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OBJECTIVE: The present study is the first to examine theory of mind (ToM) sequelae in a sample of adult survivors of primary brain tumors, and to investigate the assumed relationship between ToM and health-related quality of life (HRQoL). METHOD: Participants were 40 long-term adult survivors of primary brain tumors and 40 matched healthy controls. They completed ToM tests (Faux-Pas test and Advanced ToM task) and two questionnaires assessing HRQoL (36-Item Short-Form Health Survey and EORTC QLQ-C30/QLQ-BN20). Their relatives also completed an observer-rated version of the SF-36 questionnaire. RESULTS: Survivors performed worse than controls only on the Advanced ToM task. Overall, patients and caregivers reported more problems than healthy controls and their relatives regarding both global HRQoL and its social/emotional aspects. No relationship was found between ToM and HRQoL scores. CONCLUSION: Adult survivors of primary brain tumors may exhibit ToM deficits several years after treatment and report more problems on social/emotional HRQoL components. Our findings highlight the need to consider these late effects in survivors' long-term follow-up, even if the clinical involvement of ToM deficits still needs to be elucidated. The assessment of ToM deficits and their potential impact on survivors' everyday life is thoroughly discussed.
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Neoplasias Encefálicas , Teoria da Mente , Adulto , Humanos , Qualidade de Vida , Cognição Social , Neoplasias Encefálicas/complicações , Sobreviventes/psicologia , Testes NeuropsicológicosRESUMO
The present study focused on adult primary brain tumor (PBT) survivors' caregivers. The main objective was to study associations between PBT survivors' health-related quality of life (HRQOL), their behavioral executive functions (EF) and their caregivers' HRQOL. Forty PBT survivors of PBT and 37 caregivers (mostly patient's spouses 81.08%; n = 30) participated in the study. PBT survivors completed a cancer related Quality of Life (QOL) questionnaire. Caregivers completed informant rated HRQOL and behavioral EF reports relating to PBT survivors and a self-rated HRQOL questionnaire relating to themselves. Correlational and multiple regression analyses were conducted. No associations were found between caregivers' physical HRQOL and PBT survivors' HRQOL nor behavioral EF. Analyses yielded several significant correlations between caregivers' mental HRQOL and variables pertaining to PBT survivors' HRQOL and behavioral EF. Multiple regression analyses showed that caregivers' mental HRQOL is predicted by PBT survivors' mental HRQOL, global cancer-related QOL scores and global behavioral EF scores. This study provides evidence suggesting that during the survivorship phase, at an average of 3.67 (SD = 2.31) years following treatment for a PBT, caregivers mental HRQOL is linked to PBT survivors' long-term effects. These findings shed some light regarding post-cancer care for both PBT survivors and their caregivers.
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Neoplasias Encefálicas , Qualidade de Vida , Adulto , Humanos , Estudos Transversais , Função Executiva , Cuidadores , SobreviventesRESUMO
OBJECTIVE: The term intellectually gifted (IG) refers to children of high intelligence, which is classically measured by the intelligence quotient (IQ). Some researchers assume that the cognitive profiles of these children are characterized by both strengths and weaknesses, compared with those of their typically developing (TD) peers of average IQ. The aim of the present systematic review was to verify this assumption, by compiling data from empirical studies of cognitive functions (language, motor skills, visuospatial processing, memory, attention and executive functions, social and emotional cognition) and academic performances. METHOD: The literature search yielded 658 articles, 15 of which met the selection criteria taken from the Preferred Reporting Items for Systematic Reviews and Meta-Analyses model. We undertook a qualitative summary, to highlight any discrepancies between cognitive functions. RESULTS: IG children exhibited better skills than TD children in a number of domains, including attention, language, mathematics, verbal working memory, shifting, and social problem solving. However, the two groups had comparable skills in visuospatial processing, memory, planning, inhibition, and visual working memory, or facial recognition. CONCLUSION: Although IG children may have some strengths, many studies have failed to find differences between this population and their TD peers on many other cognitive measures. Just like any other children, they can display learning disabilities, which can be responsible for academic underachievement. Further studies are needed to better understand this heterogeneity. The present review provides pointers for overcoming methodological problems and opens up new avenues for giftedness research.
