Familial neuromyelitis optica

Background: Detection of aquaporin-4-specific immunoglobulin G (IgG) has expanded the spectrum of neuromyelitis optica (NMO). Rare reports of familial aggregation have suggested a component of genetic susceptibility but these reports mostly antedated the discovery of the NMO-IgG biomarker and recently updated diagnostic criteria. Methods: We report a case series describing the demographic, clinical, neuroimaging, and NMO-IgG serologic status of 12 multiplex NMO pedigrees with a total of 25 affected individuals. Results: Twenty-one patients (84%) were women. Families were Asian (n = 5), Latino (n = 4), white (n = 1), or African (n = 2). Apparent transmission was either maternal (n = 5) or paternal (n = 2). In 1 family, 3 individuals had NMO; in the others, 2 individuals were affected. Sibling pairs (n = 6), parent-child (n = 4), and aunt-niece (n = 3) pairs were observed. Nineteen patients (76%) were NMO-IgG positive. Twelve (48%) had clinical or serologic evidence of another autoimmune disease. Familial occurrence of NMO occurs in approximately 3% of patients with well-established diagnosis of NMO. Conclusions: A small proportion of patients with NMO have relatives with this condition, but familial occurrence is more common than would be expected from its frequency in the general population. Familial NMO is indistinguishable from sporadic NMO based on clinical symptoms, age at onset, sex distribution, and frequency of NMO-IgG detection. One or 2 generations were affected and affected individuals represented a small fraction of family members. Taken together, these data suggest complex genetic susceptibility in NMO. Neurology (R) 2010;75:310-315

A busca da comunicação na sociedade multi-ecrãs: perspectiva ecológica

Este texto reflexiona sobre una nueva forma de concebir la televisión como servicio público, resaltando las dificultades y problemas que plantea analizar el concepto de la calidad de la televisión, si no se tiene en cuenta la dimensión emotiva y de entretenimiento del medio. Posteriormente, el autor pretende ubicar la televisión en el contexto de la multiplicidad de otras pantallas y tecnologías, subrayando las cuestiones del sentido y de la calidad de vida, desde un modelo ecológico. Finalmente, se apuntan algunas contribuciones para profundizar el concepto y las experiencias de alfabetización digital.

EFNS guidelines on diagnosis and management of neuromyelitis optica.

BACKGROUND AND PURPOSE: Neuromyelitis optica (NMO) or Devic's disease is a rare inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS) characterized by recurrent attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which is distinct from multiple sclerosis (MS). The guidelines are designed to provide guidance for best clinical practice based on the current state of clinical and scientific knowledge. SEARCH STRATEGY: Evidence for this guideline was collected by searches for original articles, case reports and meta-analyses in the MEDLINE and Cochrane databases. In addition, clinical practice guidelines of professional neurological and rheumatological organizations were studied. RESULTS: Different diagnostic criteria for NMO diagnosis [Wingerchuk et al. Revised NMO criteria, 2006 and Miller et al. National Multiple Sclerosis Society (NMSS) task force criteria, 2008] and features potentially indicative of NMO facilitate the diagnosis. In addition, guidance for the work-up and diagnosis of spatially limited NMO spectrum disorders is provided by the task force. Due to lack of studies fulfilling requirement for the highest levels of evidence, the task force suggests concepts for treatment of acute exacerbations and attack prevention based on expert opinion. CONCLUSIONS: Studies on diagnosis and management of NMO fulfilling requirements for the highest levels of evidence (class I-III rating) are limited, and diagnostic and therapeutic concepts based on expert opinion and consensus of the task force members were assembled for this guideline.

Pour une histoire et une esthétique de l'écran fragmenté au cinéma

Bien que son existence soit presque aussi vieille que le cinéma, l‘écran fragmenté (que les académiciens et autres professionnels du cinéma de langue anglaise désignent communément sous l‘appellation « split screen ») n‘a jamais fait l‘objet d‘analyses véritablement approfondies. Quand il est mentionné dans les livres d‘histoire, l‘écran fragmenté est rapidement esquivé. Pourtant, ses apparitions sont nombreuses. Ce mémoire de maîtrise cherche à corriger nombre d‘idées préétablies en exposant l‘histoire de cette manifestation visuelle, en commençant des débuts (le « cinéma des premiers temps ») jusqu‘à l‘arrivée du « cinéma numérique » du nouveau millénaire.

