Abstract
The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore the profiles of social behaviour and anxiety across a broad age range of individuals with the disorder (n = 59, ages 6–36 years). We used insights from parental reports on two frequently used measures, the Spence Children’s Anxiety Scale (SCAS-P) and the Social Responsiveness Scale (SRS). Severity of anxiety was correlated with a greater degree of social dysfunction as measured by the SRS in this group. We split the group according to high or low anxiety as measured by the SCAS-P and explored the profile of social skills for the two groups. Individuals high and low in anxiety differed in their social abilities. The results emphasise the need to address anxiety issues in this disorder and to consider how components of anxiety might relate to other features of the disorder.
Similar content being viewed by others
Notes
All were biological parents of the individuals with WS and all WS individuals lived with their parents. In all cases the same respondent answered both questionnaires.
The 8 individuals who had not previously had their diagnosis confirmed with fluorescent in situ hybridisation testing to detect one deletion of the elastin gene were all older adults (>20 years) who were diagnosed prior to routine genetic testing for the disorder. The pattern of results remains consistent if these individuals are removed from the sample and therefore to allow us to analyse all available data these individuals have been left in the analysis.
The pattern of results remains consistent if we only analyse the data for those participants for whom we have verbal and nonverbal ability measures.
This correlation remains significant if T scores are used instead of SRS raw scores r = −.49, p < .001.
We also calculated the gender difference using the T scores for the SRS as these have gender-specific norms and transformations. There was no difference between genders when using the T scores for the SRS Total t(57) = 1.05, p = .30, d = .27.
We note caution due to the developmental difference between our sample and those reported elsewhere against which we are testing.
Abbreviations
- SRS:
-
Social Responsiveness Scale
- SCAS-P:
-
Spence Children’s Anxiety Scale Parent Measure
References
Bellini, S. (2006). The development of social anxiety in adolescents with autism spectrum disorders. Focus on Autism, and Other Developmental Disabilities, 21, 138–145.
Blomberg, S., Rosander, M., & Andersson, G. (2006). Fears, hyperacusis and musicality in williams syndrome. Research in Developmental Disabilities, 27, 668–680.
Constantino, J. N., & Gruber, C. P. (2005). The social responsiveness scale. Los Angeles, CA: Western Psychological Services.
Constantino, J. N., & Todd, R. D. (2005). Intergenerational transmission of subthreshold autistic traits in the general population. Biological Psychiatry, 57, 655–660.
Davies, M., Udwin, O., & Howlin, P. (1998). Adults with williams syndrome: Preliminary study of social, emotional, and behavioural difficulties. British Journal of Psychiatry, 172, 273–276.
Dimitropoulos, A., Ho, A. Y., Klaiman, C., Koenig, K., & Schultz, R. T. (2009). A comparison of behavioural and emotional characteristics in children with autism, prader-willi syndrome and williams syndrome. Journal of Mental Health Research in Intellectual Disabilities, 2, 220–243.
Dodd, H. F., & Porter, M. A. (2009). Psychopathology in williams syndrome: The effect of individual differences across the life span. Journal of Mental Health Research in Intellectual Disabilities, 2, 89–109.
Dodd, H. F., Schniering, C. A., & Porter, M. A. (2009). Beyond behaviour: Is social anxiety low in williams syndrome. Journal of Autism and Developmental Disorders, 1–9.
Dunn, L. M., Dunn, L. M., Whetton, C., & Burley, J. (1997). British picture vocabulary scale II. Windsor, UK: NFER-Nelson Publishing.
Dykens, E. M. (2003). Anxiety, fears, and phobias in persons with williams syndrome. Developmental Neuropsychology, 23, 291–316.
Dykens, E. M., Rosner, B. A., Ly, T., & Sagun, J. (2005). Music and anxiety in williams syndrome: A harmonious or discordant relationship? American Journal on Mental Retardation, 110, 346–358.
Gillott, A., & Standen, P. J. (2007). Levels of anxiety and sources of stress in adults with autism. Journal of Intellectual Disabilities, 11, 359–370.
