In this paper, vanadium dioxide thin films were successfully deposited at room temperature by high power impulse magnetron sputtering (HiPIMS) using two different magnetron configurations: (i) a conventional balanced magnetron and (ii) an unbalanced magnetron. In the case of the unbalanced magnetron, the magnetic field is extended towards the substrate and the energetic ions in the plasma actively participate in film growth allowing to improve film crystallinity. The as-deposited thin films were annealed under Ar flow for 1 h to obtain thermochromic VO2(M) phase. The 120 nm films were first characterized at room temperature by X-ray diffraction (XRD). Then, the thermochromic behavior of the VO2 films was investigated by electrical and optical characterizations. The effect of annealing temperature on thermochromic properties of VO2 was studied showing that high quality VO2(M) film can be obtained at 300 C using unbalanced HiPIMS. A very efficient change in transmittance exceeding 70% is observed in the IR spectral region. This investigation demonstrates the capability of using unbalanced HiPIMS to achieve high optical performances for VO2 at lower temperature as well as the potential of this technology for the deposition of VO2(M) films onto temperature-sensitive substrates.

W-doped monoclinic vanadium dioxide VO2(M) thin films with various W contents were successfully deposited by magnetron sputtering method. As-deposited films consisted of multilayers composed of VO2/W bilayers. Further annealing at 500 degrees C under Argon for 1 h led to crystallized and homogeneous V1-xWxO2 +/-delta films. 200 nm films were characterized at room temperature by Rutherford backscattering spectrometry (RBS), X-ray diffraction (XRD) and X-ray photoelectron spectroscopy (XPS). Besides, the films were analyzed by temperature-dependent infrared transmittance in the wavelength region 2-25 mu m and the effects of W doping on thermochromic properties of VO2 were studied. The results show that the transition temperature gradually decreases with increasing W content. A transition temperature of 21 degrees C with a high infrared transmittance modulation (approximate to 68%) was finally achieved with a W-doping level of 2.7 at.% which may indicate a strong potential for applications in optical devices as functional materials. (C) 2020 Elsevier B.V. All rights reserved.

123I-MIBG myocardial scintigraphy and clonidine growth hormone test (CGH test) may help to distinguish multiple system atrophy (MSA) from Parkinson’s disease (PD). Their relevance in the first-stage parkinsonism of uncertain etiology is unknown.

Il est parfois difficile de différencier la maladie de Parkinson (MPI) de l’atrophie multi-systématisée (MSA) à un stade débutant. La scintigraphie myocardique au MIBG et le test à la clonidine (CGH-test) peuvent aider au diagnostic mais leur efficacité n’a jamais été étudiée en cas de parkinsonisme débutant d’étiologie douteuse. Seuls les patients présentant un syndrome parkinsonien débutant d’étiologie douteuse ont été inclus dans l’étude et ont bénéficié d’une évaluation clinique permettant de suspecter un diagnostic clinique initial (DCI) selon les critères diagnostiques de MPI et de MSA. Les patients ont ensuite bénéficié d’une scintigraphie myocardique et d’un CGH-test puis d’un suivi clinique régulier jusqu’à ce que le diagnostic clinique final (DCF) ait pu être posé après au moins 5 ans d’évolution. Vingt- cinq patients inclus (DCI douteux : 15 MSA/10 MPI). Après 6 ans d’évolution, le DCF retenu était 11 MSA et 14 MPI. Le CGH-test et la scintigraphie avaient une sensibilité de 71 % et 93 % respectivement et tous deux une spécificité de 82 % pour le diagnostic de MPI. Les associations scintigraphie + hypotension orthostatique et scintigraphie + dopasensibilité >30 % augmentaient la sensibilité pour le diagnostic de MPI et étaient significativement supérieures au DCI (p = 0,04 et p = 0,005, respectivement). Les principaux biais de cette étude sont le faible effectif de patients (la MSA étant une pathologie rare) et l’absence de confirmation histologique. Nous avons tenté d’ajuster au mieux ce dernier biais par une moyenne de suivi de 6 ans et une durée d’évolution prolongée (11 ans pour les MPI et 8 ans pour les MSA). Les associations scintigraphie + dopasensibilité et scintigraphie + hypotension orthostatique sont intéressantes pour distinguer la MPI de la MSA à un stade débutant en cas de diagnostic douteux.

Essential tremor (ET) is characterized by a frequent family history. No monogenic form of ET has been identified. We aimed at exploring ET patients to identify distinct subgroups and facilitate the identification of ET genes. We tested for the presence of HTRA2 p.G399S, and ANO3 p. W490C, p. R484 W and p. S685G mutations.

