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Turbulent friction in convective regions in stars and planets is one of the key physical mechanisms that drive the dissipation of the kinetic energy of tidal flows in their interiors and the evolution of their systems. This friction acts both on the equilibrium/non-wave like tide and on tidal inertial waves in these layers. It is thus necessary to obtain a robust prescription for this friction. In the current state-of-the-art, it is modeled by a turbulent eddy-viscosity coefficient, based on mixing-length theory, applied on velocities of tides. However, none of the current prescriptions take into account the action of rotation that can strongly affects turbulent convection. Therefore, we use theoretical scaling laws for convective velocities and characteristic lengthscales in rotating stars and planets that have been recently confirmed by 3-D high-resolution nonlinear Cartesian numerical simulations to derive a new prescription. A corresponding local model of tidal waves is used to understand the consequences for the linear tidal dissipation. Finally, new grids of rotating stellar models and published values of planetary convective Rossby numbers are used to discuss astrophysical consequences. The action of rotation on convection deeply modifies the turbulent friction applied on tides. In the regime of rapid rotation (with a convective Rossby number below 0.25), the eddy-viscosity may be decreased by several ordres of magnitude. It may lead to a loss of efficiency of the viscous dissipation of the equilibrium tide and to a more efficient complex and resonant dissipation of tidal inertial waves in the bulk of convective regions. Therefore, it is necessary to have a completely coupled treatment of the tidal/rotational evolution of star-planet systems and multiple stars with a coherent treatment of the variations of tidal flows and of their dissipation as a function of rotation., 10 pages, 7 figures, accepted for publication in Astronomy & Astrophysics

Understanding the internal rotation of low mass stars all along their evolution is of primary interest when studying their rotational dynamics, internal mixing and magnetic field generation. In this context, helio- and asteroseismology probe angular velocity gradients deep within solar type stars at different evolutionary stages. Still the rotation close to the center of such stars on the main sequence is hardly detectable and the dynamical interaction of the radiative core with the surface convective envelope is not well understood. For instance, the influence of the differential rotation profile sustained by convection and applied as a boundary condition to the radiation zone is very important in the formation of tachoclines. In this work, we study a 2D hydrodynamical model of a radiative core when an imposed, solar or anti-solar, differential rotation is applied at the upper boundary. This model uses the Boussinesq approximation and we find that the shear induces a cylindrical differential rotation associated with a unique cell of meridional circulation in each hemisphere (counterclockwise when the shear is solar-like and clockwise when it is anti-solar). The results are discussed in the framework of seismic observables (internal rotation rate, core-to-surface rotation ratio) while perspectives to improve our modeling by including magnetic field or transport by internal gravity waves will be discussed., Comment: 5 pages, 4 figures. To appear in the proceedings of "Seismology of the Sun and the Distant Stars 2016, Joint TASC2 & KASC9 Workshop - SPACEINN & HELAS8 Conference" (ed. M\'ario J. P. F. G. Monteiro, Margarida S. Cunha, Jo\~ao Miguel T. Ferreira ), Azores, Portugal, 11-15 July 2016

This paper provides a brief look at dynamo scaling relationships for the degree of equipartition between magnetic and kinetic energies. Two simple models are examined, where one that assumes magnetostrophy and another that includes the effects of inertia. These models are then compared to a suite of convective dynamo simulations of the convective core of a main-sequence B-type star and applied to its later evolutionary stages., Comment: 4 pages, 2 figures, IAU Symposium 329: The lives and death-throes of massive stars

Since twenty years, a large population of close-in planets orbiting various classes of low-mass stars (from M to A-type stars) has been discovered. In such systems, the dissipation of the kinetic energy of tidal flows in the host star may modify its rotational evolution and shape the orbital architecture of the surrounding planetary system. In this context, recent works demonstrated that the amplitude of this dissipation can vary over several orders of magnitude as a function of stellar mass, age and rotation. In addition, the stellar rotation evolution strongly impacts angular momentum exchanges within star-planet systems. Therefore, it is now necessary to take into account the structural and rotational evolution of stars when studying the orbital evolution of close-in planets. The presence of planets may also modify the rotation of the host stars and as a consequence their evolution, magnetic activity and mixing. In this work, we present the first study of the dynamics of close-in planets of various masses orbiting low-mass stars (from $0.6$ to $1.2~M_\star$) where we compute the simultaneous evolution of the star's structure, rotation and tidal dissipation in its external convective enveloppe. We find that tidal friction due to the stellar dynamical tide, i.e. tidal inertial waves excited in the convection zone, can be larger by several orders of magnitude than the one of the equilibrium tide, especially during the PMS phase. Consequently, the orbital migration of the planet is now more pronounced and depends more on the stellar mass, rotation and age. This would very weakly affect the planets in the habitable zone because they are located at orbital distances such that stellar tide-induced migration happens on very long timescales. We also demonstrate that the rotational evolution of host stars is only weakly affected by the presence of planets except for massive companions., Comment: Recommended for publication in Celestial Mechanics and Dynamical Astronomy (CeMDA, special issue about tides) 24 pages, 7 figures

We used extensive ground-based multisite and archival spectroscopy to derive observational constraints for a seismic modelling of the magnetic beta Cep star V2052 Ophiuchi. The line-profile variability is dominated by a radial mode (f_1=7.14846 d^{-1}) and by rotational modulation (P_rot=3.638833 d). Two non-radial low-amplitude modes (f_2=7.75603 d^{-1} and f_3=6.82308 d^{-1}) are also detected. The four periodicities that we found are the same as the ones discovered from a companion multisite photometric campaign (Handler et al. 2012) and known in the literature. Using the photometric constraints on the degrees l of the pulsation modes, we show that both f_2 and f_3 are prograde modes with (l,m)=(4,2) or (4,3). These results allowed us to deduce ranges for the mass (M \in [8.2,9.6] M_o) and central hydrogen abundance (X_c \in [0.25,0.32]) of V2052 Oph, to identify the radial orders n_1=1, n_2=-3 and n_3=-2, and to derive an equatorial rotation velocity v_eq \in [71,75] km s^{-1}. The model parameters are in full agreement with the effective temperature and surface gravity deduced from spectroscopy. Only models with no or mild core overshooting (alpha_ov \in [0,0.15] local pressure scale heights) can account for the observed properties. Such a low overshooting is opposite to our previous modelling results for the non-magnetic beta Cep star theta Oph having very similar parameters, except for a slower surface rotation rate. We discuss whether this result can be explained by the presence of a magnetic field in V2052 Oph that inhibits mixing in its interior.

