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BackgroundNoninvasive ventilation (NIV) improves survival and quality of life in amyotrophic lateral sclerosis (ALS) patients. NIV initiation is mostly conducted at hospital, but a recurrent lack of hospital beds led to the necessity of exploring an at-home initiation process. Here, we report data from our NIV initiation cohort of ALS patients. Could our at-home NIV initiation process with telemonitoring in ALS patients be an efficient solution for adherence and nocturnal hypoxaemia correction?MethodsWe performed a retrospective analysis of data collected from 265 ALS patients treated at the Bordeaux ALS Centre for whom NIV initiation was carried out between September 2017 and June 2021, with two modalities: at-home initiation or in-hospital initiation. The primary outcome was adherence to NIV at 30 days. The secondary outcome was at-home NIV initiation process efficiency of nocturnal hypoxaemia correction.ResultsAt 30 days, NIV adherence (mean >4 h·day−1) was 66% of the total population, 70% of the at-home NIV initiation subgroup and 52% of the in-hospital NIV initiation subgroup. Nocturnal hypoxaemia correction was observed in 79% of adherent patients in the at-home NIV initiation subgroup. Mean delay of NIV prescription and at-home NIV initiation was 8.7 days (+/−6.5)versus29.5 days in hospital.ConclusionOur study shows that our at-home NIV initiation process in ALS patients is a good option to provide rapid access to NIV with good adherence and efficiency. Further literature on the benefits of at-home NIV initiation is welcomed, especially to evaluate long-term efficiency and global cost analysis.

Strong magnetic fields are observed in a substantial fraction of upper main sequence stars and white dwarfs. Many such stars are observed to exhibit photometric modulations as the magnetic poles rotate in and out of view, which could be a consequence of magnetic perturbations to the star's thermal structure. The magnetic pressure is typically larger than the gas pressure at the star's photosphere, but much smaller than the gas pressure in the star's interior, so the expected surface flux perturbations are not clear. We compute magnetically perturbed stellar structures of young $3 \, M_\odot$ stars that are in both hydrostatic and thermal equilibrium, and which contain both poloidal and toroidal components of a dipolar magnetic field as expected for stable fossil fields. This provides semi-analytical models of such fields in baroclinic stably stratified regions. The star's internal pressure, temperature, and flux perturbations can have a range of magnitudes, though we argue the most likely configurations exhibit flux perturbations much smaller than the ratio of surface magnetic pressure to surface gas pressure, but much larger than the ratio of surface magnetic pressure to central gas pressure. The magnetic pole is hotter than the equator in our models, but a cooler magnetic pole is possible depending on the magnetic field configuration. The expected flux variations for observed field strengths are $\delta L/L \! \lesssim \! 10^{-6}$, much smaller than those observed in magnetic stars, suggesting that observed perturbations stem from changes to the emergent spectrum rather than changes to the bolometric flux., Comment: Submitted to MNRAS, comments welcome!

Livedoid vasculopathy (LV) is a chronic dermatosis associated with micro-thrombosis of the vessels of the dermis, leading to ischemic lesions and painful skin ulcerations of the lower limbs. This thrombosing occlusive vasculopathy, clearly distinct from 'classical vasculitis' (not related to alteration of vessel walls), may lead to peripheral neuropathy.To clarify the main clinical, electrophysiological and pathological characteristics of peripheral neuropathy linked to LV.We presented a series of personal cases of peripheral neuropathy due to LV. We also conducted a review of the literature (since the first description of LV in 1974) using multiple combinations of keywords from 'PubMed', 'Google Scholar' and 'ScienceDirect' databases according to the 'Preferred Reporting Items for Systematic reviews and Meta-Analyses' guidelines.We identified 16 patients (6 personal cases and 10 cases from the medical literature). Our personal cases were five females and one male, with a median age (at the onset of cutaneous signs of LV) of 38 (range 25-62). Several types of skin lesions of the lower limbs were observed. Median age at the onset of peripheral neuropathy symptoms was 48 years (range 29-66), with a main clinical and electrophysiological pattern of mononeuropathy multiplex.We observed a typical pattern of peripheral neuropathy, mostly mononeuropathy multiplex, whose pathophysiology might be related to occlusions of the small vessels of the nerves, as seen in the dermis. Moreover, LV may also be associated with other types of peripheral neuropathies (sometimes of autoimmune etiology) not directly related to the skin lesions.The 'ischemic form' of peripheral neuropathy linked to LV is mainly responsible for sensory disturbances (with multifocal distribution), sometimes for motor disturbances. This type of peripheral neuropathy has to be distinguished from 'classical vasculitic neuropathies' which are usually treated with antithrombotic therapies.

