Abstract
Restricted and repetitive behavior (RRB) is a group of heterogeneous maladaptive behaviors. RRB is one of the key diagnostic features of autism spectrum disorders (ASDs) and also commonly observed in Prader–Willi syndrome (PWS). In this study, we assessed RRB using the Repetitive Behavior Scale-Revised (RBS-R) in two ASD samples (University of Illinois at Chicago [UIC] and University of Florida [UF]) and one PWS sample. We compared the RBS-R item endorsements across three ASD cohorts (UIC, UF and an ASD sample from Lam, The Repetitive Behavior Scale-Revised: independent validation and the effect of subject variables, PhD thesis, 2004), and a PWS sample. We also compared the mean RBS-R subscale/sum scores across the UIC, UF and PWS samples; across the combined ASD (UIC + UF), PWS-deletion and PWS-disomy groups; and across the combined ASD sample, PWS subgroup with a Social Communication Questionnaire (SCQ) score ≥15, and PWS subgroup with a SCQ score <15. Despite the highly heterogeneous nature, the three ASD samples (UIC, UF and Lam’s) showed a similar pattern of the RBS-R endorsements, and the mean RBS-R scores were not different between the UIC and UF samples. However, higher RRB was noted in the ASD sample compared with the PWS sample, as well as in the PWS subgroup with a SCQ score ≥15 compared with the PWS subgroup with a SCQ score <15. Study limitations include a small sample size, a wide age range of our participants, and not controlling for potential covariates. A future replication study using a larger sample and further investigation into the genetic bases of overlapping ASD and RRB phenomenology are needed, given the higher RRB in the PWS subgroup with a SCQ score ≥15.
Highlights
Restricted and repetitive behavior (RRB) is a group of heterogeneous maladaptive behaviors, such as flapping arms, lining up objects, peculiar fascination with odd objects or parts of objects, a very narrow set of restricted interests, intolerance to changes in routines, and insistence on sameness (Lewis and Kim 2009)
All three autism spectrum disorders (ASDs) cohorts (UIC, UF, and Lam) showed a similar pattern of the Repetitive Behavior Scale-Revised (RBS-R) individual item endorsements compared with the Prader–Willi syndrome (PWS) sample
The PWS-deletion and PWS-disomy subgroups did not show statistically significant group differences in the RBS-R individual item endorsement; the PWS-deletion subgroup showed a trend for higher endorsements of four items from two RBS-R subscale scores
Summary
Restricted and repetitive behavior (RRB) is a group of heterogeneous maladaptive behaviors, such as flapping arms, lining up objects, peculiar fascination with odd objects or parts of objects, a very narrow set of restricted interests, intolerance to changes in routines, and insistence on sameness (Lewis and Kim 2009). J Neurodevelop Disord (2011) 3:316–324 controlling RRB are likely independent of genes controlling social or communication deficits (Silverman et al 2002; Ronald et al 2006; Mandy and Skuse 2008). Studies have reported a high level and wide range of RRB extending beyond food-related behavior among individuals with PWS (Greaves et al 2006). A recent study (Greaves et al 2006) suggested a similar level of RRB among children with ASDs and children with PWS using the Childhood Routines Inventory (CRI) (Evans et al 1997). An increased rate of ASDs has been reported among individuals with PWS, especially among those with maternal UPD compared to those with a deletion (37.7% vs 18.6%) (Veltman et al 2005). Few studies have examined the RRB phenomenology among individuals with PWS using a comprehensive RRB measure, nor examined the characteristics of RRB across these two populations, despite the reports of increased rates of RRB as well as ASDs among individuals with PWS
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