Objectives Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder that can lead to neurological manifestations including strokes and cerebral abscesses. Our objectives were to describe clinical, radiological, bacteriological, and outcome characteristics of patients with cerebral abscess and HHT, and to concurrently compare this group with a control group with cerebral abscess, but without HHT. Patients and methods Patients with HHT and cerebral abscess in 5 French medical centers were included. Their clinical, radiological, biological data and prognosis were compared to the data of unselected patients with cerebral abscesses but without HHT included during the same period of time. Results Twenty-six patients (13 men and 13 women; 44.7 ± 17.2; range 12–79 years), with HHT and cerebral abscess were included. A pulmonary arteriovenous malformation (AVM) was present in all cases. Cerebral abscesses were solitary, supratentorial, and mostly lobar. In all cases, pathogens were anaerobic or facultative anaerobic germs (particularly streptococcus). No death was observed, but various sequels were present in up to two-thirds of the patients. We observed a recurrence of the cerebral abscess in 4 patients with a mean delay of 81 months. In comparison with the control group, cerebral abscesses were generally of later recurrence and significantly more often unique and less often due to staphylococcus. Conclusion HHT cerebral abscesses are particularly linked to pulmonary arteriovenous malformations and anaerobic germs. Their clinical, radiological and bacteriological characteristics are quite different than in a control group with more solitary brain localizations, no staphylococcus infection and a significantly longer interval to recurrence.

Polyneuropathy is a common presenting component of POEMS syndrome whose symptoms are attributed to an overproduction of vascular endothelial growth factor (VEGF). We report two female patients with POEMS syndrome presenting as a severe predominantly axonal neuropathy. A nerve biopsy was performed for these patients; pathological data confirmed unusual numerous acute axonal lesions associated with other classical signs of POEMS syndrome. POEMS syndrome is usually associated with demyelinating neuropathy (and secondary axonal loss); however, prominent axonal neuropathy (with acute axonal lesions on nerve biopsy) can also be observed in this disease. These observations illustrate the heterogeneity of peripheral nervous system involvement in POEMS syndrome.

Background: Patients with multiple sclerosis (MS) report sleep disturbances (SD) and excessive daytime sleepiness (EDS) more frequently than the general population. Our objectives were to evaluate SD and EDS in MS patients and to test the reliability of subjective sleep questionnaires. Methods: Demographic and clinical characteristics of unselected consecutive patients with MS were collected. Different questionnaires were used to assess quality of sleep, daytime sleepiness, fatigue, anxiety, depression and quality of life (QoL). Nocturnal polysomnography and Multiple Sleep Latency Test (MSLT) were performed in 25 selected MS patients with fatigue and with or without EDS. Results: 205 MS patients were enrolled. More than half of the MS patients were classified as ‘poor sleepers’. In multivariate analysis, SD were correlated with disability, fatigue, depression, QoL, and pain, but not with EDS. Subjective sleepiness evaluated with the Epworth Sleepiness Scale and SD with the Pittsburgh Sleep Quality Index were not correlated with the results of the objective assessments of vigilance (MSLT) and sleep. Conclusions: SD and EDS are frequent among MS patients. Objective assessment of vigilance and sleep can be challenging but MS patients who are poor sleepers should receive immediate assessment and treatment in order to improve QoL.

Comme le soulignait Michel Fardeau en 2010, « les travaux de Dejerine dans le domaine neuro-musculaire ne representent qu’une petite partie d’une œuvre impressionnante par son ampleur et par sa qualite puisqu’elle a resiste a l’œuvre du temps. Il n’y a pratiquement aucun chapitre de la clinique ou de l’anatomie du systeme nerveux central et peripherique auquel Dejerine n’ait apporte sa contribution ». En fait, assez tot dans sa carriere, Dejerine s’interessa au systeme nerveux peripherique. En 1875, il debuta des travaux au sein du « Laboratoire de pathologie comparee » du Pr Vulpian. L’une de ses premieres presentations porta sur l’etude de l’influence du courant electrique sur l’apparition des troubles trophiques secondaires a une lesion de nerf peripherique. La meme annee, il commenca a s’interesser a l’etude des processus physiopathologiques impliques dans la degenerescence wallerienne et s’appliqua a minutieusement decrire le processus de degenerescence distale du nerf peripherique moteur suite a la separation de son corps cellulaire (ou « centre trophique »). Par l’etude autopsique de patients presentant une amputation de membre ancienne, il confirma d’une part l’atrophie medullaire homolaterale au membre absent, mais montra aussi une alteration distale des nerfs du membre sectionne (a hauteur du moignon et ne remontant jamais jusqu’a la moelle epiniere. Dejerine et Vulpian etaient ainsi convaincus que cette atrophie medullaire n’etait que fonctionnelle, simple consequence de la suppression distale des troncs nerveux et non de l’extension d’un processus « irritatif » des nerfs et des muscles vers la moelle epiniere, contrairement a ce qui a ete propose par d’autres auteurs de l’epoque (tel que Friedreich au sujet de l’atrophie musculaire de type Aran-Duchenne). Il contribua, apres les travaux d’Octave Landry, a la description de neuropathies d’evolution aigue qui conduiront, plus tard, a l’individualisation du syndrome de Guillain-Barre-Strohl. Ainsi, en 1876, Dejerine et Goetz decrivirent le cas d’un patient ayant presente des paresthesies distales des 4 membres, de vives douleurs des membres inferieurs, puis une tetraplegie d’aggravation tres rapidement progressive sur 6 jours (dans un contexte febrile et associe a une retention urinaire aigue), aboutissant au deces du patient. L’autopsie constata une moelle epiniere indemne (tant macroscopiquement que microscopiquement) sur tout son long ; une seule racine anterieure cervicale fut etudiee, revelant une atrophie assez prononcee et une multiplication des noyaux du « tissu conjonctif inter-tubulaire » [9]. Le diagnostic de « paralysie ascendante aigue » fut alors propose par les auteurs. Les autopsies de certains autres malades le conduiront a constater la presence d’infiltrats de cellules inflammatoires au niveau des racines (1878), ce qui sera confirme en 1969 par Asbury et collaborateurs. Comparant les neuropathies d’evolution aigue a celles d’evolution chronique, Dejerine insista sur la difficulte qu’il y a a preciser les etiologies des neuropathies d’evolution chronique, ce qui s’avere etre encore une realite aujourd’hui. Par la suite, Dejerine et ses collaborateurs (1883) contribuerent a identifier des atteintes des nerfs peripheriques comme responsables « d’ataxies locomotrices » (« nervo-tabes ») qui etaient, jusqu’a ses descriptions, rapportees systematiquement a une « sclerose » des cordons posterieurs de la moelle epiniere des tabetiques. Dans le cadre d’autres concepts et syndromes concernant les neuropathies peripheriques, en particulier du fait de la realisation d’autopsies quasi systematiques, Dejerine s’affirma comme un pionnier, par exemple en demontrant que l’intensite lesionnelle de certaines « nevrites » diminuait a mesure que l’on remontait vers la racine des membres, ce qui correspond a ce qui fut decrit beaucoup plus tard comme une atteinte retrograde longueur-dependante (des axones), correspondant en Anglais au phenomene « length-dependant » de « dying back » ; il individualise et definit ce qu’il a appele la « nevrite interstitielle hypertrophique et progressive de l’enfance » qui sera reconnue par la suite comme le « syndrome de Dejerine-Sottas ». Par ailleurs, Dejerine, en association avec de prestigieux collaborateurs (comme son epouse Augusta Dejerine-Klumpke, Sottas, Andre-Thomas, et bien d’autres), rapporta de nombreuses observations d’atteinte du systeme nerveux peripherique, particulierement demonstratrices d’entites connues qu’il contribuera a mieux definir ; il s’agit des « paralysies par compression », des « nevrites peripheriques toxiques » et des « nevrites infectieuses », de plusieurs observations de « nevrite interstitielle hypertrophique », de cas de « paralysies faciales rhumatismales (dite « a frigore »), etc. Toutes ces publications et presentations sont enumerees chronologiquement et resumees dans ses interessantes (a lire) « epreuves de Titres et Travaux » publiees en 1895 et 1901. Notre presentation sera l’occasion de discuter et de rappeler ce qui caracterise, depuis les dernieres decennies, le syndrome de Dejerine-Sottas (« nevrite interstitielle hypertrophique »), de ses liens avec la maladie de Charcot-Marie-Tooth (qui curieusement avaient ete refutee par Dejerine lui-meme), et de sa signification et sa presence (utile ou non) dans la classification actuelle des neuropathies hereditaires, a l’ere de la biologie moleculaire. Par ailleurs, les travaux en microscopie electronique de Gruner (1960) et de Garcin et collaborateurs (1966) ont permis de montrer que l’hypertrophie nerveuse homogene de cette affection etait en rapport plus avec une proliferation schwanienne « tourbillonnante » (dite en « bulbes d’oignon ») autour des axones restants que d’une simple et intense augmentation du tissu conjonctif interstitiel. Il est donc evident que les travaux considerables de Dejerine ont ete determinants pour instituer, definir, et individualiser les pathologies des nerfs peripheriques ; la force de ses descriptions cliniques et neuropathologiques illustre une fois de plus l’importance de l’etude des correlations clinico-pathologiques qui furent par la suite souvent confirmees et mieux precisees du fait de l’apparition de techniques microscopiques complementaires plus performantes, comme la microscopie electronique.

