Actualité de la prise en charge des mouvements anormaux chez l’enfant : la dystonie

2,48 x 1,25 x 1,05 metres de bronze.Díaz, Lautaro (escultor)Pla mig de l'obra. A la part superior d'unes escales que voregen la mar, hi ha un home i una dona de bronze asseguts plàcidament. El que li interessa és transmetre una sensació de tranquil·litat que inciti a contemplar el paisatge

Identification de substrats arythmogènes et des mécanismes de décompensation dans une population de tétralogie de Fallot à l’âge adulte et perspectives de prise en charge ultérieure

The number of adults with a repaired tetralogy of Fallot is increasing. In the past, those patients were considered healed. Nonetheless, they present arrhythmogenic issues, with frequent sudden death, and mechanical complications: right ventricular dilation due to long lasting pulmonary valve regurgitation, secondary to surgical repair. The origin of arrhythmia and its interaction with right ventricular dysfunction is only partially understood. In this study, combining clinical with experimental data, we pointed out: 1) concerning the follow-up of this population, echocardiography is not a substitute to MRI 2) operative mortality of pulmonary valve replacement (PVR) still exists 3) a stentless bioprosthesis represents a valid solution for PVR 4) a valve repair is mandatory for severe tricuspid valve regurgitation at PVR 5) the genetic analysis carried out in an animal model of repaired tetralogy of Fallot, demonstrated the involvement of numerous genes in right ventricular remodeling 6) remodeling of the right ventricle in this animal model generates pro-arrhythmic substrate. Heart failure mechanisms in repaired tetralogy of Fallot are complex: a link between right ventricular dysfunction and arrhythmias is demonstrated. Further studies are needed to investigate other pro-arrhythmic mechanisms involving the left ventricle.Le nombre d’adultes porteurs d’une tétralogie de Fallot opérée dans l’enfance est en constante augmentation. Initialement, ces patients étaient considérés comme guéris. A l’âge adulte, ils présentent en fait des complications d’ordre rythmique, responsables de morts subites, et des complications d’ordre mécanique : dilatation du ventricule droit (VD) liée à l’insuffisance pulmonaire chronique, séquellaire de la première chirurgie de réparation de la cardiopathie. Les mécanismes de l’arythmie ainsi qu’une éventuelle interaction entre la dysfonction VD et la survenue de ces arythmies ne restent que partiellement élucidés. Dans ce travail, en couplant les données d’études cliniques et les données expérimentales issues d’un modèle animal (MA) mimant une tétralogie de Fallot réparée, nous avons montré que 1) l’échocardiographie ne pouvait pas se substituer à l’IRM pour la surveillance des patients avec tétralogie de Fallot réparée 2) la valvulation pulmonaire restait une intervention à risque de mortalité 3) une bioprothèse non stentée était une bonne solution pour effectuer cette valvulation 4) en cas de fuite tricuspidienne sévère lors de cette valvulation, une plastie était indispensable 5) plusieurs gènes participaient au remodelage ventriculaire droit (analyse génétique effectuée sur le MA) 6) le remodelage électrophysiologique du VD (MA) s’accompagnait de propriétés pro-arythmogènes. Les mécanismes de décompensation sont intriqués : un lien entre dysfonction VD et arythmie paraît bien établi. D’autres analyses électrophysiologiques sont en cours au niveau du ventricule gauche (MA), pour rechercher d’autres mécanismes pro-arythmogènes

Denis-Constant MARTIN : Sounding the Cape. Music, Identity and Politics in South Africa

Il manquait un ouvrage de référence retraçant l’histoire complexe des musiques du Cap, en Afrique du Sud. Avec Sounding the Cape. Music, Identity and Politics in South Africa, Denis-Constant Martin fait beaucoup mieux que pallier cette carence. Non seulement il propose un panorama complet des nombreux genres musicaux qui ont éclos et se sont développés au Cap à partir d’un patrimoine riche et constamment nourri d’apports extérieurs, mais il interroge aussi le rôle et la place de ces musiques ..

Catherine DEUTSCH et Caroline GIRON-PANEL, dir : Pratiques musicales féminines. Discours, normes, représentations

Cet ouvrage dirigé par Catherine Deutsch et Caroline Giron-Panel s’inscrit dans la tendance au rapprochement entre études de genre et musicologie. Une tendance encore fragile dans le contexte francophone, ce que déplorent les auteures. A travers neuf études de cas en grande partie issues de la journée d’étude « Les pratiques musicales féminines à l’Epoque moderne (XVIe-XVIIIe siècles) : discours et réalités » (5 mars 2011, Centre de musique baroque de Versailles), le livre nous plonge dans l’..

