Executive Function and Working Memory Deficits in Females with Fragile X Premutation

Executive functions (EF) are thought to be impaired in Down syndrome (DS) and sex chromosome trisomy (Klinefelter and Trisomy X syndromes; +1X). However, the syndromic specificity and developmental trajectories associated with EF difficulties in these groups are poorly understood. The current investigation (a) compared everyday EF difficulties in youth with DS, +1X, and typical development (TD); and (b) examined relations between age and EF difficulties in these two groups and a TD control group cross-sectionally. Study 1 investigated the syndromic specificity of EF profiles on the Behavior Rating Inventory of Executive Function (BRIEF) in DS (n = 30), +1X (n = 30), and a TD group (n = 30), ages 5–18 years. Study 2 examined age effects on EF in the same cross-sectional sample of participants included in Study 1. Study 3 sought to replicate Study 2's findings for DS by examining age-EF relations in a large independent sample of youth with DS (n = 85) and TD (n = 43), ages 4–24 years. Study 1 found evidence for both unique and shared EF impairments for the DS and +1X groups. Most notably, youth with +1X had relatively uniform EF impairments on the BRIEF scales, while the DS group showed an uneven BRIEF profile with relative strengths and weaknesses. Studies 2 and 3 provided support for fairly similar age-EF relations in the DS and TD groups. In contrast, for the +1X group, findings were mixed; 6 BRIEF scales showed similar age-EF relations to the TD group and 2 showed greater EF difficulties at older ages for +1X. These findings will be discussed within the context of efforts to identify syndrome specific cognitive-behavioral profiles for youth with different genetic syndromes in order to inform basic science investigations into the etiology of EF difficulties in these groups and to develop treatment approaches that are tailored to the needs of these groups.

The Behavior Rating Inventory of Executive Function (BRIEF) was one of the first attempts to measure executive function via self- and informant reports of everyday functioning in the real-world environment and was the first published measure of these self-regulatory capabilities in children and adolescents (Gioia, Isquith, Guy & Kenworthy, 2000a). The impetus for the BRIEF arose among the authors in 1994 while trying to reconcile the often discrepant parent and teacher reports of children’s everyday functioning at home and in school with their performance on putative performance measures (i.e., “tests”) of executive function. At that time, there were few such performance measures of executive function developed for children and adolescents, no rating scales or structured observational methods for evaluating executive functions, and very few published articles on executive function in children (Bernstein & Waber, 2007).

A-042 Incongruence Between Self- and Parent- Report Measures of Executive Function on the Behavior Rating Inventory of Executive Function (BRIEF) in Adolescents with Attention Deficit Hyperactivity Disorder

We examined whether parents' ratings of their children's flexibility and ability to monitor their behavior predicted adaptive skills (e.g., ability to complete day-to-day personal tasks, communicate, and socialize) and symptoms of anxiety and depression among children with and without autism spectrum disorder. For both groups, children's abilities to manage and monitor their behavior were strongly related to adaptive skills. Children's flexibility and ability to inhibit inappropriate behavior and control their emotions was associated with depression symptoms for both groups.

Executive function is very important in the children's overall development. The goal of this study was to assess the executive function in children with intellectual disability (ID) through the use of the Behavior Rating Inventory of Executive Function (BRIEF) teacher version. An additional goal was to examine the differences in executive function in relation to child's sex, level and aetiology of ID. The sample consisted of 90 children with ID attending two special education schools in Sarajevo, Bosnia and Herzegovina. There were 42 children with mild ID and 48 children with moderate ID. Of those, 54 were boys and 36 were girls. Children were classified into three etiological categories: 30 children with Down syndrome, 30 children with other genetic cause or organic brain injury and 30 children with unknown aetiology of ID. Special education teachers, who knew the children for at least 6 months filled the BRIEF. Children with ID had a significant deficit in executive function as measured by the BRIEF. There were no statistically significant differences in executive function in relation to the child's sex. Level of ID had a significant effect on executive function. In relation to the aetiology of ID, the only significant difference was on the Shift scale of the BRIEF. Knowing what executive function is most impaired in children with ID will help professionals design better intervention strategies. More attention needs to be given to the assessment of executive function and its subsequent intervention in the school settings.