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Criança Superdotada , Criança , Criança Superdotada/psicologia , Função Executiva , Humanos , Inteligência , Memória de Curto Prazo , Testes Neuropsicológicos , Resolução de ProblemasRESUMO
The health consequences of being involved in bullying and cyberbullying are well described for adolescents, but many questions related to the role played by their life skills remain unanswered. Accordingly, this systematic review aims to provide a clear overview of research on the relationships between bullying involvement as a bully, victim, bully-victim or bystander, and adolescents' life skills. This article systematically reviewed 71 relevant empirical studies that met the inclusion criteria, extracted from the PubMed, PsycINFO, Scopus, Sage, Wiley, and SpringerLink databases. Their main findings were categorized according to the three types of life skills described by the World Health Organization: decision-making/problem-solving skills, interpersonal and communication skills, and self-management skills. Results showed relatively consensual outcomes for communication and interpersonal skills (empathy, moral disengagement) and skills for managing stress (coping strategies). Other decision-making or interpersonal skills, such as executive function or theory of mind, were poorly explored, and require further research, if we are to understand how life skills may be involved in bullying. Taken together, our findings highlight methodological heterogeneity and measurement problems in bullying studies that make their results difficult to interpret. Recommendations for prevention/education health researchers and professionals are provided, emphasizing the importance of considering the sociocognitive development of adolescents in bullying prevention.
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Bullying , Cyberbullying , Adolescente , Bullying/psicologia , Cyberbullying/psicologia , Empatia , Humanos , Princípios Morais , Instituições AcadêmicasRESUMO
OBJECTIVE: To assess the Global School Adaptation (GSA) questionnaire of children's abilities and classroom behavior administered to teachers of very preterm children at 5 years of age as a predictor of the need for educational support (grade retention, special class, learning support) at age 7. STUDY DESIGN: We assessed 858 very preterm children (<33 weeks of gestation) at 5 years of age using the GSA and again at 7 years to determine the use of educational support. We examined the association between the GSA score and educational support at 7 years and performed a receiver operating characteristic curve analysis. RESULTS: At 7 years of age, 130 children had educational support (15.2%). Children with a nonoptimal GSA score (<45) at 5 years required educational support more often (57.7%) than children with a GSA score of 45 or greater (15.4%) (OR, 7.5; 95% CI, 5.02-11.21). The need for educational support was associated with male sex; a low parent socioeconomic level; lower birth weight, birth head circumference, or gestational age (28-30 weeks of gestation); severe neurologic complications; patent ductus arteriosus ligation; and the use of therapy services at 5 years of age. After adjustment, only the GSA score was associated with educational support at 7 years of age (OR, 0.86; 95% CI, 0.84-0.88). A receiver operating characteristic curve analysis of the GSA performance revealed an optimal cut-off at 48, with a sensitivity of 70.8%, a specificity of 73.5%, and an area under the curve of 0.79. CONCLUSIONS: Using a cut-off score of 48, the GSA at 5 years of age may be a useful tool to identify children born preterm at risk of school-based learning difficulties.
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Adaptação Psicológica/fisiologia , Deficiências da Aprendizagem/diagnóstico , Deficiências da Aprendizagem/etiologia , Avaliação das Necessidades , Fatores Etários , Criança , Pré-Escolar , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Valor Preditivo dos Testes , Curva ROC , Inquéritos e QuestionáriosRESUMO
Objective: Childhood brain tumors (BTs) and their treatment often negatively affect development of executive functions. Previous studies have reported executive functions deficits, particularly through questionnaires of daily life. This study aimed to assess executive functioning in everyday life following pediatric BT, in a larger and more histologically diverse sample than previously, and to study clinical and demographic factors influencing outcome.Methods: Assessment of executive functioning using parent ratings of the Behavior Rating Inventory of Executive Function (BRIEF), in a large sample of children treated for various BT (n = 153). Clinical and demographic factors were: age at diagnostic, age at assessment, parental education level, radiation therapy.Results: Significant difficulties were found in the 3 composite indices and in the majority of the BRIEF subscales. The highest level of difficulties was observed in the Working Memory subscale. Older age at assessment and younger age at diagnosis were significantly associated with higher levels of parent-reported difficulties, particularly for metacognition.Conclusions: Parents of children treated for BT report widespread and persistent deficits in executive functions that negatively affect their everyday functioning. Including analysis of all clinical scales and composite indices allows a more comprehensive approach and enables to specify the patients' executive profile.