As noted by Michel Fardeau in 2010: “les travaux de Dejerine dans le domaine neuromusculaire ne representent qu’une petite partie d’une œuvre impressionnante par son ampleur et par sa qualite puisqu’elle a resiste a l’œuvre du temps. Il n’y a pratiquement aucun chapitre de la clinique ou de l’anatomie du systeme nerveux central et peripherique auquel Dejerine n’ait apporte sa contribution” (“The studies of Dejerine on the neuromuscular system only make up a small part of an impressive body of work which has stood the test of time. He has in fact contributed to just about all clinical and anatomical aspects of the central and peripheral nervous systems”). Dejerine became interested in the peripheral nervous system rather early in his career. In 1875, it stemmed from work in the Laboratory of Comparative Pathology directed by Prof. Vulpian. One of his first presentations was a study of the influence of electric currents on the appearance of secondary trophic disorders in a peripheral nerve lesion. In the same year, he started to investigate the pathophysiological processes involved in Wallerian degeneration and made a more thorough study of the process of distal degeneration of the peripheral motor nerve following the dissection of its cellular body (’trophic center’). From autopsies of patients with a previous limb amputation, he confirmed the homolateral medullary atrophy in the missing limb, and also showed a distal deterioration of the nerves of the missing limb (at the stump and never backing up to the spinal cord). Dejerine and Vulpian were thus convinced that this medullary atrophy was simply functional, a consequence of the distal suppression of the nerve trunks and not an extension of an “irritating” process of the nerves and muscles towards the spinal cord, contrary to the descriptions of other authors at the time (such as Friedreich on muscular atrophy of the Aran-Duchenne type). Following on from the work of Octave Landry, he contributed descriptions of neuropathies of acute course, which later led to the entity of the Guillain-Barre-Strohl syndrome. Thus, in 1876, Dejerine and Goetz described the case of a patient presenting distal paraesthesias of all four limbs, sharp pains in the lower extremities, followed by a rapidly progressive quadriplegia over six days (along with fever and an urinary retention), leading to the death of the patient. On autopsy, the spinal cord was intact (both macroscopically and microscopically) throughout its length; a single anterior cervical root was studied, revealing a rather marked atrophy and a multiplication of “intertubular connective tissue” nodes. The authors proposed the diagnosis of “acute ascending paralysis”. The autopsies of other patients identified infiltrates of inflammatory cells in the nerve roots (1878) confirmed in 1969 by Asbury and colleagues. Comparing neuropathies of acute course with those of chronic course, Dejerine noted the difficulty in determining etiologies of neuropathies of chronic course. This is still true today. Thereafter, Dejerine and his collaborators (1883) helped show that impairments of peripheral nerves were responsible for “locomotor ataxias” which were thought at the time to be tabetic “sclerosis of the posterior columns”. With the thorough study of the various peripheral neuropathies, especially based on autopsy findings, Dejerine can be viewed as a pioneer in showing, for example, that nerve damage in “neuritis” decreased towards the limb roots (later identified as a length-dependant retrograde degeneration (of the axons) described in English as a “length-dependantdying back” process); he identified and defined what he called “hypertrophic and progressive interstitial neuritis of childhood”, now called Dejerine-Sottas syndrome. In addition, Dejerine (in collaboration with distinguished workers including his wife Augusta Dejerine-Klumpke, Sottas, Andre-Thomas, and numerous others) described cases of peripheral nervous system involvement that helped define known entities such as compression paralysis, toxic and infectious peripheral neuritis, several observations of “hypertrophic interstitial neuritis”, a case of “rheumatic facial palsy” (or “a frigore” facial palsy), etc. His publications and presentations are collected chronologically and summarized in the interesting “Epreuves de Titres and Travaux” published in 1895 and 1901. Our presentation will recap and discuss the characteristics of what is now called Dejerine-Sottas syndrome (hypertrophic interstitial neuritis), and its links with Charcot-Marie-Tooth (CMT) disease, which strangely was refuted by Dejerine himself, and of its significance and relevance to the current classification of hereditary neuropathies in the molecular biological age. In addition, the electron microscopic studies of Gruner (1960) and Garcin and colleagues (1966) demonstrated that the homogeneous nervous hypertrophy of this condition is more related to a swirling Schwann cell proliferation, referred to as “onion bulbs” around the remaining axons, rather than a simple and marked increase in interstitial connective tissue. The opus of Dejerine in determining, defining, and identifying peripheral nerve diseases along with the force of his clinical and neuropathologic descriptions illustrates the importance of clinical and pathological correlations. They have been subsequently confirmed and better defined using recent light and electron microscopic techniques.

