Challenges with shifting, regardless of disengagement: attention mechanisms and eye movements in Williams syndrome

TL;DR

People with Williams syndrome struggle with shifting attention, not just disengaging from stimuli, and this is linked to higher arousal levels.

Contribution

This study challenges previous theories by showing that attention challenges in Williams syndrome are more general and linked to arousal regulation.

Findings

Individuals with Williams syndrome are less likely to shift attention to upcoming targets than typically developed individuals.

Failure to shift attention in Williams syndrome is strongly predicted by higher arousal levels induced by auditory cues.

No specific difficulty with disengagement was found, contradicting prior theories about attention in Williams syndrome.

Abstract

People with Williams syndrome (WS) face challenges in various areas of cognitive processing, including attention. Previous studies suggest that these challenges are particularly pronounced when disengagement of attention from a previously attended stimulus is required, as compared to shifting attention without the need to disengage. Difficulties with attention could in turn be implicated in several of the behavioral characteristics of WS. Here, disengagement and shifting of visual attention, together with pupil dilation, were independently assessed in one of the largest eye-tracking studies of WS to date.

We investigated shifting, disengagement, and the effects of auditory alerting cues on pupil dilation in WS individuals (n = 45, age range = 9–58 years), non-WS individuals with intellectual disability (ID) (n = 36, age range = 6–59 years), and typically developed (TD) infants (n = 32, age range = 6–7 months), children and adults (n = 31, age range = 9–60 years), using a modified gap-overlap task. Data were analyzed using linear mixed-effect models (LMMs).

Individuals with WS were less likely to shift their attention to upcoming targets than TD individuals (all ages), but more likely than the ID group to do so. When they did shift attention, participants with WS and ID were slower to initiate a gaze shift than TD participants regardless of whether disengagement was needed. In the WS group, failure to shift attention was strongly predicted by higher arousal (pupil dilation), which was induced by auditory alerting cues.

Contrasting with previous theories of attention in WS, we found no evidence for a specific challenge in disengaging attention. Instead, our results point to a more general challenge in shifting attention. Reduced attention shifting in WS may be partly explained by atypical arousal regulation. These results contribute to our understanding of the WS phenotype.

The online version contains supplementary material available at 10.1186/s11689-025-09639-z.

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