A global-scale evaluation of extreme event uncertainty in the eartH2Observe project

Knowledge of how uncertainty propagates through a hydrological land surface modelling sequence is of crucial importance in the identification and characterisation of system weaknesses in the prediction of droughts and floods at global scale. We evaluated the performance of five state-of-the-art global hydrological and land surface models in the context of modelling extreme conditions (drought and flood). Uncertainty was apportioned between the model used (model skill) and also the satellite-based precipitation products used to drive the simulations (forcing data variability) for extreme values of precipitation, surface runoff and evaporation. We found in general that model simulations acted to augment uncertainty rather than reduce it. In percentage terms, the increase in uncertainty was most often less than the magnitude of the input data uncertainty, but of comparable magnitude in many environments. Uncertainty in predictions of evapotranspiration lows (drought) in dry environments was especially high, indicating that these circumstances are a weak point in current modelling system approaches. We also found that high data and model uncertainty points for both ET lows and runoff lows were disproportionately concentrated in the equatorial and southern tropics. Our results are important for highlighting the relative robustness of satellite products in the context of land surface simulations of extreme events and identifying areas where improvements may be made in the consistency of simulation models

Clinical Features And Management Of Hereditary Spastic Paraplegia.

Hereditary spastic paraplegia (HSP) is a group of genetically-determined disorders characterized by progressive spasticity and weakness of lower limbs. An apparently sporadic case of adult-onset spastic paraplegia is a frequent clinical problem and a significant proportion of cases are likely to be of genetic origin. HSP is clinically divided into pure and complicated forms. The later present with a wide range of additional neurological and systemic features. To date, there are up to 60 genetic subtypes described. All modes of monogenic inheritance have been described: autosomal dominant, autosomal recessive, X-linked and mitochondrial traits. Recent advances point to abnormal axonal transport as a key mechanism leading to the degeneration of the long motor neuron axons in the central nervous system in HSP. In this review we aim to address recent advances in the field, placing emphasis on key diagnostic features that will help practicing neurologists to identify and manage these conditions.72219-2

Aspectos clínicos e manejo das paraplegias espásticas hereditárias

Hereditary spastic paraplegia (HSP) is a group of genetically-determined disorders characterized by progressive spasticity and weakness of lower limbs. An apparently sporadic case of adult-onset spastic paraplegia is a frequent clinical problem and a significant proportion of cases are likely to be of genetic origin. HSP is clinically divided into pure and complicated forms. The later present with a wide range of additional neurological and systemic features. To date, there are up to 60 genetic subtypes described. All modes of monogenic inheritance have been described: autosomal dominant, autosomal recessive, X-linked and mitochondrial traits. Recent advances point to abnormal axonal transport as a key mechanism leading to the degeneration of the long motor neuron axons in the central nervous system in HSP. In this review we aim to address recent advances in the field, placing emphasis on key diagnostic features that will help practicing neurologists to identify and manage these conditions.Paraplegias espásticas hereditárias (PEH) constituem um grupo de desordens geneticamente determinadas caracterizadas por espasticidade e paraparesia de progressão insidiosa. Paraplegia espástica aparentemente esporádica de início no adulto constitui problema frequente na prática neurológica. Evidências recentes sugerem que uma proporção significativa destes casos é geneticamente determinada. O grupo das PEH é dividido clinicamente em formas puras e complicadas de acordo com a concomitância de outras manifestações clinicas e neurológicas. Até o momento 60 tipos genéticos foram identificados. Todos os modos de herança monogênica já foram descritos: autossômica dominante, autossômica recessiva, ligada ao X e mitocondrial. Avanços recentes indicam que alterações do transporte axonal estão implicadas na degeneração dos longos axônios motores no sistema nervoso central na PEH. Nesta revisão abordamos recentes avanços na área com ênfase nos aspectos clínicos chave que ajudam o neurologista geral no diagnóstico e manejo correto deste grupo de doenças.21922

Derek Denny-brown: The Man Behind The Ganglia

The authors present an historical review about the main contributions of Professor Derek Denny-Brown to neurology. Some of his achievements include the first description of sensory neuronopathies, and some of the essential textbooks on the function and anatomy of the basal ganglia. In 2016, on the 35th anniversary of his death, modern neurologists are still strongly influenced by his legacy.75212712

Effect of starvation and subsequent feeding on glycogen concentration, behavior and mortality in the golden mussel Limnoperna Fortunei (Dunker, 1857) (Bivalvia: Mytilidae)

