Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement

Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patient

PP270—Computational modeling of dravet syndrome

e102 Volume 35 Number 8S clorazepate (20mg 2× /d), and pregabalin (100 mg 3× /d). Because of resurgence of severe anxio-depressive symptoms, without any change of the treatment, the patient was readmitted 2 months later. Despite increasing the dose of clomipramine up to 225 mg/d, there was no clinical improvement, and the patient finally attempted to her life by abusing drugs. She then improved after 2 weeks on clomipramine IV (50 mg/d). Compliance was estimated good and no pharmacokinetic interactions with the rest of the treatment were found. C and DC plasma levels were measured, and CYP2D6/CYP2C19 genotype analyzed. Results: The plasma levels of C and DC are given in the Table below. Measures were done at the steady state and at trough concentration for IV treatment and 10 hours after the last dose for oral treatment

Contribution of EARLINET/ACTRIS to the summer 2013 Special Observing Period of the ChArMEx project: monitoring of a Saharan dust event over the western and central Mediterranean

In the framework of the Chemistry-Aerosol Mediterranean Experiment (ChArMEx; http://charmex.lsce.ipsl.fr/) initiative, a field campaign took place in the western Mediterranean Basin between 10 June and 5 July 2013 within the ADRIMED (Aerosol Direct Radiative Impact on the regional climate in the MEDiterranean region) project. The scientific objectives of ADRIMED are the characterization of the most common ‘Mediterranean aerosols’ and their direct radiative forcing (column closure and regional scale). During 15–24 June a multiintrusion dust event took place over the western and central Mediterranean Basin. Extra measurements were carried out by some EARLINET/ACTRIS (European Aerosol Research Lidar Network /Aerosols, Clouds, and Trace gases Research InfraStructure Network, http://www.actris.net/) lidar stations in Spain and Italy, in particular on 22 June in support to the flight over southern Italy of the Falcon 20 aircraft involved in the campaign. This article describes the physical and optical properties of dust observed at the different lidar stations in terms ofdust plume centre of mass, optical depth, lidar ratio, and particle depolarization ratio. To link the differences found in the origin of dust plumes, the results are discussed on the basis of back-trajectories and air- and space-borne lidars. This work puts forward the collaboration between a European research infrastructure (ACTRIS) and an international project (ChArMEx) on topics of interest for both parties, and more generally for the atmospheric community.Published4698-47114A. Clima e OceaniJCR Journalrestricte

Extrusion of Endodontic Filling Materials: Medico-Legal Aspects. Two Cases

The Authors describe two cases of alleged malpractice due to overfilling. The aim of this article is to underline some medico-legal aspects regarding the quantity of extruded material which may be considered acceptable and the consequent damage to the patient

Overview of the Chemistry-Aerosol Mediterranean Experiment/Aerosol Direct Radiative Forcing on the Mediterranean Climate (ChArMEx/ADRIMED) summer 2013 campaign

