Williams syndrome: on the role of intellectual abilities in anxiety

Charlotte Willfors; Deborah M Riby; Marcus van der Poll; Katja Ekholm; Hanna Avdic Björlin; Johan Lundin Kleberg; Ann Nordgren

doi:10.1186/s13023-021-02098-4

Williams syndrome: on the role of intellectual abilities in anxiety

Orphanet J Rare Dis. 2021 Nov 7;16(1):472. doi: 10.1186/s13023-021-02098-4.

Authors

Charlotte Willfors^{1

2}, Deborah M Riby³, Marcus van der Poll⁴, Katja Ekholm^{4

5}, Hanna Avdic Björlin⁶, Johan Lundin Kleberg^#^{4

6}, Ann Nordgren^#^{4

5}

Affiliations

¹ Rare Diseases Research Group, Department of Molecular Medicine and Surgery, Karolinska Institutet, BioClinicum, J10:20, Visionsgatan 4, 171 64, Stockholm, Sweden. charlotte.willfors@ki.se.
² Department of Clinical Genetics, Karolinska University Laboratory, Karolinska University Hospital Solna L5:03, 171 64, Stockholm, Sweden. charlotte.willfors@ki.se.
³ Department of Psychology, Durham University, South Road, Durham, DH1 3LE, UK.
⁴ Rare Diseases Research Group, Department of Molecular Medicine and Surgery, Karolinska Institutet, BioClinicum, J10:20, Visionsgatan 4, 171 64, Stockholm, Sweden.
⁵ Department of Clinical Genetics, Karolinska University Laboratory, Karolinska University Hospital Solna L5:03, 171 64, Stockholm, Sweden.
⁶ Centre for Psychiatry Research, Department of Clinical Neuroscience, Karolinska Institutet, Stockholm Health Care Services, Gävlegatan 22, 113 30, Stockholm, Sweden.

^# Contributed equally.

Abstract

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Anxiety
Anxiety Disorders
Bayes Theorem
Humans
Intelligence
Williams Syndrome*