Characterising the profile of everyday executive functioning and relation to IQ in adults with Williams syndrome : is the BRIEF adult version a valid rating scale?

Although there is evidence of a distinct profile of executive dysfunction in Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, the utility of informant reports of everyday executive function (EF) impairments and their relation to intelligence is not yet clear. Here we aimed to evaluate the functional impact of executive dysfunction in adults with WS and to establish the validity of child and adult versions of the most commonly used rating scale for EF assessment, the Behaviour Rating Inventory of Executive Function (BRIEF). We were also interested in whether distinct components of everyday EF relate to intelligence in WS. Parent report child (BRIEF-C) and adult (BRIEF-A) ratings were collected on 20 adults with WS (aged 18.5 to 53 years), with a mean IQ of 60.95 (SD = 17.67). Neuropsychological measures of EF included: The Shape School Test (Espy, 2007); select subdomains of EF from the Woodcock-Johnson III Tests of Cognitive Abilities, Australian Adaptation (WJ III COG); and select subdomains from the Vineland Adaptive Behaviour Scales, Second Edition—Parent Survey (Vineland-II). Results showed that the BRIEF-A, but not the BRIEF-C, was the most highly correlated with neuropsychological measures of EF, suggesting that it was a valid measure of the profile of EF impairments in adults with WS. The profile of everyday EF dysfunction revealed relative impairments in monitoring, working memory, planning and organisation in WS. In addition, both neuropsychological and rating scale measures showed an association between the shifting component of EF and intelligence. These findings indicate that the BRIEF-A is a valid measure of the multidimensional nature of real-world impairments in EF, and highlight its utility as a less labor intensive and low-cost screening tool for measuring specific EF impairments that could become the focus of targeted intervention in adults with WS.18 page(s

Brief Report: The Impact of Sensory Hypersensitivity and Intolerance of Uncertainty on Anxiety in Williams Syndrome

This study explored the interrelationship between intolerance of uncertainty, sensory hyper-sensitivity and anxiety in Williams syndrome (WS). Thirty-two parents or guardians of individuals with WS (Mage = 24.76 years, SD = 7.55) were included. Associations between anxiety, intolerance of uncertainty, sensory hyper-sensitivity, and ASD symptoms were assessed. Linear regression analysis revealed that intolerance of uncertainty and sensory hyper-sensitivity were unique independent predictors of anxiety, while social communication score was not. There was evidence of a mediating effect of sensory hyper-sensitivity on the relationship between intolerance of uncertainty and anxiety. These findings bear strong resemblance to the pattern seen in ASD and emphasize the need for development of anxiety interventions that attempt to reduce negative beliefs about unpredictable situations in WS

Correlations Between IQ and the BRIEF-A (T Scores).

<p><i>Note</i>. Scores represent Pearson’s correlation coefficient.</p><p>* <i>p</i> < 0.05.</p><p>Correlations Between IQ and the BRIEF-A (T Scores).</p

Correlations Between the WJ III COG and the BRIEF-A (T Scores).

<p><i>Note</i>. Scores represent Pearson’s correlation coefficient.</p><p>** <i>p</i> < 0.01.</p><p>* <i>p</i> < 0.05.</p><p>Correlations Between the WJ III COG and the BRIEF-A (T Scores).</p