Association of severe myoclonic epilepsy of infancy (SMEI) with probable autoimmune lymphoproliferative syndrome-variant

The paper reported on a case of severe myoclonic epilepsy of infancy (SMEI) associated with a probable autoimmune lymphoproliferative syndrome variant (Dianzani autoimmune lymphoproliferative disease) (DALD). A male patient with typical features of SMEI and a SCN1A gene variant presented in the first year of life with multiple lymph nodes, palpable liver at 2 cm from the costal margin, neutropenia, dysgammaglobulinemia, relative and sometimes absolute lymphocytosis. Subsequently the patient presented with constantly raised IgA in serum and positive antinuclear and thyroid antimicrosomal antibodies. The diagnosis of probable autoimmune lymphoproliferative syndrome was made; arthritis, skin and throat blisters, which appeared subsequently led to the diagnosis of linear IgA disease. On the basis of these unique associations, the Authors hypothesized that autoimmunity may be partly responsible of the severe epileptic symptomatology, perhaps mediated by autoantibodies against sodium channels or by accompanying cytotoxic T-lymphocytes. Corticosteroid treatment ameliorated the epilepsy and laboratory tests. Future studies will be necessary to evaluate the relevance of autoimmunity in SMEI

Pioneer 10 data analysis: Investigation on periodic anomalies

International audienceThe Pioneer Anomaly refers to the difference between the expected theoretical tra jectory of the Pioneer 10 and 11 spacecrafts and the observed tra jectory through Doppler measurements. It has been interpreted by the Jet Propulsion Laboratory (JPL) as a constant anomalous acceleration (Anderson et al. 2002). For this analysis, the Groupe Anomalie Pioneer (GAP) composed of several french laboratories has developped a specific tra jectography software, ODYSSEY, which enables to test different anomaly models. The paper will present, after a brief description of the software and the implemented models, the last results obtained: in addition to the constant anomaly, time dependent signatures of the anomaly have been noticed which can be described geometrically. The fit of the Pioneer 10 data with these new models yields a reduction of the standard deviation of the residual by a factor 2 with respect to the simple constant anomaly

A first analysis of the mean motion of CHAMP

The present study consists in studying the mean orbital motion of the CHAMP satellite, through a single long arc on a period of time of 200 days in 2001. We actually investigate the sensibility of its mean motion to its accelerometric data, as measures of the surface forces, over that period. In order to accurately determine the mean motion of CHAMP, we use “observed&quot; mean orbital elements computed, by filtering, from 1-day GPS orbits. On the other hand, we use a semi-analytical model to compute the arc. It consists in numerically integrating the effects of the mean potentials (due to the Earth and the Moon and Sun), and the effects of mean surfaces forces acting on the satellite. These later are, in case of CHAMP, provided by an averaging of the Gauss system of equations. Results of the fit of the long arc give a relative sensibility of about 10^-3, although our gravitational mean model is not well suited to describe very low altitude orbits. This technique, which is purely dynamical, enables us to control the decreasing of the trajectory altitude, as a possibility to validate accelerometric data on a long term basis.<br><br><b>Key words.</b> Mean orbital motion, accelerometric dat

Constraints on f(RijklRijkl)f(R_{ijkl}R^{ijkl}) gravity: An evidence against the covariant resolution of the Pioneer anomaly

