Skip to main content
SpringerLink
Log in

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

  • Brief Report
  • Published:
Journal of Autism and Developmental Disorders Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

References

  • Davies, M., Udwin, O., & Howlin, P. (1998). Adults with Williams syndrome - Preliminary study of social, emotional and behavioural difficulties. The British journal of psychiatry: the journal of mental science, 172, 273–276. doi:10.1192/bjp.172.3.273.

    Article  Google Scholar 

  • Dugas, M. J., Schwartz, A., & Francis, K. (2004). Brief report: Intolerance of uncertainty, worry, and depression. Cognitive Therapy and Research, 28(6), 835–842.

    Article  Google Scholar 

  • Dykens, E. M., Leckman, J. F., & Cassidy, S. B. (1996). Obsessions and compulsions in Prader–Willi syndrome. Journal of Child Psychology and Psychiatry, 37(8), 995–1002.

    Article  PubMed  Google Scholar 

  • Dykens, E. M., & Rosner, B. A. (1999). Refining behavioral phenotypes: Personality-motivation in Williams and Prader–Willi syndromes. American Journal on Mental Retardation, 104(2), 158–169.

    Article  PubMed  Google Scholar 

  • Green, T., Avda, S., Dotan, I., Zarchi, O., Basel-Vanagaite, L., Zalsman, G., … & Gothelf, D. (2012). Phenotypic psychiatric characterization of children with Williams syndrome and response of those with ADHD to methylphenidate treatment. American Journal of Medical Genetics Part B: Neuropsychiatric Genetics, 159(1), 13–20.

    Article  Google Scholar 

  • Howlin, P., Elison, S., & Stinton, C. (2010). Cognitive, linguistic and adaptive functioning in Williams syndrome: Trajectories from early to middle adulthood. Journal of Applied Research in Intellectual Disabilities, 23, 322–336.

    Article  Google Scholar 

  • Janes, E., Riby, D. M., & Rodgers, J. (2014). Exploring the prevalence and phenomenology of repetitive behaviours and abnormal sensory processing in children with Williams Syndrome. Journal of Intellectual Disability Research, 58(8), 746–757. doi:10.1111/jir.12086.

    Article  PubMed  Google Scholar 

  • Joyce, C., Honey, E., Leekam, S. R., Barrett, S. L., & Rodgers, J. (2017). Anxiety, intolerance of uncertainty and restricted and repetitive behaviour: Insights directly from young people with ASD. Journal of Autism and Developmental Disorders. doi:10.1007/s10803-017-3027-2.

    PubMed  Google Scholar 

  • Kushlick, A., Blunden, R., & Cox, G. (1973). A method of rating behaviour characteristics for use in large scale surveys of mental handicap. Psychological Medicine, 3(4), 466–478.

    Article  PubMed  Google Scholar 

  • Lincoln, A. J., Searcy, Y. M., Jones, W., & Lord, C. (2007). Social interaction behaviors discriminate young children with autism and Williams syndrome. Journal of the American Academy of Child & Adolescent Psychiatry, 46(3), 323–331.

    Article  Google Scholar 

  • Martens, M. A., Wilson, S. J., & Reutens, D. C. (2008). Research review: Williams syndrome: A critical review of the cognitive, behavioral, and neuroanatomical phenotype. Journal of Child Psychology and Psychiatry, 49(6), 576–608.

    Article  PubMed  Google Scholar 

  • Miyake, A., & Friedman, N. P. (2012). The nature and organization of individual differences in executive functions four general conclusions. Current Directions in Psychological Science, 21(1), 8–14.

    Article  PubMed  PubMed Central  Google Scholar 

  • Mosconi, M., Kay, M., D’Cruz, A.-M., Seidenfeld, A., Guter, S., Stanford, L., et al. (2009). Impaired inhibitory control is associated with higher-order repetitive behaviors in autism spectrum disorders. Psychological Medicine, 39(09), 1559–1566.

    Article  PubMed  PubMed Central  Google Scholar 

  • Moss, J., Oliver, C., Arron, K., Burbidge, C., & Berg, K. (2009). The prevalence and phenomenology of repetitive behavior in genetic syndromes. Journal of Autism and Developmental Disorders, 39(4), 572–588.

    Article  PubMed  Google Scholar 

  • Oglesby, M. E., Medley, A. N., Norr, A. M., Capron, D. W., Korte, K. J., & Schmidt, N. B. (2013). Intolerance of uncertainty as a vulnerability factor for hoarding behaviors. Journal of Affective Disorders, 145(2), 227–231.

    Article  PubMed  Google Scholar 

  • Pober, B. R. (2010). Medical progress: Williams-Beuren syndrome. [Review]. New England Journal of Medicine, 362(3), 239–252.

    Article  PubMed  Google Scholar 

  • Riby, D. M., Janes, E., & Rodgers, J. (2013). Brief report: exploring the relationship between sensory processing and repetitive behaviours in Williams syndrome. Journal of Autism and Developmental Disorders, 43(2), 478–482. doi:10.1007/s10803-012-1557-1.

    Article  PubMed  Google Scholar 

  • Richdale, A., Cotton, S., & Hibbit, K. (1999). Sleep and behaviour disturbance in Prader–Willi syndrome: A questionnaire study. Journal of Intellectual Disability Research, 43(5), 380–392.