We aimed to identify a brief screening measure for detection of cognitive deficit in children treated for cerebellar tumors that would be useful in clinical practice. A sample of 72 children, aged 8-14 years, and within 3 years post diagnosis for standard-risk medulloblastoma (n = 37) or low-grade cerebellar astrocytoma (n = 35) and 38 children in a nontumor group were assessed using teacher-, parent-, and child-report of the Behavior Rating Inventory of Executive Function (BRIEF), Strengths and Difficulties Questionnaire (SDQ), and Pediatric Quality of Life Inventory (PedsQL). The accuracy of these scores as a screen for a full-scale Intelligence Quotient (FSIQ) < 80 on the Wechsler Intelligence Scale for Children (WISC-IV UK) was assessed using their receiver operating characteristic (ROC) curves. The questionnaires with the highest areas under the ROC curves were the child- and parent-report PedsQL, the teacher-report BRIEF, and the SDQ. At optimal cutoff scores, their sensitivities (95% CIs) to cases of FSIQ < 80 were 84 (60-96)%, 65 (41-84)%, 79 (54-93)%, and 84 (60-96)%, and their specificities (95% CIs) were 79 (68-86)%, 87 (77-93)%, 77 (66-86)%, and 71 (64-84)% respectively. All cases of FSIQ < 80 screened positive on either teacher-report SDQ or self-report PedsQL. The PedsQL child- and parent-report and the teacher-report BRIEF and SDQ have moderately good accuracy for discriminating between children with and without a FSIQ < 80. The PedsQL could be used in a clinical setting, and the BRIEF and SDQ in an educational setting, to screen for cases with FSIQ < 80 in children treated for brain tumors.

The early assessment of the executive processes using ecologically valid instruments is essential for identifying deficits and planning actions to deal with possible adverse consequences. The present study has two different objectives. The first objective is to analyze the relationship between preschoolers’ performance on tests of Working Memory and Inhibition and parents’ and teachers’ ratings of these executive functions (EFs) using the Behavior Rating Inventory of Executive Function (BRIEF). The second objective consists of studying the predictive value of the different EF measures (performance-based test and rating scales) on Inattention and Hyperactivity/Impulsivity behaviors and on indicators of word reading performance. The participants in the study were 209 children in the last year of preschool, their teachers and their families. Performance-based tests of Working Memory and Inhibition were administered, as well as word reading measures (accuracy and speed). The parents and teachers filled out rating scales of the EF and typical behaviors of attention deficit hyperactivity disorder (ADHD) symptomatology. Moderate correlation values were found between the different EF assessments procedures, although the results varied depending on the different domains. Metacognition Index from the BRIEF presented stronger correlations with verbal working memory tests than with inhibition tests. Both the rating scales and the performance-based tests were significant predictors of Inattention and Hyperactivity/Impulsivity behaviors and the reading achievement measures. However, the BRIEF explained a greater percentage of variance in the case of the ADHD symptomatology, while the performance-based tests explained reading achievement to a greater degree. The implications of the findings for research and clinical practice are discussed.

Biomarkers of Disease Severity Predict Neurocognitive Functioning in Pediatric SCD

The present study compared parent-rated executive functioning in pediatric medulloblastoma (MB) and pilocytic astrocytoma (PA) survivors. Although standard care for both includes surgical resection, children with MB additionally receive chemotherapy and craniospinal irradiation. Given well-documented neurocognitive late effects associated with the latter, we anticipated poor parent-reported executive functioning in MB survivors. Parents/guardians of 36 MB survivors and 20 PA survivors completed the Behavior Rating Inventory of Executive Functioning (BRIEF). PA survivors were younger at diagnosis (t[51.97] = 3.07, p < .001, d = 0.86) and demonstrated higher IQ (t[54] = −3.51, p < .001, d = 0.95). However, relative to the MB group, the PA group was rated as having significantly more problems on all BRIEF scales (all p ≤ .05; d = 0.30 − 1.10), except the Shift scale. Additionally, all mean BRIEF scores for MB survivors were within normal limits, whereas for PA survivors, all mean BRIEF scores except for Organization of Materials were significantly discrepant from normative means. Overall, PA survivors were rated as demonstrating poorer executive function than MB survivors. Five theories are discussed as possible explanations for these surprising findings: two related to group differences, two related to potential sources of parental bias, and one related to the nature of questionnaire-based assessment. All these theories represent directions for future research. Parent questionnaires such as the BRIEF may have real-world implications for pediatric brain tumor survivors. Future research should explore factors affecting parent ratings of executive functioning in these populations, along with comparison to performance-based measures.