Introduction: Amyloid neuropathy is a rare peripheral neuropathy that classically presents as a progressive sensory neuropathy with prominent autonomic involvement. Methods: We describe 5 patients with amyloid neuropathy (familial amyloid polyneuropathy or acquired amyloidosis) who were initially mistaken to have chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) based on history, clinical examination, electrodiagnostic studies, and cerebrospinal fluid (CSF) analysis. Results: The diagnosis of CIDP had been retained on clinical and electrophysiological grounds for all patients, but we observed no improvement after immunomodulatory treatment. Nerve biopsy confirmed amyloid deposits in nerves, and molecular genetic analysis showed a mutation of the transthyretin (V30M) gene for 3 patients; the 2 other patients had acquired amyloidosis. Conclusions: This report emphasizes the need to look for an alternative diagnosis in CIDP patients who do not respond to treatment and to look carefully for symptoms or signs of autonomic involvement in such patients. Muscle Nerve 45: 26–31, 2012

International audience; PURPOSE OF REVIEW: Immune-mediated neuropathies are increasingly recognized as potential causes of disability. Their diagnostic criteria and management options are still a matter of debate. This review focuses on recent findings in the diagnostic and therapeutic management of these disorders and on their prognostic factors. RECENT FINDINGS: The more widespread use of consensus criteria has improved diagnostic strategies for immune-mediated neuropathies. Studies of large prospective series have defined the spectrum of these disorders and their respective prognoses. Although treatment of these disorders mostly relies on corticosteroids and intravenous immunoglobulin, recent controlled trials have indicated the value of targeted immunotherapies. The further development of these therapies requires better understanding of the pathophysiology of these disorders. Immunological and immunopathological studies have improved our understanding of immune-mediated neuropathies over the last few years. SUMMARY: Identifying immune-mediated neuropathies is important, as these disorders are treatable. Diagnostic strategies have improved our capacity to recognize affected patients who can be offered effective treatments. Therapeutic options for immune-mediated neuropathies are still rather limited, although targeted immunotherapies may improve the prognosis of these disorders in the near future.

Resume Introduction Des travaux recents montrent l’implication de la vitamine D dans la sclerose en plaques (SEP). Methodes Dans un travail pluricentrique regional (region Poitou-Charentes) realise pendant le premier trimestre 2010, nous avons dose la taux plasmatique de 25-OH vitamine D (25-OH vit D) a 170 SEP recrutees consecutivement, et 170 sujets temoins apparies en âge (± 4 ans), en sexe et en date de prelevement. Les objectifs de notre travail etaient de comparer les taux plasmatiques de 25-OH vit D dans le groupe SEP et dans le groupe temoin et de rechercher une correlation entre le taux plasmatique de 25-OH vit D et la forme de SEP, le handicap, le taux annualise de poussees de l’annee precedente dans les formes remittentes et la presence de lesions prenant le gadolinium sur une IRM datant de moins de 12 mois. Resultats Le taux plasmatique de vitamine D est effondre dans la population SEP et s’avere significativement plus bas (14,5 ± 9,2 mcg/mL) que dans le groupe temoin (16,7 ± 9,6 mcg/mL). Ce taux plasmatique est inversement correle avec le handicap mesure par le score EDSS et s’avere significativement plus bas dans les formes remittentes secondairement progressives (RR-SP) et dans les formes progressives primaires (PP) que dans les formes remittentes (RR). Il n’a pas ete retrouve de correlation entre le taux plasmatique de 25-OH vit D et le taux annualise de poussees de l’annee precedente dans les formes RR et la presence de lesions prenant le gadolinium sur l’IRM. Conclusion Le taux plasmatique de vitamine D est effondre dans les SEP, principalement les formes RR-SP et PP, et est correle au handicap. Cela incite a une detection, puis a une correction systematique de la carence en vitamine D chez les patients atteints de SEP.

With all the results now obtained by global helioseismology, it is clear that we have to go beyond the classical spherical modelling of the Sun. In this way, it is now necessary to draw a picture that takes into account internal dynamical processes such as rotation, magnetic field, and waves from the core to the surface. This is a challenging task since such mechanisms involve length and time-scales that differ from several orders of magnitude and impact on the solar behaviour both on dynamical and secular times. In this review, I will thus describe the present state of the art in the modelling of the internal dynamics of the Sun. (C) 2010 WILEY-VCH Verlag GmbH&Co. KGaA, Weinheim

Introduction Les mutations du gene VCP peuvent-etre a l’origine de diverses manifestations neuromusculaires [1] , [2] , [3] . Nous presentons le cas de trois patients suivis au CHU de Bordeaux, porteurs de VCP-opathies dont les aspects electrocliniques different, temoignant de l’heterogeneite des manifestations cliniques de cette pathologie. Observations Mr D., 55 ans, presentait une fatigabilite a la marche depuis quelques mois. L’examen clinique retrouvait une amyotrophie paravertebrale lombaire droite, un deficit moteur asymetrique des 4 membres, un decollement de l’omoplate droite et des reflexes vifs. L’ENMG montrait de nombreuses activites de repos, sans anomalie des conductions, orientant vers un diagnostic de SLA. Devant une scapula alata devenue bilaterale, un nouvel ENMG fut pratique, retrouvant un trace « myogene ». La biopsie musculaire montrait des vacuoles bordees orientant la recherche genetique de la mutation VCP. Mr C., 57 ans, presentait un deficit d’abduction des epaules asymetrique et un steppage droit, l’ensemble de ces symptomes etant apparus progressivement vers 45 ans. L’ENMG revelait un trace « myogene » dans certains muscles, « neurogene » dans d’autres, evoquant un processus myopathique chronique. La biopsie musculaire retrouvait des vacuoles bordees orientant vers la recherche de mutations du gene VCP. Mme B., 65 ans se plaignait de crampes des mollets depuis plusieurs d’annees. Cliniquement, on observait des pieds creux et un syndrome tetrapyramidal, mais l’IRM cerebromedullaire initiale etait normale. L’ENMG retrouvait une discrete atteinte sensitive d’allure demyelinisante. La recherche genetique de paraplegie spastique familiale etait negative. Une nouvelle IRM medullaire a revele des signes evocateurs d’une maladie de Paget ayant motive la recherche de mutations du gene VCP. Conclusion Les VCP-opathies peuvent revetir des aspects cliniques et electroneuromyographiques heterogenes, frequemment responsables d’une errance diagnostique. Poser un diagnostic de VCP-opathie represente donc un challenge : c’est souvent l’association d’une maladie de Paget ou d’une DFT a une atteinte neurologique (d’allure centrale ou peripherique) qui doit nous inciter a la rechercher.

