Arnaud Roy

Executive dysfunction in children with neurofibromatosis type 1: a study of action planning.

In this study, we tested the hypothesis that action planning is impaired in children with neurofibromatosis type 1 (NF1). Thirty-six children with NF1 were pair-matched to 36 healthy controls (HC) on age (range, 7-12 years), sex, and parental education level, and both groups were administered three action-planning tasks. To examine the relation of task performance to attention deficit hyperactivity disorder (ADHD), the NF1 group was divided into subsets of children who met or did not meet criteria for ADHD. Children with NF1 performed less well than HC on all planning tasks, and differences remained when controlling for IQ or a measure of visuospatial skill. Both the NF1 with ADHD subset and NF1 without ADHD subset performed more poorly than HC on two of the tasks, whereas only the NF1 with ADHD subset performed worse than HC on the third planning task. The results underscore the importance of evaluating executive function in children with NF1 and suggest that deficits in this domain may be only partially related to ADHD. Planning deficits in children with NF1 may be part of their cognitive phenotype. Identifying these deficits is relevant in determining factors contributing to learning problems and in developing appropriate interventions.

Assessment of everyday executive functioning in children with frontal or temporal epilepsies

Executive functions are particularly vulnerable in case of brain disruption during childhood, when the brain is not fully mature. Some studies showed impairments of executive functions in children with epilepsy, but only a few of them investigated the impact of executive dysfunctions on daily life. The aim of this study was to understand the everyday executive functioning of children with epilepsy both at home and in school. We administered the Behavior Rating Inventory of Executive Function to parents and teachers of 53 children (7-16 years of age) with structural epilepsies or epilepsies of unknown cause of temporal lobe (n=25) or frontal lobe (n=28). The results indicated a global executive impairment in the whole group of patients, compared with normative data, with no difference between the group with temporal lobe epilepsy (TLE) and that with frontal lobe epilepsy (FLE), except for monitor domain, which seemed more frequently impaired in the group with FLE. Congruence between parent and teacher ratings was found. The frequency of seizures was not related to executive dysfunction, whereas the number of antiepileptic drugs tended to positively correlate with working memory impairment. Onset of epilepsy at a younger age was also related to more executive difficulties but only according to teacher ratings. Lastly, duration of epilepsy was strongly associated with executive deficits reported in the context of school. Our results support the executive dysfunction hypothesis in daily life of children with structural focal epilepsy or focal epilepsy of unknown cause and are consistent with the early brain vulnerability hypothesis currently prevalent in the context of child neuropsychology. The BRIEF appears to be a clinically useful tool for assessing executive function impairment in this clinical population.

Multigroup confirmatory factor analysis and structural invariance with age of the Behavior Rating Inventory of Executive Function (BRIEF)--French version.

The parent and teacher forms of the French version of the Behavioral Rating Inventory of Executive Function (BRIEF) were used to evaluate executive function in everyday life in a large sample of healthy children (N = 951) aged between 5 and 18. Several psychometric methods were applied, with a view to providing clinicians with tools for score interpretation. The parent and teacher forms of the BRIEF were acceptably reliable. Demographic variables (such as age and gender) were found to influence the BRIEF scores. Confirmatory factor analysis was then used to test five competing models of the BRIEFs latent structure. Two of these models (a three-factor model and a two-factor model, both based on a nine-scale structure) had a good fit. However, structural invariance with age was only obtained with the two-factor model. The French version of the BRIEF provides a useful measure of everyday executive function and can be recommended for use in clinical research and practice.

Examining the Frontal Subcortical Brain Vulnerability Hypothesis in Children With Neurofibromatosis Type 1: Are T2-Weighted Hyperintensities Related to Executive Dysfunction?

OBJECTIVE It was hypothesized that neuropsychological impairments in children with neurofibromatosis type I (NF1) are associated with brain areas of increased T2-weighted signal intensity on MRI. Systematic and extensive examination of this hypothesis remains however scarce, particularly regarding executive dysfunction whereas hyperintensities are located preferentially in frontal-sub-cortical networks. In this study, we compared the executive functioning profile with characteristics of brain hyperintensities in children with NF1. METHOD A sample of 36 school-age children with NF1 (7-12 years) underwent a detailed examination of executive function, including performance-based tests and childs behavior rating in daily life. Executive function measures were compared with the characteristics of the T2-weighted hyperintensities on parallel MRI scans. The presence, number, and size of hyperintensities in the whole brain were considered as well as their main cerebral locations. RESULTS Executive dysfunction including traditional cognitive and ecological measures in children with NF1 is not significantly influenced by T2-weighted hyperintensities, in terms of presence or not, number, size, and location, whether in the whole brain or according to involved specific brain areas. CONCLUSION T2-weighted hyperintensities, as they are currently measured, cannot be used as a strong indicator of executive dysfunction in children with NF1. Based on the available NF1 cognitive impairment pathogenesis models, a critical discussion on anatomical-functional relationships between hyperintensities and neuropsychological profile is proposed, especially the executive dysfunction.

