Fenfluramine treatment is associated with improvement in everyday executive function in preschool-aged children (<5 years) with Dravet syndrome: A critical period for early neurodevelopment.

OBJECTIVE: To evaluate whether fenfluramine (FFA) is associated with improvement in everyday executive function (EF)-self-regulation-in preschool-aged children with Dravet syndrome (DS). METHODS: Children with DS received placebo or FFA in one of two phase III studies (first study: placebo, FFA 0.2 mg/kg/day, or FFA 0.7 mg/kg/day added to stiripentol-free standard-of-care regimens; second study: placebo or FFA 0.4 mg/kg/day added to stiripentol-inclusive regimens). Everyday EF was evaluated at baseline and Week 14-15 for children aged 2-4 years with parent ratings on the Behavior Rating Inventory of Executive Function®-Preschool (BRIEF®-P); raw scores were transformed to T-scores and summarized in Inhibitory Self-Control Index (ISCI), Flexibility Index (FI), Emergent Metacognition Index (EMI), and Global Executive Composite (GEC). Clinically meaningful improvement and worsening were defined using RCI ≥ 90% and RCI ≥ 80% certainty, respectively. The associations between placebo vs FFA combined (0.2, 0.4, and 0.7 mg/kg/day) or individual treatment groups and the likelihood of clinically meaningful change in BRIEF®-P indexes/composite T-scores were evaluated using Somers'd; pairwise comparisons were calculated by 2-sided Fisher's Exact tests (p ≤ 0.05) and Cramér's V. RESULTS: Data were analyzed for 61 evaluable children of median age 3 years (placebo, n = 22; FFA 0.2 mg/kg/day, n = 15; 0.4 mg/kg/day [with stiripentol], n = 10; 0.7 mg/kg/day, n = 14 [total FFA, n = 39]). Elevated or problematic T-scores (T ≥ 65) were reported in 55% to 86% of patients at baseline for ISCI, EMI, and GEC, and in ∼33% for FI. Seventeen of the 61 children (28%) showed reliable, clinically meaningful improvement (RCI ≥ 90% certainty) in at least one BRIEF®-P index/composite, including a majority of the children in the FFA 0.7 mg/kg/day group (9/14, 64%). Only 53% of these children (9/17) also experienced clinically meaningful reduction (≥50%) in monthly convulsive seizure frequency, including 6/14 patients in the FFA 0.7 mg/kg/day group. Overall, there were positive associations between the four individual treatment groups and the likelihood of reliable, clinically meaningful improvement in all BRIEF®-P indexes/composite (ISCI, p = 0.001; FI, p = 0.005; EMI, p = 0.040; GEC, p = 0.002). The FFA 0.7 mg/kg/day group showed a greater likelihood of reliable, clinically meaningful improvement than placebo in ISCI (50% vs 5%; p = 0.003), FI (36% vs 0%; p = 0.005), and GEC (36% vs 0%; p = 0.005). For EMI, the FFA 0.7 mg/kg/day group showed a greater likelihood of reliable, clinically meaningful improvement than the FFA 0.2 mg/kg/day group (29% vs 0%; p = 0.040), but did not meet the significance threshold compared with placebo (29% vs 5%; p = 0.064). There were no significant associations between treatment and the likelihood of reliable, clinically meaningful worsening (p > 0.05). SIGNIFICANCE: In this preschool-aged DS population with high baseline everyday EF impairment, FFA treatment for 14-15 weeks was associated with dose-dependent, clinically meaningful improvements in regulating behavior, emotion, cognition, and overall everyday EF. These clinically meaningful improvements in everyday EF were not entirely due to seizure frequency reduction, suggesting that FFA may have direct effects on everyday EF during the early formative years of neurodevelopment.

Univariate and Multivariate Base Rates of Score Elevations, Reliable Change, and Inter-Rater Discrepancies in the BRIEF-A Standardization Samples.