    Article  PubMed  Google Scholar 

  • Rodgers, J., Riby, D. M., Janes, E., Connolly, B., & McConachie, H. (2012). Anxiety and repetitive behaviours in autism spectrum disorders and Williams Syndrome : A cross-syndrome comparison. Journal of Autism and Developmental Disorders, 42 (Numb 2), 175–180.

    Article  PubMed  Google Scholar 

  • Royston, R., Howlin, P., Waite, J., & Oliver, C. (2016). Anxiety disorders in Williams Syndrome contrasted with intellectual disability and the general population: A systematic review and meta-analysis. Journal of Autism and Developmental Disorders. doi:10.1007/s10803-016-2909-z.

    PubMed Central  Google Scholar 

  • Sparrow, S. S., Balla, D. A., & Cicchetti, D. V. (2005). Vineland II: Vineland adaptive behavior scales. Circle Pines: American Guidance Service.

    Google Scholar 

  • Strømme, P., Bjømstad, P. G., & Ramstad, K. (2002). Prevalence estimation of Williams syndrome. Journal of Child Neurology, 17(4), 269–271.

    Article  PubMed  Google Scholar 

  • Turner, M. (1997). Towards an executive dysfunction account of repetitive behaviour in autism, In J. Russell (Ed.), Autism and developmental disorders. New York: Oxford University Press

    Google Scholar 

  • Waite, J., Moss, J., Beck, S. R., Richards, C., Nelson, L., Arron, K., et al. (2015). Repetitive behavior in Rubinstein–Taybi syndrome: Parallels with autism spectrum phenomenology. Journal of Autism and Developmental Disorders, 45(5), 1238–1253.

    Article  PubMed  Google Scholar 

  • Wechsler, D. (1999). Manual for the Wechsler abbreviated intelligence scale (WASI). San Antonio, TX: The Psychological Corporation.

    Google Scholar 

  • Wheaton, M. G., Abramowitz, J. S., Jacoby, R. J., Zwerling, J., & Rodriguez, C. I. (2016). An investigation of the role of intolerance of uncertainty in hoarding symptoms. Journal of Affective Disorders, 193, 208–214.

    Article  PubMed  Google Scholar 

  • Whittington, J., Holland, A., Webb, T., Butler, J., Clarke, D., & Boer, H. (2004). Cognitive abilities and genotype in a population-based sample of people with Prader–Willi syndrome. Journal of Intellectual Disability Research, 48(2), 172–187.

    Article  PubMed  Google Scholar 

  • Woodcock, K. A., Oliver, C., & Humphreys, G. W. (2009). Task-switching deficits and repetitive behaviour in genetic neurodevelopmental disorders: Data from children with Prader–Willi syndrome chromosome 15 q11–q13 deletion and boys with Fragile X syndrome. Cognitive Neuropsychology, 26(2), 172–194.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

This study is based on several earlier studies. It is funded by the charity Cerebra, Cornelia de Lange Syndrome Foundation, Williams Syndrome Foundation and Fondation LeJeune.

Funding

This study was funded by Cerebra, the Cornelia de Lange Foundation, Fondation LeJeune and the Williams Syndrome Foundation.

Funding

This study was funded by Cerebra, the Cornelia de Lange Foundation, Fondation LeJeune and the Williams Syndrome Foundation.

Author information

Authors and Affiliations

  1. School of Psychology, University of Birmingham, Edgbaston, Birmingham, B15 2TT, UK

    R. Royston, C. Oliver, J. Moss, D. Adams, K. Berg, C. Burbidge, L. Nelson, C. Stinton & J. Waite

  2. Griffith University, PO Box 3370, South Brisbane Qld 4101, Sidon Street, South Bank, QLD, Australia

    D. Adams

  3. School of Psychology, University of East London, AE 1.10, Stratford Campus, London, E15 4LZ, UK

    K. Berg

  4. Coventry and Warwickshire Partnership NHS Trust, Wayside House, Wilsons Lane, Coventry, CV6 6NY, UK

    C. Burbidge

  5. Department of Psychology, King’s College London, Strand, London, WC2R 2L, UK

    P. Howlin

  6. University of Sydney, Sydney, Australia

    P. Howlin

  7. University of Warwick, Coventry, CV4 7AL, UK

    C. Stinton

Authors
  1. R. Royston
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C. Oliver
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Moss
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. D. Adams
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. K. Berg
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. C. Burbidge
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. P. Howlin
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. L. Nelson
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. C. Stinton
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. J. Waite
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

RR—Data analysis, data interpretation, writing, CO—Data interpretation, writing, JM—Data collection, writing, DA—Data collection, KB—Data collection, CB - Data collection, PH - Data interpretation, writing, LN - Data collection, CS—Data collection, JW—Data analysis, data interpretation, writing.

Corresponding author

Correspondence to R. Royston.

Ethics declarations

Conflict of interest

The authors declare they have no conflict of interest.

Informed consent

Informed consent was obtained from individuals who had the capacity to provide consent themselves. For those who did not have capacity to provide their own consent, parents or carers were able to act as consultees. Consultees were asked to advise on what the wishes of the individual would be if they were able to consent from themselves.

Research Involving Human Participants and/or Animals

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 75 KB)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Royston, R., Oliver, C., Moss, J. et al. Brief Report: Repetitive Behaviour Profiles in Williams syndrome: Cross Syndrome Comparisons with Prader–Willi and Down syndromes. J Autism Dev Disord 48, 326–331 (2018). https://doi.org/10.1007/s10803-017-3319-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10803-017-3319-6

Keywords

Search

Navigation