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?

Darren R Hocking,Jessica Reeve,Melanie A Porter,Bert De Smedt

doi:10.1371/journal.pone.0137628

Abstract

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.

Highlights

It is often the case that clinicians or researchers need to utilise rating scales and questionnaires as a practical way of quantifying cognitive or psychological functioning in both typical and atypical populations
The primary goal of this study was to examine the functional impact of everyday executive function (EF) impairments in adults with Williams syndrome (WS) using an in-depth analysis of both neuropsychological measures and parent reported EF rating scales: the Behaviour Rating Inventory of Executive Function (BRIEF)
We compared the validity of parent reports on the Behavior Rating Inventory of Executive Function—Adult version (BRIEF-A) Clinical Scales, Indices, and GEC Behavioural Regulation Index (BRI) Inhibit Shift Emotional Control Self-Monitor Metacognitive Index (MI) Initiate Working Memory Planning/Organising Task-Monitor Organisation of Materials GEC

Summary

Introduction

It is often the case that clinicians or researchers need to utilise rating scales and questionnaires as a practical way of quantifying cognitive or psychological functioning in both typical and atypical populations. In atypically developing populations with intellectual disability (ID), the extent to which reliability and validity is affected based on the developmental appropriateness of a particular rating scale, and its application to a specific neurodevelopmental disorder, is an important yet neglected issue in developmental neuropsychology. EF includes complex cognitive functions such as: cognitive flexibility; task initiation and response inhibition; planning and organisation; working memory and regulation of emotion and behaviour [5,6,7]. It is well established that the central executive is further fractionated into three separable but not completely independent component processes, namely: the ability to shift between mental sets or tasks (‘shifting’); selectively attending to stimuli and inhibiting prepotent responses (‘inhibition’); and updating and monitoring in working memory (‘updating’) [10]. There has been considerable controversy as to whether these components of EF can be viewed as separable or rather as a unitary construct in early development [11,12,13], and this controversy applies to neurodevelopmental disorders associated with ID

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