Graham, J. M, Jr, Rosner, B., Dykens, E., & Visootsak, J. (2005). Behavioural features of CHARGE syndrome (hall-hittner syndrome) comparison with down syndrome, prader-willi syndrome, and williams syndrome. American Journal of Medical Genetics, 133, 240–247.
Hurtig, T., Kuusikko, S., Mattila, M. L., Haapsamo, H., Ebeling, H., Jussila, K., et al. (2009). Multi-informant reports of psychiatric symptoms among high-functioning adolescents with Asperger syndrome or autism. Autism, 13, 583–598.
Janes, E., Riby, D. M., & Rodgers, J. (2013). Exploring the prevalence and phenomenology of repetitive behaviours and sensory processing in children with WS. Journal of Intellectual Disability Research. doi: 10.1111/jir.12086.
Jawaid, A., Riby, D. M., Owens, J., White, S. W., Tarar, T., & Schulz, P. E. (2012). ‘Too withdrawn’ or ‘too friendly’: Considering social vulnerability in two neuro-developmental disorders. Journal of Intellectual Disability Research, 56, 335–350.
Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., et al. (2000). Hypersociability in Williams syndrome. Journal of Cognitive Neuroscience, 12, 30–46.
Klein-Tasman, B. P., Li-Barber, K. T., & Magargee, E. T. (2011). Honing in on the social phenotype in Williams syndrome using multiple measures and multiple raters. Journal of Autism and Developmental Disorders, 41, 341–351.
Klein-Tasman, B. P., Mervis, C. B., Lord, C. E., & Phillips, K. D. (2007). Socio-communicative deficits in young children with Williams syndrome: Performance on the autism diagnostic observation schedule. Child Neuropsychology, 13, 444–467.
Klein-Tasman, B. P., Phillips, K. D., Lord, C., Mervis, C. B., & Gallo, F. G. (2009). Overlap with the autism spectrum in young children with Williams syndrome. Journal of Developmental and Behavioral Pediatrics, 30, 289–299.
Klin, A., Volkmar, F. R., & Sparrow, S. S. (2000). Asperger syndrome. New York: The Guilford Press.
Korenberg, J. R., Bellugi, U., Salandanan, L. S., Mills, D. L., & Reiss, A. L. (2003). Williams syndrome: A neurogenetic model of human behaviour. Nature Encyclopaedia of the Human Genome, 757–766.
Leyfer, O. T., Woodruff-Borden, J., Klein-Tasman, B. P., Fricke, J. S., & Mervis, C. B. (2006). Prevalence of psychiatric disorders in 4 to 16-year-olds with williams syndrome. American Journal of Medical Genetics Part B: (Neuropsychiatric Genetics), 141(B), 615–622.
Little, K., Riby, D. M., Janes, E., Fleck, R., Clark, F., & Rodgers, J. (2013). Heterogeneity of social approach behaviours in Williams syndrome. Research in Developmental Disabilities, 34, 959–967.
Martens, M. A., Wilson, S. J., & Reutens, D. C. (2008). Research review: Williams syndrome: A critical review of the cognitive, behavioural, and neuroanatomical phenotype. Journal of Child Psychology and Psychiatry, 49, 576–608.
Mervis, C. B., Morris, C. A., Klein-Tasman, B. P., Bertrand, J., Kwitny, S., Appelbaum, L. G., et al. (2003). Attentional characteristics of infants and toddlers with Williams syndrome during triadic interactions. Developmental Neuropsychology, 23, 243–268.
Mervis, C. B., Robinson, B. F., Bertrand, J., Morris, C. A., Klein-Tasman, B. P., & Armstrong, S. C. (2000). The williams syndrome cognitive profile. Brain and Cognition, 44, 604–628.
Nauta, M. H., Scholing, A., Rapee, R. M., Abbott, M., Spence, S. H., & Waters, A. (2004). A parent-report measure of children’s anxiety: Psychometric properties and comparison with child-report in a clinic and normal sample. Behaviour Research and Therapy, 42, 813–839.
Porter, M. A., & Coltheart, M. (2005). Cognitive heterogeneity in Williams syndrome. Developmental Neuropsychology, 27, 275–306.
