Laser interstitial thermal therapy (LITT) is a minimally invasive surgical treatment for drug-resistant, focal epilepsies. With MRI guidance, LITT can also be used to treat hypothalamic hamartoma. Here, we report on the first European case of LITT for hypothalamic hamartoma. No complication occurred except a transitory peripheral facial paralysis. The short- and long term outcomes (for both epilepsy and cognitive development) were excellent.

The Nitrogen Use Efficiency (NUE) of grain cereals depends on nitrate (NO3-) uptake from the soil, translocation to the aerial parts, nitrogen (N) assimilation and remobilization to the grains. Brachypodium distachyon has been proposed as a model species to identify the molecular players and mechanisms that affects these processes, for the improvement of temperate C3 cereals. We report on the developmental, physiological and grain-characteristic responses of the Bd21-3 accession of Brachypodium to variations in NO3- availability. As previously described in wheat and barley, we show that vegetative growth, shoot/root ratio, tiller formation, spike development, tissue NO3- and N contents, grain number per plant, grain yield and grain N content are sensitive to pre- and/or post-anthesis NO3- supply. We subsequently described constitutive and NO3--inducible components of both High and Low Affinity Transport Systems (HATS and LATS) for root NO3- uptake, and BdNRT2/3 candidate genes potentially involved in the HATS. Taken together, our data validate Brachypodium Bd21-3 as a model to decipher cereal N nutrition. Apparent specificities such as high grain N content, strong post-anthesis NO3- uptake and efficient constitutive HATS, further identify Brachypodium as a direct source of knowledge for crop improvement.

Décrire un cas d’intoxication par la lamotrigine chez un nourrisson de 16 mois. Un nourrisson de 16 mois a été hospitalisé en urgence en neurologie pédiatrique pour crises tonicocloniques généralisées récidivantes avec révulsion oculaire, morsure de la langue et asthénie post-critique. L’enfant était suivi depuis 3 mois pour épilepsie généralisée idiopathique traitée par lévétiracétam (Keppra®) 300 mg matin et soir. Il avait été traité auparavant par acide valproïque (Dépakine®) et clonazépam (Rivotril®) sans grand succès. La mère, elle-même épileptique, était sous lamotrigine (Lamictal®). Après 13 jours d’hospitalisation, son état général s’est altéré brusquement de façon inexpliquée : réapparition d’oculoclonies, de clonies des paupières et d’ataxie. Un bilan toxicologique a été demandé. L’analyse toxicologique a reposé sur un dépistage sanguin par immuno-analyse des antidépresseurs tricycliques et des benzodiazépines (Vista, Siemens), un dépistage urinaire par immuno-analyse des benzodiazépines, amphétamines, opiacés, cannabis et cocaïne (Vista, Siemens), un double criblage en CL-SM/SM (ABsciex 3200 QTRAP) et CLHP-UV/BD (Waters, Alliance) sur le sérum et les urines. Un dosage spécifique de la lamotrigine a été réalisé par CLHP-UV/BD avec le prazépam comme étalon interne. Les dépistages sanguins et urinaires sont revenus négatifs. Le criblage urinaire rapportait la présence de métabolites de la lamotrigine et de lévétiracétam. Dans le sérum, les dosages montraient une concentration en lamotrigine de 74 mg/L puis 19,8 mg/L, 1,97 mg/L et 0,40 mg/L à H4, H30, H93 et H192 respectivement après la prise supposée de Lamictal® selon les éléments du dossier. La demi-vie d’élimination de la lamotrigine a été estimée à 25,6 h environ, ce qui est conforme aux données de la littérature dans cette catégorie d’âge [1]. La lamotrigine n’ayant pas été prescrite par le corps médical, un signalement au procureur de la République a été fait et, après discussion avec la mère, une ordonnance de placement provisoire a été déclarée. Par la suite, les visites de la mère ont été limitées et surveillées, l’enfant s’est nettement amélioré sur le plan clinique, sans récidives de mouvements anormaux. Les électroencéphalogrammes effectués durant les différentes hospitalisations étaient normaux et n’ont pas montré de foyer épileptique. L’amélioration clinique du patient après limitation des visites de la mère peuvent faire évoquer un syndrome de Münchhausen par procuration. À la sortie d’hospitalisation, le lévétiracétam a été diminué à 200 mg × 2/jour et l’enfant a été placé.