Ecological approach of executive functions using the Behavioural Assessment of the Dysexecutive Syndrome for Children (BADS-C): Developmental and validity study

Introduction. Ecological assessment of executive functions (EF) with tasks simulating everyday-life difficulties in children remains poorly developed. The Behavioural Assessment of the Dysexecutive Syndrome for Children (BADS-C) is one of the rare tools proposed in this perspective, for which developmental and convergent validity are, however, rather limited. The objectives of this study were to explore EF development using the BADS-C, while considering the effect of gender and parental education as well as controversial relationships between intelligence and EF. We also aimed to examine to what extent the BADS-C could reflect EF of children in everyday life, as reported by their parents on questionnaires. Method. A group of 120 healthy children aged from 7 to 12 years was recruited. Their executive performance was examined by means of the BADS-C, and their intellectual efficiency was tested with the Wechsler intelligence scales. Rating of EF in everyday life was simultaneously carried out by children’s parents, using two questionnaires: the Dysexecutive Questionnaire for Children (DEX-C) of the BADS-C and the Behavior Rating Inventory of Executive Function (BRIEF). Results. Results showed a significant effect of age on BADS-C subtests, with inconsistencies from one subtest to another. Gender effect on EF performance was nonsignificant. Weak correlations were found between EF scores and parental education or intelligence quotient. Lastly, while the two questionnaires of everyday-life EF were strongly correlated with each other, their links with BADS-C subtests scores were weak. Conclusions. This study shows differentiated developmental curves on BADS-C, reflecting a variable implication of executive resources according to subtests. The limited relations between BADS-C performance and sociodemographic variables or IQ could be due to the fact that executive difficulties are moderate in healthy children at this age. Moreover, the capacity of the BADS-C to reflect EF as perceived by the child’s relatives in everyday life remains questionable.

Involvement of Technical Reasoning More Than Functional Knowledge in Development of Tool Use in Childhood

It is well-known that even toddlers are able to manipulate tools in an appropriate manner according to their physical properties. The ability of children to make novel tools in order to solve problems is, however, surprisingly limited. In adults, mechanical problem solving (MPS) has been proposed to be supported by “technical reasoning skills,” which are thought to be involved in every situation requiring the use of a tool (whether conventional or unusual). The aim of this study was to investigate the typical development of real tool use (RTU) skills and its link with technical reasoning abilities in healthy children. Three experimental tasks were adapted from those used with adults: MPS (three different apparatus), RTU (10 familiar tool-object pairs), and functional knowledge (FK; 10 functional picture matching with familiar tools previously used). The tasks were administered to 85 healthy children divided into six age groups (from 6 to 14 years of age). The results revealed that RTU (p = 0.01) and MPS skills improve with age, even if this improvement differs according to the apparatus for the latter (p < 0.01 for the Hook task and p < 0.05 for the Sloping task). Results also showed that MPS is a better predictor of RTU than FK, with a significant and greater weight (importance weight: 0.65; Estimate ± Standard Error: 0.27 ± 0.08). Ours findings suggest that RTU and technical reasoning develop jointly in children, independently from development of FK. In addition, technical reasoning appears partially operative from the age of six onward, even though the outcome of these skills depends of the context in which they are applied (i.e., the type of apparatus).

Praxis skills and executive function in children with neurofibromatosis type 1

ABSTRACT This study aimed at examining motor and ideomotor praxis skills in children with neurofibromatosis type 1 (NF1). The impact of executive dysfunction, frequently described in children with NF1, on the expression of praxis impairments was also studied. Eighteen children with NF1 were included and matched with 20 control children for age (7–14 years), sex, laterality, and parental education level. Both groups of children underwent an assessment based on cognitive models of apraxia including visuomotor tasks, executive tests, and everyday life questionnaires. The group of children with NF1 showed a trend to weaker performances on motor and ideomotor praxis than the control group, only on the finger use condition (ps < .05; with a moderate to large effect size), but not regarding manual use condition (ps > .08). Moreover, these praxis difficulties disappeared when executive dysfunctions (planning and inhibition) were controlled. These findings support the negative impact of executive dysfunctions on praxis skills in children with NF1. The identification of praxis and executive function disorders as well as their interaction is important for differentiating primary praxic disorder from a cognitive deficit that may be expressed in gesture. Clinically, this distinction is essential to optimize targeted and effective rehabilitative interventions.

