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Introduction Les nouveaux criteres (Polman, 2010) permettent un diagnostic de SEP des le SCI. La prescription des traitements de fond reste soumise a la dissemination temporelle definie par le suivi clinico-radiologique. Objectifs Decrire la prise en charge clinique, paraclinique et therapeutique des SCI en pratique liberale ou hospitaliere et estimer le taux de conversion en SEP a 2 ans chez ces patients. Methodes Deux cent cinquante-deux patients furent inclus prospectivement par 59 neurologues sur une periode de 9 mois apres un SCI avec une IRM disponible. Le recueil prospectif des donnees observationnelles pendant 24 mois apres l’inclusion avec au moins une visite par an a permis d’analyser la conversion en SEP selon les criteres diagnostiques de 2005 et de 2010, la survenue de poussees, l’evolution du score EDSS, les resultats des examens radiologiques et biologiques, l’instauration de traitements. Resultats Des donnees completes a 2 ans furent obtenues pour 231 patients (âge moyen : 37 ans, sex-ratio F/H : 2,8) ; 26,0 % des patients presenterent une SEP definie sur la premiere IRM (2010). Les taux de conversion selon les criteres de 2010, 2005 ou SEP cliniquement definie furent respectivement de 48,6 %, 48,8 % et 38,4 % a 24 mois. Seize (14 % des 114 convertis) patients furent traites par un immunomodulateur avant la conversion selon les criteres de 2005. Discussion Les criteres diagnostiques de SEP de 2010 sont plus simples et plus sensibles que ceux de 2005 qui regissent la prescription des immunomodulateurs. Ces nouveaux criteres diagnostiques semblent accelerer la prescription des traitements de fond de la SEP pour un petit nombre de patients, qui voient leur taux de conversion clinique diminuer. Conclusion L’acceleration du diagnostic de SEP semble associee a une prescription precoce des traitements de fond apres un SCI et a une reduction du risque de conversion en SEP cliniquement definie.

Introduction Les donnees radiologiques de l’encephalite de Bickerstaff (EB) sont peu decrites dans la litterature. Nous presentons un cas d’EB avec description d’anomalies etendues en IRM et en TEP-FDG. Observation Une patiente de 36 ans fut admise pour cephalees une semaine apres une sinusite traitee par pristinamycine depuis 48 heures. La ponction lombaire (PL) montra une meningite aseptique a polynucleaires neutrophiles, avec hyperproteinorachie (1,01 g/L). Elle fut traitee par ceftriaxone et aciclovir apres une dose unique de dexamethasone. Deux jours apres l’entree, elle presenta une diplopie binoculaire avec ataxie et nystagmus. L’IRM cerebrale montra des hypersignaux (HS) diffus et symetriques de la substance blanche sus-tentorielle, cerebelleuse et du tronc cerebral en sequence FLAIR, sans prise de contraste (PdC). L’apparition d’anticorps anti-GD1a de type IgG puis leur disparition a 3 mois confirma l’hypothese d’une EB. La patiente recupera progressivement sans traitement specifique. La PL a j7 s’ameliora avec un aspect polyclonal des Ig. Le TEP-FDG a un mois montra un hypometabolisme cingulaire posterieur, des jonctions temporo-occipito-parietales, et cerebelleux. L’IRM cerebrale de suivi montra une regression incomplete des HS, qui persistent a un an, associes a une fatigabilite a l’effort et un tremblement mixte des membres superieurs. Discussion L’EB est une atteinte centrale avec diplopie, ophtalmoplegie et troubles de conscience associee a des anticorps anti-gangliosides (anti-GQ1b dans 70 % des cas). Des HS en sequence T2 sont decrits dans 11–30 % des cas, au niveau du tronc cerebral, des thalamus ou de la substance blanche supra-tentorielle. Notre cas avec anti-GD1a montre une atteinte radiologique tres etendue contrastant avec une atteinte clinique partielle s’ameliorant spontanement. Conclusion Les neurologues doivent savoir reconnaitre ces lesions IRM etendues illustrant la variabilite clinico-radiologique des EB. Le lien entre les differents anticorps anti-gangliosides et les lesions IRM merite d’etre explore.