Objective To assess differences between children with attentiondeficit/hyperactivity disorder (ADHD) and typically developing children (TDC) through the correlation between task performances and MRI measurements in the basal ganglia and thalamus. Methods Eleven TDC and 11 children who met diagnostic critaria for ADHD, combined type medication-free, underwent structural-MRI and diffusion tensor imaging (DTI). Volumetric and mean diffusivity (MD) measurements of the striatum (caudate and putamen), pallidum and thalamus were determined. Individuals reaction times (RTs) performances were assessed using a flanker task. The relationships between the individuals scores of mean and standard deviation of RTs (MRT and ISD, respectively) with the volumetric and MD measurements were assessed using the Spearman's correlation analysis. Results We did not found any significant correlation between the standard deviation of RTs with the volumetric and MD measurements neither for children with ADHD nor for controls. However, significant correlations were found between the MRTs and MD measurements increase in mean of RTs were linked (or significantly correlated with) to increase in MD measurements, in the caudate bilaterally, only for the group of children with ADHD (left Spearman's correlation coefficient (r) = 0.69, right r=0.62, P Conclusion The results substantiate support the hypothesis that dysfunctions in the striatal system may affect certain executive processes in children with ADHD. Despite of the relative low samples of children, these results may aid to extent our understanding of the microstructural substrates of ADHD.

Objective To evaluate the efectiveness of vagal stimulation in a population of children and adolescent with refractory epilepsy. Methods This is a monocentre, retrospective study, which studied the files of 29 children implanted between 1995 and 2012 with a vagus nerve stimulator. The rate of responders (reduction by more than 50% of the frequency of seizures), the antiepileptic efficacy according to the type of epilepsy or to the age of implantation or to the age of the start of epilepsy, the side effects, the overall quality of life and the number of hospitalisations were studied. Results VNS, for all types of epilepsy, brought a significant reduction of the frequency of seizures throughout the follow-up (p<0.05). The percentage of responder patients ranged between 59% at 3 months, 66% at 6 months, 70% at 18 months, 75% at 24 months then remained more or less stable afterwards Stimulation tended to be more effective on partial epilepsies than on generalised epilepsies. We did not show any other predictive factors of efficacy. An improvement in the overall quality of life was reported in 38% of patients, and a significant reduction in the number of hospitalisations was observed after implantation (p=0.03). Conclusion The stimulation of the vagus nerve is a sure and effective therapeutic alternative that would be discussed for a child with refractory epilepsy when a surgical approach is not possible. To evaluate the efectiveness of vagal stimulation in a population of children and adolescent with refractory epilepsy. This is a monocentre, retrospective study, which studied the files of 29 children implanted between 1995 and 2012 with a vagus nerve stimulator. The rate of responders (reduction by more than 50% of the frequency of seizures), the antiepileptic efficacy according to the type of epilepsy or to the age of implantation or to the age of the start of epilepsy, the side effects, the overall quality of life and the number of hospitalisations were studied. VNS, for all types of epilepsy, brought a significant reduction of the frequency of seizures throughout the follow-up (p<0.05). The percentage of responder patients ranged between 59% at 3 months, 66% at 6 months, 70% at 18 months, 75% at 24 months then remained more or less stable afterwards Stimulation tended to be more effective on partial epilepsies than on generalised epilepsies. We did not show any other predictive factors of efficacy. An improvement in the overall quality of life was reported in 38% of patients, and a significant reduction in the number of hospitalisations was observed after implantation (p=0.03). The stimulation of the vagus nerve is a sure and effective therapeutic alternative that would be discussed for a child with refractory epilepsy when a surgical approach is not possible.

