Anne Pagnier

Mineralocorticoid deficiency in post-operative cerebral salt wasting.

Acute hyponatremia, following neurosurgery, results from inappropriate antidiuretic hormone secretion (SIADH) or cerebral salt wasting (CSW). CSW is due to abnormally high atrial or brain natriuretic peptides (ANP, BNP), which block all stimulators of zona glomerulosa steroidogenesis, resulting in mineralocorticoid deficiency. A 3 year-old girl presented CSW at day 4, after resection of craniopharyngioma and hypophysectomy. Hyponatremia, hyperkalemia and high natriuresis occurred on day 8, with low renin and aldosterone and elevated BNP 120.3 ng/ml (undetectable before surgery). Fludrocortisone 100 microg/day controlled natriuresis and restored electrolytes within 24 hours. A 5 year-old boy presented CSW at day 6 after partial resection of optic glioma. Fludocortisone 100 microg/day restored electrolytes within 8 hours. ANP was elevated, 60.6 ng/l, aldosterone and renin were low. Fludrocortisone supplementation should be considered in CSW, as excessive natriuresis is controlled, and electrolytes are easily restored, avoiding life-threatening complications of this complex disorder.

Juvenile xanthogranuloma with hematological dysfunction treated with 2CDA‐AraC

Juvenile xanthogranuloma was diagnosed in two infants aged 8 and 2 months with skin lesions, hepatosplenomegaly, and pancytopenia. Disease control was not achieved with first‐line vinblastine–steroid–VP16 combination therapy. Two courses of 2‐chlorodeoxyadenosine (2CDA) (0.3 mg/kg) and cytosine arabinoside (AraC) (1 g/m2) were then administered for 5 days and were followed, after hematological recovery, by maintenance therapy. Both patients had normal complete blood cell counts and no signs of JXG, respectively, 31 and 24 months after diagnosis. Pediatr Blood Cancer. 2010;55:757–760.

SynthèseDevenir neurocognitif à long terme des enfants traités pour une tumeur cérébraleNeurocognitive outcome in childhood brain tumor survivors

Advances in treatment have significantly improved the survival rate of children with brain tumour. This review describes risk factors of late neurocognitive effect, the different type of neurocognitive side effect according to the type of tumour and the rehabilitation. The best knowledge of cognitive sequelae allowed to propose supported appropriate and specific disorders including school facilities for younger and adaptation of professional situation for adults. Furthermore, this understanding allows to link with the patients quality of life and to approach the person in its specificity and overall.

Devenir neurocognitif à long terme des enfants traités pour une tumeur cérébrale

Advances in treatment have significantly improved the survival rate of children with brain tumour. This review describes risk factors of late neurocognitive effect, the different type of neurocognitive side effect according to the type of tumour and the rehabilitation. The best knowledge of cognitive sequelae allowed to propose supported appropriate and specific disorders including school facilities for younger and adaptation of professional situation for adults. Furthermore, this understanding allows to link with the patients quality of life and to approach the person in its specificity and overall.

Modality Specific Activations in Working Memory in Children with Cerebellar Medullobastoma: A Functional MRI Study

Introduction: Medulloblastomas are the most common malignant childhood brain tumors arising in the posterior fossa. As a result of advances in treatment the number of survivors has increased significantly. In this study, children treated for a cerebellar medulloblastoma demonstrated cognitive disorders in working memory, especially the visuospatial component, leading to impairments in school performance. This study aims to describe the cerebellar involvement in specific cognitive deficits observed in these children. Method: Nine healthy volunteer children (11.1 ± 2.2 years old), were compared to 5 patients treated for cerebellar medulloblastoma (12.3 ± 0.7 years old). All subjects were native French speakers, right-handed, with a global Intelligence Quotient (IQ) of 70-130. Stimuli were presented to the participants with alternating the sensory modality (visual, auditory) and the nature of communication (verbal, nonverbal) in a blocked 1-back design during functional Magnetic Resonance Imaging (fMRI) acquisitions. Results were analyzed using a Mann-Whitney U test for neuropsychological and behavioral data; SPM8 (Statistical Parametric Mapping) and SUIT (Spatially Unbiased Atlas Template) for anatomical and functional MRI data. Results: Non-verbal working memory deficit was demonstrated in 4 of the 5 children treated for medulloblastoma. The 4 patients with working memory deficit all had surgery to the left posterior cerebellar lobe. The only patient without any working memory disorders did not have hemispheric cerebellar resection. Neuronal activations for nonverbal versus verbal contrast and visual versus auditory contrast were stronger in the left posterior cerebellar lobe. Brain activations in healthy subjects were consistent with the findings previously described in literature. Conclusion: The cerebellum plays the same role in working memory in children as that has been previously described in adults. The left posterior cerebellar lobe may involve the visuospatial working memory. Based on neuroimaging and clinical studies, attention should be paid to avoid or limit the damage of the posterior cerebellar lobe to minimize cognitive deficits and improve the quality of life for children.

Prise en charge des tumeurs cérébrales pédiatriques

Résumé:La prise en charge des tumeurs cérébrales pédiatriques vise à améliorer les taux de survie, sans accepter des séquelles incompatibles avec une bonne qualité ultérieure de vie. Le modèle du médulloblastome est exemplaire: après exérèse locale, la radiothérapie crâniospinale (± chimiothérapie) reste le standard chez l’enfant de plus de cinq ans au diagnostic. Elle est cependant pourvoyeuse de séquelles neuropsychologiques d’autant plus importantes que l’âge au diagnostic est faible. Aussi la chimiothérapie exclusive est-elle proposée en cas d’exérèse complète avant l’âge de cinq ans avec des taux élevés de guérison. La prise en charge multidisciplinaire est abordée.Abstract:The treatment of pediatric brain tumours aims at increasing survival rates while maintaining a good quality of life. The model of medulloblastoma is used for description: for children who are more than 5 year old at diagnosis, craniospinal radiation (± chemotherapy) remains a standard. However, sequelae are inversely correlated with age at diagnosis. Thus in children younger than 5 with complete removal, exclusive chemotherapy has proved effective. The multidisciplinary approach of pediatric brain tumours is described.