Abstract
BackgroundIndividuals diagnosed with fragile X syndrome (FXS), the most common known inherited form of intellectual disability, commonly exhibit significant impairments in social gaze behavior during interactions with others. Although this behavior can restrict social development and limit educational opportunities, behavioral interventions designed to improve social gaze behavior have not been developed for this population. In this proof of concept (PoC) study, we examined whether administering a behavioral skills training package—discrete trial instruction (DTI) plus relaxation training—could increase social gaze duration in males with FXS.MethodsAs part of a larger clinical trial, 20 boys with FXS, aged 8 to 18 years, were randomized to receive DTI plus relaxation training administered at one of two prescribed doses over a 2-day period at our research center. Potential improvements in social gaze behavior were evaluated by direct observations conducted across trials during the training, and generalization effects were examined by administering a social challenge before and after the treatment. During the social challenge, social gaze behavior was recorded using an eye tracker and physiological arousal levels were simultaneously recorded by monitoring the child’s heart rate.ResultsLevels of social gaze behavior increased significantly across blocks of training trials for six (60%) boys who received the high-dose behavioral treatment and for three (30%) boys who received the low-dose behavioral treatment. Boys who received the high-dose treatment also showed greater improvements in social gaze behavior during the social challenge compared to boys who received the low-dose treatment. There was no effect of the treatment on physiological arousal levels recorded on the heart rate monitor at either dose.ConclusionsThese results suggest that appropriate social gaze behavior can be successfully taught to boys with FXS using a standardized behavioral skills training approach. Future studies will need to evaluate whether younger children with FXS might benefit from this treatment, and/or whether more naturalistic forms of behavioral skills training might be beneficial, before social gaze avoidance becomes established in the child’s repertoire.Trial registrationClinicalTrials.gov, NCT02616796. Registered 30 November 2015.
Highlights
Individuals diagnosed with fragile X syndrome (FXS), the most common known inherited form of intellectual disability, commonly exhibit significant impairments in social gaze behavior during interactions with others
FXS is caused by mutations to the FMR1 gene at locus 27.3 on the long arm of the X chromosome [6] which results in excessive methylation of the gene and subsequent reduced or absent fragile X mental retardation protein (FMRP)
Studies have shown that boys with FXS typically engage in increased social gaze avoidance and social withdrawal during social interactions with unfamiliar people compared to individuals who are more familiar [10,11,12,13]
Summary
Individuals diagnosed with fragile X syndrome (FXS), the most common known inherited form of intellectual disability, commonly exhibit significant impairments in social gaze behavior during interactions with others. This behavior can restrict social development and limit educational opportunities, behavioral interventions designed to improve social gaze behavior have not been developed for this population. A subgroup of children with developmental disabilities who are prone to exhibit impairments in social gaze behavior are individuals with fragile X syndrome (FXS). Studies have shown that boys with FXS typically engage in increased social gaze avoidance and social withdrawal during social interactions with unfamiliar people compared to individuals who are more familiar [10,11,12,13]. In a naturalistic eye-tracking study, for example, Hall and colleagues [14] reported that individuals with FXS spent less time looking at an unfamiliar experimenter and exhibited shorter inter-episodes of social gaze when compared to age and IQ-matched controls [14]
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