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Neoplasias Encefálicas , Metacognição , Adolescente , Idoso , Neoplasias Encefálicas/radioterapia , Criança , Função Executiva , Humanos , Memória de Curto Prazo , Testes Neuropsicológicos , Pais , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: To provide a comprehensive systematic review of the literature by examining studies published on all cognitive aspects of children with early and continuously treated phenylketonuria (ECT-PKU) included in the databases Medline, PsycINFO, and PsycARTICLE. METHOD: In addition to a classical approach, we summarized methodology and results of each study in order to discuss current theoretical and methodological issues. We also examined recent advances in biochemical markers and treatments of PKU, with implications for future research on metabolic control and its role as a determinant of neuropsychological outcome. RESULTS: Consistent with previous reviews, the hypothesis of a specific and central executive impairment in children with ECT-PKU was suggested. However, findings are inconclusive regarding the nature of executive impairments as well as their specificity, impact on everyday life, persistence over time, and etiology. CONCLUSION: Given the current state of the science, we suggest future directions for research that utilizes a developmental and integrative approach to examine the effects of recent advances in biochemical markers and treatment of PKU. (JINS, 2019, 25, 624-643).
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Disfunção Cognitiva , Função Executiva , Fenilcetonúrias , Criança , Disfunção Cognitiva/etiologia , Humanos , Fenilcetonúrias/complicações , Fenilcetonúrias/metabolismo , Fenilcetonúrias/terapiaRESUMO
Turner syndrome (TS) is a genetic disorder, affecting 1/2500 to 1/3000 live female births, induced by partial or total deletion of one X chromosome. The neurocognitive profile of girls with TS is characterized by a normal Verbal IQ and weaknesses in visual-spatial, mathematics, and social cognitive domains. Executive functions (EFs) impairments have also been reported in these young patients. However, methodological differences across studies do not allow determination of which EFs are impaired and what is the magnitude of these impairments. The aim of this review was to clarify the EF profile of children and adolescents with TS. Sixteen samples, from thirteen studies, were included in the current meta-analysis. EFs measures used in these studies were classified into working memory, inhibitory control, cognitive flexibility, or higher-order EFs tasks in accordance with Diamond's model, Annual Review of Psychology, 64, 135-168 (2013). Results confirmed that girls with TS had significant executive impairments with effect sizes varying from small (inhibitory control) to medium (cognitive flexibility) and large (working memory, higher-order EFs). Analyses by task revealed that cognitive inhibition may be more impaired than the other inhibitory control abilities. Heterogeneity across cognitive flexibility measures was also highlighted. Between-sample heterogeneity was observed for three tasks and the impact of participants' characteristics on EFs was discussed. This meta-analysis confirms the necessity to assess, in patients living with TS, each EF by combining both visual and verbal tasks. Results also underline that, when studying girls with TS' executive profile, it is important to explore the impact of moderator variables, such as IQ, parental socio-economic status, TS karyotype, psychiatric comorbidities, and hormonal treatment status.
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Função Executiva , Síndrome de Turner/psicologia , Adolescente , Criança , HumanosRESUMO
OBJECTIVES: Neurofibromatosis type 1 (NF1) is a genetic disorder in which the most frequent complication in children is learning disabilities. Over the past decade, growing arguments support the idea that executive dysfunction is a core deficit in children with NF1. However, some data remain inconsistent. The aim of this study was to determine the magnitude of impairment for each executive function (EF) and clarify the impact of methodological choices and participant's characteristics on EFs. METHODS: In this meta-analysis, 19 studies met the selection criteria and were included with data from a total of 805 children with NF1 and 667 controls. Based on the Diamond's model (2013), EF measures were coded separately according to the following EF components: working memory, inhibitory control, cognitive flexibility, planning/problem solving. The review protocol was registered with PROSPERO (International prospective register of systematic reviews; CRD42017068808). RESULTS: A significant executive dysfunction in children with NF1 is demonstrated. Subgroup analysis showed that the impairment varied as a function of the specific component of executive functioning. The effect size for working memory and planning/problem solving was moderate whereas it was small for inhibitory control and cognitive flexibility. Executive dysfunction seems to be greater with increasing age whereas assessment tool type, intellectual performance, attention deficit hyperactivity disorder and control group composition did not seem to affect EF results. CONCLUSIONS: EF deficits are a core feature in children with NF1 and an early identification of executive dysfunctions is essential to limit their impact on the quality of life. (JINS, 2018, 24, 977-994).