The high number of discovered close-in planets motivates the improvement of tidal modeling.Among the five thousand exoplanets discovered up to now, half of them have an orbital period of less than 10 days and therefore experience some form of tidal evolution. Besides, the habitable zone of ultra-cool dwarfs is located close-in (e.g. TRAPPIST-1, Gillon et al. 2017), so planets in the habitable zone of these dwarfs are expected to undergo tidal evolution. By impacting the orbital and rotational dynamics of close-in planets, tides have an impact on their habitability. Indeed, tides drive the spin evolution of planets, influence their thermal state by internal friction (Henning & Hurford 2014, Bolmont et al. 2020), and drive their orbital evolution by exchange of angular momentum. This leads to the migration of the planets, their eccentricity and obliquity damping, and the precession of their orbits ( e.g Hut 1981, Bolmont et al. 2011, 2012, Ragozzine & Wolf 2009). Solid planetary tides also act to synchronize the spin of planets on circular orbits with their orbital motion. It is generally assumed that close-in planets (i.e. experiencing strong tidal interactions) have reached this state of synchronization, also called the 1:1 spin-orbit resonance (hereafter SOR). However, in the case of an eccentric or inclined orbit, tides can trap the spin in higher rotation rates, e.g. in the 3:2 or the 2:1 SOR. If a planet has an atmosphere, another tidal mechanism should be taken into account: the thermal tide, which is caused by the differential heating between day and night sides (Correia & Laskar 2001, Auclair-Desrotour 2017a). In the case of thick atmospheres such as that of Venus, the thermal tides acting on the atmosphere can be as strong as the gravitational tides acting on the solid core (Auclair-Desrotour et al. 2017b) and can both de-synchronize the planet and increase the spin inclination. The competition between the two tides, solid and thermal can be seen in figure [1]. Figure [1]: The two tidal contributions, gravitational and thermal, acting in opposition to each other. The solide tide drives the spin to synchronization, while the thermal tide drives it away from it. Tidal model To understand the evolution of exoplanets, we need a consistent tidal model which encapsulates the complex response of rocky planets to stress. Most models assume that planets are made of weakly viscous fluid (e.g. Hut 1981, Goldreich & Soter 1966) even for rocky planets. However, it has been shown that they do not reproduce the correct behavior for highly viscous solid bodies (Henning et al. 2009; Efroimsky & Makarov 2013). In contrast, we use here a model which accounts for more realistic anelastic behavior for the rocks (such as the Andrade rheology) for a Venus-like planet (Dumoulin et al. 2017). We choose here an approach developed by Kaula (1964), which consists in using a decomposition of the tidal potential into Fourier harmonic modes. For the thermal tide, we use the analytical formulation of Leconte et al (2015), which aims at reproducing Venus today. Our developments were added to the ESPEM code (Benbakoura et al. 2019) to compute the orbital and rotational evolution of a Venus-like planet around a Sun-like star. 2) A complex rotational evolution: Figure [2] shows the evolution of a Venus-like planet, with and without an atmosphere, orbiting a Sun-like planet with an initial obliquity of 50 degrees and a rotation of about 100 days and on a circular orbit. No atmosphere (red curve): When the planet does not have an atmosphere, the rotation spins down to synchronization in 100 million years. Interestingly and despite the absence of an eccentricity, Figure [2] shows that the obliquity of the planet allows the presence of the 2:0 spin-orbit resonance in which the planet spends a few 10 million years. The planet leaves this resonance when the obliquity becomes too low to maintain this configuration stable. With atmosphere (blue curve): The blue curve of Fig. [2] shows the effect of a strong CO2 atmosphere on the evolution of the spin and the obliquity. The evolution here is more complex. First, the combination of the two tides damps the spin toward the 2:1 SOR while increasing the obliquity. When the obliquity becomes high enough, the thermal torque begins to counter-balance the solid torque. The thermal tides continue to increase the obliquity and the spin of the planet until an equilibrium state between the solid and thermal tides is found. Depending on the atmospheric parameters, the evolutions we obtain are complex, with spin-orbit resonances, coming either from the eccentricity or from the obliquity of the planet. I will show the variety of different spin evolutions of a Venus-like planet, and how it depends on the presence of an eccentricity or obliquity and the presence or not of a thick atmosphere. Figure [2] Simulation of a Venus-like planet orbiting a Sun-like star, without an atmosphere (in red) and with an atmosphere (in blue). Top panel: evolution of the ratio of the planetary spin Ω and the mean motion n. Bottom panel: evolution of the obliquity of the planet (defined as the angle between the equatorial plane and the orbital plane).

Autoimmune neuropathies are named by eponyms, by descriptive terminology or because of the presence of specific antibodies and are traditionally classified, on the basis of pathology and electrophysiology, as primary demyelinating or axonal. However, autoimmune disorders targeting specific molecules of the nodal region, although not showing pathological evidence of demyelination, can exhibit all the electrophysiological changes considered characteristic of a demyelinating neuropathy and acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the groundwork of the classical dichotomous classification.We propose a classification of autoimmune neuropathies based on the involved domains of the myelinated fibre and, when known, on the antigen. This classification, in our opinion, helps to better systematise autoimmune neuropathies because points to the site and molecular target of the autoimmune attack, reconciles some contrasting pathological and electrophysiological findings, circumvents the apparent paradox that neuropathies labelled as axonal may be promptly reversible and finally avoids taxonomic confusion and possible misdiagnosis.

ObjectiveTo describe the clinical characteristics and outcomes of coronavirus disease 2019 (COVID-19) among patients with myasthenia gravis (MG) and identify factors associated with COVID-19 severity in patients with MG.MethodsThe CO-MY-COVID registry was a multicenter, retrospective, observational cohort study conducted in neuromuscular referral centers and general hospitals of the FILNEMUS (Filière Neuromusculaire) network (between March 1, 2020, and June 8, 2020), including patients with MG with a confirmed or highly suspected diagnosis of COVID-19. COVID-19 was diagnosed based on a PCR test from a nasopharyngeal swab or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) serology, thoracic CT scan, or typical symptoms. The main outcome was COVID-19 severity based on location of treatment/management (home, hospitalized in a medical unit, or in an intensive care unit). We collected information on demographic variables, general history, and risk factors for severe COVID-19. Multivariate ordinal regression models were used to identify factors associated with severe COVID-19 outcomes.ResultsAmong 3,558 patients with MG registered in the French database for rare disorders, 34 (0.96%) had COVID-19. The mean age at COVID-19 onset was 55.0 ± 19.9 years (mean MG duration: 8.5 ± 8.5 years). By the end of the study period, 28 patients recovered from COVID-19, 1 remained affected, and 5 died. Only high Myasthenia Gravis Foundation of America (MGFA) class (≥IV) before COVID-19 was associated with severe COVID-19 (p = 0.004); factors that were not associated included sex, MG duration, and medium MGFA classes (≤IIIb). The type of MG treatment had no independent effect on COVID-19 severity.ConclusionsThis registry-based cohort study shows that COVID-19 had a limited effect on most patients, and immunosuppressive medications and corticosteroids used for MG management are not risk factors for poorer outcomes. However, the risk of severe COVID-19 is elevated in patients with high MGFA classes (odds ratio, 102.6 [4.4–2,371.9]). These results are important for establishing evidence-based guidelines for the management of patients with MG during the COVID-19 pandemic.