Fragile X-associated tremor ataxia syndrome (FXTAS) is caused by FMR1 premutation. The features include ataxia, action tremor and middle cerebellar peduncle (MCP) hyperintensity, the latter being the only major radiological criterion in the diagnosis of definite FXTAS until very recently. The importance of corpus callosum splenium (CCS) hyperintensity was recently reported and this sign is now considered as an additional major radiological diagnostic criterion in the diagnosis of FXTAS. However, little is known about its relevance for the diagnosis of FXTAS in clinical practice. We report a practical justification of the relevance of CCS hyperintensity in parallel with MCP hyperintensity for the diagnosis of FXTAS. Clinical and radiological study of 22 FMR1 premutation carriers with neurological signs that may be encountered in FXTAS compared to series of patients with essential tremor, multiple system atrophy of cerebellar type, Parkinson’s disease, Alzheimer’s disease and stroke. Among the 22 patients with FMR1 premutation [17 men, 5 women; mean age, 63 ± 7.5 (46–84)], 14 were diagnosed with definite FXTAS with the initial criteria. Considering CCS hyperintensity as a new major radiological criterion permitted the diagnosis of definite FXTAS in 3 additional patients. Overall CCS proved as frequent as MCP hyperintensity (64 versus 64 %), while 23 % of patients had CCS but not MCP hyperintensity, 14 % of patients had CCS hyperintensity but neither MCP, nor brainstem hyperintensity. In contrast with CCS hyperintensity, MCP hyperintensity proved less frequent in women than in men. CCS and MCP hyperintensity were more frequent in FXTAS than in the other neurodegenerative disorders. The combination of CCS and MCP hyperintensity was specific of FXTAS. We confirmed the relevance of CCS hyperintensity in FXTAS and we clarified its interest compared to MCP hyperintensity. Our results support the inclusion of CCS hyperintensity in the diagnostic criteria as a new major radiological criterion.

This article presents a custom condensation model for commercial CFD code Fluent. The condensation model was developed for the species transport model in Fluent code and it is suitable for both compressible and incompressible flow of air–steam mixture with additional non-condensable gases. The condensation model consists of two parts: condensation in volume and condensation on the wall. Condensation in volume is modeled by "return to saturation in constant time scale" method. Condensation on the wall is calculated from diffusion of steam through a layer of non-condensable gases near the wall.The performance of the condensation model was tested on the CONAN experiments. In these experiments, air–steam mixture flows downwards through a vertical channel with square cross section. One vertical wall of the channel is cooled and the steam condenses on it. The same model was then applied in simulation of PANDA Test 9bis experiment with condensation. In this test, two vessels connected with a pipe were filled with air; and steam was released into the first vessel. As the steam concentration increased in the vessels, the steam started condensing on the walls. The results of CFD simulations of both CONAN and PANDA experiments compared well with the measured data.

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L'ataxie spastique autosomique récessive de Charlevoix-Saguenay (ARSACS) est une ataxie cérébelleuse de transmission autosomique récessive caractérisée par un syndrome pyramidal et une ataxie cérébelleuse débutant vers l'âge de deux ans. La paraparésie spastique s'aggrave progressivement pour dominer le tableau clinique souvent complété par une polyneuropathie sensitivomotrice axonale et démyélinisante et par la présence au fond d'œil de fibres myélinisées rétiniennes proéminentes. L'imagerie par résonance magnétique nucléaire met en évidence une atrophie cérébelleuse à prédominance vermienne. Des mutations dans le gène SACS, localisé en 13q11, sont responsables de l'ARSACS. Nous rapportons deux cas d'ARSACS, liés à deux mutations hétérozygotes composites, p.Ala2558Val et p.Pro536Leu, au sein d'une même famille française non consanguine. La présentation clinique de ces patients est assez fidèle à la description habituelle de la littérature, en dépit de l'existence d'un retard mental modéré, du caractère à prédominance démyélinisant de la neuropathie périphérique et de bouffées de pointe-ondes généralisées asymptomatiques chez les deux patients. De plus, nous décrivons l'hétérogénéité phénotypique de cette famille concernant notamment les modalités de début des symptômes et la rapidité d'aggravation de la pathologie. Nous présentons également une revue de la littérature sur cette pathologie rare décrite principalement, quoique non exclusivement, au Québec.The autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a cerebellar ataxia autosomal recessively inherited characterized by an ataxic and pyramidal syndrome usually occurring near two years of age. The spastic paraparesis progressively worsens and becomes the prominent sign. Sensorial and motor axonal peripheral neuropathy is frequently reported as well as prominent retinal myelinated fibers on fundus examination. Brain magnetic resonance imaging reveals a predominantly vermian cerebellar atrophy. Mutations in SACS gene on 13q11 are responsible for ARSACS. We report two patients from a non-consanguineous French family, affected with ARSACS, due to the compound heterozygous mutations p.Ala2558Val and p.Pro536Leu. The clinical presentation is in accordance with the previously described ARSACS cases, despite the presence of mental retardation, the predominantly demyelinating peripheral neuropathy, and the evidence of asymptomatic generalized spikes and waves on electroencephalography. We described the clinical heterogeneity in this family including the age at onset of the disease and the first signs as well as the rapidity of the disease progression. We present a review of the litterature on this rare disease mostly described in Quebec.