Context. Differential rotation has a strong influence on stellar internal dynamics and evolution, notably by triggering hydrodynamical instabilities, by interacting with the magnetic field, and more generally by inducing transport of angular momentum and chemical elements. Moreover, it modifies the way waves propagate in stellar interiors and thus the frequency spectrum of these waves, the regions they probe, and the transport they generate. Aims. We investigate the impact of a general differential rotation (both in radius and latitude) on the propagation of axisymmetric gravito-inertial waves. Methods. We use a small-wavelength approximation to obtain a local dispersion relation for these waves. We then describe the propagation of waves thanks to a ray model that follows a Hamiltonian formalism. Finally, we numerically probe the properties of these gravito-inertial rays for different regimes of radial and latitudinal differential rotation. Results. We derive a local dispersion relation that includes the effect of a general differential rotation. Subsequently, considering a polytropic stellar model, we observe that differential rotation allows for a large variety of resonant cavities that can be probed by gravito-inertial waves. We identify that for some regimes of frequency and differential rotation, the properties of gravito-inertial rays are similar to those found in the uniformly rotating case. Furthermore, we also find new regimes specific to differential rotation, where the dynamics of rays is chaotic. Conclusions. As a consequence, we expect modes to follow the same trend. Some parts of oscillation spectra corresponding to regimes similar to those of the uniformly rotating case would exhibit regular patterns, while parts corresponding to the new regimes would be mostly constituted of chaotic modes with a spectrum rather characterised by a generic statistical distribution., Comment: 17 pages, 12 figures, accepted for publication in A&A

The understanding of fossil fields origin, topology, and stability is one of the corner stones of the stellar magnetism theory. On one hand, since they survive on secular time scales, they may modify the structure and the evolution of their host stars. On the other hand, they must have a complex stable structure since it has been demonstrated that the simplest purely poloidal or toroidal fields are unstable on dynamical time scales. In this context, the only stable stellar configurations found today are those resulting from numerical simulations by Braithwaite and collaborators who studied the evolution of an initial stochastic magnetic field, which relaxes with a selective decay of magnetic helicity and energy, on mixed stable configurations (poloidal and toroidal) that seem to be in equilibrium and then diffuse. In this talk, we report the semi-analytical investigation of such an equilibrium field in the axisymmetric case. We use variational methods, which describe selective decay of magnetic helicity and energy during MHD relaxation, and we identify a supplementary invariant due to the stable stratification of stellar radiation zones. This leads to states that generalize force-free Taylor's relaxation states studied in plasma laboratory experiments that become non force-free in the stellar case. Moreover, astrophysical applications are presented and the stability of obtained configurations is studied.

International audience

Introduction Les mutations du gene VCP peuvent etre a l’origine de diverses manifestations neuromusculaires. Nous presentons le cas de quatre patients porteurs d’un phenotype clinique atypique. Observation M. C., 57 ans, presentait un deficit d’abduction de l’epaule initialement asymetrique, puis bilateral, avec steppage droit, sans atteinte faciale, apparus progressivement vers 45 ans. La recherche genetique FSHD 1 etait negative. La biopsie musculaire retrouvait des vacuoles bordees orientant la recherche de la mutation VCP, positive. M. D., 55 ans, presentait une fatigabilite a la marche. L’examen clinique retrouvait une amyotrophie avec un deficit moteur asymetrique, un decollement de l’omoplate droite et des reflexes vifs. L’EMG etait evocateur de SLA. Devant un decollement scapulaire devenu bilateral, la mutation FSHD1 fut demandee, negative. La biopsie musculaire montrait des vacuoles bordees orientant la recherche genetique de la mutation VCP, positive. Mme B., 65 ans exprimait un tableau de paraplegie spastique. La recherche de la paraplegie spastique familiale et de la maladie de Friedreich etaient negatives. L’IRM medullaire retrouvait des signes evocateurs d’une maladie de Paget. La recherche genetique pour la mutation VCP est revenue positive. M. G., 38 ans, presentait un deficit de la ceinture scapulaire avec difficulte a lever les bras. On retrouvait la meme symptomatologie dans sa famille evoquant une transmission autosomique dominante. La biopsie musculaire a retrouve des vacuoles bordees orientant l’analyse genetique de la mutation VCP, positive. Discussion L’alteration du gene VCP est a l’origine d’une proteinopathie systemique dont les manifestations cliniques apparaissent proteiformes : myogenes, neurogenes et centrales. L’expression clinique musculaire des VCP-opathies prend le plus souvent l’aspect d’une dystrophie musculaire des ceintures. Les tableaux scapulo-peronier ou de paraplegie spastique, comme ceux que nous rapportons, paraissent plus rares. Conclusion Devant tableau clinique FSH-like ou de paraparesie spastique, il faut savoir evoquer le diagnostic de myopathie a VCP responsables de diverses manifestations neuromusculaires.

Resume Introduction L’atteinte neoplasique du muscle est une entite assez peu repandue en myologie et les metastases musculaires, pour des raisons mal determinees, sont assez peu observees. Methode Il s’agit d’une etude retrospective portant sur l’analyse de la litterature medicale et l’observation d’un cas original de metastase musculaire revele par une « incapacite a ouvrir la bouche » chez un sujet âge de 58 ans (metastase du muscle pterygoidien medial droit secondaire a un adenocarcinome du tiers inferieur de l’oesophage). Resultats Apres etude de la litterature medicale, nous avons recense 174 cas (dont notre observation). La decouverte d’une lesion musculaire de type « adenocarcinome » orientait prioritairement vers l’appareil digestif (35 %), le rein (20 %), ainsi que le poumon chez l’homme (18 %) et la sphere gynecologique chez la femme (23,5 % des adenocarcinomes de la femme). La decouverte d’une lesion musculaire de type « carcinome epidermoide » orientait prioritairement vers le poumon chez l’homme (81 %) et le col uterin chez la femme (64 %). Conclusion Il est important d’evoquer la possibilite d’une metastase musculaire devant l’apparition d’une lesion musculaire focale, douloureuse, notamment en cas d’alteration de l’etat general. En fonction de l’histologie de la metastase et de l’immunohistochimie, il est possible d’orienter rapidement les recherches vers le cancer primitif.

Aims: To perform a large and detailed epidemiologic study on restless legs syndrome (RLS) during pregnancy in a European country. Methods: A cross-sectional questionnaire survey. The questionnaire was distributed by the medical staff in different outpatient waiting rooms (obstetrics and gynecology department of the university hospital, obstetrics and gynecology department of a private clinic, private midwives, private obstetrician-gynecologists, radiological centers before fetal ultrasound examination and general practitioners) in a French town and its surrounding area (200,000 inhabitants): A woman was considered affected if she met the International RLS Study Group criteria for RLS diagnosis. Results: 1,022 pregnant women living in a French town were included. 24% of women were affected by RLS during their pregnancy. The disease was strongly related to the third trimester of pregnancy and had a significant impact on sleep leading to severe nocturnal and diurnal consequences with a high consumption of sleep medication. Conclusions: RLS affects one quarter of pregnant women, essentially during the third trimester and represents an important public health issue with sleep medication intake.

Aims: To describe the frequency of restless legs syndrome (RLS) in a French population of randomly selected women during their third trimester of pregnancy and its evolution up to 3 months after delivery and to identify potential factors associated with the improvement of RLS after delivery. Methods: A cross-sectional questionnaire survey. Results: 186 pregnant women living in a French town were included. 32% of women were affected by RLS during the third trimester of their pregnancy. Multiple pregnancies and iron intake during pregnancy were significantly associated with RLS during the third trimester. RLS disappeared after delivery among 64.8% of the women, and by less than 2 weeks after delivery in half of them. This improvement after delivery was not associated with the number of previous pregnancies, the RLS severity and iron intake during pregnancy, peridural anaesthesia, caesarean section, delivery complications, newborn weight, breastfeeding, dopaminergic agent intake after delivery, and with the absence of RLS before pregnancy. Conclusions: RLS affects one third of pregnant women during their third trimester and usually improves after delivery. Although there is no allowed treatment, most of the time only counselling and iron status assessment should be provided.