Monoclonal gammopathy is encountered quite frequently in the general population. This type of hematologic abnormality may be mild, referred to as monoclonal gammopathy of undetermined significance or related to different types of hematologic malignancies. The association of a peripheral neuropathy with monoclonal gammopathy is also fairly common, and hemopathy may be discovered in an investigation of peripheral neuropathy. In such a situation, it is essential to determine the exact nature of the hematologic process in order not to miss a malignant disease and thus initiate the appropriate treatment (in conjunction with hematologists and oncologists). In this respect, nerve biopsy (discussed on a case-by-case basis) is of great value in the management of such patients. We therefore propose to present the objectives and main interests of nerve biopsy in this situation.

To study the pathological characteristics of acute and chronic ataxic peripheral neuropathy at the level of the node of Ranvier.We performed the pathological study (nerve biopsy of a sural nerve) of two patients, one with an acute form of ataxic peripheral neuropathy called 'Miller Fisher syndrome' (MFS), the other one with a chronic form called 'chronic ataxic neuropathies with disialosyl antibodies' (CANDA).A dysimmune process involving peripheral nerves commences in myelin, at the internodal area or/and in the paranodal and nodal regions. Our electron microscopic observations suggest that both patients present lesions in favor of a paranodopathy.Many of the immune-mediated peripheral neuropathies are now classified as nodoparanodopathies. This subtype of auto-immune neuropathy may present various clinical phenotypes such as 'Acute Motor Axonal Neuropathy' (AMAN), 'Acute Motor and Sensory Neuropathy' (AMSAN) or 'chronic inflammatory demyelinating polyradiculoneuropathy' (CIDP), and are associated with anti-disialosyl antibodies. In our two cases, some paranodes seem to be associated with macrophages and we hypothesized that these lesions are in favor of a complement-mediated dysfunction/disruption of the nodal region due to disialosyl antibodies against gangliosides which are mainly located at the level of the axolemma of the paranode.

International audience; The history of mankind is marked by numerous epidemics, some of which involved diseases of the peripheral nervous system, either infectious or otherwise. We describe here the three main infectious causes of epidemics that affect the peripheral nervous system: leprosy, poliomyelitis and diphtheria. We then discuss the main epidemics of immune-mediated origin.

International audience; Peripheral neuropathies have various causes, both infectious and non-infectious. When we think of “epidemics”, we often refer to an infectious or even post-infectious origin. Nevertheless, the history of mankind is marked by episodes of epidemics of peripheral neuropathies of non-infectious nature, either of nutritional or toxic origin: we present here the main causes of such epidemics.

Introduction. Amyotrophic lateral sclerosis (ALS), usually fatal in a few years, is a neurodegenerative disorder where the diagnostic delay, although variable according to the studies, remains too long. The main objective of this study was to determine the average time to diagnose ALS and the role of each physician, general practitioner (GP), or specialist (neurologist or not) involved in the management of these patients. The secondary objective was to propose some simple schemes to quickly identify an ALS suspicion with the aim to reduce this delay. Patients and Methods. This retrospective study evaluated the diagnostic delay (and other intermediate delays) of 90 ALS patients registered in the ALS Center of Bordeaux (France) in 2013. The main clinical signs encountered (and their order of appearance) were studied. Results. The average diagnostic delay was 17 months, with a median diagnostic delay of 12 months. The average diagnostic delay was 2.7 months between the first symptoms and the first complaint to GP, followed by an additional 6.5 month delay before the patient’s first visit to a neurologist. This period could be shortened, especially if GP performed additional tests quickly (p=0.01), as the time spent consulting various specialists often extends this crucial step. Overall, diagnostic delay accounted for 40% of the total duration of the disease progression. Conclusion. In relation to total survival time, the diagnostic delay of ALS appears to be proportionately very long, sometimes longer than that observed in previous studies (because it also included the total delay to diagnostic or treatment initiation). The rapid execution of useful additional tests by the first medical doctor, often GP (with the help of a neurologist), considerably reduces the diagnostic delay. The central role of GP seems to be crucial in the management of patients with ALS. The main objective is, of course, to initiate appropriate treatment and care as soon as possible. Finally, based on our results, we also provide a short practical diagram to help nonneurologist practitioners to quickly discuss the diagnosis of ALS in case of some specific symptoms (“red flags”).

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy described in 1916 by Guillain, Barré, and Strohl. However, many similar cases had been reported earlier under various terms, with less detail and with various explanations about its pathophysiologic origin. Based on the analysis of old articles, we propose an overview of the history of acute inflammatory polyradiculoneuropathy before the official description of GBS.

Although Guillain-Barre syndrome was officially described in 1916, other cases had been reported earlier, such as some cases of Landry's paralysis. This year is the 250th anniversary of the birth of Georges Cuvier (1769–1832), one of the fathers of comparative anatomy and palaeontology: he died at age 63 from an unknown disease. By reading medical reports about his last days and hours, we conclude Cuvier died from a severe form of Guillain-Barre syndrome. Moreover, we think this observation could be the first complete report of acute polyradiculoneuropathy with pharyngeal-cervical-brachial onset.