L'hémangiomatose miliaire correspond à l'efflorescence de 5 ou 6, et jusqu'à plusieurs centaines, d'hémangiomes infantiles associée à une localisation viscérale. La localisation viscérale la plus fréquente est hépatique. Nous rapportons le cas exceptionnel d'une localisation piemérienne symptomatique. Une enfant prématurée née à 34 SA (PN 2415 g, TN 46 cm, PCN 34 cm, Apgar 10/10) présentait à la naissance 3 hémangiomes (paupière supérieure gauche, pied droit et fesse droite). À 2 mois apparaissaient 25 hémangiomes ponctiformes sous forme de miliaire disséminée. Les échographies cardiaque et hépatique étaient normales. À 6 mois, l'enfant était hospitalisée pour spasmes avec fixité oculaire. L'examen neurologique était normal. Le TDM cérébral et l'IRM montraient un hémangiome piemérien temporal droit sans hémangiome associé du parenchyme cérébral. Un traitement par vigabatrine (Sabril®) et valproate de sodium (Dépakine®) était maintenu jusqu'à l'âge de 2 ans. Aucun traitement complémentaire n'était associé, étant donné l'état clinique stable sous traitement antiépileptique et l'involution spontanée de la localisation méningée. À 2 ans, l'hémangiome piemérien avait régressé aux scanner et IRM cérébraux comme la majorité des hémangiomes cutanés, à l'exception d'un hémangiome vulvaire stable. Les atteintes cérébrales de l'hémangiomatose miliaire sont exceptionnelles : 9 cas sont rapportés, comprenant un cas asymptomatique, 6 hydrocéphalies et 2 hémorragies méningées. Sept des neuf cas étaient associés à d'autres localisations viscérales : hépatique (n = 5), pulmonaire (n = 3), rénale (n = 1), digestive (n = 1). Dans la littérature, les crises convulsives ont pu être rattachées à des perturbations hémodynamiques causées par de très gros hémangiomes céphaliques, à des anomalies artérielles intracrâniennes associées, à des hydrocéphalies ou encore des hémorragies méningées mais non à des localisations d'hémangiomes méningées comme c'est le cas de notre patiente. La proximité des structures épithéliales dans des sites riches en CCL20 (surexpression des ligands CCR6 dans les cellules endothéliales des hémangiomatoses néonatales diffuses) explique peut-être le tropisme cérébral. Notre observation est particulière de part la localisation viscérale unique, piemérienne, symptomatique.

Les hématomes sous-duraux (HSD) sont fréquents dans la population pédiatrique et sont une cause de morbi-mortalité importante. L'objectif est de décrire la présentation clinique, les caractéristiques TDM et IRM, les examens complémentaires, la prise en charge thérapeutique, le devenir médical et socio-judiciaire chez les enfants souffrant d'HSD. Dans le cadre de cette revue rétrospective, les 88 dossiers d'HSD survenus dans la population pédiatrique, entre 1998 et 2012, ont été étudiés. 72% des enfants avaient moins de 12 mois. Les motifs de consultation et les signes cliniques à l'entrée étaient multiples et non spécifiques. Il n'y avait pas de caractéristique radiologique significative permettant d'affirmer avec certitude l'étiologie de l'HSD. Dans 51% des cas il était également retrouvé des hémorragies rétiniennes. 49% des HSD étaient dus à un traumatisme crânien accidentel, 36% à un syndrome du bébé secoué et 15% à une pathologie identifiée ou non. 37,5% des dossiers ont fait l'objet d'un signalement. 46% souffraient à posteriori de séquelles cliniques de gravité variable. Chaque acteur de santé a un rôle primordial face à un enfant consultant pour HSD et notamment afin de ne pas méconnaître une situation de maltraitance.

L'hamartome hypothalamique (HH) est une tumeur neurale bénigne rare à l'origine d'épilepsie, de troubles du comportement et de puberté précoce. Nous rapportons le cas d'un garçon de 3 ans, adressé pour troubles autistiques et absence de langage. La marche a été acquise à 14 mois. Dès la naissance, il a présenté des crises gélastiques puis des spasmes infantiles dès 6 mois non diagnostiqués. Une avance staturopondérale liée à une puberté précoce centrale a été diagnostiquée. L'IRM cérébrale a mis en évidence une masse tumorale hypothalamique appendue au plancher du 3ème ventricule évocatrice d'un hamartome. Une déconnexion par voie endoscopique a été réalisée permettant la disparition des manifestations épileptiques, une diminution du syndrome autistique et des progrès majeurs sur le langage. L'hamartome hypothalamique est révélé très fréquemment par des crises gélastiques. Les spasmes infantiles précoces sont également des manifestations épileptiques décrites dans l'HH. Le diagnostic d'hamartome hypothalamique doit toujours être évoqué devant des crises gélastiques. Ceci permet d'introduire précocement un traitement antiépileptique afin de limiter le risque de retard psychomoteur et de troubles autistiques.