The success of Limnoperna fortunei as an invasive species is related to its physiological plasticity that allows them to endure adverse environmental conditions. Starvation tolerance is considered to be an important trait associated with bivalve invasiveness. In natural ecosystems, food resources can vary during the year, exposing mussels to variable periods of starvation or limited food availability. Thus, mussels have developed physiological strategies to tolerate fluctuations in food availability. Glycogen concentration has been used in different monitoring studies as an indicator of the nutritional condition of bivalves. The aim of this study was to investigate the physiological responses of L. fortunei based on the glycogen concentrations of specimens under four treatments, comprising differentcombinations of feeding and starvation, during 125 days. The experiment was carried out in two phases. In the phase I, mussels were divided in two treatments: starvation (S) and feeding (F). After 100 days, tissue samples were collected to quantify glycogen concentrations and, each phase I group was divided in two subgroups: starvation (S) and feeding (F), resulting in four treatments. In the phase II, that lasted 25 days, starvation specimens (S) from phase I were allowed to feed (starvation-feeding treatment, or S-F), or continued to undergo starvation (starvation-starvation treatment, or S-S) and the feeding specimens (F) continued feeding (feeding-feeding group, or F-F), or were subjected to starvation (feeding-starvation treatment, or F-S). Behavior (valve-closing) and mortality were recorded in 24 h intervals. After the 25 days (phase II) all specimens were killed, and thei r soft tissue was removed to quantify glycogen concentrations. The glycogen concentration of the S-F treatment was lower than that of the F-S treatment, which was initially allowed to feed (phase I) and then subjected to starvation (phase II). Stability in the glycogen concentrations was observed when the phase II feeding conditions were maintained during the experiments, as observed in the S-S (continued starvation) and F-F (continued feeding) treatments. Based on our glycogen concentrations results, the golden mussel shows a higher tolerance to starvation (125 days) than has previously been published, which suggests that its tolerance strongly influences its invasive behavior.Fil: Cordeiro, Nelmara I. S.. Universidade Federal de Minas Gerais; BrasilFil: Andrade, Jennifer T. M.. Universidade Federal de Minas Gerais; BrasilFil: Montresor, Lângia C.. Fundación Oswaldo Cruz; BrasilFil: Luz, Dalva M. R.. Universidade Federal de Minas Gerais; BrasilFil: Martinez, Carlos B.. Universidade Federal de Minas Gerais; BrasilFil: Darrigran, Gustavo Alberto. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Naturales y Museo. División Zoología Invertebrados; ArgentinaFil: Pinheiro, Jairo. Universidade Federal Rural Do Rio de Janeiro;Fil: Vidigal, Teofânia H. D. A.. Universidade Federal de Minas Gerais; Brasi

Experiencia clínica del tratamiento con onabotulinumtoxin A en pacientes con migraña refractaria

To analyse our experience in the treatment of refractory chronic migraine, episodic frequent refractory migraine (>= 10 days/month), with onabotulinumtoxin A (OnabotA). PATIENTS AND METHODS. Retrospective analysis of patients with refractory migraine who underwent, at least two sessions of OnabotA pericranial injections following the PREEMPT protocol between 2008 and 2012. The efficacy of OnabotA was evaluated comparing the basal situation with 12-16 weeks after the second session. We analysed the subjective improvement of the patients, number of days with headache, preventive and abortive drugs consumption, and adverse effects. RESULTS. Forty-one patients (37 women, 4 male) were identified. 65.8% patients experienced subjective improvement after OnabotA treatment. 36.58% responded (reduction of > 50% in headache days). Differences between days with headache before the first session (24.5 +/- 7.3), and 12-16 weeks after the second session (17.4 +/- 11.6), as well as the differences between the number of abortive drugs taken before the first session (26.8 +/- 23.1) and 12-16 weeks after the second session (16.7 +/- 19.3), were statistically significant (p < 0.001). Subgroups analysis showed that all differences were significant, except for the reduction of the number of days with headache in patients with episodic frequent refractory migraine. CONCLUSION. Our work shows that treatment with OnabotA is safe and useful in patients with episodic and chronic refractory migraine, including those patients with medication overuse headache

Carbono y nitrógeno recalcitrante en un suelo con dos sistemas de labranza: efectos de largo plazo

Los cambios de las formas más activas de la materia orgánica (MO) a formas intermedias o pasivas favorece el secuestro de carbono (C) en los suelos. Los objetivos del trabajo fueron i) evaluar los cambios de largo plazo en las fracciones recalcitrantes de C y nitrógeno (N) y ii) calcular el índice de recalcitrancia (IR) de estas fracciones en un suelo ubicado en un sitio subhúmedo del sudoeste bonaerense. La experiencia se realizó sobre un ensayo de larga duración en un suelo Argiudol Típico con dos sistemas de labranza contrastantes: labranza convencional (LC) y siembra directa (SD). Se tomaron muestras de suelo en los años 2003, 2009 y 2015 en las profundidades de 0-5 y 0-20 cm. Se determinó C orgánico total (COT), N total (Nt), C y N orgánico recalcitrante (COR y NOR). Además se calculó el IR de C (IRC) y de N (IRN). Los niveles de COT y Nt fueron mayores en SD en ambas profundidades y para todos los años. El COR en la profundidad 0-5 cm mostró diferentes efectos tanto por la labranza como por los años En 0-20 cm, sólo mostró diferencias significativas (p&amp;lt;0,05) entre años, sin efecto por la labranza. Por otra parte, el NOR evidenció diferencias altamente significativas entre sistemas de labranza y años en 0-5 cm, y diferencias significativas entre años para 0-20 cm. El IRC en superficie mostró diferencias entre los sistemas de labranza. La mayor acumulación del C y N en SD no resultó en una mayor humificación de las fracciones lábiles a formas estables. El secuestro de C en superficie mostró variabilidad con respecto al tiempo, mientras que en 0-20 cm no demostró estar asociado a los sistemas de labranza pero sí al efecto del año.Publicado en las Actas de las III Jornadas Nacionales de Suelos de Ambientes Semiáridos