We present an analysis of the first 20 second cadence light curves obtained by the TESS space telescope during its extended mission. We find improved precision of 20 second data compared to 2 minute data for bright stars when binned to the same cadence (˜10%-25% better for T ? 8 mag, reaching equal precision at T ˜ 13 mag), consistent with pre-flight expectations based on differences in cosmic-ray mitigation algorithms. We present two results enabled by this improvement. First, we use 20 second data to detect oscillations in three solar analogs (? Pav, ? Tuc, and p Men) and use asteroseismology to measure their radii, masses, densities, and ages to ˜1%, ˜3%, ˜1%, and ˜20% respectively, including systematic errors. Combining our asteroseismic ages with chromospheric activity measurements, we find evidence that the spread in the activity-age relation is linked to stellar mass and thus the depth of the convection zone. Second, we combine 20 second data and published radial velocities to recharacterize p Men c, which is now the closest transiting exoplanet for which detailed asteroseismology of the host star is possible. We show that p Men c is located at the upper edge of the planet radius valley for its orbital period, confirming that it has likely retained a volatile atmosphere and that the "asteroseismic radius valley"remains devoid of planets. Our analysis favors a low eccentricity for p Men c (, D.H. acknowledges support from the Alfred P. Sloan Foundation, the National Aeronautics and Space Administration (80NSSC19K0379, 80NSSC21K0652), and the National Science Foundation (AST-1717000). T.S.M. acknowledges support from NASA grant 80NSSC20K0458. Computational time at the Texas Advanced Computing Center was provided through XSEDE allocation TG-AST090107. A.C. acknowledges support from the National Science Foundation through the Graduate Research Fellowship Program (DGE 1842402). W.H.B. performed computations using the University of Birmingham's BlueBEAR High Performance Computing service. T.R.B. acknowledges support from the Australian Research Council through Discovery Project DP210103119. Funding for the Stellar Astrophysics Centre is provided by The Danish National Research Foundation (Grant DNRF106). M.S.C. and M.D. acknowledge the support by FCT/MCTES through the research grants UIDB/04434/2020, UIDP/04434/2020 and PTDC/FIS-AST/30389/2017, and by FEDER—Fundo Europeu de Desenvolvimento Regional through COMPETE2020—Programa Operacional Competitividade e Internacionalização (grant: POCI-01-0145-FEDER-030389). T.L.C. is supported by Fundação para a Ciência e a Tecnologia (FCT) in the form of a work contract (CEECIND/00476/2018). M.S.C. is supported by national funds through FCT in the form of a work contract. H.K. and E.P. acknowledge the grant from the European Social Fund via the Lithuanian Science Council (LMTLT) grant No. 09.3.3-LMT-K-712-01-0103. R.A.G. and S.N.B. acknowledge the support received from the CNES with the PLATO and GOLF grants. B.N. acknowledges postdoctoral funding from the Alexander von Humboldt Foundation and "Branco Weiss fellowship Science in Society" through the SEISMIC stellar interior physics group. S.M. acknowledges support by the Spanish Ministry of Science and Innovation with the Ramon y Cajal fellowship number RYC-2015-17697 and the grant number PID2019-107187GB-I00. T.W. acknowledges support from the B-type Strategic Priority Program of the Chinese Academy of Sciences (grant No. XDB41000000) from the NSFC of China (grant Nos. 11773064, 11873084, and 11521303), from the Youth Innovation Promotion Association of Chinese Academy of Sciences, and from the Ten Thousand Talents Program of Yunnan for Top-notch Young Talents. T.W. also gratefully acknowledges the computing time granted by the Yunnan Observatories and provided by the facilities at the Yunnan Observatories Supercomputing Platform. T.D. acknowledges support from MIT's Kavli Institute as a Kavli postdoctoral fellow.

We examine the dynamics of low-frequency gravito-inertial waves (GIWs) in differentially rotating deformed radiation zones in stars and planets by generalising the traditional approximation of rotation (TAR). The TAR treatment was built on the assumptions that the star is spherical and uniformly rotating. However, it has been generalised in our previous work by including the effects of the centrifugal deformation using a non-perturbative approach in the uniformly rotating case. We aim to carry out a new generalisation of the TAR treatment to account for the differential rotation and the strong centrifugal deformation simultaneously. We generalise our previous work by taking into account the differential rotation in the derivation of our complete analytical formalism that allows the study of the dynamics of GIWs in differentially and rapidly rotating stars. We derived the complete set of equations that generalises the TAR, simultaneously taking the full centrifugal acceleration and the differential rotation into account. Within the validity domain of the TAR, we derived a generalised Laplace tidal equation for the horizontal eigenfunctions and asymptotic wave periods of the GIWs, which can be used to probe the structure and dynamics of differentially rotating deformed stars with asteroseismology. A new generalisation of the TAR, which simultaneously takes into account the differential rotation and the centrifugal acceleration in a non-perturbative way, was derived. This generalisation allowed us to study the detectability and the signature of the differential rotation on GIWs in rapidly rotating deformed stars and planets. We found that the effects of the differential rotation in early-type deformed stars on GIWs is theoretically largely detectable in modern space photometry using observations from $\textit{Kepler}$ and TESS., 13 pages, 11 figures, 1 table, abstract shortened for arXiv. Accepted for publication in A&A. arXiv admin note: substantial text overlap with arXiv:2104.09302

Giant cell arteritis, the most frequent form of vasculitis in persons over 50 years of age, is a granulomatous chronic vasculitis involving large and medium-sized vessels, most commonly the temporal and other cranial arteries. This common, treatable condition is associated with various clinical symptoms, including neurological ones, affecting both the central and peripheral nervous systems. In this review, we discuss the cranial and extra cranial neurological complications of giant cell arteritis, to help avoid the many pitfalls in the diagnosis of giant cell arteritis.