We review the interaction in intermediate and high mass stars between their evolution and magnetic and chemical properties. We describe the theory of Ap-star `fossil' fields, before touching on the expected secular diffusive processes which give rise to evolution of the field. We then present recent results from a spectropolarimetric survey of Herbig Ae/Be stars, showing that magnetic fields of the kind seen on the main-sequence already exist during the pre-main sequence phase, in agreement with fossil field theory, and that the origin of the slow rotation of Ap/Bp stars also lies early in the pre-main sequence evolution; we also present results confirming a lack of stars with fields below a few hundred gauss. We then seek which macroscopic motions compete with atomic diffusion in determining the surface abundances of AmFm stars. While turbulent transport and mass loss, in competition with atomic diffusion, are both able to explain observed surface abundances, the interior abundance distribution is different enough to potentially lead to a test using asterosismology. Finally we review progress on the turbulence-driving and mixing processes in stellar radiative zones., Proceedings of IAU GA in Rio, JD4 on Ap stars; 10 pages, 7 figures

International audience; Context: We examine the dynamics of low-frequency waves in differentially rotating stellar radiation zones, the angular velocity being taken as generally as possible depending both on radius and on latitude in stellar interiors. The associated induced transport of angular momentum, which plays a key role in the evolution of rotating stars, is derived. Aims: We focus on the wave-induced transport of angular momentum, taking into account the Coriolis acceleration in the case of strong radial and latitudinal differential rotation. We thus go beyond the ``weak differential rotation'' approximation, where rotation is almost a solid-body one plus a residual radial differential rotation. As has been shown in previous works, the Coriolis acceleration modifies such transport. Methods: We built analytically a complete formalism that allows the study of rotational transport in stellar radiation zones taking into account the wave action modified by a general strong differential rotation. Results: The different approximations possible for low-frequency waves in a differentially rotating stably stratified radiative region, namely the traditional and the JWKB approximations, are examined and discussed. The complete bidimensional structure of regular elliptic gravito-inertial waves, which verify these approximations, is derived and compared to those in the ``weak differential rotation'' case. Next, associated transport of energy and of angular momentum in the vertical and in the horizontal directions and the dynamical equations, respectively for the mean radial differential rotation and the latitudinal one, are obtained. Conclusions: The complete formalism, which takes into account low-frequency wave action, is derived and can be used for the study of secular hydrodynamics of radiative regions and of the associated mixing. The modification of waves due to general strong differential rotation and their feed-back on the angular momentum transport are treated rigourously. In a forthcoming paper (Paper II), this formalism will be applied to the case of solar differential rotation. However, the case of hyperbolic gravito-inertial waves should be carefully studied.

La douleur oculaire represente une manifestation rare des accidents vasculaires cerebraux (AVC). Nous rapportons le cas d’un patient âge de 65 ans qui a presente un accident ischemique lateroprotuberantiel posterieur gauche (explore par imagerie encephalique et electrophysiologie), dont la manifestation initiale etait une douleur de la partie posterieure de la paupiere inferieure gauche, completee par des troubles sensitifs controlateraux epargnant la face. Il s’agissait d’une forme peu frequente de syndrome alterne du tronc cerebral.

We study the impact on the stellar structure of a large-scale magnetic field in stellar radiation zones. The field is in magneto-hydrostatic (MHS) equilibrium and has a non force-free character, which allows us to study its influence both on the mechanical and and on the energetic balances. This approach is illustrated in the case of an Ap star where the magnetic field matches at the surface with an external potential one. Perturbations of the stellar structure are semi-analytically computed. The relative importance of the magnetic physical quantities is discussed and a hierarchy, aiming at distinguishing various refinement degrees in the implementation of a large-scale magnetic field in a stellar evolution code, is established. This treatment also allows us to deduce the gravitational multipolar moments and the change in effective temperature associated with the presence of a magnetic field.

International audience; Internal gravity waves constitute an efficient process for angular momentum transport over large distances. They are now seen as an important ingredient in understanding the evolution of stellar rotation and can explain the Sun's quasi-flat internal-rotation profile. Because the Sun's rotation frequency is of the same order as that of the waves, it is now necessary to refine our description of wave propagation and to take into account the action of the Coriolis acceleration in a coherent way. To achieve this goal, we adopt the traditional approximation, which can be applied to stellar radiation zones under conditions that are given. We present the modified transport equations and their numerical evaluation in a parameter range that is significant for the Sun. Consequences for the transport of angular momentum inside solar and stellar radiative regions are discussed.

Resume Introduction L'histiocytose langerhansienne est une pathologie a developpement multivisceral. Une atteinte neurologique est possible mais peu frequente. Exegese Nous rapportons un cas de tumeur intracerebrale prerolandique droite revelee par une crise epileptique partielle motrice chez un homme âge de 31 ans. L'analyse anatomopathologique retrouvait une histiocytose langerhansienne (avec expression immunohistochimique par les cellules de Langerhans de la proteine S100 et de CD1a) dont le bilan d'extension fut negatif. L'evolution etait favorable apres resection chirurgicale complete. Une revue exhaustive de la litterature de ces formes pseudotumorales cerebrales de l'histiocytose ainsi qu'une discussion des manifestations neurologiques de cette maladie a ete realisee. Conclusion L'histiocytose langerhansienne peut se reveler par une lesion neurologique cerebrale focale.

Resume Propos La radiotherapie peut se compliquer de manifestations neurologiques, precoces ou tardives, de localisation centrale ou peripherique. Methodes Nous rapportons trois cas de neuropathie motrice pure observes plusieurs annees apres une radiotherapie pour lymphome de Hodgkin ou seminome testiculaire. Resultats Trois patients (une femme et deux hommes) âges de 35 a 65 ans ont presente une neuropathie motrice pure dans un delai de 8,5 a 21 ans apres une radiotherapie pour lymphome de Hodgkin (deux cas) ou seminome testiculaire (un cas). Dans tous les cas, il s'agissait d'un deficit proximodistal, a nette predominance proximale, confirme par l'electroneuromyogramme. Apres une phase d'installation progressive, l'evolution s'est faite vers la stabilite avec un traitement par anticoagulant dans un cas et par pentoxifylline dans deux cas. Conclusion La neuropathie motrice pure represente une complication rare, et generalement tardive, de la radiotherapie. En l'absence de consensus therapeutique, un traitement par anticoagulant ou par pentoxifylline, en association a du tocopherol, a ete propose.