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Função Executiva , Neurofibromatose 2/psicologia , Criança , HumanosRESUMO
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.
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Lesões Encefálicas Traumáticas/reabilitação , Função Executiva/fisiologia , Transtornos da Memória/diagnóstico , Monitorização Fisiológica/métodos , Inquéritos e Questionários , Adolescente , Lesões Encefálicas Traumáticas/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Feminino , Humanos , Escala de Gravidade do Ferimento , Masculino , Transtornos da Memória/epidemiologia , Testes Neuropsicológicos , Pais , Análise de Regressão , Instituições AcadêmicasRESUMO
Neurofibromatosis type 1 (NF1) is a genetic disease that can lead to impaired social adaptation and functioning, thus affecting quality of life. To date, studies of these children's social cognition abilities have been scant and far from exhaustive. Therefore, the purpose of the present study was to assess the ability of children with NF1, compared with controls, to process facial expressions of emotions - not only including the usual primary emotions (happiness, anger, surprise, fear, sadness and disgust), but secondary emotions, too. To do so, the links between this ability and the characteristics of the disease (mode of transmission, visibility, and severity) were examined. A total of 38 children with NF1 aged 8-16 years 11 months (mean = 11.4, SD = 2.3) and 43 sociodemographically comparable control children performed the emotion perception and recognition tests of a social cognition battery. Results confirmed that the processing of primary and secondary emotions is impaired in children with NF1, but there were no significant links with either mode of transmission, severity, or visibility. These results encourage further comprehensive assessments of emotions in NF1, and suggest that investigations should be extended to higher level social cognition skills, such as theory of mind and moral judgments.
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Neurofibromatose 1 , Criança , Humanos , Emoções , Expressão Facial , Medo/psicologia , Neurofibromatose 1/complicações , Percepção , Qualidade de Vida , AdolescenteRESUMO
INTRODUCTION: Neurofibromatosis type 1 (NF1) is a genetic pathology that can lead to impaired social functioning that has a negative impact on patients' quality of life. To date, although the hypothesis of impaired social cognition has been proposed as a potential explanation for these difficulties, very few studies have focused on theory of mind in children with NF1. Furthermore, other complex sociocognitive abilities have never been investigated. The aim of the present study was to assess theory of mind, moral reasoning, and social information processing in children with NF1 compared with a control group. METHOD: We administered the Paediatric Evaluation of Emotions, Relationships and Socialization® to 38 children with NF1 aged between 8 years and 16 years 11 months (mean = 11.4, SD = 2.3) and 43 control children with comparable sociodemographic characteristics. RESULTS: Patients performed significantly worse than controls on moral reasoning and social information processing tests, but there was no significant difference on theory of mind. CONCLUSIONS: These results seem to confirm the presence of social cognition difficulties in NF1 that could explain, at least in part, their social difficulties, although not all dimensions are concerned. The differences between the processes we assessed are discussed in relation to the methodologies used to measure them, and raises questions about the complementarity of traditional tools and more ecological assessments.
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Neurofibromatose 1 , Cognição Social , Teoria da Mente , Humanos , Neurofibromatose 1/complicações , Neurofibromatose 1/psicologia , Criança , Masculino , Feminino , Adolescente , Teoria da Mente/fisiologia , Testes Neuropsicológicos , Princípios Morais , Transtornos Cognitivos/etiologiaRESUMO
Medical practitioners are entrusted with the pivotal task of making optimal decisions in healthcare delivery. Despite rigorous training, our confidence in reasoning can fail when faced with pressures, uncertainties, urgencies, difficulties, and occasional errors. Day-to-day decisions rely on swift, intuitive cognitive processes known as heuristic or type 1 decision-making, which, while efficient in most scenarios, harbor inherent vulnerabilities leading to systematic errors. Cognitive biases receive limited explicit discussion during our training as junior doctors in the domain of paediatric cardiology. As pediatric cardiologists, we frequently confront emergencies necessitating rapid decision-making, while contending with the pressures of stress, fatigue, an earnest interest in "doing the right thing" and the impact of parental involvement. This article aims to describe cognitive biases in pediatric cardiology, highlighting their influence on therapeutic interventions for congenital heart disease. Whether future pediatric cardiologists or experienced professionals, understanding and actively combating cognitive biases are essential components of our ongoing medical education. Furthermore, it is our responsibility to thoroughly examine our own practices in our unwavering commitment to providing high-quality care.