Animal envenomation in humans is usually accidental or for defensive purposes. Depending on the venom composition and administration, different reactions can be observed. After reporting the first case of acute polyradiculitis in a 57-year-old healthy male after red lionfish envenomation, we propose to analyze rare similar cases of acute neuritis after animal envenomation published in the medical literature. Including our case, we found 54 patients who developed acute peripheral neuropathy after having been stung or bitten by various animals, mainly hymenoptera (in half of the cases) but also jellyfishes, snakes, corals or nonhooked arthropods. We observed two distinct patterns of peripheral neuropathy: more than half of them were polyneuropathy while the others were focal neuropathy. The prognosis was favorable in most cases. The pathophysiological mechanism associated with these rare complications remain unknown, although some hypotheses may be proposed. A direct action of certain components of the venom, such as phospholipase-A2, could explain the focal forms of peripheral neuropathy trough toxic reactions and/or vasculitis processes. The more diffuse clinical situations could be due to an allergy-triggered immune-mediated reaction (possibly linked to a molecular mimicry mechanism between venom proteins and some myelin proteins of the peripheral nervous system), or to the action of some venom components on membrane ionic channels particularly at the node of Ranvier. Even if acute peripheral neuropathies are rare after envenomation, they may occur after envenomation from various animals, and their usually favorable prognoses should be known by neurologists.

The Traditional Approximation of Rotation (TAR) is a treatment of the hydrodynamic equations of rotating and stably stratified fluids in which the action of the Coriolis acceleration along the direction of the entropy and chemical stratifications is neglected because it is weak in comparison with the buoyancy force. The dependent variables in the equations for the dynamics of gravito-inertial waves (GIWs) then become separable into radial and horizontal parts as in the non-rotating case. The TAR is built on the assumptions that the star is spherical (i.e. its centrifugal deformation is neglected) and uniformly rotating. We study the feasibility of carrying out a generalisation of the TAR to account for the centrifugal acceleration in the case of strongly deformed uniformly and rapidly rotating stars (and planets), and to identify the validity domain of this approximation. We built analytically a complete formalism that allows the study of the dynamics of GIWs in spheroidal coordinates which take into account the flattening of rapidly rotating stars by assuming the hierarchies of frequencies adopted within the TAR in the spherical case and by deriving a generalised Laplace tidal equation for the horizontal eigenfunctions of the GIWs and their asymptotic wave periods, which can be used to probe the structure and dynamics of rotating deformed stars with asteroseismology. Using 2D ESTER stellar models, we determine the validity domain of the generalised TAR as a function of the rotation rate of the star normalised by its critical angular velocity and its pseudo-radius. This generalisation allows us to study the signature of the centrifugal effects on GIWs in rapidly rotating deformed stars. We found that the effects of the centrifugal acceleration in rapidly rotating early-type stars on GIWs are theoretically detectable in modern space photometry using observations from Kepler., Comment: 17 pages, 14 figures, 1 table, abstract shortened for arXiv. Accepted for publication in A&A

Gaseous giant planets (Jupiter and Saturn in our solar system and hot Jupiters around other stars) are turbulent rotating magnetic objects that have strong and complex interactions with their environment (their moons in the case of Jupiter and Saturn and their host stars in the case of hot Jupiters/Saturns). In such systems, the dissipation of tidal waves excited by tidal forces shape the orbital architecture and the rotational dynamics of the planets. During the last decade, a revolution has occurred for our understanding of tides in these systems. First, Lainey et al. (2009, 2012, 2017) have measured tidal dissipation stronger by one order of magnitude than expected in Jupiter and Saturn. Second, unexplained broad diversity of orbital architectures and large radius of some hot Jupiters are observed in exoplanetary systems. Finally, new constraints obtained thanks to Kepler/K2 and TESS indicate that tidal dissipation in gaseous giant exoplanets is weaker than in Jupiter and in Saturn (Ogilvie 2014, Van Eylen et al. 2018, Huber et al. 2019). Furthermore, the space mission JUNO and the grand finale of the CASSINI mission have revolutionized our knowledge of the interiors of giant planets. We now know, for example, that Jupiter is a very complex planet: it is a stratified planet with, from the surface to the core, a differentially rotating convective envelope, a first mixing zone (with stratified convection), a uniformly rotating magnetised convective zone, a second magnetized mixing zone (the diluted core, potentially in stratified convection) and a solid core (Debras & Chabrier 2019). So far, tides in these planets have been studied by assuming a simplified internal structure with a stable rocky and icy core (Remus et al. 2012, 2015) and a deep convective envelope surrounded by a thin stable atmosphere (Ogilvie & Lin 2004) where mixing processes, differential rotation and magnetic field were completely neglected. Our objective is thus to predict tidal dissipation using internal structure models, which agree with these last observational constrains. In this work, we build a new ab-initio model of tidal dissipation in giant planets that coherently takes into account the interactions of tidal waves with their complex stratification induced by the mixing of heavy elements, their zonal winds, and (dynamo) magnetic fields. This model is a semi-global model in the planetary equatorial plane. We study the linear excitation of tidal magneto-gravito-inertial progressive waves and standing modes. We take into account the buoyancy, the compressibility, the Coriolis acceleration (including differential rotation), and the Lorentz force. The tidal waves are submitted to the different potential dissipative processes: Ohmic, thermal, molecular diffusivities, and viscosity. We here present the general formalism and the potential regimes of parameters that should be explored. The quantities of interest such as tidal torque, dissipation, and heating are derived. This will pave the way for full 3D numerical simulations that will take into account complex internal structure and dynamics of gaseous giant (exo-)planets in spherical/spheroidal geometry.

