103 Atypical Progressive Bulbar Palsy presenting with Dropped Head

Introduction: Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. There are a few case reports of dropped head syndrome in ALS, mainly in patients with the limb involvement variant. Methods: Case report Results: A 56 year old right-handed male, presented to the clinic with four months of dysphagia to liquids and solids, neck pain and progressive neck weakness causing constant drop head. No dysarthria or other neurological symptoms, no dyspnea. Neurological examination: Cranial Nerve (CN) CN XII: Nasal voice, bilateral atrophy of the tongue with tremor and fasciculations. Motor: Diffuse atrophy and decreased tone of the sternocleidomastoid and trapezii bilaterally, strength: 2/5 in neck flexors and extensors. Sensory: Hypoesthesia of the tongue. The rest of his neurological examination was normal. Labs: Routine blood work, thyroid function tests, collagen vascular work-up, and protein electrophoresis were normal. Creatine Phosphokinase (CPK) and Acetylcholine Receptor Antibodies (AChR Ab) were negative. Brain and Spinal Cord MRI: Showed mild brainstem, cerebellar and cervical spinal atrophy. Conclusions: Patients with ALS initially present with symptoms localized to the limbs or bulbar muscles. A very small percentage 1-2% of ALS patients had neck muscle weakness with head drop (Jokelainen et al, 1977; Gourie-Devi et al, 2003). However, in all the previously reported cases, the patients had limb involvement at the time of presentation which was absent in this case, and the head drop occurred after the onset of symptoms (Lange et al, 1986; Katz et al; 1996). Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients with atypical presentations

How (Not) to Palatini

We revisit the problem of defining non-minimal gravity in the first order formalism. Specializing to scalar-tensor theories, which may be disguised as `higher-derivative' models with the gravitational Lagrangians that depend only on the Ricci scalar, we show how to recast these theories as Palatini-like gravities. The correct formulation utilizes the Lagrange multiplier method, which preserves the canonical structure of the theory, and yields the conventional metric scalar-tensor gravity. We explain the discrepancies between the na\"ive Palatini and the Lagrange multiplier approach, showing that the na\"ive Palatini approach really swaps the theory for another. The differences disappear only in the limit of ordinary General Relativity, where an accidental redundancy ensures that the na\"ive Palatini works there. We outline the correct decoupling limits and the strong coupling regimes. As a corollary we find that the so-called `Modified Source Gravity' models suffer from strong coupling problems at very low scales, and hence cannot be a realistic approximation of our universe. We also comment on a method to decouple the extra scalar using the chameleon mechanism.Comment: 18 pages, LaTeX; added references and minor improvements in sec

Spanish Research Report for 1999

14 páginas, 18 tablasPeer reviewe

Adaptive Autoimmunity and Foxp3-Based Immunoregulation in Zebrafish

Background: Jawed vertebrates generate their immune-receptor repertoire by a recombinatorial mechanism that has the potential to produce harmful autoreactive lymphocytes. In mammals, peripheral tolerance to self-antigens is enforced by Foxp3+ regulatory T cells. Recombinatorial mechanisms also operate in teleosts, but active immunoregulation is thought to be a late incorporation to the vertebrate lineage. Methods/Principal Findings: Here we report the characterization of adaptive autoimmunity and Foxp3-based immunoregulation in the zebrafish. We found that zebrafish immunization with an homogenate of zebrafish central nervous system (zCNS) triggered CNS inflammation and specific antibodies. We cloned the zebrafish ortholog for mammalian Foxp3 (zFoxp3) which induced a regulatory phenotype on mouse T cells and controlled IL-17 production in zebrafish embryos. Conclusions/Significance: Our findings demonstrate the acquisition of active mechanisms of self-tolerance early in vertebrate evolution, suggesting that active regulatory mechanisms accompany the development of the molecular potential for adaptive autoimmunity. Moreover, they identify the zebrafish as a tool to study the molecular pathways controlling adaptive immunity

Energy optimization of a light projection system for buildings that virtually restores artworks

Published March 2022: Erratum regarding missing Declaration of Competing Interest statements in previously published articles - Part 1 (Digital Applications in Archaeology and Cultural Heritage, Volume 24, March 2022, Pages e00209). DOI: https://doi.org/10.1016/j.daach.2021.e00209; Erratum incluido en el documentoThe need to achieve energy efficiency standards in the lighting systems of buildings makes it necessary to optimize all aspects of them. Here, the development of a light projection system that achieves this goal by studyingand modifying the spectral output, compared to conventional illumination, is described. A lighting system thatestimates the reflectance characteristics of artwork and emits optimized lighting can reduce light absorption. Adamage-minimizing point-by-point light projection system is developed using an optimization algorithm, toimprove the appearance of the surfaces of artworks whose color has faded. In this case, a simulation of an aged oilpainting was made by manipulating the original photograph, which was printed and to which the proposedsystem was applied. The results show that, when the aged printed image is illuminated with the optimized lightsource, it appears indistinguishable from the non-aged oil painting.This research was supported by project RTI2018-097633-A-I00 from the Spanish Ministry funding "Proyectos I + D Retos de Investigación" entitled Photonic restoration applied to cultural heritage: application to Dali's picture "Dos Figuras