Oncostatin M is a novel glucocorticoid-dependent neuroinflammatory factor that enhances oligodendrocyte precursor cell activity in demyelinated sites

The innate immune reaction to tissue injury is a natural process, which can have detrimental effects in the absence of negative feedbacks by glucocorticoids (GCs). Although acute lipopolysaccharide (LPS) challenge is relatively harmless to the brain parenchyma of adult animals, the endotoxin is highly neurotoxic in animals that are treated with the GC receptor antagonist RU486. This study investigated the role of cytokines of the gp130-related family in these effects, because they are essential components of the inflammatory process that provide survival signals to neurons. Intracerebral LPS injection stimulated expression of several members of this family of cytokines, but oncostatin M (Osm) was the unique ligand to be completely inhibited by the RU486 treatment. OSM receptor (Osmr) is expressed mainly in astrocytes and endothelial cells following LPS administration and GCs are directly responsible for its transcriptional activation in the presence of the endotoxin. In a mouse model of demyelination, exogenous OSM significantly modulated the expression of genes involved in the mobilization of oligodendrocyte precursor cells (OPCs), differentiation of oligodendrocyte, and production of myelin. In conclusion, the activation of OSM signaling is a mechanism activated by TLR4 in the presence of negative feedback by GCs on the innate immune system of the brain. OSM absence is associated with detrimental effects of LPS, whereas exogenous OSM favors repair response to demyelinated regions. (C) 2010 Elsevier Inc. All rights reserved.

Luz industrial e imagen tecnificada: de Moholy Nagy al C.A.V.S. (Center for Advanced Visual Studies)

