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Agreement Between Self- and Caregiver-Report of Thought Disturbances in Adults with Williams Syndrome and Intellectual Disability

Vassall, S. G.; Kittleson, A. R.; Halverson, A. S.; Schock, G. V.; Leslie, E.; Dykens, E. M.; Roof, E.; Sheffield, J. M.; Bress, K. S.

2025-12-18 psychiatry and clinical psychology
10.64898/2025.12.15.25341842 medRxiv
Show abstract

Williams Syndrome (WS) is a rare neurodevelopmental disorder associated with intellectual disability and increased vulnerability to traits such as anxiety, perseveration, and belief inflexibility. In the general population, these traits are linked to self-reported thought disturbances such as paranoia and delusions. However, little is known about how such disturbances present in WS, largely due to concerns regarding the validity of self-report in this population. To address this gap, we collected self- and caregiver-reported measures characterizing thought disturbances and related cognitive traits in adults with WS, assessing inter-rater reliability and correlations among measures. Total scores were similar across reporters, except for delusional ideation, which participants endorsed more strongly than caregivers. Several participants also reported clinically significant levels of paranoia, delusions, and worry that were not captured by caregiver report. These findings suggest that self-report is a valid method for accurately characterizing the severity and nature of thought disturbances in WS.

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