We consider corrections in the form of $\Delta L(R_{ijkl}R^{ijkl})$ to the Einstein-Hilbert Lagrangian. Then we compute the corrections to the Schwarszchild geometry due to the inclusion of this general term to the Lagrangian. We show that $\Delta L_3=\alpha_{{1/3}}(R_{ijkl}R^{ijkl})^{{1/3}}$ gives rise to a constant anomalous acceleration for objects orbiting the Sun onward the Sun. This leads to the conclusion that $\alpha_{{1/3}}=(13.91\pm 2.11) \times 10^{-26}(\frac{1}{\text{meters}})^{{2/3}}$ would have covariantly resolved the Pioneer anomaly if this value of $\alpha_{{1/3}}$ had not contradicted other observations. We notice that the experimental bounds on $\Delta L_3$ grows stronger in case we examine the deformation of the space-time geometry around objects lighter than the Sun. We therefore use the high precision measurements around the Earth (LAGEOS and LLR) and obtain a very strong constraint on the corrections in the form of $\Delta L(R_{ijkl}R^{ijkl})$ and in particular $\Delta L=\alpha_n(R_{ijkl}R^{ijkl})^n$. This bound requires $\alpha_{{1/3}}\leq6.12\times 10^{-29}(\frac{1}{\text{meters}})^{{2/3}}$. Therefore it refutes the covariant resolution of the Pioneer anomaly.Comment: ...v5: references added, new discussions adde

Grown-up stars physics with MATISSE

MATISSE represents a great opportunity to image the environment around massive and evolved stars. This will allow one to put constraints on the circumstellar structure, on the mass ejection of dust and its reorganization , and on the dust-nature and formation processes. MATISSE measurements will often be pivotal for the understanding of large multiwavelength datasets on the same targets collected through many high-angular resolution facilities at ESO like sub-millimeter interferometry (ALMA), near-infrared adaptive optics (NACO, SPHERE), interferometry (PIONIER, GRAVITY), spectroscopy (CRIRES), and mid-infrared imaging (VISIR). Among main sequence and evolved stars, several cases of interest have been identified that we describe in this paper.Comment: SPIE, Jun 2016, Edimbourgh, Franc

Squared visibility estimator. Calibrating biases to reach very high dynamic range

In the near infrared where detectors are limited by read-out noise, most interferometers have been operated in wide band in order to benefit from larger photon rates. We analyze in this paper the biases caused by instrumental and turbulent effects to $V^2$ estimators for both narrow and wide band cases. Visibilities are estimated from samples of the interferogram using two different estimators, $V^{2}_1$ which is the classical sum of the squared modulus of Fourier components and a new estimator $V^{2}_2$ for which complex Fourier components are summed prior to taking the square. We present an approach for systematically evaluating the performance and limits of each estimator, and to optimizing observing parameters for each. We include the effects of spectral bandwidth, chromatic dispersion, scan length, and differential piston. We also establish the expression of the Signal-to-Noise Ratio of the two estimators with respect to detector and photon noise. The $V^{2}_1$ estimator is insensitive to dispersion and is always more sensitive than the $V^{2}_2$ estimator. However, the latter allows to reach better accuracies when detection is differential piston noise limited. Biases and noise directly impact the dynamic range of reconstructed images. Very high dynamic ranges are required for direct exoplanet detection by interferometric techniques thus requiring estimators to be bias-free or biases to be accurately calibrated. We discuss which estimator and which conditions are optimum for astronomical applications especially when high accuracy visibilities are required. We show that there is no theoretical limit to measuring visibilities with accuracies as good as $10^{-5}$ which is important in the prospect of detecting faint exoplanets with interferometers.Comment: 23 pages, 6 figures, accepted for publication in Ap

Spectral and spatial imaging of the Be+sdO binary phi Persei

The rapidly rotating Be star phi Persei was spun up by mass and angular momentum transfer from a now stripped-down, hot subdwarf companion. Here we present the first high angular resolution images of phi Persei made possible by new capabilities in longbaseline interferometry at near-IR and visible wavelengths. We observed phi Persei with the MIRC and VEGA instruments of the CHARA Array. Additional MIRC-only observations were performed to track the orbital motion of the companion, and these were fit together with new and existing radial velocity measurements of both stars to derive the complete orbital elements and distance. The hot subdwarf companion is clearly detected in the near-IR data at each epoch of observation with a flux contribution of 1.5% in the H band, and restricted fits indicate that its flux contribution rises to 3.3% in the visible. A new binary orbital solution is determined by combining the astrometric and radial velocity measurements. The derived stellar masses are 9.6+-0.3Msol and 1.2+-0.2Msol for the Be primary and subdwarf secondary, respectively. The inferred distance (186 +- 3 pc), kinematical properties, and evolutionary state are consistent with membership of phi Persei in the alpha Per cluster. From the cluster age we deduce significant constraints on the initial masses and evolutionary mass transfer processes that transformed the phi Persei binary system. The interferometric data place strong constraints on the Be disk elongation, orientation, and kinematics, and the disk angular momentum vector is coaligned with and has the same sense of rotation as the orbital angular momentum vector. The VEGA visible continuum data indicate an elongated shape for the Be star itself, due to the combined effects of rapid rotation, partial obscuration of the photosphere by the circumstellar disk, and flux from the bright inner disk.Comment: 16 pages, 6 figures, 1 Anne