The Chemistry-Aerosol Mediterranean Experiment (ChArMEx; http://charmex.lsce.ipsl.fr) is a collaborative research program federating international activities to investigate Mediterranean regional chemistry-climate interactions. A special observing period (SOP-1a) including intensive airborne measurements was performed in the framework of the Aerosol Direct Radiative Impact on the regional climate in the MEDiterranean region (ADRIMED) project during the Mediterranean dry season over the western and central Mediterranean basins, with a focus on aerosol-radiation measurements and their modeling. The SOP-1a took place from 11 June to 5 July 2013. Airborne measurements were made by both the ATR-42 and F-20 French research aircraft operated from Sardinia (Italy) and instrumented for in situ and remote-sensing measurements, respectively, and by sounding and drifting balloons, launched in Minorca. The experimental setup also involved several ground-based measurement sites on islands including two ground-based reference stations in Corsica and Lampedusa and secondary monitoring sites in Minorca and Sicily. Additional measurements including lidar profiling were also performed on alert during aircraft operations at EARLINET/ACTRIS stations at Granada and Barcelona in Spain, and in southern Italy. Remote-sensing aerosol products from satellites (MSG/SEVIRI, MODIS) and from the AERONET/PHOTONS network were also used. Dedicated meso-scale and regional modeling experiments were performed in relation to this observational effort. We provide here an overview of the different surface and aircraft observations deployed during the ChArMEx/ADRIMED period and of associated modeling studies together with an analysis of the synoptic conditions that determined the aerosol emission and transport. Meteorological conditions observed during this campaign (moderate temperatures and southern flows) were not favorable to producing high levels of atmospheric pollutants or intense biomass burning events in the region. However, numerous mineral dust plumes were observed during the campaign, with the main sources located in Morocco, Algeria and Tunisia, leading to aerosol optical depth (AOD) values ranging between 0.2 and 0.6 (at 440 nm) over the western and central Mediterranean basins. One important point of this experiment concerns the direct observations of aerosol extinction onboard the ATR-42, using the CAPS system, showing local maxima reaching up to 150Mm(-1) within the dust plume. Non-negligible aerosol extinction (about 50Mm(-1)) has also been observed within the marine boundary layer (MBL). By combining the ATR- 42 extinction coefficient observations with absorption and scattering measurements, we performed a complete optical closure revealing excellent agreement with estimated optical properties. This additional information on extinction properties has allowed calculation of the dust single scattering albedo (SSA) with a high level of confidence over the western Mediterranean. Our results show a moderate variability from 0.90 to 1.00 (at 530 nm) for all flights studied compared to that reported in the literature on this optical parameter. Our results underline also a relatively low difference in SSA with values derived near dust sources. In parallel, active remote-sensing observations from the surface and onboard the F-20 aircraft suggest a complex vertical structure of particles and distinct aerosol layers with sea spray and pollution located within the MBL, and mineral dust and/or aged North American smoke particles located above (up to 6–7 km in altitude). Aircraft and balloon-borne observations allow one to investigate the vertical structure of the aerosol size distribution showing particles characterized by a large size (> 10 μm in diameter) within dust plumes. In most of cases, a coarse mode characterized by an effective diameter ranging between 5 and 10 μm, has been detected above the MBL. In terms of shortwave (SW) direct forcing, in situ surface and aircraft observations have been merged and used as inputs in 1-D radiative transfer codes for calculating the aerosol direct radiative forcing (DRF). Results show significant surface SW instantaneous forcing (up to (-90)Wm(-2) at noon). Aircraft observations provide also original estimates of the vertical structure of SW and LW radiative heating revealing significant instantaneous values of about 5 K per day in the solar spectrum (for a solar angle of 30 ) within the dust layer. Associated 3-D modeling studies from regional climate (RCM) and chemistry transport (CTM) models indicate a relatively good agreement for simulated AOD compared with observations from the AERONET/PHOTONS network and satellite data, especially for long-range dust transport. Calculations of the 3-D SW (clear-sky) surface DRF indicate an average of about -10 to -20Wm(-2) (for the whole period) over the Mediterranean Sea together with maxima (-50Wm(-2)) over northern Africa. The top of the atmosphere (TOA) DRF is shown to be highly variable within the domain, due to moderate absorbing properties of dust and changes in the surface albedo. Indeed, 3-D simulations indicate negative forcing over the Mediterranean Sea and Europe and positive forcing over northern Africa. Finally, a multiyear simulation, performed for the 2003 to 2009 period and including an ocean–atmosphere (O–A) coupling, underlines the impact of the aerosol direct radiative forcing on the sea surface temperature, O–A fluxes and the hydrological cycle over the Mediterranean.French National Research Agency (ANR) ANR-11-BS56-0006ADEMEFrench Atomic Energy CommissionCNRS-INSU and Meteo-France through the multidisciplinary programme MISTRALS (Mediterranean Integrated Studies aT Regional And Local Scales)CORSiCA project - Collectivite Territoriale de Corse through Fonds Europeen de Developpement Regional of the European Operational ProgramContrat de Plan Etat-RegionEuropean Union's Horizon 2020 research and innovation program 654169Spanish Ministry of Economy and Competitivity TEC2012-34575Science and Innovation UNPC10-4E-442European Union (EU)Department of Economy and Knowledge of the Catalan Autonomous Government SGR 583Andalusian Regional Government P12-RNM-2409Spanish Government CGL2013-45410-R 26225

Common Promoter Elements in Odorant and Vomeronasal Receptor Genes

In mammals, odorants and pheromones are detected by hundreds of odorant receptors (ORs) and vomeronasal receptors (V1Rs and V2Rs) expressed by sensory neurons that are respectively located in the main olfactory epithelium and in the vomeronasal organ. Even though these two olfactory systems are functionally and anatomically separate, their sensory neurons show a common mechanism of receptor gene regulation: each neuron expresses a single receptor gene from a single allele. The mechanisms underlying OR and VR gene expression remain unclear. Here we investigated if OR and V1R genes share common sequences in their promoter regions