The Behavior Rating Inventory of Executive Function-Adult Version (BRIEF-A) is a standardized rating scale of subjective executive functioning. We provide univariate and multivariate base rates (BRs) for scale/index scores in the clinical range (T scores ≥65), reliable change, and inter-rater information not included in the Professional Manual. Participants were adults (ages = 18-90 years) from the BRIEF-A self-report (N = 1,050) and informant report (N = 1,200) standardization samples, as well as test-retest (n = 50 for self, n = 44 for informant) and inter-rater (n = 180) samples. Univariate BRs of elevated T scores were low (self-report = 3.3%-15.4%, informant report = 4.5%-16.3%). Multivariate BRs revealed the common occurrence of obtaining at least one elevated T-score across scales (self-report = 26.5%-37.3%, informant report = 22.7%-30.3%), whereas virtually none had elevated scores on all scales. Test-retest scores were highly correlated (self = .82-.94; informant = .91-.96). Inter-rater correlations ranged from .44 to .68. Significant (p < .05) test-retest T-score differences ranged from 7 to 12 for self-report, from 6 to 8 for informant report, and from 16 to 21 points for inter-rater T-score differences. Applications of these findings are discussed.

[Formula: see text]Multivariate base rates of score elevations on the BRIEF2 in children with ADHD, autism spectrum disorder, or specific learning disorder with impairment in reading.

There is a paucity of research examining multivariate base rates (MBRs) of elevated scores in pediatric rating scales of cognition. We present novel MBR information on the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF2) for several clinical groups: Attention-Deficit/Hyperactivity Disorder Combined Presentation (ADHD-C); ADHD Inattentive Presentation (ADHD-I); Autism Spectrum Disorder (ASD); and Specific Learning Disorder with impairment in Reading (SLD-R). Participants included children diagnosed as having ADHD-C (n = 350), ADHD-I (n = 343), ASD (n = 390), or SLD-R (n = 240). Cumulative MBRs (e.g., the % of a sample having one or more elevated scores) were examined for each BRIEF2 form (Parent, Teacher, and Self-Report) and at three T-score cutoffs (T ≥ 60, T ≥ 65, and T ≥ 70). The MBR of obtaining at least one elevated score was common across clinical groups and forms at T ≥ 60 (ADHD-C = 90.5-98.1%; ADHD-I = 83.9-98.7%; ASD = 90.3-96.9%, SLD-R = 60.0-78.4%), T ≥ 65 (ADHD-C = 66.7-97.2%; ADHD-I = 77.5-94.9%; ASD = 77.3-92.7%; SLD-R = 38.5-64.0%), and T ≥ 70 (ADHD-C = 52.4-89.4%; ADHD-I = 64.8-84.2%; ASD = 54.5-83.2%; SLD-R = 26.9-44.1%). MBRs appeared to differ as a function of group (ADHD-C > ADHD-I > ASD > SLD-R) and form (Parent > Teacher > Self-Report) though future research with well-defined samples is needed to investigate this. We provide novel MBR information to enhance clinical interpretation of BRIEF2 data.

[Formula: see text]Enhanced interpretation of the BRIEF2: multivariate base rates of elevated scores in the standardization samples.

The Behavior Rating Inventory of Executive Function, Second Edition (BRIEF2) is a standardized rating (self, parent, and teacher) scale of executive functioning in children and adolescents. Here, we provide multivariate base rate (MBR) information (for the Self, Parent, and Teacher forms), which is not included in the BRIEF2 Professional Manual. Participants were children and adolescents for the BRIEF2 Self-Report (ages = 11-18; N = 803), Parent-Report (ages = 5-18; N = 1,400), and Teacher-Report (ages = 5-18; N = 1,400) standardization samples. We focused on cumulative (e.g., % of sample with oneor more elevated scores) MBRs across scales, which were examined at three elevation levels on each form: T≥ 60, ≥65, and ≥70. Across forms, MBRs predictably decreased with increasing number of elevated scores and at higher cutoffs. The cumulative MBR of having at least one score at T≥ 60 was common (37.5-42.2%), but less frequent at T≥ 70 (15.4-17.4%). The probability of having elevated scores on all scales was very low, irrespective of form, age, or elevation threshold (T≥ 60 = 2.4-4.4%; T≥ 65 = 1.0-1.4%; T≥ 70 = 0.0-0.7%). There was no clinically meaningful relation between demographic factors (age, gender, race, and parental education) and MBRs. These data provide clinicians and researchers with an enhanced way of concurrently interpreting multiple BRIEF2 scales.