Porter, M. A., Dodd, H., & Cairns, D. (2009). Psychopathological and behaviour impairments in williams-beuren syndrome: The influence of gender, chronological age, and cognition. Child Neuropsychology, 15, 359–374.
Raven, J. C., Court, J. H., & Raven, J. (1990). Raven’s coloured progressive matrices. Oxford: Oxford Psychologists Press.
Riby, D. M., Hancock, P. J. B., Jones, N., & Hanley, M. (2013). Spontaneous and cued gaze-following in autism and Williams syndrome. Journal of Neurodevelopmental Disorders. (in press).
Riby, D. M., Kirk, H., Hanley, M., & Riby, L. M. (2013). Stranger danger awareness in Williams syndrome. Journal of Intellectual Disability Research. (in press).
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, 175–180.
Searcy, Y. M., Lincoln, A. J., Rose, F. E., Klima, E. S., Bavar, N., & Korenberg, J. R. (2004). The relationship between age and IQ in adults with Williams syndrome. American Journal on Mental Retardation, 109, 231–236.
Sofronoff, K., Attwood, T., & Hinton, S. (2005). A randomised controlled trial of a CBT intervention for anxiety in children with Asperger syndrome. Journal of Child Psychology and Psychiatry, 46(11), 1152–1160.
Spence, S. H. (1998). A measure of anxiety symptoms among children. Behaviour Research and Therapy, 36, 545–566.
Spence, S. H., Barrett, P. M., & Turner, C. M. (2003). Psychometric properties of the Spence Children’s Anxiety Scale with young adolescents. Anxiety Disorders, 17, 605–625.
Stinton, C., Elison, S., & Howlin, P. (2010). Mental health problems in adults with Williams syndrome. American Journal on Intellectual and Developmental Disabilities, 115, 3–18.
Stinton, C., Tomlinson, K., & Estes, Z. (2012). Examining reports of mental health in adults with Williams syndrome. Research in Developmental Disabilities, 33, 144–152.
Strømme, P., Bjørnstad, P. G., & Ramstad, K. (2002). Prevalence estimation of Williams syndrome. Journal of Child Neurology, 17, 269–271.
Sukhodolsky, D. G., Scahill, L., Gadow, K. D., Arnold, L. E., Aman, M. G., McDougle, C. J., et al. (2008). Parent-rated anxiety symptoms in children with pervasive developmental disorders: Frequency and association with core autism symptoms and cognitive functioning. Journal of Abnormal Child Psychology, 36, 117–128.
Tantam, D. (2003). The challenge of adolescents and adults with Asperger syndrome. Child and Adolescent Psychiatric Clinics of North America, 12, 143–163.
Udwin, O., Yule, W., & Martin, N. (1987). Cognitive abilities and behavioural characteristics of children with idiopathic infantile hypercalcaemia. Journal of Child Psychology and Psychiatry, 28, 297–309.
van der Fluit, F., Gaffrey, M. S., & Klein-Tasman, B. P. (2012). Social cognition in Williams syndrome: Relations between performance on the social attribution task and cognitive and behavioral characteristics. Frontiers in Developmental Psychology, 3, 197.
van Steensel, F. J. A., Bogels, S. M., & Wood, J. J. (2013). Autism spectrum traits in children with anxiety disorders. Journal of Autism and Developmental Disorders, 43, 361–370.
White, S. W., Oswald, D., Ollendick, T., & Scahill, L. (2009). Anxiety in children and adolescents with autism spectrum disorders. Clinical Psychology Review, 29, 216–229.
Wood, J. J., Drahota, A., Sze, K., Har, K., Chiu, A., & Langer, D. A. (2009). Cognitive behavioral therapy for anxiety in children with autism spectrum disorders: A randomized, controlled trial. Journal of Child Psychology and Psychiatry, 50, 224–234.
Acknowledgments
The authors would like to thank all the families and schools who participated in the research reported here.
Conflict of interest
The authors report no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Riby, D.M., Hanley, M., Kirk, H. et al. The Interplay Between Anxiety and Social Functioning in Williams Syndrome. J Autism Dev Disord 44, 1220–1229 (2014). https://doi.org/10.1007/s10803-013-1984-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10803-013-1984-7