El desarrollo de la tecnología de la luz implicará la transformación de la vida social, cultural y económica. Tanto las consideraciones espaciales del Movimiento Moderno, como los efectos producidos por la segunda Guerra Mundial, tendrán efectos visibles en las nuevas configuraciones espaciales y en la relación simbiótica y recíproca que se dará entre ideología y tecnología. La transformación en la comprensión de la articulación espacial, asociada al desarrollo tecnológico, afectará al modo en que este espacio es experimentado y percibido. El espacio expositivo y el espacio escénico se convertirán en laboratorio práctico donde desarrollar y hacer comprensible todo el potencial ilusorio de la luz, la proyección y la imagen, como parámetros modificadores y dinamizadores del espacio arquitectónico. Esta experimentación espacial estará precedida por la investigación y creación conceptual en el mundo plástico, donde los nuevos medios mecánicos serán responsables de la construcción de una nueva mirada moderna mediatizada por los elementos técnicos. La experimentación óptica, a través de la fotografía, el cine, o el movimiento de la luz y su percepción, vinculada a nuevos modos de representación y comunicación, se convertirá en elemento fundamental en la configuración espacial. Este ámbito de experimentación se hará patente en la Escuela de la Bauhaus, de la mano de Gropius, Schlemmer o Moholy Nagy entre otros; tanto en reflexiones teóricas como en el desarrollo de proyectos expositivos, arquitectónicos o teatrales, que evolucionarán en base a la tecnología y la modificación de la relación con el espectador. El espacio expositivo y el espacio escénico se tomarán como oportunidad de investigación espacial y de análisis de los modos de percepción, convirtiéndose en lugares de experimentación básicos para el aprendizaje. El teatro se postula como punto de encuentro entre el arte y la técnica, cobrando especial importancia la intersección con otras disciplinas en la definición espacial. Las múltiples innovaciones técnicas ligadas a los nuevos fundamentos teatrales en la modificación de la relación con la escena, que se producen a principios del siglo XX, tendrán como consecuencia la transformación del espacio en un espacio dinámico, tanto física como perceptivamente, que dará lugar a nuevas concepciones espaciales, muchas de ellas utópicas. La luz, la proyección y la creación de ilusión en base a estímulos visuales y sonoros, aparecen como elementos proyectuales efímeros e inmateriales, que tendrán una gran incidencia en el espacio y su modo de ser experimentado. La implicación de la tecnología en el arte conllevará modificaciones en la visualización, así como en la configuración espacial de los espacios destinados a esta. Destacaremos como propuesta el Teatro Total de Walter Gropius, en cuyo desarrollo se recogen de algún modo las experiencias espaciales y las investigaciones desarrolladas sobre la estructura formal de la percepción realizadas por Moholy Nagy, además de los conceptos acerca del espacio escénico desarrollados en el taller de Teatro de la Bauhaus por Oskar Schlemmer. En el Teatro Total, Gropius incorporará su propia visión de cuestiones que pertenecen a la tradición de la arquitectura teatral y las innovaciones conceptuales que estaban teniendo lugar desde finales del s.XIX, tales como la participación activa del público o la superación entre escena y auditorio, estableciendo en el proyecto una nueva relación perceptual entre sala, espectáculo y espectador; aumentando la sensación de inmersión, a través del uso de la física, la óptica, y la acústica, creando una energía concéntrica capaz de extenderse en todas direcciones. El Teatro Total será uno de los primeros ejemplos en los que desde el punto de partida del proyecto, se conjuga la imagen como elemento comunicativo con la configuración espacial. Las nuevas configuraciones escénicas tendrán como premisa de desarrollo la capacidad de transformación tanto perceptiva, como física. En la segunda mitad del s.XX, la creación de centros de investigación como el CAVS (The Center for Advanced Visual Studies,1967), o el EAT (Experiments in Art and Technology, 1966), favorecerán la colaboración interdisciplinar entre arte y ciencia, implicando a empresas de carácter tecnológico, como Siemens, HP, IBM o Philips, facilitando soporte técnico y económico para el desarrollo de nuevos sistemas. Esta colaboración interdisciplinar dará lugar a una serie de intervenciones espaciales que tendrán su mayor visibilidad en algunas Exposiciones Universales. El resultado será, en la mayoría de los casos, la creación de espacios de carácter inmersivo, donde se establecerá una relación simbiótica entre espacio, imagen, sonido, y espectador. La colocación del espectador en el centro de la escena y la disposición dinámica de imagen y sonido, crearán una particular narrativa espacial no lineal, concebida para la experiencia. Desde las primeras proyecciones de cine a la pantalla múltiple de los Eames, las técnicas espaciales de difusión del sonido en Stockhausen, o los experimentos con el movimiento físico interactivo, la imagen, la luz en movimiento y el sonido, quedan inevitablemente convertidos en material arquitectónico. ABSTRACT. Light technology development would lead to a social, cultural and economic transformation. Both spatial consideration of “Modern Movement” and Second World War effects on technology, would have a visible aftereffect on spatial configuration and on the symbiotic and mutual relationship between ideology & technology. Comprehension adjustment on the articulation of space together with technology development, would impact on how space is perceived and felt. Exhibition space and scenic space would turn into a laboratory where developing and making comprehensive all illusory potential of light, projection and image. These new parameters would modify and revitalize the architectonic space. as modifying and revitalizing parameters of architectonic space. Spatial experimentation would be preceded by conceptual creation and investigation on the sculptural field, where new mechanic media would be responsible for a fresh and modern look influenced by technical elements. Optical experimentation, through photography, cinema or light movement and its perception, would turn into essential components for spatial arrangement linked to new ways of performance and communication. This experimentation sphere would be clear at The Bauhaus School, by the hand of Gropius, Schlemmer or Moholy Nag among others; in theoretical, theatrical or architectural performance’s projects, that would evolve based on technology and also based on the transformation of the relationship with the observer. Exhibition and perfor-mance areas would be taken as opportunities of spatial investigation and for the analysis of the different ways of perception, thus becoming key places for learning. Theater is postulated as a meeting point between art and technique, taking on a new significance at its intersection with other disciplines working with spatial definition too. The multiple innovation techniques linked to the new foundations for the theater regarding stage relation, would have as a consequence the regeneration of the space. Space would turn dynamic, both physically and perceptibly, bringing innovative spatial conceptions, many of them unrealistic. Light, projection and illusory creation based on sound and visual stimulus would appear as intangible and momentary design components, which would have a great impact on the space and on the way it is experienced. Implication of technology in art would bring changes on the observer as well as on the spatial configuration of the art spaces2. It would stand out as a proposal Walter Groupis Total Theater, whose development would include somehow the spatial experiments and studies about formal structure of perception accomplished by Moholy Nagy besides the concepts regarding stage space enhanced at the Bauhaus Theater Studio by Oskar Schlemmer. Within Total Theater, Groupis would incorporate his own view about traditional theatric architecture and conceptual innovations that were taking place since the end of the nineteenth century, such as active audience participation or the diffusing limits between scene and audience, establishing a new perception relationship between auditorium, performance and audience, improving the feeling of immersion through the use of physics, optics and acoustics, creating a concentric energy capable of spreading in all directions. Total Theater would be one of the first example in which, from the beginning of the Project, image is combined as a communicating element with the spatial configuration. As a premise of development, new stage arrangement would have the capacity of transformation, both perceptive and physically. During the second half or the twentieth century, the creation of investigation centers such as CAVS (Center for Advanced Visual Studies, 1967) or EAT (Experiments in Art and Technology, 1966), would help to the interdisciplinary collaboration between art and science, involving technology companies like Siemens, HP, IBM or Philips, providing technical and economic support to the development of new systems. This interdisciplinary collaboration would give room to a series of spatial interventions which would have visibility in some Universal Exhibitions. The result would be, in most cases, the creation of immersive character spaces, where a symbiotic relationship would be stablished between space, image, sound and audience. The new location of the audience in the middle of the display, together with the dynamic arrangement of sound and image would create a particular, no lineal narrative conceived to be experienced. Since the first cinema projections, the multiple screen of Eames, the spatial techniques for sound dissemination at Stockhausen or the interactive physical movement experimentation, image, motion light and sound would turn inevitably into architectural material.

