Further evidence suggests anxiety is part of behavioural phenotype of Williams syndrome


Williams syndrome is genetic condition caused by gene deletion and is estimated to have a prevalence of 1 in 7,500 to 1 in 20,000 live births. Previous studies, which have relied mainly on the testimony of parents and carers, have suggested that people with Williams syndrome may have a disposition towards anxiety. The authors of this study were keen to look at the perspectives of the people with Williams syndrome themselves in exploring this issue. They worked with 19 adults with Williams syndrome using psychiatric interviews and and  a modified Stroop task to compare the differences between psychiatric symptoms reported by parents and carers and the people with Williams syndrome themselves – the respondents. (The Stroop effect is a demonstration of the reaction time of a task, particularly when there is a mixed stimulus, for example when the name of a colour is printed in a colour not denoted by the name e.g., the word “red” printed in blue ink instead of red ink. This will result in a longer reaction time to name the colour written on the card. The test from this effect, the Stroop Test is widely used in clinical settings.) They found that both informants and respondents reported more symptoms of anxiety than depression. They also found strong positive correlations between informant and respondent reports of symptoms of mental health problems. The respondents reported significantly more symptoms overall and more symptoms of anxiety. Results from the Stroop task suggested that adults with Williams syndrome were more vigilant to anxiety related words than to depression-related words. The authors conclude that their findings add to the evidence of anxiety as part of the behavioural phenotype of Williams syndrome. Examining reports of mental health in adults with Williams syndrome, Stinton C et al., in Research in Developmental Disabilities, 33, 1, 144-152

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John Northfield

After qualifying as a social worker, John worked in community learning disability teams before getting involved in a number of long-stay hospital closure programmes, working to develop individual plans for people moving into their own homes. He worked for BILD, helping to develop the Quality Network and was editorial lead for the NHS electronic library learning disabilities specialist collection. This led him to found the Learning Disabilities Elf site with Andre Tomlin as a way of making the evidence accessible to practitioners in health and social care. Most recently he has worked as part of Mencap's national quality team and also been involved in a number of national website developments, including the General Medical Council's learning disabilities site.

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