A likely pathogenic variant in the SLC20A2 gene presenting with progressive myoclonus

A 60-year-old man is presented with progressive involuntary muscle movements and neuropsychiatric symptoms who developed a variety of additional complaints over 2 years. Brain imaging revealed bilateral basal ganglia calcifications suggesting primary familial brain calcification. Analysis of the SLC20A2 gene revealed a missense mutation (c.541C>T, p.(Arg181Trp)), in silico predicted to be deleterious and not found in available databases. Segregation analysis confirmed his asymptomatic father to harbor the same mutation, though on brain imaging basal ganglia calcifications were found. This report illustrates the intrafamilial variability of the phenotype and generalized myoclonus as the presenting symptom

The LBV HR Car has a partner: Discovery of a companion with the VLTI

Luminous Blue Variables (LBVs) are massive stars caught in a post-main sequence phase, during which they are losing a significant amount of mass. As, on one hand, it is thought that the majority of massive stars are close binaries that will interact during their lifetime, and on the other, the most dramatic example of an LBV, Eta Car, is a binary, it would be useful to find other binary LBVs. We present here interferometric observations of the LBV HR Car done with the AMBER and PIONIER instruments attached to ESO's Very Large Telescope Interferometer (VLTI). Our observations, spanning two years, clearly reveal that HR Car is a binary star. It is not yet possible to constrain fully the orbit, and the orbital period may lie between a few years and several hundred years. We derive a radius for the primary in the system and possibly resolve as well the companion. The luminosity ratio in the H-band between the two components is changing with time, going from about 6 to 9. We also tentatively detect the presence of some background flux which remained at the 2% level until January 2016, but then increased to 6% in April 2016. Our AMBER results show that the emission line forming region of Br gamma is more extended than the continuum emitting region as seen by PIONIER and may indicate some wind-wind interaction. Most importantly, we constrain the total masses of both components, with the most likely range being 33.6 and 45 solar masses. Our results show that the LBV HR Car is possibly an Eta Car analog binary system with smaller masses, with variable components, and further monitoring of this object is definitively called for.Comment: A&A, in pres

Max Stirner’s Early French Reception (1844-1892)

The focus of this dissertation is the French reception of German philosopher Max Stirner (1806-56) during the period spanning the release of his magnum opus, Der Einzige und sein Eigenthum, in Germany in 1844 to the appearance of its first partial French translations in the early 1890s. This phase of Stirner’s French reception has been widely overlooked by Stirner scholars, or at any rate approached in an unsystematic, somewhat dismissive manner. The prolonged lack of interest in this particular timeframe owes much to the uncritical acceptance of an old but still predominant narrative according to which, soon after the appearance of Der Einzige, Stirner ‘fell into oblivion’ and was only ‘rediscovered’ in the 1880s and 1890s. This study aims to show that, far from being a ‘forgotten’ figure, during the period 1844-1892 Stirner’s name was often invoked in French literary, political, philosophical, and religious discourse where he came to personify many of the worst features of a (perceived) German cultural and even military threat. This dissertation is conceived not only as a transnational reception history of Stirner (and, by extension, of Hegelianism) in France, but also as an intellectual history of France itself. As such, it will provide significant insights into French responses in the nineteenth century to a variety of radical philosophical ideas or traditions (such as materialism, sensualism, egoism, pantheism or atheism, and nihilism) which were commonly associated in France with German contemporary philosophy and of which Stirner became, for many, the archetype. This thesis seeks to explain why French intellectuals interpreted Stirner’s thought as they did, to understand what they were doing by engaging with it in the way they did and the effects that they hoped to produce by doing so

Do tonic itch and pain stimuli draw attention towards their location?

Background. Although itch and pain are distinct experiences, both are unpleasant, may demand attention, and interfere with daily activities. Research investigating the role of attention in tonic itch and pain stimuli, particularly whether attention is drawn to the stimulus location, is scarce. Methods. In the somatosensory attention task, fifty-three healthy participants were exposed to 35-second electrical itch or pain stimuli on either the left or right wrist. Participants responded as quickly as possible to visual targets appearing at the stimulated location (ipsilateral trials) or the arm without stimulation (contralateral trials). During control blocks, participants performed the visual task without stimulation. Attention allocation at the itch and pain location is inferred when responses are faster ipsilaterally than contralaterally. Results. Results did not indicate that attention was directed towards or away from the itch and pain location. Notwithstanding, participants were slower during itch and pain than during control blocks. Conclusions. In contrast with our hypotheses, no indications were found for spatial attention allocation towards the somatosensory stimuli. This may relate to dynamic shifts in attention over the time course of the tonic sensations. Our secondary finding that itch and pain interfere with task performance is in-line with attention theories of bodily perception

Moduli spaces of compact RCD(0,N)-structures

The goal of the paper is to set the foundations and prove some topological results about moduli spaces of non-smooth metric measure structures with non-negative Ricci curvature in a synthetic sense (via optimal transport) on a compact topological space; more precisely, we study moduli spaces of RCD(0,N)-structures. First, we relate the convergence of RCD(0,N)-structures on a space to the associated lifts' equivariant convergence on the universal cover. Then we construct the Albanese and soul maps, which reflect how structures on the universal cover split, and we prove their continuity. Finally, we construct examples of moduli spaces of RCD(0,N)-structures that have non-trivial rational homotopy groups.Comment: 40 pages. Final version to appear in Math. Annale

TESS asteroseismology of the known red-giant host stars HD 212771 and HD 203949

International audienc

4CaaSt: Comprehensive management of Cloud services through a PaaS

The 4CaaSt project aims at developing a PaaS framework that enables flexible definition, marketing, deployment and management of Cloud-based services and applications. The major innovations proposed by 4CaaSt are the blueprint and its management and lifecycle, a one stop shop for Cloud services and the management of resources in the PaaS level (including elasticity). 4CaaSt also provides a portfolio of ready to use Cloud native services and Cloud- aware immigrant technologies

Asset management contracts and equilibrium prices

We study the joint determination of fund managers' contracts and equilibrium asset prices. Because of agency frictions, investors make managers' fees more sensitive to performance and benchmark performance against a market index. This makes managers unwilling to deviate from the index and exacerbates price distortions. Because trading against overvaluation exposes managers to greater risk of deviating from the index than trading against undervaluation, agency frictions bias the aggregate market upwards. They can also generate a negative relationship between risk and return because they raise the volatility of overvalued assets. Socially optimal contracts provide steeper performance incentives and cause larger pricing distortions than privately optimal contracts