Requirements modelling and formal analysis using graph operations

The increasing complexity of enterprise systems requires a more advanced analysis of the representation of services expected than is currently possible. Consequently, the specification stage, which could be facilitated by formal verification, becomes very important to the system life-cycle. This paper presents a formal modelling approach, which may be used in order to better represent the reality of the system and to verify the awaited or existing system’s properties, taking into account the environmental characteristics. For that, we firstly propose a formalization process based upon properties specification, and secondly we use Conceptual Graphs operations to develop reasoning mechanisms of verifying requirements statements. The graphic visualization of these reasoning enables us to correctly capture the system specifications by making it easier to determine if desired properties hold. It is applied to the field of Enterprise modelling

Constraining Disk Parameters of Be Stars using Narrowband H-alpha Interferometry with the NPOI

Interferometric observations of two well-known Be stars, gamma Cas and phi Per, were collected and analyzed to determine the spatial characteristics of their circumstellar regions. The observations were obtained using the Navy Prototype Optical Interferometer equipped with custom-made narrowband filters. The filters isolate the H-alpha emission line from the nearby continuum radiation, which results in an increased contrast between the interferometric signature due to the H-alpha-emitting circumstellar region and the central star. Because the narrowband filters do not significantly attenuate the continuum radiation at wavelengths 50 nm or more away from the line, the interferometric signal in the H-alpha channel is calibrated with respect to the continuum channels. The observations used in this study represent the highest spatial resolution measurements of the H-alpha-emitting regions of Be stars obtained to date. These observations allow us to demonstrate for the first time that the intensity distribution in the circumstellar region of a Be star cannot be represented by uniform disk or ring-like structures, whereas a Gaussian intensity distribution appears to be fully consistent with our observations.Comment: 23 pages, 14 figures, accepted for publication in A

Line Forces in Keplerian Circumstellar Disks and Precession of Nearly Circular Orbits

We examine the effects of optically thick line forces on orbiting circumstellar disks, such as occur around Be stars. For radially streaming radiation, line forces are only effective if there is a strong radial velocity gradient, as occurs, for example, in a line-driven stellar wind. However, within an orbiting disk, the radial shear of the azimuthal velocity leads to strong line-of-sight velocity velocity gradients along nonradial directions. As such, in the proximity of a stellar surface extending over a substantial cone angle, the nonradial stellar radiation can impart a significant line force, even in the case of purely circular orbits. Given the highly supersonic nature of orbital velocity variations, we use the Sobolev approximation, thereby extending to the disk case the standard CAK formalism developed for line-driven winds. We delineate the parameter regimes for which radiative forces might alter disk properties; but even when radiative forces are small, we analytically quantify higher-order effects in the linear limit, including the precession of weakly elliptical orbits. We find that optically thick line forces can have observable implications for the dynamics of disks around Be stars, including the generation of either prograde or retrograde precession in slightly eccentric orbits. However, our analysis suggests a net retrograde effect, in apparent contradiction with observed long-term variations of violet/red line profile asymmetries from Be stars, which are generally thought to result from prograde propagation of a so-called ``one arm mode''. We also conclude that radiative forces may alter the dynamical properties at the surface of the disk where disk winds originate, and may even make low-density disks vulnerable to being blown away.Comment: 31 pages, Latex, aaspp4 macro, 4 figure