Introduction Chitinase-3 like protein 1 (CHI3L1) est une proteine de l inflammation identifiee comme biomarqueur sensible de sclerose en plaques (SEP) dans le liquide cephalorachidien (LCR) et le serum mais semble peu specifique. Objectifs Evaluer l’interet diagnostique en routine de la CHI3L1 dans le LCR et le serum pour differentes maladies neurologiques inflammatoires et non inflammatoires, en comparaison avec les parametres immunologiques du LCR. Methodes Etude de cohorte monocentrique retrospective de patients du CHU de Nimes ayant subi une ponction lombaire avec analyse immunologique du LCR, classes parmi syndrome cliniquement isole (SCI), SEP, controle neurologique inflammatoire (INDC), controle neurologique inflammatoire peripherique (PINDC), controle neurologique non inflammatoire (NINDC) et controle symptomatique (SC). La concentration de CHI3L1 dans le LCR et le serum ont ete determinees par Elisa et les bandes oligoclonales par immunoelectrophorese. Resultats Deux cent deux patients dont 17 SEP, 35 SCI, 17 INDC, 12 PINDC, 62 NINDC et 59 SC furent analyses. Dans les LCR, on observât une elevation significative de la concentration de CHI3L1 de tous les groupes par rapport aux SC ( p p = 0,06). Dans le serum, seuls SC et NINDC etaient significativement differents ( p Discussion Cette etude de la concentration de CHI3L1 dans le LCR et le serum en routine met en evidence une mauvaise specificite de ce marqueur pour la SEP par rapport aux autres atteintes neuro-inflammatoires et non inflammatoires, contrairement aux BOC. En revanche, CHI3L1 a une excellente sensibilite pour differencier les SC de tous les autres groupes alors que les BOC ne sont presentes que chez 12 % des INDC et 25 % des PINDC et NINDC. Conclusion CHI3L1 dans le LCR apporte des informations complementaires par rapport a l’analyse immunologique classique pour le diagnostic de la SEP et d’autres maladies neuro-inflammatoires, justifiant son utilisation en routine. Informations complementaires Cette etude a ete financee par le CHU de Nimes.

Objective: Coma or respiratory failure in patients with acute ischemic stroke (IS) may require mechanical ventilation (MV). The inclusion criteria used in previous studies on MV for patients with stroke have been very heterogeneous. We carried out this prospective study in our neurologic stroke and intensive care department to assess clinical and radiologic features, mortality, outcome, and prognosis factors for patients presenting with acute IS involving the middle cerebral artery (MCA) and requiring MV. Methods: Of 470 patients admitted with acute IS involving the MCA territory, we prospectively enrolled 50 patients requiring MV. Indications for intubation were a Glasgow coma score Results: The survival at 1 year was 30%. The mortality (70%) was independent of the reason for ventilation. Survivors had a mean Barthel index of 59 ± 25 and a mean modified Rankin score of 4 ± 1. Logistic regression showed that survivors were characterized by incomplete MCA territory involvement and atherosclerosis origin. Conclusion: Patients with MCA IS who require MV have high mortality regardless of the cause of intubation. Survival is associated with incomplete MCA territory involvement and atherosclerosis origin.

Endocrine disturbance in multiple sclerosis (MS) is a rare condition and involvement of the hypothalamus is rarely described.1 The hypothalamus encompasses many nuclei where demyelinating lesions are hardly detectable using conventional MRI.2 Deficiency of antidiuretic hormone (ADH) can be encountered in inflammatory processes such as sarcoidosis, but may be idiopathic, possibly related to pathogenic antibodies. In MS, only 2 cases of ADH deficiency have been reported without overt hypothalamic lesions.3,4 Herein, we present the case of a 28-year-old man who developed diabetes insipidus (DI) 7 years before diagnosis of MS. MRI revealed the presence of bilateral MS lesions in the supraoptic nuclei, suggesting that DI was the first manifestation of MS. ### Case report. A 28-year-old man was referred after discovering cerebral white matter abnormalities on an MRI prescribed to explore DI. The patient had ingested >10 L of water every day since the age of 21. He had neither familial history of DI nor personal antecedent head trauma or neurosurgery. Neurologic and general examinations were normal. The patient had no history of asthenia, fever, dyspnea, coughs, arthritis, parotitis, …