La plupart des types d’épilepsie sont susceptibles d’être améliorés par la stimulation du nerf vague et ce à n’importe quel moment de leur évolution. L’objectif de cette étude est d’évaluer son efficacité globale, d’identifier d’éventuels facteurs prédictifs d’efficacité, d’évaluer sa tolérance et ses bénéfices médico-économiques. Dans le cadre de cette revue rétrospective, les dossiers des 29 enfants âgés de 3,5 à 18 ans implantés dans notre CHU entre 1995 et 2012 ont été étudiés. La stimulation du nerf vague a permis une réduction significative de la fréquence des crises et ce à chaque instant du suivi (p < 0,05). Après 3 mois, la stimulation du nerf vague avait tendance à être plus efficace sur les épilepsies partielles. Nous n’avons pas mis en évidence d’autres facteurs prédictifs d’efficacité (p > 0,05). Nous notons une amélioration globale de la qualité de vie chez 38% des patients et une réduction significative du nombre d’hospitalisations après implantation (p=0,03). La stimulation du nerf vague est une alternative thérapeutique sûre et efficace chez les enfants non candidats à la chirurgie souffrant d’une épilepsie partielle pharmacorésistante.

Globalization has altered the way we live and earn a livelihood. Consequently, trade and travel have been recognized as significant determinants of the spread of disease. Additionally, the rise in urbanization and the closer integration of the world economy have facilitated global interconnectedness. Therefore, globalization has emerged as an essential mechanism of disease transmission. This paper aims to examine the potential impact of COVID-19 on globalization and global health in terms of mobility, trade, travel, and countries most impacted.The effect of globalization were operationalized in terms of mobility, economy, and healthcare systems. The mobility of individuals and its magnitude was assessed using airline and seaport trade data and travel information. The economic impact was measured based on the workforce, event cancellations, food and agriculture, academic institutions, and supply chain. The healthcare capacity was assessed by considering healthcare system indicators and preparedness of countries. Utilizing a technique for order of preference by similarity to ideal solution (TOPSIS), we calculated a pandemic vulnerability index (PVI) by creating a quantitative measure of the potential global health. The pandemic has placed an unprecedented burden on the world economy, healthcare, and globalization through travel, events cancellation, employment workforce, food chain, academia, and healthcare capacity. Based on PVI results, certain countries were more vulnerable than others. In Africa, more vulnerable countries included South Africa and Egypt; in Europe, they were Russia, Germany, and Italy; in Asia and Oceania, they were India, Iran, Pakistan, Saudi Arabia, and Turkey; and for the Americas, they were Brazil, USA, Chile, Mexico, and Peru. The impact on mobility, economy, and healthcare systems has only started to manifest. The findings of this study may help in the planning and implementation of strategies at the country level to help ease this emerging burden.

ObjectivesThe aim of this study was to investigate whether the quadrivalent human papillomavirus (HPV) vaccine Gardasil is associated with a change in the risk of autoimmune disorders (ADs) in young female subjects.DesignSystematic case-control study of incident ADs associated with quadrivalent HPV vaccination in young women across France.Participants and settingA total of 113 specialised centres recruited (from December 2007 to April 2011) females aged 14-26years with incident cases of six types of ADs: idiopathic thrombocytopenic purpura (ITP), central demyelination/multiple sclerosis (MS), Guillain-Barre syndrome, connective tissue disorders (systemic lupus erythematosus, rheumatoid arthritis/juvenile arthritis), type 1 diabetes mellitus and autoimmune thyroiditis. Control subjects matched to cases were recruited from general practice.AnalysisMultivariate conditional logistic regression analysis; factors included age, geographical origin, smoking, alcohol consumption, use of oral contraceptive(s) or vaccine(s) other than Gardasil received within 24months before the index date and personal/family history of ADs.ResultsOverall, 211 definite cases of ADs were matched to 875 controls. The adjusted odds ratio (OR) for any quadrivalent HPV vaccine use was 0.9 [95% confidence interval (CI) 0.5-1.5]. The individual ORs were 1.0 (95% CI 0.4-2.6) for ITP, 0.3 (95% CI 0.1-0.9) for MS, 0.8 (95% CI 0.3-2.4) for connective disorders and 1.2 (95% CI 0.4-3.6) for type 1 diabetes. No exposure to HPV vaccine was observed in cases with either Guillain-Barre syndrome or thyroiditis.ConclusionsNo evidence of an increase in the risk of the studied ADs was observable following vaccination with Gardasil within the time periods studied. There was insufficient statistical power to allow conclusions to be drawn regarding individual ADs.

