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Background: Prader-Willi syndrome (PWS) is a neurodevelopmental genetic disorder involving executive deficits notably with planning. The main objective of the study is to assess the effectiveness of cognitive training on daily life planning difficulties in PWS patients. Methods/design: The study is a double-blind randomized controlled trial which will compare the effectiveness of a metacognitive strategy intervention designed to improve planning difficulties for PWS patients to usual occupational therapy. Sixty adults will be included over 20 months. The main outcome measure will be the performance on the Modified Six Elements Test from the BADS; secondary outcome measures will be computerized executive tasks and questionnaires. Daily life planning difficulties will be identified and transformed into measurable goals using Goal Attainment Scaling. Discussion: The project will provide knowledge on the difficulties experienced by PWS patients, in relation to their executive functioning in order to implement effective intervention for planning in daily life.

Background Executive function (EF) impairment is a major predictor of overall outcome after traumatic brain injury (TBI). TBI severity is a factor of poor outcome, but most studies include a majority of children with mild and moderate TBI. The aims of this study were to estimate EF impairment after severe childhood TBI and to explore factors predicting EF outcome. The secondary aim was to compare recovery trajectories by age-at-injury groups. Methods This was a prospective longitudinal study of children with severe TBI who were tested for EFs by performance-based tests and questionnaires at 3, 12 and 24 months. Results Children with TBI (n = 65) showed significant impairment in working memory, inhibition, attention and global EF, with little or no recovery at 24 months. For flexibility and performance-based EF score, children were impaired at 3 months only and showed normal scores by 12 months. No impairment was found in planning. At 3 and 24 months, Glasgow Coma Scale score and parental education predicted global EF. Coma length was not a significant predictor of outcome. Age at injury predicted progress in EF, but the relationship was not linear; children 10–12 years old at injury showed better outcome than older and younger children. Conclusions EFs are impaired after severe TBI in childhood. The relationship between age at injury and outcome is not linear. Relying on only performance-based EF tests can underestimate EF impairment.

To compare three ways of assessing self-awareness in children with traumatic brain injury (TBI) and to propose a model of child anosognosia.Five single cases of children with severe TBI, aged 8-14, undergoing metacognitive training. Awareness was assessed using three different measures: two measures of metacognitive knowledge/intellectual awareness (a questionnaire and illustrated stories where child characters have everyday problems related to their executive dysfunction) and one measure of on-line/emergent awareness (post-task appraisal of task difficulty).All three measures showed good feasibility. Analysis of awareness deficit scores indicated large variability (1-100%). Three children showed dissociated scores.Based on these results, we propose a model of child self-awareness and anosognosia and a framework for awareness assessment for rehabilitation purposes. The model emphasizes (1) the role of on-line error detection in the construction of autobiographical memories that allow a child to build a self-knowledge of his/her strengths and difficulties; (2) the multiple components of awareness that need to be assessed separately; (3) the implications for rehabilitation: errorless versus error-based learning, rehabilitation approaches based on metacognition, rationale for rehabilitation intervention based on child's age and impaired awareness component, ethical and developmental consideration of confrontational methods.Self-awareness has multiple components that need to be assessed separately, to better adapt cognitive rehabilitation. Using questionnaires and discrepancy scores are not sufficient to assess awareness, because it does not include on-line error detection, which can be massively impaired in children, especially those with impaired executive functions. On-line error detection is important to promote and error-based learning is useful to allow a child to build a self-knowledge of his/her strengths and difficulties, in the absence of severe episodic memory problems. Metacognitive trainings may not be appropriate for younger children who have age appropriate developmentally immature self-awareness, nor for patients with brain injury if they suffer anosognosia because of their brain injury.

International audience; Purpose: To determine if Goal Management Training (GMT) is effective for the rehabilitation of executive functions following brain injury when administered alone or in combination with other interventions.Method: Systematic review, with quality appraisal specific to executive functions research and calculation of effect sizes.Results: Twelve studies were included. Four studies were “Proof-of-principle” studies, testing the potential effectiveness of GMT and eight were rehabilitation studies. Effectiveness was greater when GMT was combined with other interventions. The most effective interventions appeared to be those combing GMT with: Problem Solving Therapy; personal goal setting; external cueing or prompting apply GMT to the current task; personal homework to increase patients’ commitment and training intensity; ecological and daily life training activities rather than paper-and-pencil, office-type tasks. Level of support for GMT was higher for studies measuring outcome in terms of increases in participation in everyday activities rather than on measures of executive impairment.Conclusion: Comprehensive rehabilitation programs incorporating GMT, but integrating other approaches, are effective in executive function rehabilitation following brain injury in adults. There is insufficient evidence to support use of GMT as a stand-alone intervention.

International audience; Objectives: To assess the effectiveness of a metacognitive training intervention, based on an adapted Goal Management Training and Ylvisaker's principles, on 3 activity domains of executive functions: (1) prospective memory (PM) performance in ecological setting, (2) complex cooking task management, and (3) daily executive functioning (EF) at home and at school.Participants: Five children aged 8 to 14 years, who were 3 to 11 years post–severe traumatic brain injury, experiencing severe EF difficulties in daily life.Design: Single-case experimental design and assessment of EF twice prior to intervention, postintervention, and 3 and 6 months postintervention. Progress was monitored by a weekly ecological PM score. The effect on EF was assessed using the Children's Cooking Task. Transfer to the child's natural context was assessed by parental and teacher questionnaires and Goal Attainment Scaling.Results: All children improved both on the measure of PM and on questionnaires of daily EF. Two children improved on the Children's Cooking Task but returned to their preintervention level in a novel cooking task at follow-up. Participation of school personnel and parents in the program was low.Conclusions: It is feasible but challenging to use Goal Management Training in children with traumatic brain injury. Further research is needed in relation to how to promote generalization and how to increase the involvement of the child's “everyday people” in the intervention.

Introduction Cognitive and behavioural aspects of executive functioning (EF) are frequently impaired following childhood TBI. The Behavior Rating Inventory of Executive Function (BRIEF) questionnaire provides an ecological assessment of EFs in everyday life in home and school environments. The aims of this study were to describe the dysexecutive disorders in children with TBI using the BRIEF; to compare parent- and teacher-ratings and to analyse the demographic and medical variables influencing outcome. Methods Participants: Children/adolescents aged 5–17 years 11 months, referred to a paediatric rehabilitation department following TBI. Outcome measures: the parent–and the teacher-report of the BRIEF were collected during neuropsychological assessment (2009–2014), as well as the teacher-report (from 2014). Age at injury and assessment, parental education and TBI severity were collected. Results 194 patients (142 boys) participated in the study [mild (n = 13), moderate (n = 12) or severe (n = 169: mean duration of coma 7.2 days; SD = 6.5)]. 193 parent-reports and 28 complete teacher reports of the BRIEF were available. Mean age at injury/assessment were 6.9 (SD = 4.4), and 11.8 (SD = 3.5) years respectively. According to parent-ratings, children had significantly elevated scores in all BRIEF indices [Global Executive Composite (GEC), Behaviour Regulation Index (BRI), Metacognition Index (MI)], and subscales (mean T-scores 61–64; all P