Olfaction, one of our five main qualitative sensory abilities, is the action of smelling or the capacity to smell. Olfactory impairment can be a sign of a medical problem, from a benign nasal/sinus problem up to a potentially serious brain injury. However, although clinicians (neurologists or not) usually test the olfactory nerves in specific clinical situations (for example, when a neurodegenerative disorder is suspected), they may omit such tests in many other situations. With the recent COVID-19 pandemic, the resurgence of anosmia has reminded us of the importance of testing this sensorineural function. We retrace here the main historical steps and discoveries concerning olfaction and anosmia.

Anti-MAG polyneuropathy (anti-MAG PN) is an immune-mediated peripheral sensorimotor neuropathy characterized by distal demyelination and ataxia. However, this disorder, unlike other immune-mediated neuropathies, is difficult to treat in most cases. We retrospectively collected all anti-MAG PN patients followed in two hospitals for a period of 12 years to determine prognostic factors, especially those that indicated a good response to the various therapeutic strategies used. Forty-seven patients were included in the study; of these, 61% had a classical ‘distal demyelinating pattern’, 34.2% had a ‘CIDP-like pattern’, and the others had an ‘axonal pattern’. The most commonly used treatments were intravenous immunoglobulin (IVIg) as the first-line treatment and rituximab as the second- or third-line treatment. No prognostic factor was identified for IVIg, but electrophysiological parameters at onset were better in patients with a good response to rituximab than in non-responder patients, even though mild or high disability was observed in nearly half the patients at last examination. Even though disability seems to progress in most cases despite the treatments used, our results suggest that an early electrophysiological reduction in sensory nerves could be considered a ‘red flag’ for the prompt initiation of rituximab to try to delay long-term disability.

Nodoparanodopathy is a recent concept in the field of peripheral neuropathy, corresponding to peripheral nerve disorders stemming from an autoimmune attack directed and limited to the nodal region. This concept was identified using modern techniques of electrophysiology, immunology, and pathology (including electron microscopy). We present here what we believe to be the earlier well-documented case of nodoparanodopathy in the medical literature, based on an article written by Samuel Gilbert Webber (1838–1926) in 1884.

Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our observation with the nineteen other cases found in the medical literature. Such an association (especially with suggestive skin abnormalities) has to be known for two reasons. First, this diagnosis might be quite challenging because the myopathy may precede the typical skin changes. Secondly, conversely to other forms of vacuolar myopathy, some of the symptoms may respond (even partially) to immunomodulatory and/or immunosuppressant therapeutics.

International audience; In humans, hereditary sensory neuropathies (HSN), also known as hereditary sensory and autonomic neuropathies (HSAN), constitute a clinically and genetically heterogeneous group of disorders characterized by progressive sensory loss, often accompanied by chronic skin ulcerations and nail dystrophic changes. To date, although around 20 genes have already been discovered, they do not explain the genetic causes of all patients. In dogs, similar neuropathies are also diagnosed, several breeds being predisposed to specific forms of the disease. Indeed, the breed specificity of most canine genetic diseases is due to the small numbers of founders and high levels of inbreeding. Recent knowledge and tools developed to study the canine genome efficiently allows deciphering the genetic bases of such diseases. To date, a dozen breeds are recognized to develop specific HSN. For the Border collie and hunting dog breeds, the genes involved have recently been discovered. Other affected breeds thus constitute potential genetic models, with new genes to be found in dogs that can be considered as candidate genes for human HSAN/HSN. Here, we review the different forms of human and canine HSAN/HSN and we present a novel form in Fox terrier cases, highlighting the advantages of the dog model for such rare human diseases. © 2019, Springer-Verlag GmbH Germany, part of Springer Nature.

Vitamin B12 (B12) has a fundamental role in both central and peripheral nervous system function at all ages. Neurologic manifestations may be the earliest and often the only manifestation of B12 deficiency. Mostly because of the poor sensitivity of methods of determination for B12 levels, peripheral neuropathy remains a classical but underdiagnosed complication of B12 deficiency. So the clinical and electrophysiological characteristics of B12-responsive neuropathy are not well known.A retrospective study of patients with B12-responsive neuropathy was conducted at our hospital on a 3-year period. The criteria for inclusion were: (a) neuropathy confirmed by the electrophysiological study (nerve conduction study); and (b) improvement of at least 1 point of the total Overall Neuropathy Limitations Scale score after vitamin B12 treatment.Nine patients were identified. Serum B12 level was low in only four. Four patients had sensorimotor (predominantly sensory) axonal polyneuropathy while five had only sensory neuronopathy. Six improved in less than 1 month after B12 supplementation.B12-responsive neuropathy is a more heterogeneous group of neuropathy than previously described. B12 deficiency is a cause of peripheral neuropathy and should systematically be ruled out in the clinical setting of idiopathic neuropathy or sensory neuronopathy because of potential reversibility.B12: vitamin B12; CMAP: compound muscle action potentials; DRG: dorsal root ganglia; ENMG: electroneuromyography; MCCT: motor central conduction time; MEP: motor evoked potentials; MMA: methylmalonic acid; MMCoAM: L-methylmalonyl-CoenzymeA mutase; ONLS: overall neuropathy limitations scale; SCV: sensory conduction velocities; SNAP: sensory nerve action potentials; SNN: sensory neuronopathy; SSS: SNAP sum score.

International audience; Background: Charcot-Marie-Tooth diseases (CMT) are due to abnormalities of many genes, the most frequent being linked to PMP22 (Peripheral Myelin Protein 22). In the past, only spontaneous genetic anomalies occurring in mouse mutants such as Trembler (Tr) mice were available; more recently, several rodent models have been generated for exploration of the pathophysiological mechanisms underlying these neuropathies.Methods: Based on the personal experience of our team, we describe here the pathological hallmarks of most of these animal models and compare them to the pathological features observed in some CMT patient nerves (CMT types 1A and E; hereditary neuropathy with liability to pressure palsies, HNPP).Results: We describe clinical data and detailed pathological analysis mainly by electron microscopy of the sciatic nerves of these animal models conducted in our laboratory; lesions of PMP22 deficient animals (KO and mutated PMP22) and PMP22 overexpressed models are described and compared to ultrastructural anomalies of nerve biopsies from CMT patients due to PMP22 gene anomalies. It is of note that while there are some similarities, there are also significant differences between the lesions in animal models and human cases. Such observations highlight the complex roles played by PMP22 in nerve development.Conclusion: It should be borne in mind that we require additional correlations between animal models of hereditary neuropathies and CMT patients to rationalize the development of efficient drugs.

