical neural circuitry involved in face processing and social cognition may exist in individuals in FXS (Dalton et al. 2007; Holsen et al. 2008). "
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    ABSTRACT: Recent insight into the underlying molecular and cellular mechanisms of fragile X syndrome (FXS) has led to the proposal and development of new pharmaceutical treatment strategies, and the initiation of clinical trials aimed at correcting core symptoms of the developmental disorder. Consequently, there is an urgent and critical need for outcome measures that are valid for quantifying specific symptoms of FXS and that are consistent across time. We used eye tracking to evaluate test-retest reliability of gaze and pupillometry measures in individuals with FXS and we demonstrate that these measures are viable options for assessing treatment-specific outcomes related to a core behavioral feature of the disorder.
    Journal of Autism and Developmental Disorders 11/2011; 41(11):1515-22. DOI:10.1007/s10803-011-1176-2 · 3.06 Impact Factor
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