Improved everyday executive functioning following profound reduction in seizure frequency with fenfluramine: Analysis from a phase 3 long-term extension study in children/young adults with Dravet syndrome.

OBJECTIVE: Individuals with Dravet syndrome (DS) experience frequent pharmacoresistant seizures beginning in infancy. Most exhibit poor neurodevelopmental outcomes including motor function difficulties, behavior problems, and cognitive impairment. Cognitive deficits in children with DS have been associated with seizure frequency and antiseizure medication (ASM) use. Recent research in children and young adults with DS has begun to examine the role of executive functions (EFs), as these include higher-order cognitive functions and may mediate the relationship between risk factors and cognitive impairment. Current conceptualizations, however, of EFs involve the broader self-regulation of cognitive, behavioral, and emotional domains. We explored relationships between reduction in convulsive seizure frequency and everyday EFs in a subset of children and young adults with DS treated with adjunctive fenfluramine for 1 year. METHODS: This is a post-hoc analysis of data from children and young adults with Dravet syndrome aged 5-18 years who participated in a phase 3 randomized, placebo-controlled clinical trial (core study) followed by completion of at least 1 year of fenfluramine treatment in an open-label extension (OLE) study. Eligible children and young adults started the OLE study at 0.2 mg/kg/day fenfluramine and were titrated to optimal seizure control and tolerability (maximum daily dose: 26 mg/day). Parents/caregivers documented convulsive seizure frequency per 28 days (i.e., monthly convulsive seizure frequency [MCSF]) by electronic diary. A parent/caregiver for each child also completed the Behavior Rating Inventory of Executive Function (BRIEF®) parent form, a questionnaire capturing parents'/caregivers' perceptions of everyday EF that was included as a safety measure to assess treatment-related adverse effects on EF during the trial. Ratings on BRIEF® were mapped to the current edition, the BRIEF®2 parent form, and were used to calculate T-scores for the Behavior Regulation Index (BRI), Emotion Regulation Index (ERI), Cognitive Regulation Index (CRI), and Global Executive Composite (GEC). Change in BRIEF®2 T-scores from baseline in the core study to Year 1 of the OLE study was calculated. Spearman's rho correlation coefficients assessed associations between change in BRIEF®2 indexes/composite T-scores and percentage change in MCSF. Children and young adults were divided into 2 groups based on percentage of MCSF reduction achieved from pre-randomization baseline in the core study to Year 1 of the OLE study: <50% and ≥50% MCSF reduction. Changes in the distribution of BRIEF®2 indexes/composite T-scores were compared between MCSF reduction groups using Mann-Whitney U tests. The proportions of children and young adults in these groups who showed clinically meaningful improvement in everyday EF, defined as Reliable Change Index (RCI) values ≥95% certainty relative to a reference population of neurotypically developing healthy volunteers, were then assessed by cross-tabulations and Somers' D tests (p ≤ 0.05). When there was a significant meaningful improvement in an index score, post-hoc analyses using the same statistical methods were conducted to evaluate the individual BRIEF®2 scales composing that index. Supplemental analyses examined the proportions of patients in MCSF reduction groups <25% and ≥75% who achieved clinically meaningful improvement or worsening in everyday EF using RCI values ≥95% certainty and ≥80% certainty, respectively, relative to the reference population. RESULTS: At the time of analysis, 58 children and young adults (mean age: 11 ±â€¯4 years) had reached OLE Year 1 of fenfluramine treatment with a 75% median percentage reduction in seizure frequency from pre-randomization baseline. Overall, there was a significant correlation between change in MCSF and change in BRIEF®2 T-scores for ERI (p = 0.008), but not for BRI, CRI, or GEC (p > 0.05). At OLE Year 1, 78% (n = 45) of total children/young adults had ≥50% MCSF reduction (50% [n = 29] achieved ≥75% MCSF reduction) and 22% (n = 13) of total children/young adults had <50% MCSF reduction (12% [n = 7] showed <25% MCSF reduction). The ≥50% MCSF reduction group was significantly more likely to achieve clinically meaningful improvement (RCI ≥ 95% certainty) in ERI (p = 0.002) and in CRI (p = 0.001) than the <50% MCSF reduction group. There were no significant differences in the proportions of children and young adults in the 2 MCSF reduction groups showing clinically meaningful worsening (RCI ≥ 80% certainty) on the BRIEF®2 indexes/composite. SIGNIFICANCE: In children and young adults with DS, the magnitude of reduction in MCSF after long-term treatment with adjunctive fenfluramine was associated with clinically meaningful levels of improvement in everyday EF. Seventy-eight percent (78%) of children and young adults treated with adjunctive fenfluramine for 1 year in the OLE study achieved ≥50% reduction in MCSF, for a magnitude of efficacy associated with a significantly greater likelihood of experiencing clinically meaningful improvement in emotion regulation and cognitive regulation.