In this lecture opening the session focused on tides in stellar and planetary systems, I will review the Jean-Paul Zahn's key contributions to the theory of tidal dissipation in stars and fluid planetary layers. I will first recall the general principles of tidal friction in celestial bodies. Then, I will focus on the theories of the stellar equilibrium and dynamical tides founded by Jean-Paul and their predictions for the evolution of binary stars. I will underline their essential legacy for ongoing studies of tidal dissipation in stars hosting planets and in fluid planetary regions. I will also discuss his pioneering work on the turbulent friction applied on tidal flows by stellar convection and the corresponding still unsolved challenging problems. Next, I will present the results we obtained on tidal dissipation in the potential dense rocky/icy core of gaseous giant planets such as Jupiter and Saturn within the Encelade international team. This mechanism provides important keys to interpret the high-precision astrometric measurements of the rates of tidal orbital migration of the moons of these planets, which are found to be larger than expected. This corresponds to a Jovian and Saturnian tidal frictions which are higher by one order of magnitude than the usually used values calibrated on formation scenarios. Finally, I will review the work done by Jean-Paul and Michel Rieutord on potential Ekman boundary layers associated to tidal flows. As a consequence, a coherent physical modeling of tides is now mandatory to understand the properties and the evolution of stellar and planetary systems. To progress on this forefront research subject, we are walking on the path first drawn by Jean-Paul.

Atmospheric tides can have a strong impact on the rotational dynamics of planets. They are of most importance for terrestrial planets located in the habitable zone of their host star, where their competition with solid tides is likely to drive the body towards non-synchronized rotation states of equilibrium, as observed in the case of Venus. Contrary to other planetary layers, the atmosphere is sensitive to both gravitational and thermal forcings, through a complex dynamical coupling involving the effects of Coriolis acceleration and characteristics of the atmospheric structure. These key physics are usually not taken into account in modelings used to compute the evolution of planetary systems, where tides are described with parametrised prescriptions. In this work, we present a new ab initio modeling of atmospheric tides adapting the theory of the Earth's atmospheric tides (Chapman & Lindzen 1970) to other terrestrial planets. We derive analytic expressions of the tidal torque, as a function of the tidal frequency and parameters characterizing the internal structure (e.g. the Brunt-V��is��l�� frequency, the radiative frequency, the pressure heigh scale). We show that stratification plays a key role, the tidal torque being strong in the case of convective atmospheres (i.e. with a neutral stratification) and weak in case of atmosphere convectively stable. In a second step, the model is used to determine the non-synchronized rotation states of equilibrium of Venus-like planets as functions of the physical parameters of the system. These results are detailed in Auclair-Desrotour et al. (2017a) and Auclair-Desrotour et al. (2017b)., Proceedings for Astro Fluid conference in memory of Jean-Paul Zahn (Paris, June 2016), 9 pages, 5 figures

The Kepler and TESS missions delivered high-precision, long-duration photometric time series for hundreds of main-sequence stars with gravito-inertial (g) pulsation modes. This high precision allows us to evaluate increasingly detailed theoretical stellar models. Recent theoretical work extended the traditional approximation of rotation (TAR), a framework to evaluate the effect of the Coriolis acceleration on g-modes, to include the effects of the centrifugal acceleration in the approximation of slightly deformed stars, which so far had mostly been neglected in asteroseismology. This extension of the TAR was conceived by rederiving the TAR in a centrifugally deformed, spheroidal coordinate system. We explore the effect of the centrifugal acceleration on g modes and assess its detectability in space-based photometry. We implement the new framework to calculate the centrifugal deformation of precomputed 1D spherical stellar structure models and compute the corresponding g-mode frequencies, assuming uniform rotation. The framework is evaluated for a grid of stellar structure models covering a relevant parameter space for observed g-mode pulsators. The centrifugal acceleration modifies the effect of the Coriolis acceleration on g modes, narrowing the equatorial band in which they are trapped. Furthermore, the centrifugal acceleration causes the pulsation periods and period spacings of the most common g modes (prograde dipole modes and r modes) to increase with values similar to the observational uncertainties in Kepler and TESS data. The effect of the centrifugal acceleration on g~modes is formally detectable in modern space photometry. Implementation of the new theoretical framework in stellar structure and pulsation codes will allow for more precise asteroseismic modelling of centrifugally deformed stars, to assess its effect on mode excitation, -trapping and -damping., Comment: 14 pages, 14 figures, 1 table. Resubmitted to A&A after a positive referee report