Objective:Sluggish cognitive tempo (SCT) is an attentional disorder characterized by excessive daydreaming, reduced alertness, slowed motor behavior, and mental fogginess. The purpose of the present study was to examine potential executive functioning group differences between children with high SCT symptoms versus those with low SCT symptoms. It was hypothesized that children with high SCT symptoms would have greater executive functioning deficits than children with low SCT symptoms, as reported by their teachers.Participants and Methods:There were 32 children in this study, between the ages of 6 to 13 (M = 8.94; SD = 1.97). To measure the level of SCT symptomology, an average rating on four items from the Child Behavior Checklist (CBCL; Items 13, 17, 80, 102) and an average rating from five items from the Teacher's Report Form (TRF; Items 13, 17, 60, 80, 102) were acquired and averaged to produce a combined measure of SCT. The present study had fair to good reliability for CBCL and TRF with Cronbach alpha values of .71 and .82 respectively. Eighteen participants had SCT scores above the Garner et al. (2010) cutoff criteria for the CBCL (SCT over 0.67) or the TRF (SCT over 0.75) which placed them in the high SCT group. The 13 participants who did not meet criteria for high SCT were considered the low SCT group. To measure executive function, Behavior Rating Inventory of Executive Function (BRIEF) teacher ratings were used. A general linear model multivariate analysis was conducted on each measure of the BRIEF teacher reports with ADHD-Inattentive (ADHD-IN) and Verbal Comprehension Index (VCI) scores as covariates.Results:There were significant group differences between the BRIEF Teacher Global Executive Composite scores of the high SCT group (M = 60.81, SD = 7.78) versus the low SCT group (M = 50.31, SD = 6.87), F(1, 30) = 11.73, p &lt; .001, np2 = .59. The high SCT group scored significantly higher than the low SCT group on the Initiate (p &lt; .001), Working Memory (p &lt; .001), Plan/Organize (p &lt; .001), Monitor (p &lt; .01), and Organization of Materials (p &lt; .05) subscales. These findings indicate that the children in the high SCT group had greater executive functioning difficulties overall than the low SCT group.Conclusions:Children with high SCT symptoms demonstrated greater executive functioning deficits than children with low SCT symptoms regarding metacognition but not behavioral regulation. This means that children with SCT likely struggle more with initiating tasks, planning, organization, memory, and monitoring their thinking and behaviors than children without SCT. These skills are important for learning, which may at least partially help explain why children with SCT experience problems in school.

Difficulties in executive functioning (EF) have been consistently reported in autistic individuals, but less is known about the attentional and neural mechanisms driving these difficulties. We explored the associations between EF abilities and sustained attention, measured with eye-tracking, and spontaneous measures of EEG spectral power density in 176 2–8 year-old autistic children with a wide range of cognitive abilities. EF was measured using the Behavior Rating Inventory of Executive Function (BRIEF). We found that EF abilities were positively associated with look durations while watching complex, audiovisual stimuli involving social content and dyadic speech. We also found that EF was negatively associated with scalp-wide theta power and positively associated with frontal beta and gamma power. These results shed light on attentional and neural associations with EF abilities and underscore the role of frontal brain activity for EF in autism.

The authors aim was to examine associations of breastfeeding duration and exclusivity in infancy with executive function, behavior, and social-emotional development in mid-childhood. The authors studied 1037 participants in Project Viva, a prebirth cohort that enrolled pregnant mothers from 1999 to 2002 and followed children for 7 to 10 years. Main exposures were: (1) duration of any breastfeeding in the first 12 months and (2) duration of exclusive breastfeeding in the first 6 months. Main outcomes were child executive function, behavior, and social-emotional development, assessed by (1) the Behavior Rating Inventory of Executive Function (BRIEF) and (2) the Strengths and Difficulties Questionnaire (SDQ), completed independently by parents and teachers. Higher scores indicate greater problems. In linear regression models adjusted for sociodemographics, maternal intelligence, home environment, early child care, and maternal depression, longer breastfeeding duration was not associated with substantially better executive function, behavior, or social-emotional development. For example, for each additional month of any breastfeeding, the BRIEF Global Executive Composite score (parent) was 0.10 points higher (95% confidence interval, -0.01 to 0.22) and the SDQ total difficulties score was 0.06 points higher (-0.01, 0.12). Breastfeeding duration was also not associated with BRIEF or SDQ subscales, nor was exclusive breastfeeding duration associated with any of the outcomes analyzed. Despite beneficial effects on general intelligence, longer duration of any breastfeeding or of exclusive breastfeeding was not associated with better executive function, behavior, or social-emotional development in mid-childhood.

