Multiprofessional follow-up programmes are needed to address psychosocial, neurocognitive and educational issues in children with brain tumours.

AIM: The aim of this study was to coordinate the structured psychosocial, neurocognitive and educational follow-up of children treated for brain tumours with the medical protocol and apply the model in two Swedish healthcare regions. METHODS: We invited all children living in the two regions, who had been diagnosed with a brain tumour from October 1, 2010, through June 30, 2012, to participate along with their parents. The follow-up programme evaluated the emotional status of the parents and patients and assessed the children's general cognitive level, working memory, speed of performance, executive functions and academic achievement from diagnosis through to adult care. RESULTS: During the study period, 61 children up to the age of 17.1 years were diagnosed with a brain tumour, but 18 of these were excluded for various reasons. The majority of the mothers (70%) displayed significantly poor emotional status, as did 34% of the fathers and 21% of the children. The majority of the children (57%) also showed poor neurocognitive performance and needed special adaptations at school (66%). CONCLUSION: Our findings indicate the need for coordinated, multiprofessional follow-up programmes, well anchored in the healthcare organisation, for children diagnosed with brain tumours.

Central nervous system involvement in severe congenital neutropenia: neurological and neuropsychological abnormalities associated with specific HAX1 mutations.

OBJECTIVES: Homozygous mutations in the HAX1 gene were recently identified in severe congenital neutropenia patients belonging to the original Kostmann family in northern Sweden. Our observations suggested that these patients also develop neurological and neuropsychological symptoms. METHODS: Detailed clinical studies and mutation analyses were performed in the surviving patients belonging to the Kostmann kindred and in two patients not related to this family, along with studies of HAX1 splice variant expression in normal human tissues. RESULTS: Five of six Kostmann family patients and one other patient from northern Sweden harboured homozygous HAX1 mutations (568C-->T, Q190X) and one carried a heterozygous ELA2 gene mutation. One Swedish patient of Kurdish extraction carried alternative homozygous HAX1 mutations (131G-->A, W44X). All the three patients with Q190X mutations who were alive and available for evaluation developed neurological disease with decreased cognitive function, and three of four patients who reached 10 years developed epilepsy. In contrast, the patients with the ELA2 and W44X HAX1 mutations, respectively, showed no obvious neurological abnormalities. Moreover, two alternative HAX1 splice variants were identified in normal human tissues, including the brain. Both transcripts contained exon 5, harbouring the Q190X mutation, whereas the 5' end of exon 2 containing the W44X mutation was spliced out from the second transcript. CONCLUSIONS: We describe neurological and neuropsychological abnormalities for the first time in Kostmann disease patients. These central nervous system symptoms appear to be associated with specific HAX1 mutations.

Attention and memory training in children with acquired brain injuries.

AIM: To test the feasibility of the Amsterdam memory and attention training for children (Amat-c) in Swedish children with acquired brain damage. METHODS: Amat-c consists of structured exercises in specific attention and memory techniques. Three Swedish children aged 9-16 y with acquired brain injuries and related memory and attention deficits trained with the Amat-c method for half an hour a day in school or at home interactively with a teacher or parent for a period of 20 wk. RESULTS: All children and their coaches completed the training without interruption. The results showed an improvement in several neuropsychological tests of sustained and selective attention as well as in memory performance. Questionnaires filled in by parents and teachers indicate that, using the Amat-c method, the children learned strategies that improved their school achievement and self-image. CONCLUSIONS: The Amat-c is a valuable treatment option for improving cognitive efficiency in children with acquired brain injuries. The results indicate improved performance in several psychometric measurements. On the basis of these results, the second step will be to modify the complexity and duration of the method, as well as to integrate a reward system before further evaluating the efficacy in a larger controlled study.

Measurements of attention deficits and impulsivity: a Swedish study of the Gordon Diagnostic System.

The Gordon Diagnostic System (GDS) is a portable easily operated computerized tool developed to measure impulse control, attention and vigilance. In 1988, it was standardized for use among American children. The aim of this study was to evaluate the GDS for use among Swedish children. A clinical sample of 71 Swedish children, mean age 10.5 y, fulfilling the ADHD criteria according to the DSM-IV was compared with a control sample of 88 children, mean age 10.2-y, with no known psychiatric diagnosis. The clinical sample showed lower GDS scores in all age groups, with some exceptions. The GDS scores were not associated with gender, but strongly associated with age, especially in the control sample. The accuracy of the GDS referring a specific child to either of the samples was, as expected, not impressive. With respect to the practical usefulness, the GDS was well accepted by the children and parents in both samples. The findings in age variation and when comparing children with ADHD and controls are in agreement with results from other studies.