Caracteristiques et prise en charge initiale des patients presentant un syndrome cliniquement isole (SCI) en France en 2012 E. Thouvenot , D. Audry-Chaboud , B. Salin , M.L. Camut , J.P. Meunier , M. Hellet , M. Coustans e a Service de neurologie, CHU de Nimes, Nimes, France b Cabinet de neurologie, Dijon, France c TEVA, Paris La Defense, Paris, France d AXONAL, Nanterre, France e Service de Neurologie, CHI de Cornouaille, Quimper, France

Introduction Le diagnostic positif des affections neurodegeneratives et en particulier de la maladie d’Alzheimer (MA) devient une exigence pour la prise en charge des patients et pour l’evaluation des nouvelles molecules “anti-demence”. Actuellement, le diagnostic de MA combine de donnees anamnestiques, cliniques, neuropsychologiques, d’imagerie morphologique ou fonctionnelle, et recemment des biomarqueurs dans le liquide cephalo-rachidien. Les donnees sur les marqueurs plasmatiques, en particulier l’Abeta restent decevantes. L’objectif principal de notre etude est d’identifier de nouvelles proteines plasmatiques ou seriques par technique de proteomique permettant d’optimiser le diagnostic positif de la MA. Materiel/methode 70 prelevements de patients atteints de troubles cognitifs sont analyses par proteomique differentielle de type SELDI (“Surface enhanced laser desorption/ionization time-of-flight mass spectrometry”). Ce systeme de “puces a proteine” (Bio-Rad) permet de capturer des proteines sur differentes surfaces chromatographiques et de les detecter en spectrometrie de masse. Trois groupes de patients sont compares : 1/ MA, 2/ demence non MA, 3/ affection neurologique sans atteinte cognitive. Ce protocole est valide par le comite d’ethique, le CPP et la CNIL. Resultats La premiere partie du travail a permis de valider une methode de prefractionnement des prelevements permettant d’optimiser la detection des marqueurs proteiques de faible concentration. La detection proteique par SELDI a permis de selectionner de nouveaux marqueurs potentiels permettant de differencier le groupe MA des autres demences. Certains sont en cours d’identification en spectrometrie de masse. Conclusion La recherche de biomarqueurs sanguins represente un enjeu majeur dans le cadre du diagnostic positif de la MA. L’interet clinique et la pertinence physiopathologique des biomarqueurs potentiels selectionnes dans notre etude semblent des plus prometteurs.

Pax-6 is a transcription factor containing both a homeodomain (HD) and a Paired domain (PD). It functions as an essential regulator of eye development in both Drosophila and vertebrates, suggesting an evolutionarily conserved origin for different types of metazoan eyes. Classical morphological and phylogenetic studies, however, have concluded that metazoan eyes have evolved many times independently. These apparently contradictory findings may be reconciled if the evolutionarily ancient role of Pax-6 was to regulate structural genes (e.g., rhodopsin) in primitive photoreceptors, and only later did it expand its function to regulate the morphogenesis of divergent and complex eye structures. In support of this, we present evidence that eyeless (ey), which encodes the Drosophila homolog of Pax-6, directly regulates rhodopsin 1 (rh1) expression in the photoreceptor cells. We detect ey expression in both larval and adult terminally differentiated photoreceptor cells. We show that the HD of Ey binds to a palindromic HD binding site P3/RCS1 in the rh1 promoter, which is essential for rh1 expression. We further demonstrate that, in vivo, P3/RCS1 can be replaced by binding sites specific for the PD of Ey. P3/RCS1 is conserved in the promoters of all Drosophila rhodopsin genes as well as in many opsin genes in vertebrates. Mutimerized P3 sites in front of a basal promoter are able to drive the expression of a reporter gene in all photoreceptors. These results suggest that Pax-6/Ey directly regulates rhodopsin 1 gene expression by binding to the conserved P3/RCS1 element in the promoter. ispartof: GENES & DEVELOPMENT vol:11 issue:9 pages:1122-1131 ispartof: location:United States status: published

The aim of the small satellite project VAGSAT is to provide measurement of the directional wave spectrum over the globe to improve the climatology models. The system definition is based upon the nadir beam measurement to normalize the wave spectrum, and the spectrum beam measurement to provide the directional wave spectrum. The dual beam antenna sub- system with one rotative beam and the on-board processing design with the pulse compression solution selected are presented. The first VAGSAT design is encouraging; the payload conception demonstrates the feasibility with an overall mass and power consumption compatible with a small satellite platform.