Education as a mirror of Spanish society: challenges and policies towards multiple diversity

The ways in which the dominant cultural majority frames the educationalsystem determine perceptions of its own identity and understandings ofthe ‘other.’ In this article I take a political approach, by examining themanagement of cultural diversity within Spanish education policies, treating“education as the mirror of society”. This article analyzes Spanish challengesand policies approaches towards the management of immigrationrelated diversity in education. The main finding is that there is not one approach,but several, due to both the decentralized character of the educationsystem and the multiplicity of diversity that is at stake (i.e. language,religion, culture etc.)

From Offshore to Onshore: Multiple Origins of Shallow-Water Corals from Deep-Sea Ancestors

Shallow-water tropical reefs and the deep sea represent the two most diverse marine environments. Understanding the origin and diversification of this biodiversity is a major quest in ecology and evolution. The most prominent and well-supported explanation, articulated since the first explorations of the deep sea, holds that benthic marine fauna originated in shallow, onshore environments, and diversified into deeper waters. In contrast, evidence that groups of marine organisms originated in the deep sea is limited, and the possibility that deep-water taxa have contributed to the formation of shallow-water communities remains untested with phylogenetic methods. Here we show that stylasterid corals (Cnidaria: Hydrozoa: Stylasteridae)-the second most diverse group of hard corals-originated and diversified extensively in the deep sea, and subsequently invaded shallow waters. Our phylogenetic results show that deep-water stylasterid corals have invaded the shallow-water tropics three times, with one additional invasion of the shallow-water temperate zone. Our results also show that anti-predatory innovations arose in the deep sea, but were not involved in the shallow-water invasions. These findings are the first robust evidence that an important group of tropical shallow-water marine animals evolved from deep-water ancestors.

The right to the screen: from media education to educommunication in Brazil

Nowadays in Brazil, some social organizations, governments and mass media are discussing the need to establish an oversight committee to guarantee the quality of television programmes, as well as the need to set a system to determine what kind of pro, gram is appropriate for every television time slot. Across Brazil, a representative body of children and young people have come to the conclusion that the right to receive quality television programmes is not enough. The children of the new generations think they have the right to access new technologies and the production of their own messages, in accordance with their own creativity, interests and lifestyle projects within society.