Everyday executive function in focal onset pediatric epilepsy on the parent-report BRIEF2.

Executive function (EF) difficulties are a core neuropsychological feature of pediatric epilepsy, and parent-report measures of EF concerns are an important complement to task-based EF measures. The Behavior Rating Inventory of Executive Function (BRIEF) has shown sensitivity to parent-reported EF concerns in epilepsy and other pediatric populations. We compared profiles of parent-reported EF concerns using the BRIEF and its revision, the BRIEF2, in 117 pediatric patients with focal onset epilepsy to examine the clinical utility of the revised scale. We then compared BRIEF2 profiles between patients and age- and gender-matched healthy controls. Among patients, profiles on the BRIEF did not globally differ from the BRIEF2, and agreement was very good across scales. Patients and controls differed significantly on the BRIEF2, with patients showing higher EF difficulties reported by parents across most scales. High rates of clinical elevation among patients emerged on the Task Monitor, Plan/Organize, Working Memory, and Shift scales. Younger age of epilepsy onset, chronic epilepsy, and right hemisphere seizure focus were associated with higher parent-reported EF concerns. Findings suggest that the BRIEF2 demonstrates similar performance to the BRIEF among pediatric patients with focal onset epilepsy who are most at risk in the areas of task monitoring, working memory, planning and organization, and flexibility. These findings are informative when comparing literature across versions and provide additional insight into the nature of parent-reported EF difficulties among children with focal onset epilepsy.

Distinct Patterns of Everyday Executive Function Problems Distinguish Children With Tourette Syndrome From Children With ADHD or Autism Spectrum Disorders.

OBJECTIVE: The aim is to investigate the everyday executive function (EF) in children with Tourette syndrome (TS), Inattentive or Combined presentations of ADHD (ADHD-I/ADHD-C), autism spectrum disorders (ASD), and typically developing children (TDC). METHOD: Nineteen TS, 33 ADHD-C, 43 ADHD-I, 34 ASD, and 50 TDC participated (8-17 years). Parents completed the Behavior Rating Inventory of Executive Function (BRIEF). RESULTS: TS, ADHD-C, ADHD-I, or ASD were rated with significantly more regulation problems on all scales compared with TDC. Considerable overlap of symptoms between clinical groups made differentiation difficult on individual scales. Scale configurations showed children with TS to have more problems with emotional control (EC) than cognitive flexibility in relation to children with ASD, more problems with EC than inhibitory control in relation to ADHD-C, and more problems with EC than planning/organizing in relation to ADHD-I. CONCLUSION: Paired BRIEF scales dissociated EF problems in children with TS from children with ADHD-C, ADHD-I, or ASD. Clinical relevance is discussed.

Executive Function and Emotion Regulation Strategy Use in Adolescents.