ObjectiveWe performed a prospective, cross‐sectional cognitive assessment in subjects with Duchenne Muscular Dystrophy (DMD) and their biological mothers.MethodsThirty subjects with out‐of‐frame mutations in the dystrophin (DMD) gene, and 25 biological mothers were evaluated using the National Institutes of Health Toolbox Cognition Battery (NIHTB‐CB). A parent completed the Behavior Rating Inventory of Executive Functioning (BRIEF), a standardized rating scale of executive functioning, for their child. Mothers completed self‐reports of BRIEF and Neuro Quality‐of‐Life (NeuroQoL) Cognitive Function.ResultsOverall, the subjects with DMD scored approximately one standard deviation (SD) below age‐corrected norms on the NIHTB‐CB Total Cognition score. They scored 1.5 SD below age‐corrected norms in Fluid Cognition, which evaluates the cognitive domains of executive function, working memory, episodic memory, attention, and processing speed. Their performance was consistent with age expectations (i.e., within 1 SD below age‐corrected norms) in Crystalized Cognition, which evaluates vocabulary and reading. Subjects with DMD had higher T‐scores in several domains of BRIEF, demonstrating greater difficulty in executive functioning. The biological mothers had overall average or above average T‐scores on NIHTB‐CB. Mothers who were carriers of DMD mutation performed lower overall compared to mothers who were not carriers of DMD mutation (Cohen’s d = −1.1). Carrier mothers performed lower than average (1.5 SD) in Executive Function, measured by Flanker Inhibitory Control and Attention. Biological mothers scored within expected score ranges for adults in BRIEF and NeuroQoL.InterpretationThe NIHTB‐CB, combined with standardized self‐reported measures, can be a sensitive screening tool for cognitive surveillance in DMD.

&lt;p style="text-align: justify;"&gt;El objetivo de este estudio fue determinar la estructura de la Función Ejecutiva (FE) desde el dominio conductual derivado de las subescalas de padres y maestros del BRIEF &lt;em&gt;(Behavior Rating Inventory of Executive Function) &lt;/em&gt;en adolescentes infractores con Trastorno Disocial de la Conducta (TDC) y un grupo control (no-TDC) del valle del Aburrá . La muestra fue de 128 adolescentes: 56 infractores con TDC y 72 no infractores no-TDC. Se realizó un análisis factorial con las puntuaciones de las subescalas del BRIEF. Se observó una estructura factorial de las conductas de la FE con un único factor en ambos grupos. A este factor se le denominó Sistema de Supervisión Conductual (SSC). El BRIEF evalúa un Sistema de Supervisión Conductual (SSC) que diferencia significativamente ambos grupos.&lt;/p&gt;&lt;p style="text-align: justify;"&gt;&lt;strong&gt;Palabras clave: &lt;/strong&gt;trastorno de conducta, BRIEF, Función ejecutiva, infractores, adolescentes, TDC.&lt;/p&gt;

ABSTRACTAboriginal children in Canada are at high risk of fetal alcohol spectrum disorder (FASD) but there is little research on the cognitive impact of prenatal alcohol exposure (PAE) in this population. This paper reviews the literature on parent report of executive functioning in children with FASD that used the Behavior Rating Inventory of Executive Function (BRIEF). New data on the BRIEF is then reported in a sample of 52 Aboriginal Canadian children with FASD for whom a primary caregiver completed the BRIEF. The children also completed a battery of neuropsychological tests. The results reveal mean scores in the impaired range for all three BRIEF index scores and seven of the eight scales, with the greatest difficulties found on the Working Memory, Inhibit and Shift scales. The majority of the children were reported as impaired on the index scores and scales, with Working Memory being most commonly impaired scale. On the performance-based tests, Trails B and Letter Fluency are most often reported as impaired, though the prevalence of impairment is greater for parent ratings than test performance. No gender difference is noted for the parent report, but the boys had slightly slower intellectual functioning and were more perseverative than the girls on testing. The presence of psychiatric comorbidity is unrelated to either BRIEF or test scores. These findings are generally consistent with prior studies indicating that parents observe considerable executive dysfunction in children with FASD, and that children with FASD may have more difficulty with executive functions in everyday life than is detected by laboratory-based tests alone.