Long-memory recursive prediction error method for identification of continuous-time fractional models

This paper deals with recursive continuous-time system identification using fractional-order models. Long-memory recursive prediction error method is proposed for recursive estimation of all parameters of fractional-order models. When differentiation orders are assumed known, least squares and prediction error methods, being direct extensions to fractional-order models of the classic methods used for integer-order models, are compared to our new method, the long-memory recursive prediction error method. Given the long-memory property of fractional models, Monte Carlo simulations prove the efficiency of our proposed algorithm. Then, when the differentiation orders are unknown, two-stage algorithms are necessary for both parameter and differentiation-order estimation. The performances of the new proposed recursive algorithm are studied through Monte Carlo simulations. Finally, the proposed algorithm is validated on a biological example where heat transfers in lungs are modeled by using thermal two-port network formalism with fractional models

Truncation of fractional derivative for online system identification

Fractional derivatives are non local operators that has compacity property in terms of parameter number for modeling diffusive phenomenon with very few parameters. One of its main properties is its non-local behavior, as it can be exploited to model long-memory phenomena such as heat transfers. However, such non-locality implies a constant knowledge of the full past of the function to be differentiated. In the context of real-time system identification, this may limit the experiences as calculations become slower as time progresses. This study deals with the relationship between frequency content of a signal and its truncation error in order to obtain real-time exploitable algorithms

Modeling thermal systems with fractional models: human bronchus application

System thermal modeling allows heat and temperature simulations for many applications, such as refrigeration design, heat dissipation in power electronics, melting processes and bio-heat transfers. Sufficiently accurate models are especially needed in open-heart surgery where lung thermal modeling will prevent pulmonary cell dying. For simplicity purposes, simple RC circuits are often used, but such models are too simple and lack of precision in dynamical terms. A more complete description of conductive heat transfer can be obtained from the heat equation by means of a two-port network. The analytical expressions obtained from such circuit models are complex and nonlinear in the frequency ω. This complexity in Laplace domain is difficult to handle when it comes to control applications and more specifically during surgery, as heat transfer and temperature control of a tissue may help in reducing necrosis and preserving a greater amount of a given organ. Therefore, a frequency-domain analysis of the series and shunt impedances will be presented and different techniques of approximations will be explored in order to obtain simple but sufficiently precise linear fractional transfer function models. Several approximations are proposed to model heat transfers of a human middle bronchus and will be quantified by the absolute errors

A novel mutation of AFG3L2 might cause dominant optic atrophy in patients with mild intellectual disability

Dominant optic neuropathies causing fiber loss in the optic nerve are among the most frequent inherited mitochondrial diseases. In most genetically resolved cases, the disease is associated to a mutation in OPA1, which encodes an inner mitochondrial dynamin involved in network fusion, cristae structure and mitochondrial genome maintenance. OPA1 cleavage is regulated by two m-AAA proteases, SPG7 and AFG3L2, which are, respectively involved in Spastic Paraplegia 7 and Spino-Cerebellar Ataxia 28. Here, we identified a novel mutation c.1402C>T in AFG3L2, modifying the arginine 468 in cysteine in an evolutionary highly conserved arginine-finger motif, in a family with optic atrophy and mild intellectual disability. Ophthalmic examinations disclosed a loss of retinal nerve fibers on the temporal and nasal sides of the optic disk and a red-green dyschromatopsia. Thus, our results suggest that neuro-ophthalmological symptom as optic atrophy might be associated with AFG3L2 mutations, and should prompt the screening of this gene in patients with isolated and syndromic inherited optic neuropathies

Proarrhythmic remodelling of the right ventricle in a porcine model of repaired tetralogy of Fallot

OBJECTIVE: The growing adult population with surgically corrected tetralogy of Fallot (TOF) is at risk of arrhythmias and sudden cardiac death. We sought to investigate the contribution of right ventricular (RV) structural and electrophysiological remodelling to arrhythmia generation in a preclinical animal model of repaired TOF (rTOF). METHODS AND RESULTS: Pigs mimicking rTOF underwent cardiac MRI functional characterisation and presented with pulmonary regurgitation, RV hypertrophy, dilatation and dysfunction compared with Sham-operated animals (Sham). Optical mapping of rTOF RV-perfused wedges revealed a significant prolongation of RV activation time with slower conduction velocities and regions of conduction slowing well beyond the surgical scar. A reduced protein expression and lateralisation of Connexin-43 were identified in rTOF RVs. A remodelling of extracellular matrix-related gene expression and an increase in collagen content that correlated with prolonged RV activation time were also found in these animals. RV action potential duration (APD) was prolonged in the epicardial anterior region at early and late repolarisation level, thus contributing to a greater APD heterogeneity and to altered transmural and anteroposterior APD gradients in rTOF RVs. APD remodelling involved changes in Kv4.3 and MiRP1 expression. Spontaneous arrhythmias were more frequent in rTOF wedges and more complex in the anterior than in the posterior RV. CONCLUSION: Significant remodelling of RV conduction and repolarisation properties was found in pigs with rTOF. This remodelling generates a proarrhythmic substrate likely to facilitate re-entries and to contribute to sudden cardiac death in patients with rTOF