Script-event representation in patients with severe traumatic brain injury.

The aim of the present study was to examine the syntactic and semantic dimensions of script representation in patients with structural damage within the cerebral cortex following a severe traumatic brain injury (TBI). Forty TBI patients and 38 healthy control subjects (HC) were asked to sort cards describing actions belonging to eight scripts according to the script to which they belonged and according to their order of execution. Each script included actions which were low in centrality and distinctiveness (NCA & NDA), and high in centrality (CA), distinctiveness (DA), and CA and DA. Actions were presented in three conditions. In the scripts with headers (SH) condition, the actions were given with each script header written on a separate card. In the scripts without headers condition (SwH) no script header was provided. In the scripts with distractor header (SDH) condition, the actions were given with each script header and a distractor header written on separate cards. The results showed that performance of TBI patients was significantly lower in all conditions. Overall, TBI patients made significantly more sequencing and sorting errors (for all types of actions) than HC subjects. These data are consistent with the view that TBI produces impairment of both the syntactic and semantic dimensions of script representation.

Ecological Assessment of Everyday Executive Functioning at Home and at School Following Childhood Traumatic Brain Injury Using the Brief Questionnaire

Objectives: To describe dysexecutive symptoms in children with traumatic brain injury (TBI) using the Behavior Rating Inventory of Executive Function (BRIEF); to compare parent- and teacher-ratings, to analyze the differential impairment in the BRIEF subscales, and factors influencing outcome. Participants: Children aged 5 to 18 years 11 months, referred to a rehabilitation department following TBI. Outcome Measures: Parent- and teacher reports of the BRIEF. Results: A total of 194 patients participated in the study: mild (n = 13), moderate (n = 12), severe (n = 169); mean 4.92 (standard deviation = 3.94) years post-injury. According to parent ratings (n = 193), all BRIEF subscales and indices were significantly elevated (23.8%–48% in the clinical range). The Working Memory subscale score was significantly higher than all other subscales. Results of teacher ratings (n = 28) indicated similar significantly elevated scores in all subscales (39.3%–57.2% in the clinical range). No significant difference was found between parent and teacher ratings, which were significantly correlated. Regression analyses indicated that, in children with severe TBI, parental BRIEF overall and metacognition indices were significantly predicted by younger age at injury and older age at assessment, whereas no significant predictor of behavioral regulation index was identified. Discussion and Conclusion: This study highlights significant executive dysfunction in everyday life several years after childhood TBI, evident in home and school environments.

Health-Related Quality of Life for Children and Adolescents with Specific Language Impairment: A Cohort Study by a Learning Disabilities Reference Center

Objectives To assess the health-related quality of life (HRQOL) of children with specific language impairment (SLI). Study Design In a prospective sample at a Learning Disabilities Reference Center, proxy-rated HRQOL (KIDSCREEN-27) was assessed for children with SLI and unaffected children from January 1, 2014 to March 31, 2015. Quality of life predictors for children with SLI were evaluated by recording the length and number of speech therapy and psychotherapy sessions and the specific school organization that the children had participated in. The KIDSCREEN scores of the two groups were compared using nonparametric statistics. Results The questionnaires were completed by the parents of 67 children with SLI and 67 unaffected children. For children with SLI, the mean HRQOL scores were significantly lower for physical and psychological well-being, autonomy and parent relation, social support, and school environment compared to the reference group, controlling for age and parental education (β = -6.7 (-12.7;-.7) P = 0.03, β = -4.9 (-9.5;-.3) P = 0.04, β = -8.4 (-14.2;-2.6) P = 0.005, β = -11.6 (-19.5;-3.7) P = 0.004, β = -7.1(-12.4;-1.7) P = 0.010, respectively). Multivariate analyses in the group of children with SLI found that children who had undergone psychotherapy sessions or who had been enrolled in specific schooling programs had reduced HRQOL scores in social support and school environment and that children who were in a special class had higher scores in physical well-being. Conclusion Children with SLI had significantly lower HRQOL scores as compared to unaffected children. Measurement of HRQOL could serve as one of the strategies employed throughout the follow-up of these individuals to provide them with the most appropriate and comprehensive care possible.