A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes.
A quick five-minute social anxiety checklist shows Fragile X and Rubinstein-Taybi kids are anxious with everyone, while Cornelia de Lange kids only freeze with strangers.
01Research in Context
What this study did
The team watched children with four rare syndromes meet an adult they knew and an adult they did not know. They used a simple checklist to score signs of social anxiety and social motivation. The kids had Fragile X, Cornelia de Lange, Rubinstein-Taybi, or Down syndrome.
What they found
Fragile X and Rubinstein-Taybi children showed high social anxiety with both familiar and unfamiliar adults. Cornelia de Lange children looked calm with people they knew but shut down with strangers. Down syndrome children showed the least anxiety in both setups.
How this fits with other research
Minhas et al. (2025) later linked the same Fragile X anxiety to lower quality-of-life scores, so the worry you see on the checklist has real-life weight.
Protic et al. (2022) recommend early combined behavioral and drug treatment for Fragile X; Hayley’s data give you the easy scale to start that track.
Jackson et al. (2025) found that Down syndrome youth with poor shifting and working memory later show more anxiety. Hayley saw less anxiety in Down syndrome overall, so the two papers together say: watch EF skills in Down syndrome to keep anxiety low.
Why it matters
You can use the same brief checklist during intake. Start social skills work with a trusted adult for Cornelia de Lange kids and plan extra anxiety supports for Fragile X and Rubinstein-Taybi clients no matter who is in the room. The tool is free and takes five minutes.
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02At a glance
03Original abstract
Unique socio-behavioural phenotypes are reported for individuals with different neurodevelopmental disorders. Here, the effects of adult familiarity and nature of interaction on social anxiety and social motivation were investigated in individuals with fragile X (FXS; n = 20), Cornelia de Lange (CdLS; n = 20) and Rubinstein-Taybi (RTS; n = 20) syndromes, compared to individuals with Down syndrome (DS; n = 20). The Social Anxiety and Motivation Rating Scale was employed whilst participants completed four social tasks, each administered separately by a familiar adult, and also by an unfamiliar adult. Compared to participants with DS, those with FXS and RTS exhibited high levels of social anxiety but similar levels of social motivation. Participants with CdLS showed heightened social anxiety and reduced social motivation only during interactions with an unfamiliar adult when active participation was voluntary.
Journal of autism and developmental disorders, 2020 · doi:10.1007/s10803-019-04232-5