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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.

Single-case experimental designs (SCED) are experimental designs aiming at testing the effect of an intervention using a small number of patients (typically one to three), using repeated measurements, sequential (±randomized) introduction of an intervention and method-specific data analysis, including visual analysis and specific statistics. The aim of this paper is to familiarise professionals working in different fields of rehabilitation with SCEDs and provide practical advice on how to design and implement a SCED in clinical rehabilitation practice. Research questions suitable for SCEDs and the different types of SCEDs (e.g., alternating treatment designs, introduction/withdrawal designs and multiple baseline designs) are reviewed. Practical steps in preparing a SCED design are outlined. Examples from different rehabilitation domains are provided throughout the paper. Challenging issues such as the choice of the repeated measure, assessment of generalisation, randomization, procedural fidelity, replication and generalizability of findings are discussed. Simple rules and resources for data analysis are presented. The utility of SCEDs in physical and rehabilitation medicine (PRM) are discussed.

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.

International audience; BackgroundGoal Attainment Scaling (GAS) is a method for writing personalized evaluation scales to quantify progress toward defined goals. It is useful in rehabilitation but is hampered by the experience required to adequately “predict” the possible outcomes relating to a particular goal before treatment and the time needed to describe all 5 levels of the scale. Here we aimed to investigate the feasibility of using GAS in a clinical setting of a pediatric spasticity clinic with a shorter method, the “3-milestones” GAS (goal setting with 3 levels and goal rating with the classical 5 levels). Secondary aims were to (1) analyze the types of goals children's therapists set for botulinum toxin treatment and (2) compare the score distribution (and therefore the ability to predict outcome) by goal type.MethodsTherapists were trained in GAS writing and prepared GAS scales in the regional spasticity-management clinic they attended with their patients and families. The study included all GAS scales written during a 2-year period. GAS score distribution across the 5 GAS levels was examined to assess whether the therapist could reliably predict outcome and whether the 3-milestones GAS yielded similar distributions as the original GAS method.ResultsIn total, 541 GAS scales were written and showed the expected score distribution. Most scales (55%) referred to movement quality goals and fewer (29%) to family goals and activity domains.ConclusionThe 3-milestones GAS method was feasible within the time constraints of the spasticity clinic and could be used by local therapists in cooperation with the hospital team.

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. Children aged 5 to 18 years 11 months, referred to a rehabilitation department following TBI. Parent- and teacher reports of the BRIEF. 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. This study highlights significant executive dysfunction in everyday life several years after childhood TBI, evident in home and school environments.

International audience; Objective: To investigate the long-term outcome in prospective memory (PM), seven years after childhood severe traumatic brain injury (TBI), in a prospective longitudinal cohort. Participants: 76 young individuals (aged 7–22 years): 39 patients with a severe accidental TBI included prospectively seven years earlier, aged 0–15 years at injury, and 37 controls individually matched on age, gender and parental education. Main outcome measures: Three novel short PM tasks varying in the delay, motivation and context (ecological versus paper and pencil task). Results: Individuals with severe TBI showed significantly poorer PM than matched controls in the two low-motivation PM tasks: (1) the ecological long-delay task consisting of sending a letter on a rainy day (p=0.047, odds ratio = 2.6); (2) the non-ecological short-delay task consisting of taking off post-its while identifying facial emotions (p=0.004, r=0.34). Differences in PM on the high motivation were not significant. PM is impaired several years post severe TBI.

International audience; Goal Attainment Scaling (GAS) is a method for writing personalized evaluation scales in order to quantify progress toward defined rehabilitation goals. In published literature, GAS methodology is used with different levels of rigor, ranging from precisely written GAS scales, that ensure minimal bias, explicitly describing five levels of goal attainment; to subjective rating of goal attainment by adjectives such as “worse/better than expected”, which are transformed into a T-score, wrongly giving the reader the impression of a truly standardized, interval scale. A drawback of GAS methodology is that it is highly dependent on the ability of the GAS setting team/person to generate valid, reliable and meaningful scales, therefore reliability and validity of GAS scales are idiosyncratic to each study. The aims of this article were to: (1) increase awareness of potential sources of bias in GAS processes; (2) propose Goal Attainment Scaling quality appraisal criteria, allowing judgment of the quality of GAS methodology in individual rehabilitation studies; and (3) propose directions to improve GAS implementation in order to increase its reliability and validity as a research measurement tool. Our proposed quality appraisal criteria are based on critical appraisal of GAS literature, and published GAS validity studies that have demonstrated that precision, validity and reliability can be obtained when using GAS as an outcome measure in clinical trials. We recommend that authors using GAS report accurately how GAS methodology was used based on these criteria.

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.