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Williams syndrome: On the role of intellectual abilities in anxiety

Willfors, C.; Riby, D.M.; Van Der Poll, M.; Ekholm, K.; Avdic Björlin, H.; Kleberg, J.L.; Nordgren, A.

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C. Willfors

M. Van Der Poll

K. Ekholm

H. Avdic Björlin

J.L. Kleberg

A. Nordgren


Background: Individuals with Williams syndrome (WS) have an elevated risk for anxiety disorders throughout the life span, making it a research priority to identify the individual factors associated with anxiety. Most of the existing literature is based on questionnaire data and suggests that impaired executive functions (EF) increase the risk for anxiety in WS. The aim of this study was to use direct measures by trained clinicians to investigate the effects of general intelligence, inhibition, sustained attention, and working memory on anxiety in WS, to further elucidate potential underlying mechanisms. Method: Twenty-four individuals with WS participated in the study (mean age: 29 years, range: 9–53 years), together with at least one of their parents. The MINI international neuropsychiatric interview for DSM-5 was completed to establish clinical diagnosis of anxiety, and the Clinical Global Impression Scale – Severity was used for an expert rating of symptom severity. Intellectual abilities were measured using the Wechsler scales, and attention and inhibition using the Conner’s Continuous Performance Test. In addition, a parent-report questionnaire measuring EF, learning and memory was collected. Results: In contrast to the apriori hypothesis, there was no significant association between anxiety and core elements of EF such as working memory, sustained attention, and inhibition (i.e. the process of restraining one’s impulses or behaviour). Using ordinal logistic regression analyses, we showed that decreasing intelligence quotient (IQ) and age are associated with elevated anxiety. We confirmed these results in between-groups analyses (anxiety disorder vs no current anxiety disorder), and low IQ was associated with higher risk of having an anxiety diagnosis. In addition, Bayesian statistics gave substantial evidence for no significant association between anxiety and inhibition. Conclusion: By using direct measures of psychological pathology and functioning, the current results provide a deeper characterisation of the WS phenotype and provide novel insights into the potential mechanisms underpinning anxiety.


Willfors, C., Riby, D., Van Der Poll, M., Ekholm, K., Avdic Björlin, H., Kleberg, J., & Nordgren, A. (2021). Williams syndrome: On the role of intellectual abilities in anxiety. Orphanet Journal of Rare Diseases, 16, Article 472.

Journal Article Type Article
Acceptance Date Oct 24, 2021
Online Publication Date Nov 7, 2021
Publication Date 2021
Deposit Date Oct 20, 2021
Publicly Available Date Jan 19, 2022
Journal Orphanet Journal of Rare Diseases
Publisher BioMed Central
Peer Reviewed Peer Reviewed
Volume 16
Article Number 472


Published Journal Article (944 Kb)

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