Les troubles mnésiques, plus particulièrement ceux de la mémoire épisodique verbale (MEV), constituent un motif fréquent de plainte cognitive chez les patients atteints de sclérose en plaques (SEP). Outre la gêne occasionnée dans la vie quotidienne, ces troubles peuvent avoir un impact sur les activités professionnelles. L'évaluation neuropsychologique des patients doit donc comporter des tests explorant la MEV, de façon à décrire le degré d'atteinte des différents processus cognitifs contribuant à la MEV et, le cas échéant, pouvoir proposer des stratégies rééducatives adaptées. L'objectif de cet article est de proposer une revue critique de la littérature portant sur les capacités de MEV dans la SEP. Cette revue permettra de présenter les références conceptuelles et les caractéristiques psychométriques des principaux tests de MEV appliqués dans cette pathologie (épreuves isolées ou intégrées à des batteries cognitives plus globales développées pour la SEP). Dans un second temps, nous proposons un inventaire, par processus cognitifs, des travaux réalisés dans cette affection. Cette approche permettra d'aborder les limites de chaque conception et les éventuelles ambiguïtés terminologiques. Les contributions de ces travaux aux connaissances sur les troubles mnésiques de la SEP seront précisées, ainsi que l'impact des caractéristiques de la maladie (formes de SEP, durée d'évolution, EDSS).Memory impairment, especially verbal episodic memory (VEM), represents a common ground for cognitive complaint in patients with multiple sclerosis (MS). Beyond the difficulty caused in daily life, these deficits may impact on occupational activities. Neuropsychological assessment of these patients has to include VEM tests, to describe the level of dysfunction of the different processes contributing to VEM and, if required, to guide adapted cognitive rehabilitation. The objective of the present paper is to propose a critique review of the literature on VEM abilities in MS. This review will present the conceptual references and the psychometric characteristics of the main VEM tests applied in MS (isolated tests or included within more general batteries developed specifically for MS). In a second phase, we propose an inventory of work on MS presented as a function of the cognitive processes involved. This approach provides an approach to the limitations of each conception and possible terminological ambiguities. Contributions to knowledge of MS memory impairments will be clarified, as well as the impact of the disease characteristics (MS forms, disease duration, EDSS).

Les mouvements anormaux (tremblement, myoclonies, tics, choree, dystonie) sont des symptomes frequents dont la prevalence augmente avec l’âge. L’examen clinique permet le plus souvent de caracteriser le mouvement anormal. Les causes iatrogenes ou metaboliques sont les plus frequentes et doivent etre systematiquement recherchees et traitees avant d’envisager d’autres explorations. Des examens electrophysiologiques peuvent aider a preciser les caracteristiques du mouvement anormal et a proposer un traitement adapte au generateur ou a la pathologie sous-jacente. Outre les traitements medicamenteux per os adaptes a chaque mouvement anormal, la prise en charge therapeutique peut passer, notamment pour les dystonies, par l’utilisation de la toxine botulique, ou pour les tremblements, les dystonies et certaines formes graves de syndrome de Gilles de la Tourette, par la stimulation cerebrale profonde.

Introduction. - L-2-hydroxyglutaric aciduria is a rare metabolic disorder with quite typical radiological abnormalities and various clinical symptoms.Observation. - A 19-year-old girl presented with ataxia, facial dyskinesia, and mild cognitive impairment. Cerebral magnetic resonance imaging demonstrated subcortical white matter T2 abnormalities and a suggestive rim hyperintensity around the caudate nuclei and the putamen. Diagnosis was confirmed by increased 2-hydroxyglutaric acid in urine and a genetic study (Gly260Ala mutation in the L-2-hydroxyglutarate dehydrogenase (L2HGDH) gene).Discussion. - This case highlights the movement disorder onset and radiological aspects that should indicate the L-2-hydroxyglutaric aciduria diagnosis. (C) 2011 Elsevier Masson SAS. All rights reserved.

Thin films of indium zinc oxide so called IZO were prepared with pulsed laser deposition. It was found that the crystalline structure, the composition and the morphology of the films as well as the optical and electrical properties were quite sensitive to the deposition conditions namely to the temperature and oxygen pressure. The crystallinity of the ZnkIn2O3+k (k from 1 to 5) thin films increases as the substrate temperature increases. An average transmittance of 85 % in the visible region was obtained for any k values. Optical measurements show a continuous decrease of the band gap as the zinc amount increases. The highest conductivity reported is for the ZnIn2O4, thin films deposited at 300 °C (σ = 1.2 103 S/cm). Increasing the amount of Zn (i.e. k value) was found to result in a conductivity decrease. Finally, a good correlation between the electric mobility and the optical mobility is obtained.