Medulloblastoma patients treated at the Institute Curie between 1980 and 2000 were reviewed. Only patients whose primary treatment included craniospinal radiation were considered. Surviving patients ...were identified and evaluated by means of self-report questionnaires using the Health Utility Index (HUI). Psychosocial functioning, employment, and other health-related indicators were recorded. Seventy-three patients were treated during the study period. At a median follow-up from diagnosis of 14.4 years, 49 patients were alive and 45 surviving patients could be contacted. Late sequelae were frequent, particularly neurological deficits (71%) and endocrine complications (52%). Impairments of psychosocial functioning, including employment, driving capacity, independent living, and marital status, were identified in most patients. Most long-term medulloblastoma survivors suffer persistent deficits in several domains, with a significant impact on their psychosocial functioning. These findings reinforce the importance of early intervention programs for all survivors in order to reduce the psychosocial impacts of their disease.
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 child's 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.
The highest incidence rate of childhood brain tumours is in children below the age of five years, who are particularly vulnerable to the effects of treatments. The assessment of quality of survival ...(QoS) in multiple domains is essential to compare the outcomes for different tumour types and treatment regimens.
The aim of this position statement is to present the domains of health and functioning to be assessed in children from birth to five years, to advance the collection of a common QoS data set in European brain tumour trials.
The QoS group of the European Society of Paediatric Oncology (SIOP-E) Brain Tumour group conducted consensus discussions over a period of six years to establish domains of QoS that should be prioritised in clinical trials involving children under 5 years.
The domains of health and functioning that were agreed to affect QoS included: medical outcomes (e.g. vision, hearing, mobility, endocrine), emotion, behaviour, adaptive behaviour, and cognitive functioning.
As for children aged five years and older, a ‘core plus’ approach is suggested in which core assessments are recommended for all clinical trials. The core component for children from birth to three years includes indirect assessment which, in this age-group, requires proxy assessment by a parent, of cognitive, emotional and behaviour variables and both direct and indirect endocrine measures. For children from four years of age direct cognitive assessment is also recommended as ‘core’. The ‘plus’ components enable the addition of assessments which can be selected by individual countries and/or by, age-, treatment-, tumour type- and tumour location-specific trials.
•We consider the range of functioning affected by children under 5 years of age.•We present domains of function with measures in use across much of Europe.•We present core areas of function that should be measured in all clinical trials.•We discuss additional areas of function that can be identified for specific trials.
Un déficit des fonctions exécutives est fréquent dans le vieillissement normal. Des lésions cérébrales affectant les fonctions exécutives pourraient donc avoir des répercussions cliniques plus ...sévères chez des patients plus âgés. Notre objectif était d’étudier l’effet de l’âge sur l’évaluation neuropsychologique et sur le bilan écologique des fonctions exécutives dans une population d’adultes cérébrolésés.
Nous avons analysé l’effet de l’âge sur les résultats de l’évaluation classique des fonctions exécutives et sur les résultats d’une tâche écologique complexe réalisée en situation de cuisine, sensible aux troubles des fonctions exécutives, chez 45 patients cérébrolésés présentant un syndrome dysexécutif, âgés de 17 à 63 ans.
Une dégradation significative avec l’âge des performances a été notée dans plusieurs tests papier-crayon évaluant les fonctions exécutives, ainsi que dans l’exécution de la tâche écologique (nombre d’erreurs et comportements dangereux).
Cette étude montre un effet cumulé de l’âge et des lésions cérébrales sur l’importance des difficultés d’adaptation comportementale, retentissant sur les activités de vie quotidienne, et ce même dans une population d’adultes encore jeunes. Ces résultats sont à prendre en compte lors de la prise en charge de ces patients.
Executive functioning deficits have often been described in normal aging. They are also known to be a frequent sequel of traumatic brain injury, where patients may exhibit severe long-standing impairments in instrumental activities of daily living. One could therefore expect that cerebral lesions affecting executive functioning would result in more severe impairments in older patients. We previously developed an ecological assessment of executive functions, consisting of a cooking task, requiring multitasking abilities and known to be sensitive to a dysexecutive syndrome Cortex 36 (2000) 649–669. The aim of this study was to analyze the effect of age on the cognitive and ecological assessments of executive functions in a group of patients with acquired brain injury (ABI) resulting in a dysexecutive syndrome. Studies in this area usually focus on patients older than 60 or 65, but we chose to analyze the effect of age in a younger population. We hypothesized that older patients would have poorer performances on the cognitive and ecological tests of executive functioning, when compared to younger patients.
Forty-five patients with ABI resulting in frontal lesions and a dysexecutive syndrome participated in this study. Patients underwent a comprehensive battery of cognitive tests assessing executive functioning, as well as the cooking task. We also studied a group of 12 control subjects who performed the cooking task.
No effect of age was found on performance in the cooking task in the control group. Age was not related to demographic parameters or injury severity in the ABI group. Although the ABI group was relatively young (mean age: 40.3 years (S.D.=12.5), ranging from 17 to 63), results indicated a significant deleterious effect of age on the cognitive tests of executive functioning in the ABI group. We also highlighted a significant worsening of patients’ performance in the cooking task with age, and this effect was found on several variables of task analysis: the number of errors and occurrence of dangerous behaviors.