The adult outcome after childhood onset epilepsy is a complex subject because seizure types and severity are diverse, comorbidities are common, and additional factors influence social outcome. We review selected data about seizure remission or persistence and social outcome in adulthood.Information came from published literature, especially population-based studies.In general, approximately 50-60% of children with epilepsy eventually have complete seizure remission (i.e., seizure free and off antiepileptic drug treatment): with longer follow-up, the remission rate improves. Predicting remission, persistent or intractable epilepsy is often inaccurate for an individual patient. A tiny proportion of children with epilepsy die as the result of seizures or sudden unexpected death in epilepsy patients; however, an otherwise normal child has the same risk of death as the reference population. When uncontrolled epilepsy persists into adulthood, the rate of sudden unexpected death in epilepsy patients possibly increases. Reports about social outcome in adulthood are increasing. For those with intellectual disability, a lifetime of dependency is to be expected. For those with normal intelligence, adult life is often unsatisfactory with high rates of incomplete education, unemployment, poverty, social isolation, inadvertent pregnancy, and psychiatric disorders. Seizure remission does not ensure good adult social outcome.Although seizure control in childhood is important, anticipating poor social outcome in adulthood may allow earlier interventions. A well-orchestrated transition from pediatric to adult health care may be beneficial for the 40-50% with persistent seizures and for the majority who are at risk for adult social difficulties.

Objective. - This case study aims to demonstrate that spatiotemporal spike discrimination and source analysis are effective to monitor the development of sources of epileptic activity in time and space. Therefore, they can provide clinically useful information allowing a better understanding of the pathophysiology of individual seizures with time- and space-resolved characteristics of successive epileptic states, including interictal, preictal, postictal, and ictal states.Methods. - High spatial resolution scalp EEGs (HR-EEG) were acquired from a 2-year-old girl with refractory central epilepsy and single-focus seizures as confirmed by intracerebral EEG recordings and ictal single-photon emission computed tomography (SPEC). Evaluation of HR-EEG consists of the following three global steps: (1) creation of the initial head model, (2) automatic spike and seizure detection, and finally (3) source localization. During the source localization phase, epileptic states are determined to allow state-based spike detection and localization of underlying sources for each spike. In a final cluster analysis, localization results are integrated to determine the possible sources of epileptic activity. The results were compared with the cerebral locations identified by intracerebral EEG recordings and SPECT.Results. - The results obtained with this approach were concordant with those of MRI, SPECT and distribution of intracerebral potentials. Dipole cluster centres found for spikes in interictal, preictal, ictal and postictal states were situated an average of 6.3 mm from the intracerebral contacts with the highest voltage. Both amplitude and shape of spikes change between states. Dispersion of the dipoles was higher in the preictal state than in the postictal state. Two clusters of spikes were identified. The centres of these clusters changed position periodically during the various epileptic states.Conclusion. - High-resolution surface EEG evaluated by an advanced algorithmic approach can be used to investigate the spatiotemporal characteristics of sources located in the epileptic focus. The results were validated by standard methods, ensuring good spatial resolution by MRI and SPECT and optimal temporal resolution by intracerebral EEG. Surface EEG can be used to identify different spike clusters and sources of the successive epileptic states. The method that was used in this study will provide physicians with a better understanding of the pathophysiological characteristics of epileptic activities. In particular, this method may be useful for more effective positioning of implantable intracerebral electrodes. (C) 2011 Elsevier Masson SAS. All rights reserved.