Resume Introduction Le syndrome de Wolfram est une maladie genetique, a transmission autosomique recessive, associant un diabete sucre juvenile et une atrophie optique bilaterale. Observation Nous rapportons le cas d’un patient âge de 47 ans pour lequel nous avons porte le diagnostic de syndrome de Wolfram devant l’apparition d’un tableau neurologique tardif associant ataxie et nystagmus horizontal bilateral. L’IRM encephalique revelait une atrophie majeure du tronc cerebral et du cervelet. Conclusion Le syndrome de Wolfram est une pathologie rare, dont l’issue est fatale avant l’âge de 50 ans dont le diagnostic doit etre evoque devant l’association d’un diabete sucre juvenile et d’une atrophie optique, en particulier en presence d’autres symptomes tels qu’ataxie, hypoacousie, diabete insipide, troubles neuro-psychiatriques ou troubles vesico-renaux.

Background Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration. Methods Eighty-six patients with acute/subacute inflammatory SNN (paraneoplastic, associated with dysimmune diseases, or idiopathic) were retrospectively studied. The monthly SNAP reduction was determined and normalized with the lower limit of normal. Disability progression was expressed by the modified Rankin score and correlated with SNAP reduction. Results The monthly SNAP reduction was similar in the four limbs although the median nerve was less severely affected. The monthly SNAP reduction was very severe within the first two months of evolution, began to slow down after seven months, and stabilized after ten months. It was tightly correlated with disability progression. Kaplan–Meier analysis showed that the median time until matching the diagnostic criteria of SNN was 8.5 months. Within this period, 42% of nerves remained excitable. Conclusions Developing treatment aiming at the stabilization of SNN is possible within the first 8 months of evolution. An improvement of the disease is possible if patients are treated within two months, which needs an early referral to an expert center and ENMG testing of the radial and ulnar nerves, which are most sensitive to changes.

International audience; Charcot-Marie-Tooth (CMT) diseases represent a heterogeneous genetic disorder (more than 80 genes are implicated in these inherited neuropathies), but sharing a similar phenotype. In recent years, advances in molecular genetics and molecular biology, and also the development of various animal models of CMT, have led to a better understanding. Taken together, this knowledge represents a prerequisite for the development of future therapies in CMT, and in peripheral nervous system disorders in general. The efficacy of various substances has been shown in vitro and also in vivo (in animal models); but, no significant positive effect has yet been confirmed in humans. However, some of these trials are still in development, and we may expect positive results in the future. Although CMT is still an incurable disease, symptomatic treatments (physiotherapy, surgery, analgesic, etc.) are crucial to improve the quality of life of CMT patients.

Since 1995, more than 1500 exoplanets have been discovered around a large diversity of host stars (from M- to A-type stars). Tidal dissipation in stellar convective envelopes is a key actor that shapes the orbital architecture of short-period systems. Our objective is to understand and evaluate how tidal dissipation in the convective envelope of low-mass stars (from M to F types) depends on their mass, evolutionary stage and rotation. Using a simplified two-layer assumption, we compute analytically the frequency-averaged tidal dissipation in their convective envelope. This dissipation is due to the conversion into heat of the kinetic energy of tidal non wave-like/equilibrium flow and inertial waves because of the viscous friction applied by turbulent convection. Using grids of stellar models allows us to study the variation of the dissipation as a function of stellar mass and age on the Pre-Main-Sequence and on the Main-Sequence for stars with masses spanning from $0.4$ to $1.4M_{\odot}$. As shown by observations, tidal dissipation in stars varies over several orders of magnitude as a function of stellar mass, age and rotation. During their Pre-Main-Sequence, all low-mass stars have an increase of the frequency-averaged tidal dissipation for a fixed angular velocity in their convective envelope until they reach a critical aspect and mass ratios. Next, the dissipation evolves on the Main Sequence to an asymptotic value that becomes maximum for $0.6M_{\odot}$ K-type stars and that decreases by several orders of magnitude with increasing stellar mass. Finally, the rotational evolution of low-mass stars strengthens the importance of tidal dissipation during the Pre-Main-Sequence for star-planet and multiple star systems., Comment: 5 pages, 4 figures, accepted for publication as a Letter in Astronomy & Astrophysics

Resume Introduction Le zona est une pathologie secondaire a une reactivation du varicella-zoster virus (VZV), frequent dans la population generale. Etat des connaissances Le zona est susceptible d’entrainer de nombreuses complications, parmi lesquelles des manifestations neurologiques peripheriques ou centrales de gravite variable. Dans tous les cas, et particulierement concernant les complications neurologiques, un traitement associant un antiviral ainsi que des mesures locales et antalgiques doit etre debute le plus precocement possible. Une corticotherapie peut etre proposee, en particulier en cas de syndrome de Ramsay-Hunt ou de complications neurologiques centrales dont le pronostic fonctionnel et vital reste reserve. Conclusion Le zona est une pathologie virale banale, mais potentiellement grave dans certaines situations. La detection et la precocite du traitement sont gages d’une diminution de la severite des complications neurologiques.

The seismology and physics of localized structures beneath the surface of the Sun takes on a special significance with the completion in 2006 of a solar cycle of observations by the ground-based Global Oscillation Network Group (GONG) and by the instruments on board theSolar and Heliospheric Observatory(SOHO). Of course, the spatially unresolved Birmingham Solar Oscillation Network (BiSON) has been observing for even longer. At the same time, the testing of models of stellar structure moves into high gear with the extension of deep probes from the Sun to other solar-like stars and other multi-mode pulsators, with ever-improving observations made from the ground, the success of theMOSTsatellite, and the recently launchedCoRoTsatellite. Here we report the current state of the two closely related and rapidly developing fields of helio- and asteroseimology.

International audience; Inherited white matter diseases are rare and heterogeneous disorders usually encountered in infancy. Adult-onset forms are increasingly recognized. Our objectives were to determine relative frequencies of genetic leukoencephalopathies in a cohort of adult-onset patients and to evaluate the effectiveness of a systematic diagnostic approach. Inclusion criteria of this retrospective study were: (i) symmetrical involvement of white matter on the first available brain MRI; (ii) age of onset above 16 years. Patients with acquired diseases were excluded. Magnetic resonance imaging analysis identified three groups (vascular, cavitary and non-vascular/non-cavitary) in which distinct genetic and/or biochemical testing were realized. One hundred and fifty-four patients (male/female = 60/94) with adult-onset leukoencephalopathies were identified. Mean age of onset was 38.6 years. In the vascular group, 41/55 patients (75%) finally had a diagnosis [including CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, n = 32) and COL4A1 mutation, n = 7]. In the cavitary group, 13/17 (76%) patients had a diagnosis of EIF2B-related disorder. In the third group (n = 82), a systematic biological screening allowed a diagnosis in 23 patients (28%) and oriented direct genetic screening identified 21 additional diseases (25.6%). Adult-onset genetic leukoencephalopathies are a rare but probably underestimated entity. Our study confirms the use of a magnetic resonance imaging-based classification with a final diagnosis rate of 64% (98/154) cases.

