M. Chevignard

Quality-of-life, mood and executive functioning after childhood craniopharyngioma treated with surgery and proton beam therapy.

Primary objective: Childhood craniopharyngioma, a benign tumour with a good survival rate, is associated with important neurocognitive and psychological morbidity, reducing quality‐of‐life (QoL). Method: This retrospective study analysed QoL, mood disorders, everyday executive functioning and diseases impact on family life in 29 patients (mean age at diagnosis 7 years 10 months (SD = 4.1); mean follow‐up period 6 years 2 months (SD = 4.5)) treated for childhood craniopharyngioma by surgery combined with radiotherapy using proton beam. Assessment included a semi‐structured interview and standardized scales evaluating self‐report of QoL (Kidscreen 52) and depression (MDI‐C) and proxy‐reports of QoL (Kidscreen 52), executive functioning (BRIEF) and diseases impact (Hoare and Russel Questionnaire). Results: Twenty‐three families answered the questionnaires completely. Overall QoL self‐report was within the normal range. QoL proxy‐report was lower than self‐report. Eleven patients reported depression; 24–38% had dysexecutive symptoms. A majority of families felt ‘very concerned’ by the disease. Depression and low parental educational level were associated with lower QoL and higher levels of executive dysfunction. Conclusion: Given the high morbidity of childhood craniopharyngioma, screening for psychosocial outcome, cognitive functioning, including executive functions, mood and QoL should be systematic and specific interventions should be developed and implemented.

A comprehensive model of care for rehabilitation of children with acquired brain injuries

BACKGROUND Acquired brain injury (ABI) is a leading cause of death and lifelong acquired disability in children and remains a significant public health issue. Deficits may only become fully apparent when developmental demands increase and once cognitive processes are expected to be fully developed. It is therefore necessary to provide organized long-term follow-up for children post ABI. Despite these recommendations, it has been shown that only a small proportion of children received specialized rehabilitation and adequate follow-up after ABI. AIMS The aims are: (i) to describe a comprehensive model of care devoted to children with acquired brain injuries; and (ii) to provide descriptive data analysing the characteristics of children followed up, the type/amount of services provided and general outcomes. PROGRAMME DESCRIPTION The programme features an in- and outpatient rehabilitation facility, where multidisciplinary rehabilitation and specialized schooling are provided. The ultimate goal of the programme is to promote each childs successful reintegration in school and in the community. Adequate preparation of discharge is essential, long-term follow-up is organized, and an outreach programme has been developed to deal with the complex delayed psychosocial issues. RESULTS Overall outcome, as measured by the Glasgow Outcome Scale, improved dramatically between admission (3.3; SD = 0.45) and discharge (2.15; SD = 0.74). Most of the children were discharged home with an adequate personalized plan for ongoing rehabilitation and school adaptations. Analysis of the outreach programme underlines the more challenging issues arising in late adolescence-early adulthood. CONCLUSION Given the specificities of childhood ABI, long-term specific care must be organized and co-ordinated, regardless of injury severity.

Early electro-clinical features may contribute to diagnosis of the anti-NMDA receptor encephalitis in children

OBJECTIVE To describe initial and follow-up electroencephalographic (EEG) characteristics in anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. METHODS Consecutive polygraphic video-EEG recordings were analyzed in nine pediatric patients with anti-NMDAR encephalitis at the initial stage of the disease and during the intermediate period until motor recovery. EEG characteristics in waking and sleep stages as well as EEG correlates of abnormal movements are described. RESULTS In six patients, [corrected] the waking EEG showed preserved background activity and either focal or unilateral hemispheric slowing. These children had more favorable outcome than the three children with diffuse slowing. Unilateral [corrected] abnormal movements contra-lateral to hemispheric or focal slowing were also indicative of milder severity when compared to generalized abnormal movements and diffuse slowing. During non-rapid eye movement (NREM) sleep, a decrease in the expected slow waves and unilateral or diffuse theta-alpha band rhythms were observed in six children, not correlated with the outcome, representing a suggestive EEG pattern of anti-NMDAR encephalitis. [corrected]. CONCLUSIONS In pediatric patients presenting behavioral disorders and abnormal movements, early EEG patterns may be suggestive of anti-NMDAR encephalitis. Moreover early electro-clinical presentation contributes to outcome prediction. SIGNIFICANCE This case series demonstrates that early EEG patterns may be suggestive of anti-NMDAR encephalitis in pediatric patients with behavioral disorders and abnormal movements.

Spatial and motor neglect in children

Objective: To determine if unilateral spatial neglect can be found in very young children with acquired brain lesions. Methods: A retrospective study was carried out over a 10-year period in a pediatric rehabilitation department specializing in acquired brain lesions. Twelve cases were selected involving children aged 7 months to 14 years. Neglect was assessed by behavior observation for all children and, depending on age, by drawing, copying, reading, writing, and arithmetic tasks. Seven patients underwent the experimental “Teddy Bear” cancellation task, specially adapted to children. Results: All children exhibited spatial and motor neglect in daily activities. In addition, children who were over 2 years of age made lateralized omissions in paper and pencil tasks as well as in the “Teddy Bear” cancellation task. Nine of the 12 children exhibited left spatial and motor neglect, whereas the other three, all of whom were very young (under 4 years), exhibited right spatial neglect. In three cases, cortical lesions were restricted to one lobe, either the parietal, frontal, or temporal. In eight children, lesions extended over more than one lobe, and one child sustained only subcortical lesions. Conclusion: Unilateral spatial and motor neglect occurs even in very young children. Right spatial neglect described in very young children may be related to the development of hemispheric specialization.