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BACKGROUND: The risk of neuropsychological disorders appears to be high in hyperphenylalaninemia (HPA). The hypothesis of executive function impairment is prominent in accounting for the neuropsychological phenotype in phenylketonuria (PKU) and is suspected in moderate hyperphenylalaninemia (MHP). However, the issue of early onset of executive disorders remains. The aim of this study was to explore the hypothesis of early executive dysfunction in HPA patients and the possible links with certain metabolic variables according to the new international classifications for patients with PKU and MHP. A group of 23 HPA children (12 PKU, 11 MHP) aged 3 to 5 years was included and compared to 50 control children. The two groups were comparable in terms of socio-demographics (age, sex, parental education level). Executive functions were assessed using performance-based tests and daily life questionnaires (parents and teachers). RESULTS: Preschool HPA patients have comparable executive scores to control subjects. In contrast, PKU patients score significantly worse than MHP patients on 3 executive tests (verbal working memory, visual working memory and cognitive inhibition. There is no executive complaints in daily life (parents and teachers) for the 2 groups of patients. In addition, 3 correlations were identified between executive scores and Phe levels at inclusion, mean Phe level and variability of Phe levels throughout life. CONCLUSIONS: Thus, there appears to be evidence of early executive dysfunction in PKU preschool-children, but not in MHP children. Occasionally, certain metabolic indicators can predict executive difficulties in young children with PKU.
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Função Executiva , Fenilcetonúrias , Humanos , Função Executiva/fisiologia , Estudos Prospectivos , Memória de Curto Prazo/fisiologia , FenótipoRESUMO
Background: Executive functions (EFs) are a set of neuropsychological skills permitting solving problems in a new situation by regulating action, behavior, and emotional response. As cerebral maturation remains vulnerable in preterm children, a higher risk of developing cognitive disorders including EFs exist compared to term children. Aims: The aim of this study was to estimate the incidence of preschool EF impairments through proxy reports in children born preterm before 34 weeks of gestational age using the Behavior Rating Inventory of Executive Function-Preschool (BRIEF-P) version. Secondary aims were to report neonatal, child, or socioeconomic factors associated with EF disorders. Results: Parents of 357 children born preterm aged 3-4â years old completed the BRIEF-P version. Impairment in EFs was clinically significant for 13.5% of preterm children (n = 47; 95% CI = 0.10-0.18) compared to 5.1% in term children. A low parental socioeconomic level was significantly associated with impaired parent-rated EF (19.1% vs. 5.3%, p = 0.003). Conclusions: Proxy reports of EF impairment are about twice as frequent as in term children. EF difficulties are not related to neonatal or child severity factors in contrast with the parental socioeconomic level. Using a parent-rated questionnaire may be a useful and easy tool to identify early the daily life impact of EF disorders on clinical follow-up of preterm children.This study was recorded in the Clinical Trials Register under identifier NCT03700463.
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Objectives: To perform a detailed description of executive functioning following moderate-to-severe childhood traumatic brain injury (TBI), and to study demographic and severity factors influencing outcome. Methods: A convenience sample of children/adolescents aged 7-16 years, referred to a rehabilitation department after a TBI (n = 43), was compared to normative data using a newly developed neuropsychological test battery (Child Executive Functions Battery-CEF-B) and the BRIEF. Results: Performance in the TBI group was significantly impaired in most of the CEF-B subtests, with moderate to large effect sizes. Regarding everyday life, patients were significantly impaired in most BRIEF clinical scales, either in parent or in teacher reports. Univariate correlations in the TBI group did not yield significant correlations between the CEF-B and socio-economic status, TBI severity, age at injury, or time since injury. Conclusion: Executive functioning is severely altered following moderate-to-severe childhood TBI and is best assessed using a combination of developmentally appropriate neuropsychological tests and behavioral ratings to provide a comprehensive understanding of children's executive functions.
