Assessment & Research

Brief Report: Repetitive Behaviour Profiles in Williams syndrome: Cross Syndrome Comparisons with Prader-Willi and Down syndromes.

Royston et al. (2018) · Journal of autism and developmental disorders 2018
★ The Verdict

Repetitive behaviors look almost the same across Williams, Prader-Willi, and Down syndromes, so treat the person, not the label.

✓ Read this if BCBAs who work with adults with genetic syndromes in day or residential programs.
✗ Skip if Clinicians looking only for autism-specific RRB interventions.

01Research in Context

01

What this study did

Cohen et al. (2018) sent a survey to parents of adults with Williams, Prader-Willi, or Down syndrome.

Parents checked boxes on the Repetitive Behaviour Questionnaire. The team then lined up the three profiles to see where they differed.

02

What they found

All three groups showed almost the same repetitive behavior pattern.

Williams syndrome had only tiny bumps: a bit more body rocking and hoarding. Otherwise the profiles sat on top of each other.

03

How this fits with other research

South et al. (2005) used the same survey tool on kids with Asperger and high-functioning autism. They also found no big split between groups, showing the questionnaire can pick up fine shades but not wide gaps.

Mukherjee et al. (2015) argue that precise behavioral maps like this one are the bridge that lets future gene studies turn into real help for families.

Koller et al. (2022) looked at repetitive behaviors in autistic children and linked parent accommodation to later problem behavior. Their focus is changeable parent actions, while R et al. paint a fixed picture; the two views sit side-by-side, not in conflict.

04

Why it matters

If you assess adults with genetic syndromes, expect similar repetitive topographies. Spend your energy on supports for daily living, not on hunting for big syndrome-only quirks. Track small differences like hoarding only when they create safety issues.

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Pull the Repetitive Behaviour Questionnaire for your new adult client and use it as a baseline instead of syndrome-specific checklists.

02At a glance

Intervention
not applicable
Design
survey
Sample size
277
Population
down syndrome, other
Finding
not reported

03Original abstract

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation.

Journal of autism and developmental disorders, 2018 · doi:10.1007/s10803-017-3319-6