Goal Attainment Scaling in rehabilitation: A literature-based update

Goal Attainment Scaling (GAS) is a method for quantifying progress on personal goals. Turner-Stokess guide to GAS is a method for quantifying progress towards personal goals. Turner-Stokess guide and the use of Kiresuks T-score are the most widely used GAS-based approaches in rehabilitation. However, the literature describes a number of other approaches and emphasizes the need for caution when using the T-score. This article presents the literature debates on GAS, variations of GAS (in terms of the score level assigned to the patients initial status and description of the scales different levels), the precautions to be taken to produce valid GAS scales and the various ways of analyzing GAS results. Our objective is to (i) provide clinical teams with a critical view of GAS (the application of which is not limited to a single research groups practices) and (ii) present the most useful resources and guidelines on writing GAS scales. According to the literature, it appears to be preferable to set the patients initial level to -2 (even when worsening is a possible outcome) and to describe all five GAS levels in detail. The use of medians and rank tests appears to be appropriate, given the ordinal nature of GAS.

Goal Management Training for rehabilitation of executive functions: a systematic review of effectivness in patients with acquired brain injury

Abstract 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. Implications for Rehabilitation Goal Management Training (GMT) is a well-studied metacognitive intervention for executive dysfunction. Comprehensive rehabilitation programs relying partly on GMT and integrating other approaches are effective in executive function rehabilitation following brain injury in adults. GMT alone does not have sufficient evidence to support it as a training on its own.

Assessment of executive functioning in children after TBI with a naturalistic open-ended task: A pilot study

Purpose: Traumatic brain injury (TBI) often leads to executive functions (EF) deficits, resulting in severe longstanding disabilities in daily life activities. The sensitivity and ecological validity of neuropsychological tests have been questioned. The aim of this study was to pilot a novel open-ended naturalistic task and to compare it to other standardized assessments of EF in children post-TBI. Methods: Ten children aged 8–14, with moderate-to-severe TBI, and 18 matched controls participated in the study. The clinical group was assessed using cognitive tests and parent-based questionnaires of EF. An interactive ecological cooking task was designed. Results: Analyses indicated mild-to-moderate executive deficits in the cognitive tests in approximately half of the TBI group. For the experimental cooking task, all quantitative and qualitative variables were significantly impaired for the TBI group compared to the control group and failure in the cooking task was associated with lower scores in cognitive tests of EF. The task was able to discriminate the TBI children from the control group. Conclusion: This pilot study highlights the role of naturalistic assessments, to complement standardized tests in assessing patients’ dysexecutive impairments in complex activities of daily living post-childhood TBI.

Ecological assessment of cognitive functions in children with acquired brain injury: A systematic review

Background: Childhood acquired brain injury (ABI) often leads to impairment in cognitive functioning, resulting in disabilities in both the home and school environment. Assessing the impact of these cognitive deficits in everyday life using traditional neuropsychological tests has been challenging. This study systematically reviewed ecological measures of cognitive abilities available for children with ABI. Method: Eight databases were searched (until October 2011) for scales: (1) focused on ecological assessment of cognitive functioning; (2) with published data in an ABI population; (3) applicable to children up to 17;11 years of age; and (4) in English. The title and abstract of all papers were reviewed independently by two reviewers. Results: Database searches yielded a total of 12 504 references, of which 17 scales met the inclusion criteria for the review, focusing on executive functions (n = 9), memory (n = 3), general cognitive abilities (n = 2), visuo-spatial skills (n = 2) and attention (n = 1). Four tasks used observation of actual performance in a natural environment, five were proxy-reports and six were functional paper and pencil type tasks, performed in an office. Conclusion: Overall, few measures were found; eight were still experimental tasks which did not provide norms. Executive functions were better represented in ecological assessment, with relatively more standardized scales available.

Context-sensitive goal management training for everyday executive dysfunction in children after severe traumatic brain injury

Objectives:To assess the effectiveness of a metacognitive training intervention, based on an adapted Goal Management Training and Ylvisakers 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 Childrens Cooking Task. Transfer to the childs 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 Childrens 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 childs “everyday people” in the intervention.

Long-term outcome of the shaken baby syndrome and medicolegal consequences: A case report

INTRODUCTION Studies of long-term outcome of the shaken baby syndrome (SBS) are scarce, but they usually indicate poor outcome. OBJECTIVES To describe long-term outcome of a child having sustained a SBS, to ascertain possible delayed sequelae and to discuss medicolegal issues. METHODS We report a single case study of a child having sustained a SBS, illustrating the initial clinical features, the neurological, cognitive and behavioural outcomes as well as her social integration. RESULTS The child sustained diffuse brain injuries, responsible for spastic right hemiplegia leading to secondary orthopaedic consequences, as well as severe cognitive impairment, worsening over time: the developmental quotient measured at 15 months of age was 55 and worsened as age increased. At 6 years and 8 months, the childs IQ had fallen to 40. Behavioural disorders became apparent only after several months and precluded any social integration. The child eventually had to be placed in a specialised education centre at age 5. DISCUSSION AND CONCLUSION The SBS has a very poor outcome and major long-standing sequelae are frequent. Cognitive or behavioural sequelae can become apparent only after a long sign-free interval, due to increasing demands placed on the child during development. This case report confirms severity of early brain lesions and necessity for an extended follow-up by a multi-disciplinary team. From a medicolegal point of view, signaling the child to legal authorities allows protection of the child, but also conditions later compensation if sequelae compromise autonomy.