Lepidopteran larva consumption of soybean foliage: basis for developing multiple-species economic thresholds for pest management decisions

BACKGROUND: Defoliation by Anticarsia gemmatalis (Hubner), Pseudoplusia includens (Walker), Spodoptera eridania (Cramer), S. cosmioides (Walker) and S. frugiperda (JE Smith) (Lepidoptera: Noctuidae) was evaluated in four soybean genotypes. A multiple-species economic threshold (ET), based upon the species` feeding capacity, is proposed with the aim of improving growers` management decisions on when to initiate control measures for the species complex. RESULTS: Consumption by A. gemmatalis, S. cosmioides or S. eridania on different genotypes was similar. The highest consumption of P. includens was 92.7 cm(2) on Codetec 219RR; that of S. frugiperda was 118 cm(2) on Codetec 219RR and 115.1 cm(2) on MSoy 8787RR. The insect injury equivalent for S. cosmoides, calculated on the basis of insect consumption, was double the standard consumption by A. gemmatalis, and statistically different from the other species tested, which were similar to each other. CONCLUSIONS: As S. cosmioides always defoliated nearly twice the leaf area of the other species, the injury equivalent would be 2 for this lepidopteran species and 1 for the other species. The recommended multiple-species ET to trigger the beginning of insect control would then be 20 insect equivalents per linear metre. (C) 2010 Society of Chemical Industry

Effect of multiple transverse modes in self-mixing sensors based on vertical-cavity surface-emitting lasers

We investigate the effect of coexisting transverse modes on the operation of self-mixing sensors based on vertical-cavity surface-emitting lasers (VCSELs). The effect of multiple transverse modes on the measurement of displacement and distance were examined by simulation and in laboratory experiment. The simulation model shows that the periodic change in the shape and magnitude of the self-mixing signal with modulation current can be properly explained by the different frequency-modulation coefficients of the respective transverse modes in VCSELs. The simulation results are in excellent agreement with measurements performed on single-mode and multimode VCSELs and on self-mixing sensors based on these VCSELs.

Constitutive mutants of the GM-CSF receptor reveal multiple pathways leading to myeloid cell survival, proliferation, and granulocyte-macrophage differentiation

Activation of the granulocyte-macrophage colony-stimulating factor (GM-CSF) family of receptors promotes the survival, proliferation, and differentiation of cells of the myeloid compartment. Several signaling pathways are activated downstream of the receptor, however it is not clear how these induce specific biologic outcomes. We have previously identified 2 classes of constitutively active mutants of the shared signaling subunit, human (h) betac, of the human GM-CSF/interieukin-3 (IL-3)/IL-5 receptors that exhibit different modes of signaling. In a factor-dependent bipotential myeloid cell line, FDB1, an activated mutant containing a substitution in the transmembrane domain (V449E) induces factor-independent proliferation and survival, while mutants in the extracellular domain induce factor-independent granulocyte-macrophage differentiation. Here we have used further mutational analysis to demonstrate that there are nonredundant functions for several regions of the cytoplasmic domain with regard to mediating proliferation, viability, and differentiation, which have not been revealed by previous studies with the wild-type GM-CSF receptor. This unique lack of redundancy has revealed an association of a conserved membrane-proximal region with viability signaling and a critical but distinct role for tyrosine 577 in the activities of each class of mutant.

Ethical preferences for influencing superiors: A 41-society study

With a 41-society sample of 9990 managers and professionals, we used hierarchical linear modeling to investigate the impact of both macro-level and micro-level predictors on subordinate influence ethics. While we found that both macro-level and micro-level predictors contributed to the model definition, we also found global agreement for a subordinate influence ethics hierarchy. Thus our findings provide evidence that developing a global model of subordinate ethics is possible, and should be based upon multiple criteria and multilevel variables. Journal of International Business Studies (2009) 40, 1022-1045. doi:10.1057/jibs.2008.109