Intellectual, educational, and situation-based social outcome in adult survivors of childhood medulloblastoma.

PURPOSE: To investigate intellectual and situation-based social outcome and educational achievement in adult survivors of childhood medulloblastoma and analyse factors influencing outcome Methods: We collected demographic, medical and cognitive data, and social and educational outcome at a mean time since the end of treatments of 14.9 years in 58 adults, aged 19-35 years, consecutively treated in a single cancer center between 1989 and 2005. RESULTS: Ten survivors had severe intellectual disability, 12 were still studying, 23 had a regular employment and 13 were unemployed. Full Scale Intellectual Quotient, assessed 6.6 years after the end of treatments, ranged from 46 to 131. It was strongly associated with educational achievement and significantly lower in patients who experienced postoperative cerebellar mutism, and when parental education level was low. CONCLUSION: These factors should be systematically considered at diagnosis in order to offer adequate and timely assessments and interventions.

Cognitive Deficits Underlying Error Behavior on a Naturalistic Task after Severe Traumatic Brain Injury.

People with severe traumatic brain injury (TBI) often make errors on everyday tasks that compromise their safety and independence. Such errors potentially arise from the breakdown or failure of multiple cognitive processes. This study aimed to investigate cognitive deficits underlying error behavior on a home-based version of the Cooking Task (HBCT) following TBI. Participants included 45 adults (9 females, 36 males) with severe TBI aged 18-64 years (M = 37.91, SD = 13.43). Participants were administered the HBCT in their home kitchens, with audiovisual recordings taken to enable scoring of total errors and error subtypes (Omissions, Additions, Estimations, Substitutions, Commentary/Questions, Dangerous Behavior, Goal Achievement). Participants also completed a battery of neuropsychological tests, including the Trail Making Test, Hopkins Verbal Learning Test-Revised, Digit Span, Zoo Map test, Modified Stroop Test, and Hayling Sentence Completion Test. After controlling for cooking experience, greater Omissions and Estimation errors, lack of goal achievement, and longer completion time were significantly associated with poorer attention, memory, and executive functioning. These findings indicate that errors on naturalistic tasks arise from deficits in multiple cognitive domains. Assessment of error behavior in a real life setting provides insight into individuals' functional abilities which can guide rehabilitation planning and lifestyle support.

Quality of survival assessment in European childhood brain tumour trials, for children aged 5 years and over.

INTRODUCTION: There is increasing recognition of the long-term sequelae of brain tumours treated in childhood. Five year survival rates now exceed 75% and assessing the quality of survival (QoS) in multiple domains is essential to any comparison of the benefits and harms of treatment regimens. AIM: The aim of this position statement is to rationalise assessments and facilitate collection of a common data set across Europe. Sufficient numbers of observations can then be made to enable reliable comparisons between outcomes following different tumour types and treatments. METHODS: This paper represents the consensus view of the QoS working group of the Brain Tumour group of the European Society of Paediatric Oncology regarding domains of QoS to prioritise for assessment in clinical trials. This consensus between clinicians and researchers across Europe has been arrived at by discussion and collaboration over the last eight years. RESULTS: Areas of assessment discussed include core medical domains (e.g. vision, hearing, mobility, endocrine), emotion, behaviour, adaptive behaviour and cognitive functioning. CONCLUSIONS: A 'core plus' approach is suggested in which core assessments (both direct and indirect tests) are recommended for all clinical trials. The core component is a relatively brief screening assessment that, in most countries, is a sub-component of routine clinical provision. The 'plus' components enable the addition of assessments which can be selected by individual countries and/or tumour-, age-, and location-specific groups. The implementation of a QoS protocol common to all European clinical studies of childhood brain tumours is also discussed.

Long-term outcome of abusive head trauma.

Abusive head trauma is a severe inflicted traumatic brain injury, occurring under the age of 2 years, defined by an acute brain injury (mostly subdural or subarachnoidal haemorrhage), where no history or no compatible history with the clinical presentation is given. The mortality rate is estimated at 20-25% and outcome is extremely poor. High rates of impairments are reported in a number of domains, such as delayed psychomotor development; motor deficits (spastic hemiplegia or quadriplegia in 15-64%); epilepsy, often intractable (11-32%); microcephaly with corticosubcortical atrophy (61-100%); visual impairment (18-48%); language disorders (37-64%), and cognitive, behavioral and sleep disorders, including intellectual deficits, agitation, aggression, tantrums, attention deficits, memory, inhibition or initiation deficits (23-59%). Those combined deficits have obvious consequences on academic achievement, with high rates of special education in the long term. Factors associated with worse outcome include demographic factors (lower parental socioeconomic status), initial severe presentation (e.g., presence of a coma, seizures, extent of retinal hemorrhages, presence of an associated cranial fracture, extent of brain lesions, cerebral oedema and atrophy). Given the high risk of severe outcome, long-term comprehensive follow-up should be systematically performed to monitor development, detect any problem and implement timely adequate rehabilitation interventions, special education and/or support when necessary. Interventions should focus on children as well as families, providing help in dealing with the child's impairment and support with psychosocial issues. Unfortunately, follow-up of children with abusive head trauma has repeatedly been reported to be challenging, with very high attrition rates.

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.

Community integration following severe childhood traumatic brain injury.

PURPOSE OF REVIEW: Severe childhood traumatic brain injury (TBI) is the leading cause of death and acquired disability in children, causing impairments in children's sensory-motor, cognitive and behavioural functioning, with devastating consequences on community integration. Community integration is the ultimate goal of rehabilitation; it is a complex outcome, with many variables contributing to it. RECENT FINDINGS: Community integration and quality of life (QOL) are lower in children who sustained severe TBI at a younger age. Further, a wide range of injury-related, demographic and postinjury factors influence outcomes, and should serve as targets for specific interventions. An increasing number of interventions targeting cognitive, behavioural or family-related issues have been developed, with promising results. SUMMARY: Children should benefit from early integrated patient and family-centred specific care, and receive long-term follow-up until early adulthood, with regular assessments, enabling detection and treatment of any emerging problem, and to ensure the acquisition of independent living skills and stable vocational outcome when this is possible. So far, few well conducted intervention studies are available, but their number is increasing with positive results on the trained skills. Well designed studies using large samples and looking at generalization of the skills in everyday life are needed.

Management of spasticity and dystonia in children with acquired brain injury with rehabilitation and botulinum toxin A.

OBJECTIVE: To investigate the effect of a combination of botulinum toxin A (BTX-A) and rehabilitation on spasticity, pain and motor functioning in children with acquired brain injury (ABI). METHODS: All children and adolescents with ABI, aged 2-20 years, consecutively treated in the department over a 22-month period, were prospectively followed-up and clinically assessed pre- and post-treatment. They had spasticity and/or dystonia leading to impairment in activities of daily living, orthopaedic deformations and/or pain. Injections were performed using electro-stimulation. Doses of BTX-A (Botox) were administered using recent recommendations. RESULTS: Twenty-five children (mean age 6.3 years) participated in the study (51 injection sessions). All patients received BTX-A injections, followed with physical and/or occupational therapy. Significant improvement was achieved for spasticity reduction (p < 0.0001), command on antagonist muscles (p = 0.03 for the tibialis anterior) and goniometry assessment (p < 0.05). Pain relief was achieved in patients in a minimally responsive state. Functional goals were achieved, such as improving transfers or gait, grasping and releasing abilities, with significant transfer in activities of daily living (p < 0.0001). CONCLUSION: A combination of BTX-A injection with rehabilitation is an interesting option for treatment of muscle tone disorders in children with ABI.

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.