Resume Les statines sont devenues un des premiers postes budgetaires en France tant leur champ de prescription s’est elargi depuis ces cinq dernieres annees sous l’impulsion d’etudes prospectives multicentriques. Ainsi, outre leur vertu preventive dans les affections coronariennes, il semble actuellement demontre qu’elles protegent egalement de la survenue d’accidents vasculaires cerebraux (AVC) chez les sujets coronariens (etudes CARE et LIPID) comme chez les sujets a risque (etudes HPS et ASCOT-LLA), et ce, quel que soit le taux plasmatique de cholesterol avec une diminution de 30 p. 100 environ du risque relatif d’AVC par rapport au placebo. Cette vertu preventive s’exerce a priori par d’autres voies que la diminution du cholesterol et/ou du LDL-cholesterol, probablement par un effet direct sur l’inflammation, la coagulation et la modulation de certains processus atherogenes et de la fonction endotheliale. Neanmoins, il reste a l’heure actuelle, de nombreuses zones d’incertitudes quant au role exact des statines sur la prevention secondaire des AVC (etude SPARCL), a leur utilite reelle chez le sujet âge (etude PROSPER) et a la superiorite eventuelle d’une statine par rapport a l’autre.

Introduction La classification des maladies neurogenetiques s’est complexifiee en raison d’une incrementation numerique systematique au fur et a mesure de la decouverte de nouveaux genes, sans reference claire au phenotype et au mode de transmission. Objectifs Identifier les difficultes liees a la classification actuelle et proposer des modifications simples et utiles aux praticiens, en tenant compte pour chaque maladie, du mode de transmission, du phenotype, et du gene implique. Patients et methodes Suite a des discussions informelles et apres analyse de la litterature, nous avons conclu que la classification des maladies neurogenetiques etait percue comme trop complexe. Lors de sessions de travail, nous avons identifie comme elements cles d’une classification utile, le mode de transmission, le phenotype clinique et les donnees de la genetique moleculaire. Nous proposons sur ces bases une classification qui peut notamment s’appliquer aux neuropathies, aux ataxies cerebelleuses et aux paraparesies spastiques hereditaires. Resultats Pour les maladies de Charcot-Marie-Tooth, nos propositions aboutiraient a transformer par exemple CMT1A (forme dominante demyelinisante par duplication de la PMP22) en AD-CMTde-PMP22dup. De meme, l’ataxie spinocerebelleuse de type 1 (SCA1 dominante par mutation de ATXN1) deviendrait AD-CA-ATXN1, et la forme dominante de paraparesie spastique liee au gene ATL1 (SPG3A) deviendrait AD-SPG-ATL1. Pour les phenotypes dits d’ataxie spastique, l’acronyme SPAX pourrait etre utilise dans les memes conditions. Discussion Nos propositions simplifient grandement la classification des maladies neurogenetiques en introduisant le mode de transmission, des informations phenotypiques basiques, et les donnees de la genetique moleculaire. Elles permettraient d’eviter d’avoir a se referer a des tableaux de correspondance, facilitant le dialogue entre cliniciens, neurogeneticiens et biologistes. En outre, cette classification peut s’incrementer d’elle-meme au fil des decouvertes genetiques. Conclusion Les maladies neurogenetiques sont complexes et leur classification actuelle ajoute a la confusion pour les cliniciens. Nous proposons une classification de ces maladies plus simple, comprehensible, et aisement actualisable.

A 45-year-old man presented with an 11-year history of severe refractory chronic inflammatory demyelinating polyneuropathy (CIDP). Clinical examination revealed distal weakness of all 4 limbs, ataxia, facial diplegia, dysphonia, and facial sensory disturbance. Several therapeutics were tried: IV immunoglobulins (IVIg), corticosteroids, plasma exchange, cyclophosphamide, and mycophenolate mofetil. Anti-NF155 immunoglobulin G4 (IgG4) antibodies were detected by ELISA (1:2,000). Brain MRI revealed diffuse hypertrophy of multiple cranial nerves (figure).

We estimate the amount of angular momentum transferred by the low-frequency oscillations detected in the rapidly rotating hot Be star HD 51452. Here, we assume that the oscillations detected are stochastically excited by convective motions in the convective core of the star, that is, we treat the oscillations as forced oscillations excited by the periodic convective motions of the core fluids having the frequencies observationally determined. With the observational amplitudes of the photometric variations, we determine the oscillation amplitudes, which makes it possible to estimate the net amount of angular momentum transferred by the oscillations using the wave-meanflow interaction theory. Since we do not have any information concerning the azimuthal wavenumber $m$ and spherical harmonic degree $l$ for each of the oscillations, we assume that all the frequencies detected are prograde or retrograde in the observer's frame and they are all associated with a single value of $m$ both for even modes ($l=|m|$) and for odd modes ($l=|m|+1$). We estimate the amount of angular momentum transferred by the oscillations for $|m|=1$ and 2, which are typical $|m|$ values for Be stars, and find that the amount is large enough for a decretion disc to form around the star. Therefore, transport of angular momentum by waves stochastically excited in the core of Be stars might be responsible for the Be phenomenon., 9 pages, 6 figures

Asteroseismology and spectropolarimetry have allowed us to progress significantly in our understanding of the physics of hot stars over the last decade. It is now possible to combine these two techniques to learn even more information about hot stars and constrain their models. While only a few magnetic pulsating hot stars are known as of today and have been studied with both seismology and spectropolarimetry, new opportunities - in particular Kepler2 and BRITE - are emerging and will allow us to rapidly obtain new combined results., proceedings of the IAU Symposium 307 held in Geneva in June 2014

The most common clinical manifestations following ymenoptera stings are local reactions; the occurrence of severe llergic reactions is less frequent [1]. Non allergic reactions intravascular thrombolysis, renal failure, rhabdomyolysis and yspnea) are rare, and strokes are particularly uncommon [2]. e are reporting on a patient with both hemorrhagic (cerebral nd subarachnoid) and ischemic strokes following multiple Asian lack hornet stings.

Tidal dissipation is known as one of the main drivers of the secular evolution of planetary systems. It directly results from dissipative mechanisms that occur in planets and stars' interiors and strongly depends on the structure and dynamics of the bodies. This work focuses on the mechanism of viscous friction in stars and planetary layers. A local model is used to study tidal dissipation. It provides general scaling laws that give a qualitative overview of the different possible behaviors of fluid tidal waves. Furthermore, it highlights the sensitivity of dissipation to the tidal frequency and the roles played by the internal parameters of the fluid such as rotation, stratification, viscosity and thermal diffusivity that will impact the spins/orbital architecture in planetary systems., 4 pages, 3 figures, IAU 310 symposium proceedings, Complex planetary systems

In this article, we show how asteroseismology and spectropolarimetry allow to probe dynamical processes in massive star interiors. First, we give a summary of the state-of-the-art. Second, we recall the MHD mechanisms that take place in massive stars. Next, we show how asteroseimology gives strong constraints on the internal mixing and transport of angular momentum while spectropolarimetry allows to unravel the role played by magnetic fields., 5 pages, 1 figure, IAU 307 symposium proceedings, New windows on massive stars: asteroseismology, interferometry, and spectropolarimetry, Eds. G. Meynet, C. Georgy, J. H. Groh, and P. Stee