Maternal imprinting on cognition markers of wild type and transgenic Alzheimer's disease model mice

The risk of suffering from Alzheimer’s disease (AD) is higher in individuals from AD-affected mothers. The purpose of this investigation was to study whether maternal transmission might produce AD-related alterations in progenies of mice that do not have any genotypic alteration. We used cognitively-intact mothers harbouring in heterozygosity the transgene for overexpressing the Swedish double mutant version of the human amyloid precursor protein (hAβPPswe). The phenotype of the offspring with or without the transgene resulting from crossing young Tg2576 females with wild-type males were compared with those of the offspring resulting from crossing wild-type females with Tg2576 males. The hAβPPswe-bearing offspring from Tg2576 mothers showed an aggravated AD-like phenotype. Remarkably, cognitive, immunohistochemical and some biochemical features displayed by Tg2576 heterozygous mice were also found in wild-type animals generated from Tg2576 females. This suggests the existence of a maternal imprinting in the wild-type offspring that confers a greater facility to launch an AD-like neurodegenerative cascade. Such progeny, lacking any mutant amyloid precursor protein, constitutes a novel model to study maternal transmission of AD and, even more important, to discover early risk markers that predispose to the development of AD

Filtration efficiency of air conditioner filters and face masks to limit exposure to aerosolized algal toxins

Harmful algal blooms (HABs) can generate toxins that can be aerosolized and negatively impact human health through inhalation. HABs are often found in waterways near residences, therefore, aerosolized HAB toxins can potentially affect both indoor and outdoor air quality. Given that HABs are predicted to increase worldwide, effective mitigation strategies are needed to prevent the inhalation of aerosolized HAB toxins. In this work, we characterized both the particle filtration efficiency using particle sizing instruments as well as the mass concentration of different congeners of aerosolized microcystin (MC) toxins that penetrate through commercially available face masks and air conditioner (AC) filters. Particles were generated from cultures of the toxin-producing cyanobacteria Microcystis aeruginosa. Hydrophobic congeners of microcystin including MC-LF and MC-LW were enriched in aerosols compared to water, with MC-LR being the most abundant, which has implications for the toxicity of inhalable particles generated from HAB-contaminated waters. Particle transmission efficiencies and toxin filtration efficiencies scaled with the manufacturer-provided filter performance ratings. Up to 80% of small, microcystin-containing aerosols were transmitted through AC filters with low filter performance ratings. In contrast, both face masks as well as AC filters with high filter performance ratings efficiently removed toxin-containing particles to below limits of quantification. Our findings suggest that face masks and commercially available AC filters with high filtration efficiency ratings are suitable mitigation strategies to avoid indoor and outdoor air exposure to aerosolized HAB toxins. This work also has relevance for reducing airborne exposure to other HAB toxins, non-HAB toxins, pathogens, and viruses, including SARS-CoV-2, the virus responsible for the COVID-19 pandemic

Characterization of 8p21.3 chromosomal deletions in B-cell lymphoma: TRAIL-R1 and TRAIL-R2 as candidate dosage-dependent tumor suppressor genes

Deletions of chromosome 8p are a recurrent event in B-cell non-Hodgkin lymphoma (B-NHL), suggesting the presence of a tumor suppressor gene. We have characterized these deletions using comparative genomic hybridization to microarrays, fluorescence in situ hybridization (FISH) mapping, DNA sequencing, and functional studies. A minimal deleted region (MDR) of 600 kb was defined in chromosome 8p21.3, with one mantle cell lymphoma cell line (Z138) exhibiting monoallelic deletion of 650 kb. The MDR extended from bacterial artificial chromosome (BAC) clones RP11-382J24 and RP11-109B10 and included the tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) receptor gene loci. Sequence analysis of the individual expressed genes within the MDR and DNA sequencing of the entire MDR in Z138 did not reveal any mutation. Gene expression analysis and quantitative reverse transcriptase-polymerase chain reaction (QRT-PCR) showed down-regulation of TRAIL-R1 and TRAIL-R2 receptor genes as a consistent event in B-NHL with 8p21.3 loss. Epigenetic inactivation was excluded via promoter methylation analysis. In vitro studies showed that TRAIL-induced apoptosis was dependent on TRAIL-R1 and/or -R2 dosage in most tumors. Resistance to apoptosis of cell lines with 8p21.3 deletion was reversed by restoration of TRAIL-R1 or TRAIL-R2 expression by gene transfection. Our data suggest that TRAIL-R1 and TRAIL-R2 act as dosage-dependent tumor suppressor genes whose monoallelic deletion can impair TRAIL-induced apoptosis in B-cell lymphoma