Development of emotion regulation strategy use involves a transition from reliance on suppression during childhood to greater use of reappraisal in adolescence and adulthood-a transition that parallels developmental changes in executive functions. We evaluated the relationship between emotion regulation strategy use and executive functioning in the everyday life of 70 typically developing adolescents who completed the Emotion Regulation Questionnaire for Youth and the Behavior Rating Inventory of Executive Function-Self-Report. Results indicated that greater reliance on reappraisal was associated with better executive functions, while reliance on suppression was related to poorer executive functions. Findings suggest that adolescents who rely on reappraisal may have more cognitive resources to help them remain attentive and well regulated in their daily lives. On the other hand, if better executive functions facilitate the use of reappraisal, adolescents' ability to regulate their emotions could potentially be enhanced via supports for executive functions.

Much ado about norming: the Behavior Rating Inventory of Executive Function.

The Behavior Rating Inventory of Executive Function (BRIEF) is a rating scale designed to assess executive functions in everyday life that is widely used in school and clinical settings and in research studies. It has been recently suggested, however, that the limited geographic stratification of the standardization sample renders the measure overly sensitive. We evaluated this hypothesis by examining BRIEF scores across studies of typically developing children and adolescents. Thirty-nine studies were identified that included at least one of three possible index scores. Mean scores across studies were (a) within one to two T-score units from the standardization sample mean of 50, (b) tended to be slightly lower than 50, and (c) were unrelated to geographic location (US Census regions or internationally). These findings refute recent claims that the BRIEF is overly sensitive and further add to the large body of literature supporting the validity of the measure.

Contribution of rating scales to intervention for executive dysfunction.

Executive dysfunction is present in children, adolescents, and adults with a wide range of clinical conditions. A growing body of literature has demonstrated the usefulness of rating scales designed to gauge executive functioning in everyday life. In this article, we discuss evidence supporting the use of such rating scales to assess intervention outcome, how they may inform development of interventions, and how comparing rater perspectives can assess awareness of cognitive dysfunction. We provide an example of how an executive function rating scale helped define intervention targets and measured outcomes in a recently published real-world intervention for children with autism spectrum disorder. Rating scales of executive function provide valuable information with respect to treatment planning and assessment of intervention outcome.

Confirmatory factor analysis of the Behavior Rating Inventory of Executive Function-Adult version in healthy adults and application to attention-deficit/hyperactivity disorder.

The Behavior Rating Inventory of Executive Function-Adult Version (BRIEF-A) is a questionnaire measure designed to assess executive functioning in everyday life. Analysis of data from the BRIEF-A standardization sample yielded a two-factor solution (labeled Behavioral Regulation and Metacognition). The present investigation employed confirmatory factor analysis (CFA) to evaluate four alternative models of the factor structure of the BRIEF-A self-report form in a sample of 524 healthy young adults. Results indicated that a three-factor model best fits the data: a Metacognition factor, a Behavioral Regulation factor consisting of the Inhibit and Self-Monitor scales, and an Emotional Regulation factor composed of the Emotional Control and Shift scales. The three factors contributed 14%, 19%, and 24% of unique variance to the model, respectively, and a second-order general factor accounted for 41% of variance overall. This three-factor solution is consistent with recent CFAs of the Parent report form of the BRIEF. Furthermore, although the Behavioral Regulation factor score in the two-factor model did not differ between adults with attention-deficit/hyperactivity disorder and a matched healthy comparison group, greater impairment on the Behavioral Regulation factor but not the Emotional Regulation factor was found using the three-factor model. Together, these findings support the multidimensional nature of executive function and the clinical relevance of a three-factor model of the BRIEF-A.

Contribution of rating scales to the assessment of executive functions.

Executive functions play a vital role in the everyday functioning of healthy individuals across the lifespan and have been implicated in a wide variety of clinical conditions. Historically, the assessment of executive functions in clinical and research settings relied on performance-based measures. A number of authors have argued, however, that such measures have limited ecological validity. In response to this limitation of performance-based measures, several rating scales have been developed that seek to gauge a person's or their knowledgeable informant's (e.g., parent or teacher) subjective view of executive functioning in everyday life. In this article we review evidence supporting the use of rating scales of executive function including profiles in clinical populations, biological correlates, relationships to relevant outcome measures such as academic performance, and correlations with performance-based measures. We conclude that performance-based and rating scale measures provide complementary information with respect to a child's executive functions, offering a more comprehension view than either approach alone.

