Background Repetitive behaviours are frequently observed in individuals with intellectual disability. The present study examined the profile, inter-correlations, and predictive correlates of repetitive behaviours in males with FXS, the leading inherited cause of intellectual disability. Specific child characteristics examined as predictors included: anxiety, nonverbal cognition, and autism social-affective symptomatology. Method Participants were 39 boys with FXS (aged 6–10 years). Repetitive behaviours were measured using the Repetitive Behavior Scale – Revised (RBS-R) – a 43-item caregiver-report measure normed on individuals with intellectual disability. Results Restricted Interests and Sensory Motor behaviours were reported as most problematic for this sample of boys, whereas Self-injurious behaviours were less problematic. All subscales of the RBS-R were significantly inter-correlated. Nonverbal IQ was negatively related, whereas anxiety and social affective symptoms of ASD were positively related, to scores for Restricted Interests. Anxiety was also positively related to scores for Compulsive behaviours and Ritualistic Sameness behaviours. Conclusions This study provides a preliminary description of repetitive behaviours in males with FXS, which may form the groundwork for future research.

A small randomized group design (N = 20) was used to examine a parent-implemented intervention designed to improve the spoken language skills of school-aged and adolescent boys with FXS, the leading cause of inherited intellectual disability. The intervention was implemented by speech-language pathologists who used distance video-teleconferencing to deliver the intervention. The intervention taught mothers to use a set of language facilitation strategies while interacting with their children in the context of shared story-telling. Treatment group mothers significantly improved their use of the targeted intervention strategies. Children in the treatment group increased the duration of engagement in the shared story-telling activity as well as use of utterances that maintained the topic of the story. Children also showed increases in lexical diversity, but not in grammatical complexity.

Using a single case design, a parent-mediated spoken language intervention was delivered to three mothers and their school-aged sons with fragile X syndrome, the leading inherited cause of intellectual disability. The intervention was embedded in the context of shared story-telling using wordless picture books and targeted three empirically-derived language support strategies. All sessions were implemented via distance video-teleconferencing. Parent education sessions were followed by 12 weekly clinician coaching and feedback sessions. Data was collected weekly during independent homework and clinician observation sessions. Relative to baseline, mothers increased their use of targeted strategies and dyads increased the frequency and duration of story-related talking. Generalized effects of the intervention on lexical diversity and grammatical complexity were observed. Implications for practice are discussed.

Background: The purpose of this study was to conduct a 20-week controlled trial of lovastatin (10 to 40 mg/day) in youth with fragile X syndrome (FXS) ages 10 to 17 years, combined with an open-label treatment of a parentimplemented language intervention (PILI), delivered via distance video teleconferencing to both treatment groups, lovastatin and placebo. Method: A randomized, double-blind trial was conducted at one site in the Sacramento, California, metropolitan area. Fourteen participants were assigned to the lovastatin group; two participants terminated early from the study. Sixteen participants were assigned to the placebo group. Lovastatin or placebo was administered orally in a capsule form, starting at 10 mg and increasing weekly or as tolerated by 10 mg increments, up to a maximum dose of 40 mg daily. A PILI was delivered to both groups for 12 weeks, with 4 activities per week, through video teleconferencing by an American Speech-Language Association-certified Speech-Language Pathologist, in collaboration with a Board-Certified Behavior Analyst. Parents were taught to use a set of language facilitation strategies while interacting with their children during a shared storytelling activity. The main outcome measures included absolute change from baseline to final visit in the means for youth total number of story-related utterances, youth number of different word roots, and parent total number of story-related utterances.