Quantifying tidal interactions in close-in two-body systems is of prime interest since they have a crucial impact on the architecture and on the rotational history of the bodies. Various studies have shown that the dissipation of tides in either body is very sensitive to its structure and to its dynamics, like differential rotation which exists in the outer convective enveloppe of solar-like stars and giant gaseous planets. In particular, tidal waves may strongly interact with zonal flows at the so-called corotation resonances, where the wave's Doppler-shifted frequency cancels out. We aim to provide a deep physical understanding of the dynamics of tidal inertial waves at corotation resonances, in the presence of differential rotation profiles typical of low-mass stars and giant planets. By developping an inclined shearing box, we investigate the propagation and the transmission of free inertial waves at corotation, and more generally at critical levels, which are singularities in the governing wave differential equation. Through the construction of an invariant called the wave action flux, we identify different regimes of wave transmission at critical levels, which are confirmed with a one-dimensional three-layer numerical model. We find that inertial waves can be either fully transmitted, strongly damped, or even amplified after crossing a critical level. The occurrence of these regimes depends on the assumed profile of differential rotation, on the nature as well as the latitude of the critical level, and on wave parameters such as the inertial frequency and the longitudinal and vertical wavenumbers. Waves can thus either deposit their action flux to the fluid when damped at critical levels, or they can extract action flux to the fluid when amplified at critical levels. Both situations could lead to significant angular momentum exchange between the tidally interacting bodies., 25 pages, 12 figures, 4 tables, accepted for publication in Astronomy & Astrophysics

Context. Turbulent transport in stellar radiative zones is a key ingredient of stellar evolution theory, but the anisotropy of the transport due to the stable stratification and the rotation of these regions is poorly understood. The assumption of shellular rotation, which is a cornerstone of the so-called rotational mixing, relies on an efficient horizontal transport. However, this transport is included in many stellar evolution codes through phenomenological models that have never been tested. Aims. We investigate the impact of horizontal shear on the anisotropy of turbulent transport. Methods. We used a relaxation approximation (also known as {\tau} approximation) to describe the anisotropising effect of stratification, rotation, and shear on a background turbulent flow by computing velocity correlations. Results. We obtain new theoretical scalings for velocity correlations that include the effect of horizontal shear. These scalings show an enhancement of turbulent motions, which would lead to a more efficient transport of chemicals and angular momentum, in better agreement with helio- and asteroseismic observations of rotation in the whole Hertzsprung-Russell diagram. Moreover, we propose a new choice for the non-linear time used in the relaxation approximation, which characterises the source of the turbulence. Conclusions. For the first time, we describe the effect of stratification, rotation, and vertical and horizontal shear on the anisotropy of turbulent transport in stellar radiative zones. The new prescriptions need to be implemented in stellar evolution calculations. To do so, it may be necessary to implement non-diffusive transport., Comment: 7 pages, accepted for publication in A&A

The history of mankind is marked by numerous epidemics, some of which involved diseases of the peripheral nervous system, either infectious or otherwise. We describe here the three main infectious causes of epidemics that affect the peripheral nervous system: leprosy, poliomyelitis and diphtheria. We then discuss the main epidemics of immune-mediated origin.

Multi-dimensional (magneto-)hydrodynamical simulations of physical processes in stellar interiors depend on a multitude of uncalibrated free parameters, which set the spatial and time scales of their computations. We aim to provide an asteroseismic calibration of the wave and convective Rossby numbers, and of the stiffness at the interface between the convective core and radiative envelope of intermediate-mass stars. We deduce these quantities for rotating dwarfs from the observed properties of their identified gravity and gravito-inertial modes. We rely on near-core rotation rates and asteroseismic models of 26 B- and 37 F-type dwarf pulsators derived from 4-year Kepler space photometry, high-resolution spectroscopy and Gaia astrometry in the literature to deduce their convective and wave Rossby numbers. We compute the stiffness at the convection/radiation interface from the inferred maximum buoyancy frequency at the interface and the convective turnover frequency in the core. We use those asteroseismically inferred quantities to make predictions of convective penetration levels, local flux levels of gravito-inertial waves triggered by the convective core, and of the cores' potential rotational and magnetic states. Our sample of 63 gravito-inertial mode pulsators covers near-core rotation rates from almost zero up to the critical rate. The frequencies of their identified modes lead to models with stiffness values between $10^{2.69}$ and $10^{3.60}$ for the B-type pulsators, while those of F-type stars cover the range from $10^{3.47}$ to $10^{4.52}$. The convective Rossby numbers derived from the maximum convective diffusion coefficient in the convective core, based on mixing length theory and a value of the mixing length coefficient relevant for these pulsators, vary between $10^{-2.3}$ and $10^{-0.8}$ for B-type stars and $10^{-3}$ and $10^{-1.5}$ for F-type stars. (abridged), Comment: 10 pages, 8 figures, accepted for publication in Astronomy & Astrophysics, version adapted to proofs

[Abridged] Most exoplanets detected so far are close-in planets, which are likely to be affected by tidal dissipation in their host star. To get a complete picture of the evolution of star-planet systems one needs to consider the impact of tides within both stellar radiative and convective zones. We aim to provide a general formalism to assess tidal dissipation in stellar radiative zones for all spectral types, allowing for the study of the dynamics of a given system throughout stellar evolution. We investigate the influence of stellar structure and evolution on tidal dissipation in the radiative core of low-mass stars. From the study of adiabatic oscillations throughout the star, we compute the energy flux transported by progressive internal gravity waves and the induced tidal torque. We then study the influence of stellar structure and evolution on tidal dissipation of solar-type stars from the pre-main sequence (PMS) to the red giant branch (RGB). For a given star-planet system, tidal dissipation reaches a maximum value on the pre-main sequence for all stellar masses. On the main sequence (MS), it decreases to become almost constant. The dissipation is then several orders of magnitude smaller for F-type stars than for G and K-type stars. During the Sub-Giant phase and the RGB, tidal dissipation increases by several orders of magnitude, along with the expansion of the stellar envelope. We show that the dissipation of the dynamical tide in the convective zone dominates the evolution of the system during most of the PMS and the beginning of the main sequence. Tidal dissipation in the radiative zone then becomes the strongest contribution during the Sub-Giant phase and the RGB. We also find that the dissipation of a metal-poor star is stronger than the dissipation of a metal-rich star during the PMS, the Sub-Giant phase and the RGB., Comment: 25 pages, 14 figures, accepted for publication in Astronomy and Astrophysics