Response-time variability is related to parent ratings of inattention, hyperactivity, and executive function.

OBJECTIVE: Individuals with ADHD are often characterized as inconsistent across many contexts. ADHD is also associated with deficits in executive function. We examined the relationships between response time (RT) variability on five brief computer tasks to parents' ratings of ADHD-related features and executive function in a group of children with a broad range of ADHD symptoms from none to full diagnosis. METHODS: We tested 98 children (mean age 9.9 ± 1.4 years; 66 boys) from community clinics on short tasks of executive control (TEC) and the Eriksen Flanker task, while a parent completed the Conners' Parent Rating Scale and Behavior Rating Inventory of Executive Function. RESULTS: Variability for two of the TEC tasks explained significant proportions of the variance of all five ADHD-related Conners' subscales and several executive function subscales. By contrast, variability on the flanker task or mean RTs for any task were not associated with any rating scale. CONCLUSION: The significant dimensional relationships observed between variability measures and parent ratings supported the utility of RT variability as an objective measure in ADHD and aspects of executive functioning that is superior to RT means or accuracy measures.

Executive Function in the Real World: BRIEF lessons from Mark Ylvisaker.

It is well established that brain injuries adversely affect the executive functions and their development. Mark Ylvisaker had a substantial impact on how we assess and treat children and adolescents with disrupted executive functions secondary to traumatic brain injury. He articulated core principles for effective assessment and intervention that emphasized the importance of real-world meaning and application. He taught us that assessment that captures a child's everyday functioning in the context of real-world demands is often more informative than traditional neuropsychological measures alone and that interventions that improve functioning in the real-world environment are most useful. His pragmatic model influenced our own efforts to measure executive function and to develop intervention systems for children with executive function deficits. This article reviews the development of the Behavior Rating Inventory for Executive Function as an approach to ecological assessment of children and adolescents with traumatic brain injury. As we take what we learned from Mark Ylvisaker, it becomes increasingly apparent that our assessment and intervention methods, especially as related to the executive functions, demand an everyday, real-world context.

Subjective rating of working memory is associated with frontal lobe volume in schizophrenia.

BACKGROUND: Patients with schizophrenia commonly show deficits in working memory on objective neuropsychological measures, and brain imaging studies have documented neural abnormalities during performance of working memory tasks. It remains unclear to what extent such patients are able to accurately gauge the integrity of their working memory in their daily lives. AIMS: We evaluated the relationship between subjective rating of working memory integrity in daily life and volumes of the frontal, temporal, and parietal lobes in patients with schizophrenia. METHODS: Participants included 29 patients with schizophrenia and 26 healthy comparison subjects. Participants completed a structural magnetic resonance imaging (MRI) scan, the Self Report form of the Behavioral Rating Inventory of Executive Function - Adult version (BRIEF-A), and Digit Span Backwards as an objective measure of working memory. Lobar volumes were obtained using an automated processing package and adjusted for total intracranial volume. RESULTS: The patient group reported worse working memory in daily life, and performed worse on Digit Span Backwards, than the comparison group. Within the patient group, poorer working memory in daily life was associated with smaller left and right frontal lobe volumes. Shorter backwards digit span was associated with smaller left frontal and left and right temporal lobe volumes. CONCLUSIONS: The significant relationship between frontal lobe volumes and subjective working memory in daily life provides some support for the validity of self report measures of cognitive functioning in patients with schizophrenia, and provides further evidence for a contribution of frontal lobe abnormality to executive dysfunction in the illness.

Improving identification and diagnosis of mild traumatic brain injury with evidence: psychometric support for the acute concussion evaluation.