Our study demonstrates the deleterious effect of aging on cognitive and ecological assessment of executive functioning after ABI. The strength of this study is that it deviated from the traditional age considered in studies of elderly populations and focused on younger patients. It is therefore important to consider the implication that this may have on a patient's rehabilitation program and postinjury discharge.
Children who have been treated for a medulloblastoma often suffer long-term cognitive impairments that often negatively affect their academic performance and quality of life. In this article, we will ...review the neuropsychological consequences of childhood medulloblastoma and discuss the risk factors known to influence the presence and severity of these cognitive impairments and possible interventions to improve their quality of life.
This narrative review was based on electronic searches of PubMed to identify all relevant studies.
Although many types of cognitive impairments often emerge during a child's subsequent development, the core cognitive domains that are most often affected in children treated for a medulloblastoma are processing speed, attention and working memory. The emergence and magnitude of these deficits varies greatly among patients. They are influenced by demographic (age at diagnosis, parental education), medical and treatment-related factors (perioperative complications, including posterior fossa syndrome, radiation therapy dose, etc.), and the quality of interventions such as school adaptations provided to the child or rehabilitation programs that focus on cognitive skills, behavior and psychosocial functioning.
These patients require specialized and coordinated multidisciplinary rehabilitation follow-up that provides timely and adapted assessments and culminates in personalized intervention goals being set with the patient and the family. Follow-up should be continued until referral to adult services.
Objective: The first aim of this study was to estimate the prevalence of TBI and epilepsy in a French prison population and to study variables known to be associated with TBI. The second aim was to ...compare prisoners with and without a history of TBI.
Participants: All offenders (females, males and juveniles) admitted consecutively to Fleury-Mérogis prison over a period of 3 months were included in the study.
Design: During the admission procedure, offenders were interviewed by healthcare staff using a self-reported questionnaire.
Results: In all, 1221 prisoners were included. The rates of TBI and epilepsy were high, with a prevalence of 30.6% and 5.9%, respectively. Psychiatric care, anxiolytic and antidepressant treatment, use of alcohol and cannabis were all significantly higher among offenders with a history of TBI. Moreover, the number of times in custody and the total time spent in jail over the preceding 5 years were significantly higher among offenders with a history of TBI.
Conclusions: These results provide further evidence that specific measures need to be developed such as, first of all, screening for TBI upon arrival in prison.
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 disease's 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 disease's 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.
Patients with a dysexecutive syndrome often have severe disabilities in daily life activities. The aims of this study were to use a naturalistic experimental task to assess patients' disabilities, ...and to study the nature of the cognitive disorders underlying them. Execution of a cooking task involving multi-tasking (Chevignard et al.,
2000
) was studied in 45 patients with a dysexecutive syndrome following acquired brain injury. Patients made significantly more errors and were slower than controls; more than half of the patients did not achieve the goal and demonstrated dangerous behaviours. Those results were significantly correlated to the results of the Six Elements Task and to a behavioural questionnaire. They were also correlated to brain injury severity and to patients' cooking habits.
This naturalistic assessment is clinically relevant to better assess patients' dysexecutive impairments in complex activities of daily living. Correlations of the results in the cooking task with the neuropsychological assessment highlighted the role of the dysexecutive syndrome in patients' disabilities, indicating control alterations rather than planning disorders, difficulty in dealing with the environment, and inhibiting inappropriate actions. The role of attention and prospective memory was also underlined, whereas other cognitive functions did not influence task performance.
It is increasingly accepted that survival alone is an inadequate measure of the success of childhood brain tumour treatments. Consequently, there is growing emphasis on capturing quality of survival. ...Ependymomas are the third most frequently occurring brain tumours in childhood and present significant clinical challenges. European Society of Paediatric Oncology Ependymoma II is a comprehensive international program aiming to evaluate outcomes under different treatment regimens and improve diagnostic accuracy. Importantly, there has been agreement to lower the age at which children with posterior fossa ependymoma undergo focal irradiation from three years to either eighteen months or one year of age. Hitherto radiotherapy in Europe had been reserved for children over three years due to concerns over adverse cognitive outcomes following irradiation of the developing brain. There is therefore a duty of care to include longitudinal cognitive follow-up and this has been agreed as an essential trial outcome. Discussions between representatives of 18 participating European countries over 10 years have yielded European consensus for an internationally accepted test battery for follow-up of childhood ependymoma survivors. The ‘Core-Plus’ model incorporates a two-tier approach to assessment by specifying core tests to establish a minimum dataset where resources are limited, whilst maintaining scope for comprehensive assessment where feasible. The challenges leading to the development of the Core-Plus model are presented alongside learning from the initial stages of the trial. We propose that this model could provide a solution for future international trials addressing both childhood brain tumours and other conditions associated with cognitive morbidity.
•Quality of survival is an important consideration in childhood brain tumour trials.•We present European consensus for a cognitive follow-up model in a clinical trial.•The Core-Plus battery incorporates a minimum dataset and comprehensive assessment.•Challenges encountered during development and implementation are described.
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