Synthetic aperture radar is an active ranging sensor that is very sensitive to terrain slope. From available information included in radar image pixel, a Digital Elevation Model (DEM) may be generated. The radargrammetry is a method that derives a topographic map from two overlapping radar images, a set of homologous points and platform motion and geometry parameters. This method is based on the parallax in range direction. The stereoscopic radar image analysis consists of three main processing steps: (1) selection and plotting of homologous points, (2) elevation calculation based on the geometry of radar images (height and distance between the antennas, and look angles), and (3) elevation interpolation in order to generate a DEM. We develop an automatic research of homologous points location in the two images, taking into account geometry of radar image acquisition and curvature of the planet. This is based on automatic analysis of shape recognition determined by a threshold on pixel radiometric gradient. We use this method by an iterative way. Their elevation is calculated and interpolated on a regular grid to generate a DEM. The elevation accuracy depends on spatial resolution. This method has been tested on AIRSAR and Magellan images.© (1995) COPYRIGHT SPIE--The International Society for Optical Engineering. Downloading of the abstract is permitted for personal use only.

Magellan synthetic aperture radar data reveal numerous surface features that are attributed to aeolian, or wind processes. Wind streaks are the most common aeolian feature. They consist of radar backscatter patterns that are high, low, or mixed in relation to the surface on which they occur. A data base of more than 3400 wind streaks shows that low backscatter linear forms (long, narrow streaks) are the most common and that most streaks occur between 17°S to 30°S and 5°N to 53°N on smooth plains. Moreover, most streaks are associated with deposits from certain impact craters and some tectonically deformed terrains. We infer that both of these geological settings provide fine particulate material that can be entrained by the low-velocity winds on Venus. Turbulence and wind patterns generated by the topographic features with which many streaks are associated can account for differences in particle distributions and in the patterns of the wind streaks. Thus, some high backscatter streaks are considered to be zones that are swept free of sedimentary particles to expose rough bedrock; other high backscatter streaks may be lag deposits of dense materials from which low-density grains have been removed (dense materials such as ilmenite or pyrite have dielectric properties that would produce high backscatter patterns). Wind streaks generally occur on slopes < 2° and tend to be oriented toward the equator, consistent with the Hadley model of atmospheric circulation. In addition to wind streaks, other aeolian features on Venus include yardangs(?) and dune fields. The Aglaonice dune field, centered at 25°S, 340°E, covers ∼1290 km^2 and is located in an ejecta flow channel from the Aglaonice impact crater. The Meshkenet dune field, located at 67°N, 90°E, covers ∼17,120 km^2 in a valley between Ishtar Terra and Meshkenet Tessera. Wind streaks associated with both dune fields suggest that the dunes are of transverse forms in which the dune crests are perpendicular to the prevailing winds. Dunes on Venus signal the presence of sand-size (∼60 to 2,000 μm) grains. The possible yardangs are found at 9°N, 60.5°E, about 300 km southeast of the crater Mead. Although most aeolian features are concentrated in smooth plains near the equator, the occurrence of wind streaks is widespread, and some have been found at all latitudes and elevations. They demonstrate that aeolian processes operate widely on Venus. The intensity of wind erosion and deposits, however, varies with locality and is dependent on the wind regime and supply of particles.

A CCD camera was built in 1984 using a linear detector (Thomson TH7810A) with 1728 pixels. The simplicity of this sort of detector enabled us to gain experience with CCDs. With a linear detector it is possible to carry out spectroscopy or even two-dimensional imaging by shifting the observed field at right angles to the photosensitive strip and by acquiring data in a regular manner (the scanning technique). The limitations of this equipment soon became apparent when trying to image deep-sky objects, so a second camera was built in 1985 using a CCD array, a Thomson TH7851 with 208 × 144 pixels. Numerous improvements were later made and the most advanced model is described.

A CCD camera was built in 1984 using a linear detector (Thomson TH7810A) with 1728 pixels. The simplicity of this sort of detector enabled us to gain experience with CCDs. With a linear detector it is possible to carry out spectroscopy or even two-dimensional imaging by shifting the observed field at right angles to the photosensitive strip and by acquiring data in a regular manner (the scanning technique). The limitations of this equipment soon became apparent when trying to image deep-sky objects, so a second camera was built in 1985 using a CCD array, a Thomson TH7851 with 208 × 144 pixels. Numerous improvements were later made and the most advanced model is described.