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North African countries such as Morocco are scarcely the focus of neuropsychological studies, although the role of culture in cognition processes is widely recognized. Currently, studies on flexibility and planning skills in the Moroccan context are still lacking and there are no adapted tools to assess these functions in the country. In this scenario, this study aimed to adapt the Child Executive Functions Battery (CEF-B) tasks and the playing cards task of the Behavioral Assessment of the Dysexecutive Syndrome for Children (BADS-C) to Morocco and provide preliminary normative data on the development of flexibility and planning. In addition, this study proposed to examine the effects of gender, parents' education level and multilingualism on executive development. To this end, six tasks of the CEF-B were adapted through a translation and back-translation process and administered to 115 children aged 7-12 years. Results showed that the adopted procedure was sufficient for the cross-cultural adaptation of the tasks. Analyses showed a continuous increase with age on executive performance in most of the variables. However, gender and parents' level of education showed mostly non-significant effects. On the other hand, a significant effect of multilingualism was found on the two analyzed functions, with better results for multilingual children if compared to their bilingual peers. In general, results suggest that the identified pattern of development is consistent with international studies. Although normative data on executive functions are still lacking in Morocco, this unprecedented study will help better understand their development in this context.
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Função Executiva , Multilinguismo , Criança , Cognição , Escolaridade , Humanos , Testes NeuropsicológicosRESUMO
Studies regarding executive functions (EFs) in children rarely focus on populations of North Africa countries. In this context, this research aimed to adapt EFs tests to the Moroccan context and provide preliminary normative data on the development of inhibitory control and working memory (WM). In addition, the executive performance of school-age Moroccan children was examined, as well as the effects of gender, parents' education level and multilingualism. The sample included 115 children at age 7 to 12 from Fes city. Results showed that the translation and adaptation steps were sufficient for the cross-cultural adaptation of the tasks. In addition, an overall effect of age on inhibition and WM performances was found, whereas gender and parents' education level showed non-significant effects. Multilingualism effects had a partial positive influence on EFs, with improved scores on a few executive tasks for multilingual children compared to their bilingual peers. Overall, results suggest that EFs in school-age Moroccan children operate on relatively homogeneous development trajectories, marked by improvements that differ according to tasks. As neuropsychological tools and normative data are still lacking in Morocco, this study helps better understand EFs development of children in this country.
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Memória de Curto Prazo , Multilinguismo , Criança , Função Executiva/fisiologia , Humanos , Inibição Psicológica , Instituições AcadêmicasRESUMO
The goal of this review is to perform a critical analysis of the instruments used to assess executive functions (EFs) in preschool and school-age children in Brazil. We identified 37 assessment measures through a systematic review. Some performance-based tests for assessing working memory and inhibition were identified. However, there is a lack of rating measures and instruments to assess flexibility and planning in clinical practice. We observed regional differences in the performance of EFs measures. One possible explanation may be the use of normative samples from more highly-developed regions to characterize performance in less-developed regions. However, there may be alternative explanations, such as variations in the exposure to testing and the adequacy of test materials in different regions. Joint efforts among research groups should be encouraged in order to obtain normative references that are more representative of the socio-cultural diversity of the country. This improvement is essential to better understand the typical and atypical development of EFs and how the peculiarities of each country's context and culture can impact its trajectory.
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Função Executiva , Memória de Curto Prazo , Brasil , Criança , Pré-Escolar , Função Executiva/fisiologia , Humanos , Inibição Psicológica , Instituições AcadêmicasRESUMO
Background: Mitochondrial disorders (MD) are metabolic diseases related to genetic mutations in mitochondrial DNA and nuclear DNA that cause dysfunction of the mitochondrial respiratory chain. Cognitive impairment and psychiatric symptoms are frequently associated with MD in the adult population. The aim of this study is to describe the neuropsychological profile in children and adolescents with MD. Methods: We prospectively recruited a sample of 12 children and adolescents between February 2019 and February 2020 in the Reference Center for Mitochondrial Disorders of Angers (France). Participants and their parents completed an anamnestic form describing socio-demographic data and completed the WISC-V (Wechsler Intelligence Scale for Children, 5th edition) and the Parent and Teacher forms of the BRIEF (Behavior Rating Inventory of Executive Function). Results: In our sample, the mean IQ (Intellectual Quotient) score was 87.3 ± 25.3. The score ranged from 52 to 120. Concerning executive functions, a significant global clinical complaint was found for parents (six times more than normal) and to a lesser extent, for teachers (among 3 to 4 times more). Levels of intelligence and executive functioning were globally linked in our cohort but dissociation remains a possibility. Conclusion: The results of this study show that MD can be associated to neuropsychological disorders in children and adolescents, especially regarding the intelligence quotient and the executive function. Our study also highlights the need for regular neuropsychological assessments in individuals with MD and developing brains, such as children and adolescents.