OBJECTIVES: A dearth of standardized assessment tools exists to properly assess and triage mild traumatic brain injury (mTBI) in primary care and acute care settings. This article presents evidence of appropriate psychometric properties for the Acute Concussion Evaluation (ACE), a new structured clinical interview. PARTICIPANTS: Parent informants of 354 patients, aged 3 to 18 years, with suspected mTBI completed the ACE via telephone interview. MEASURE: Acute Concussion Evaluation. RESULTS: Evidence is presented for appropriate item-scale membership, internal consistency reliability as well as content, predictive, convergent/divergent, and construct validity of the ACE symptom checklist. CONCLUSIONS: Overall, the ACE symptom checklist exhibits reasonably strong psychometric properties as an initial assessment tool for mTBI.

Self- and informant reports of executive function on the BRIEF-A in MCI and older adults with cognitive complaints.

Amnestic mild cognitive impairment (MCI) is characterized by impaired episodic memory, although subtle executive problems have been noted on neuropsychological tests. Recent research also has described a group of healthy, non-depressed older adults with significant cognitive complaints (CC) but normal performance on neuropsychological testing. These individuals show structural and functional brain changes intermediate between those seen in MCI and healthy older adults without such complaints (HC). We evaluated executive functions in MCI and CC using the Behavior Rating Inventory of Executive Function-Adult version (BRIEF-A), a newly developed self- and informant report questionnaire in 29 patients with amnestic MCI, 28 CCs, and 30 demographically matched HCs. MCI and CC participants reported significant difficulties with selective aspects of executive functioning relative to HCs despite clinically normal performance on neuropsychological tests of this cognitive domain. Scores were generally in the pattern of MCI>CC>HC, and findings were most pronounced for working memory. Additionally, MCI and CC participants were more likely than their informants to report clinically meaningful executive problems, though informants identified a similar pattern of difficulty overall. Results failed to reveal strong relations between the BRIEF-A and standardized neuropsychological tests of executive function. Overall findings indicate that the BRIEF-A is sensitive to subtle executive changes in MCI and CC and suggest the need for research to determine if executive complaints are predictive of clinical course.

Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome.

OBJECTIVE: Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS: A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 +/- 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS: The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 +/- 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS: Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.

Assessment of executive function in preschool-aged children.

Assessment of the overarching self-regulatory mechanisms, or executive functions, in any age group is challenging, in part due to the complexity of this domain, in part due to their dynamic essence, and in part due to the inextricable links between these central processes and the associated domain-specific processes, such as language, motor function, and attention, over which they preside. While much progress has been made in clinical assessment approaches for measuring executive functions in adults and to some extent in adolescents and school-aged children, the toolkit for the preschool evaluator remains sparse. The past decade, however, has seen a substantial increase in attention to executive functions in very young children from a developmental neuropsychological perspective. With this has come a necessity for better, more specific, and more internally valid performance measures, many of which are now described in the experimental literature. Few such tasks, however, have adequately demonstrated psychometric properties for clinical application. We present two performance tasks designed to tap selective aspects of executive function in preschoolers that are emerging from the experimental laboratory and hold promise of appropriate reliability and validity for the clinical laboratory. Performance tests alone, however, are insufficient to develop a comprehensive picture of a child's executive functioning. Thus, we present a rating scale of preschoolers' executive function in the everyday context, and advocate a model of executive function assessment that incorporates both controlled performance tasks that target specific aspects of executive function and parent/teacher ratings that target more global aspects of self-regulation in the everyday context.

Psychosocial treatment of children in foster care: a review.

A substantial number of children in foster care exhibit psychiatric difficulties. Recent epidemiological and historical trends in foster care, clinical findings about the adjustment of children in foster care and adult outcomes are reviewed, followed by a description of current approaches to treatment and extant empirical support. Available interventions for these children can be categorized as either symptom-focused or systemic, with empirical support for specific methods ranging from scant to substantial. Even with treatment, behavioral and emotional problems often persist into adulthood resulting in poor functional outcomes. We suggest that self-regulation may be an important mediating factor in the appearance of emotional and behavioral disturbance in these children.