A Recent Class of Chemosensory Neurons Developed in Mouse and Rat

In most animal species, the vomeronasal organ ensures the individual recognition of conspecifics, a prerequisite for a successful reproduction. The vomeronasal organ expresses several receptors for pheromone detection. Mouse vomeronasal type-2 receptors (V2Rs) are restricted to the basal neurons of this organ and organized in four families. Family-A, B and D (family ABD) V2Rs are expressed monogenically (one receptor per neuron) and coexpress with either Vmn2r1 or Vmn2r2, two members of family-C V2Rs. Thus, basal neurons are characterized by specific combinations of two V2Rs. To investigate this issue, we raised antibodies against all family-C V2Rs and analyzed their expression pattern. We found that six out of seven family-C V2Rs (Vmn2r2-7) largely coexpressed and that none of the anti-Vmn2r2-7 antibodies significantly stained Vmn2r1 positive neurons. Thus, basal neurons are divided into two complementary subsets. The first subset (Vmn2r1-positive) preferentially coexpresses a distinct group of family-ABD V2Rs, whereas the second subset (Vmn2r2-7-positive) coexpresses the remaining group of V2Rs. Phylogenetic reconstruction and the analysis of genetic loci in various species reveal that receptors expressed by this second neuronal subset are recent branches of the V2R tree exclusively present in mouse and rat. Conversely, V2Rs expressed in Vmn2r1 positive neurons, are phylogenetically ancient and found in most vertebrates including rodents. Noticeably, the more recent neuronal subset expresses a type of Major Histocompatibility Complex genes only found in murine species. These results indicate that the expansion of the V2R repertoire in a murine ancestor occurred with the establishment of a new population of vomeronasal neurons in which coexists the polygenic expression of a recent group of family-C V2Rs (Vmn2r2-7) and the monogenic expression of a recent group of family-ABD V2Rs. This evolutionary innovation could provide a molecular rationale for the exquisite ability in individual recognition and mate choice of murine species

Ethological principles predict the neuropeptides co-opted to influence parenting

Ethologists predicted that parental care evolves by modifying behavioural precursors in the asocial ancestor. As a corollary, we predict that the evolved mechanistic changes reside in genetic pathways underlying these traits. Here we test our hypothesis in female burying beetles, Nicrophorus vespilloides, an insect where caring adults regurgitate food to begging, dependent offspring. We quantify neuropeptide abundance in brains collected from three behavioural states: solitary virgins, individuals actively parenting or post-parenting solitary adults and quantify 133 peptides belonging to 18 neuropeptides. Eight neuropeptides differ in abundance in one or more states, with increased abundance during parenting in seven. None of these eight neuropeptides have been associated with parental care previously, but all have roles in predicted behavioural precursors for parenting. Our study supports the hypothesis that predictable traits and pathways are targets of selection during the evolution of parenting and suggests additional candidate neuropeptides to study in the context of parenting

Genetic hitchhiking and resistance evolution to transgenic Bt toxins: insights from the African stalk borer Busseola fusca (Noctuidae)

Since transgenic crops expressing Bacillus thuringiensis (Bt) toxins were first released, resistance evolution leading to failure in control of pests populations has been observed in a number of species. Field resistance of the moth Busseola fusca was acknowledged 8 years after Bt maize was introduced in South Africa. Since then, field resistance of this corn borer has been observed at several locations, raising questions about the nature, distribution and dynamics of the resistance trait. Using genetic markers, our study identified four outlier loci clearly associated with resistance. In addition, genetic structure at neutral loci reflected extensive gene flow among populations. A realistically parameterised model suggests that resistance could travel in space at speed of several kilometres a year. Markers at outlier loci delineated a geographic region associated with resistance spread. This was an area of approximately 100 km radius, including the location where resistance was first reported. Controlled crosses corroborated these findings and showed significant differences of progeny survival on Bt plants depending on the origin of the resistant parent. Last, our study suggests diverse resistance mutations, which would explain the widespread occurrence of resistant larvae in Bt fields across the main area of maize production in South Africa