Spanish Research Report for 2002

26 páginas, 24 tablasPeer reviewe

Spanish Research Report for 2003

24 páginas, 22 tablasPeer reviewe

Spanish Research Report for 2000

24 páginas, 1 figura, 20 tablasPeer reviewe

Nueva economía 20+20

276 p : il.El fuerte avance de las Tecnologías de la Información y las Comunicaciones (TIC) ha provocado profundos cambios económicos, sociales y culturales en las últimas décadas (Cohen, De Long y Zysman, 2000). La gran velocidad con la que se ha producido este cambio ha quedado reflejada en cifras, como el avance de Internet que, desde 1969, fecha de su nacimiento, hasta la actualidad, se ha extendido a más del 25% de la población mundial, con más de 1.700 millones de usuarios en todo el mundo, siendo especialmente significativos los casos de Europa y Estados Unidos, con penetraciones del 52% y 74,2%, respectivamente (Internet World Stats, 2009). Es igualmente significativo el fuerte crecimiento experimentado por los ordenadores personales que, desde 1977, año en el que vio la luz el primero de ellos, se estima que alcanzaron los 1.000 millones en el 2008, y se hacen predicciones de que se doblará dicha cifra en torno al año 2014 (Gartner, 2008). El trabajo, el ocio, el transporte o, incluso, las relaciones personales se encuentran en un proceso de cambio permanente debido a la profunda influencia que estas tecnologías han tenido sobre la sociedad. Tanto es así que en la nueva realidad, a la que el conocimiento y la tecnología trasladan al ser humano, existen nuevas concepciones del tiempo o del espacio (Castells, 2005). Desde el punto de vista económico, se han producido cambios en la estructura mundial que han desembocado en una nueva economía. Por una parte, las nuevas tecnologías han transformado la economía tradicional, dando lugar a una compleja estructura interconectada de forma global, en la que el desarrollo de las comunicaciones ha jugado un papel fundamental. Adicionalmente, la incorporación de la tecnología en el proceso productivo ha transformado elementos tales como la localización, el tamaño, las estructuras o las relaciones entre las empresas (Brynjolfsson y Kahln, 2000). Por otra parte, las nuevas tecnologías dan lugar a un nuevo mercado de enormes dimensiones, formado por todos aquellos bienes y servicios que dependen de forma crítica de las tecnologías digitales o lo son en sí mismas, lo cual constituye la base de la economía digital (Kling y Lamb, 1999). Con todo ello, el presente informe se encarga, en primer lugar, de identificar en qué consiste la economía digital y cuáles son sus principales componentes. En el siguiente apartado se intenta medir la importancia de este sector, a través de los datos más significativos, que reflejan el crecimiento que dicho sector de la economía ha sufrido en los últimos años, y la importancia relativa del mismo respecto al resto de los sectores. A continuación se presenta una caracterización de los perfiles de empresa que constituyen la economía digital y se busca plantear, después, un modelo de variables significativas que nos permita medir de forma adecuada el sector.Esta publicación ha contado con la cofinancia- ción del Fondo Social Europeo a través del Pro- grama Operativo Plurirregional de Adaptabili- dad y Empleo 2007-2013.ÍNDICE Capítulo 0 EL PROYECTO SECTORES DE LA NUEVA ECONOMÍA 20+20 Capítulo 1 LA ECONOMÍA DIGITAL 1 Introducción 2 ¿Qué es la economía digital? 3 Importancia de la economía digital 4 Las empresas de la economía digital 5 ¿Cómo analizar la economía digital? 6 Conclusiones Capítulo 2 EXPERIENCIAS EMPRESARIALES 11870.COM AGNITIO ASPgems BARRABESBIZ BITDEFENDER BUONGIORNO BUYVIP COMEX GRUPO IBÉRICA DAEDALUS DEIMOS SPACE EPTISA TI ÍNCIPY ISDE ING KERAJET LA CIGÜEÑA DEL BEBÉ MICROGÉNESIS QAPACITY ÍNDICE TERRITORIO CREATIVO TOPRURAL TYVEN Capítulo 3 CONCLUSIONES Capítulo 4 BIBLIOGRAFÍ

Tribological Behavior of Nanolubricants Based on Coated Magnetic Nanoparticles and Trimethylolpropane Trioleate Base Oil

The main task of this work is to study the tribological performance of nanolubricants formed by trimethylolpropane trioleate (TMPTO) base oil with magnetic nanoparticles coated with oleic acid: Fe3O4 of two sizes 6.3 nm and 10 nm, and Nd alloy compound of 19 nm. Coated nanoparticles (NPs) were synthesized via chemical co-precipitation or thermal decomposition by adsorption with oleic acid in the same step. Three nanodispersions of TMPTO of 0.015 wt% of each NP were prepared, which were stable for at least 11 months. Two different types of tribological tests were carried out: pure sliding conditions and rolling conditions (5% slide to roll ratio). With the aim of analyzing the wear by means of the wear scar diameter (WSD), the wear track depth and the volume of the wear track produced after the first type of the tribological tests, a 3D optical profiler was used. The best tribological performance was found for the Nd alloy compound nanodispersion, with reductions of 29% and 67% in friction and WSD, respectively, in comparison with TMPTO. On the other hand, rolling conditions tests were utilized to study friction and film thickness of nanolubricants, determining that Fe3O4 (6.3 nm) nanolubricant reduces friction in comparison to TMPTOThis research was supported by the Spanish Ministry of Economy and Competitiveness and the ERDF programme through the ENE2017-86425-C2-2-R project. Moreover, this work was funded by the Xunta de Galicia (ED431E 2018/08, GRC ED431C 2017/22 and GRC ED431C 2016/001). These three funders also financed the acquisition of the 3D Optical Profile Sensofar S Neox (UNST15-DE-3156)S