Repetitive Behaviours in Williams Syndrome: A Cross‐Cultural Comparison Between the United Kingdom and Japan

BACKGROUND: Williams syndrome (WS) is a relatively rare neurodevelopmental condition characterised by distinctive cognitive and behavioural phenotypes, including restricted and repetitive behaviours (RRBs). Although cross-cultural studies suggest that caregiver reports of autism-related RRBs may be culturally subjective, little is known about whether caregiver-reported RRB profiles in WS are similar across cultural contexts. Additionally, because RRB profiles may vary with age, examining cross-sectional age-related patterns is important. This study explored between-country variation and cross-sectional age-related patterns in caregiver-reported Repetitive Behaviour Questionnaire (RBQ) scores among individuals with WS in the United Kingdom and Japan. METHODS: Eighty primary caregivers of individuals with WS from Japan (n = 40) and the United Kingdom (n = 40) completed the Repetitive Behaviour Questionnaire (RBQ). Bayesian negative binomial regression models were used to examine between-country differences and cross-sectional age-related associations in caregiver-reported RBQ total, sensory/motor and sameness/circumscribed interests scores. RESULTS: UK caregivers reported directionally higher Total RBQ and Sameness/Circumscribed Interests scores than Japanese caregivers, with the clearest contrast observed for the Sameness/Circumscribed Interests domain. No clear evidence of a between-country difference was found for caregiver-reported Sensory/Motor Behaviours scores, although a weak directional tendency toward higher UK scores was observed. Moreover, age showed weak cross-sectional negative trends for total RBQ and Sensory/Motor Behaviours scores, although these estimates were imprecise and were attenuated in the verbal mental age-adjusted analyses. CONCLUSIONS: These findings are consistent with previous studies of caregiver-reported RRBs among autistic individuals, suggesting that insistence on sameness and circumscribed interests may be reported differently across cultural contexts. In WS, caregiver-reported RBQ profiles may vary by cultural context and show tentative cross-sectional age-related patterns, highlighting the need to consider contextual and developmental factors when interpreting parent-report measures of these behaviours.

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Journal
Journal of Intellectual Disability Research
Published
2026-06-28
DOI
https://doi.org/10.1111/jir.70133
Primary Topic
Williams Syndrome Research
Type
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article

Repetitive Behaviours in Williams Syndrome: A Cross‐Cultural Comparison Between the United Kingdom and Japan

Mary Hanley, Kosuke Asada, Deborah M. Riby, Masahiro Hirai
Journal of Intellectual Disability Research
Williams Syndrome Research
article

Repetitive Behaviours in Williams Syndrome: A Cross‐Cultural Comparison Between the United Kingdom and Japan

Mary Hanley, Kosuke Asada, Deborah M. Riby, Masahiro Hirai
article en

Abstract

BACKGROUND: Williams syndrome (WS) is a relatively rare neurodevelopmental condition characterised by distinctive cognitive and behavioural phenotypes, including restricted and repetitive behaviours (RRBs). Although cross-cultural studies suggest that caregiver reports of autism-related RRBs may be culturally subjective, little is known about whether caregiver-reported RRB profiles in WS are similar across cultural contexts. Additionally, because RRB profiles may vary with age, examining cross-sectional age-related patterns is important. This study explored between-country variation and cross-sectional age-related patterns in caregiver-reported Repetitive Behaviour Questionnaire (RBQ) scores among individuals with WS in the United Kingdom and Japan. METHODS: Eighty primary caregivers of individuals with WS from Japan (n = 40) and the United Kingdom (n = 40) completed the Repetitive Behaviour Questionnaire (RBQ). Bayesian negative binomial regression models were used to examine between-country differences and cross-sectional age-related associations in caregiver-reported RBQ total, sensory/motor and sameness/circumscribed interests scores. RESULTS: UK caregivers reported directionally higher Total RBQ and Sameness/Circumscribed Interests scores than Japanese caregivers, with the clearest contrast observed for the Sameness/Circumscribed Interests domain. No clear evidence of a between-country difference was found for caregiver-reported Sensory/Motor Behaviours scores, although a weak directional tendency toward higher UK scores was observed. Moreover, age showed weak cross-sectional negative trends for total RBQ and Sensory/Motor Behaviours scores, although these estimates were imprecise and were attenuated in the verbal mental age-adjusted analyses. CONCLUSIONS: These findings are consistent with previous studies of caregiver-reported RRBs among autistic individuals, suggesting that insistence on sameness and circumscribed interests may be reported differently across cultural contexts. In WS, caregiver-reported RBQ profiles may vary by cultural context and show tentative cross-sectional age-related patterns, highlighting the need to consider contextual and developmental factors when interpreting parent-report measures of these behaviours.

Journal of Intellectual Disability Research
Jichi Medical University (JP), Toyo University (JP), Durham University (GB), Nagoya Gakuin University (JP), Nagoya University (JP)
Quality Education
Openalex Percentile: Top 9%
Williams Syndrome Research
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