Executive function in phenylketonuria (PKU): Insights from the Behavior Rating Inventory of Executive Function (BRIEF) and a large sample of individuals with PKU.

OBJECTIVE: Previous research has documented executive function (EF) impairments in individuals with early treated phenylketonuria (ETPKU). It remains unclear, however, whether some aspects of EF may be more affected than others. A number of factors, including small sample sizes and variability in EF tasks, have likely contributed to past mixed findings. The present objective was to elucidate further the EF profile associated with ETPKU, particularly as it relates to report-based assessment of EF. METHOD: Data from 286 individuals (5-48 years of age) with ETPKU on the child and adult versions of the Behavior Rating Inventory of Executive Function (BRIEF), a well-established report-based assessment tool, were analyzed. RESULTS: The Working Memory scale showed the largest effect size in both young and older ETPKU samples, with 19% of children and 29% of adults scoring in the "abnormally elevated" range. In addition, EF impairment appeared more general (i.e., affecting more domains) in the adult sample as compared to the child sample. Exploratory analyses also suggested that the presence/absence of overall impairment on the BRIEF among our ETPKU participants could be predicted based on a small subset of items. A 10-item subset showed total classification accuracy values of 90% and above for both groups. CONCLUSIONS: Working memory represents an aspect of EF that appears to be particularly affected in individuals with ETPKU. Findings also provide preliminary support of the viability for the development and/or adoption of an abbreviated screening measure for EF difficulties in children and adults with ETPKU. (PsycInfo Database Record (c) 2020 APA, all rights reserved).

[Access to the early diagnosis of dementia in New Brunswick: perceptions of potential users of services depending on the language and the middle of life]. / Accès au diagnostic précoce de démence au Nouveau-Brunswick : perceptions d'usagers potentiels de services selon la langue et le milieu de vie.

OBJECTIVES: The early diagnosis of dementia (EDD) enables the identification of reversible causes of dementia and allows the timely implementation of secondary preventive and therapeutic interventions. This study explores New Brunswick seniors' perceptions of the accessibility and availability of EDD services as well as their satisfaction with them while taking into account their language of use and place of residence (urban or rural). METHOD: Self-administered survey exploring perceptions of EDD services in Francophone and Anglophone seniors from rural and urban areas of New Brunswick. Univariate and bivariate analyses were carried out. RESULTS: Of the 157 participants aged 65 years and over who filled out the survey and whose data were analyzed, 84 identified as Francophone, 72 of whom lived in rural areas. Bivariate analyses showed that linguistic groups were comparable with regard to their perceptions of the availability, access to, and satisfaction with EDD services. However, when taking the geographic dimension into account, linguistic intergroup and intragroup disparities were observed, notably in the areas pertaining to the type of services available in the area. CONCLUSIONS: These results suggest that seniors who live in rural areas of New Brunswick are a particularly vulnerable group with perceived limited access to EDD services in their area.