Clinical Association of White Matter Hyperintensities Localization in a Mexican Family with Spastic Paraparesis Carrying the PSEN1 A431E Mutation

Presenilin 1 gene (PSEN1) mutations are the most common cause of familial Alzheimer’s disease (FAD). One of the most abundant FAD mutations, PSEN1 A431E, has been reported to be associated with spastic paraparesis in about half of its carriers, but the determining mechanisms of this phenotype are still unknown. In our study we characterized three A431E mutation carriers, one symptomatic and two asymptomatic, from a Mexican family with a history of spastic paraparesis in all of its affected members. At cognitive assessment and MRI, the symptomatic subject showed an atypical non-amnestic mild cognitive impairment with visuospatial deficits, olfactory dysfunction and significant parieto-occipital brain atrophy. Furthermore, we found several periventricular white matter hyperintensities whose progression pattern and localization correlated with their motor impairment, cognitive profile, and non-motor symptoms. Together, our data suggests that in this family the A431E mutation leads to a divergent neurological disorder in which cognitive deterioration was clinically exceeded by motor impairment and that it involves early glial and vascular pathological changes

Structural and magnetic characterization of the intermartensitic phase transition in NiMnSn Heusler alloy ribbons

"Phase transitions and structural and magnetic properties of rapidly solidified Ni50Mn38Sn12 alloy ribbons have been studied. Ribbon samples crystallize as a single-phase, ten-layered modulated (10M) monoclinic martensite with a columnar-grain microstructure and a magnetic transition temperature of 308 K. By decreasing the temperature, martensite undergoes an intermartensitic phase transition around 195 K. Above room temperature, the high temperature martensite transforms into austenite. Below 100 K, magnetization hysteresis loops shift along the negative H-axis direction, confirming the occurrence of an exchange bias effect. On heating, the thermal dependence of the coercive field H-C shows a continuous increase, reaching a maximum value of 1017 Oe around 50 K. Above this temperature, H-C declines to zero around 195 K. But above this temperature, it increases again up to 20 Oe falling to zero close to 308 K. The coercivity values measured in both temperature intervals suggest a significant difference in the magnetocrystalline anisotropy of the two martensite phases.

In-situ growth rate assessment of hexactinellid sponge Asconema setubalense using 3D photogrammetric reconstruction

We describe the first application of a non-invasive and novel approach to estimate the growth rate of Asconema setubalense (Porifera, Hexactinellida) through the use of 3D photogrammetric methodology. Structure-from-Motion techniques (SfM) were applied to videos acquired with the Politolana ROTV in the El Cachucho Marine Protected Area (MPA) (Cantabrian Sea) on three different dates (2014, 2017, and 2019) over six years. With these data, a multi-temporal study was conducted within the framework of MPA monitoring. A complete 3D reconstruction of the deep-sea floor was achieved with Pix4D Mapper Pro software for each date. Having 3D point clouds of the study area enabled a series of measurements that were impossible to obtain in 2D images. In 3D space, the sizes (height, diameter, cup-perimeter, and cup-surface area) of several A. setubalense specimens were measured each year. The annual growth rates recorded ranged from zero (“no growth”) for a large size specimen, to an average of 2.2 cm year�����1 in cup-diameter, and 2.5 cm year�����1 in height for developing specimens. Von Bertalanffy growth parameters were estimated. Taking into account the size indicators used in this study and based on the von Bertalanffy growth model, this sponge reaches 95% maximum size at 98 years of age. During the MPA monitoring program, a high number of specimens disappeared. This raised suspicions of a phenomenon affecting the survival of this species in the area. This type of image-based methodology does not cause damage or alterations to benthic communities and should be employed in vulnerable ecosystem studies and MPA monitoring. Keywords: underwater photogrammetry, Asconema setubalense, Marine ProtectedPostprin

Community composition and habitat characterization of a rock sponge aggregation (Porifera, Corallistidae) in the Cantabrian Sea.

Deep-sea sponge-dominated communities are complex habitats considered hotspots of biodiversity and ecosystem functioning. They are classified as Vulnerable Marine Ecosystem and are listed as threatened or declining as a result of anthropogenic activities. Yet, studies into the distribution, community structure and composition of these habitats are scarce, hampering the development of appropriate management measures to ensure their conservation. In this study we describe a diverse benthic community, dominated by a lithistid sponge, found in two geomorphological features of important conservation status —Le Danois Bank and El Corbiro Canyon— of the Cantabrian Sea. Based on the analyses of visual transects using a photogrammetric towed vehicle and samples collected by rock dredge, we characterize the habitat and the associated community in detail. This deep-sea sponge aggregation was found on bedrock. It is dominated by one lithistid sponge, Neoschrammeniella aff. bowerbankii (0.2 ind./m2) and further composed of various sponge species as well as of other benthic invertebrates such as cnidarians, bryozoans and crustaceans. Using a non-invasive methodology (SfM – Structure from Motion) and empirical relationships of individuals size and biomass/volume obtained in laboratory for N. aff. bowerbankii, we were able to estimate a total biomass of 41 kg and volume of 39 l of this species in the surveyed area. This approach allows a fine tune methodology for estimating biomass and volume by image-based-observed area avoiding destructive techniques for this species.Postprin

Influence of crystalline orientation on the thermal-energy convertion efficiency in shape memory alloys

A theoretical study about the influence of crystalline orientation on the energy conversion efficiency of two shape memory alloys is present. The study consider single crystal samples in simple tension. The results showed higher properties for of the NiTi than Cu-Al-Be, especially in the temperature-increment (ΔT) necessary for get a conversion energy cycle. However, the study showed a crystalline orientation favorable for Cu-Al-Be

P9 23. Seudoaneurisma aórtico con infeción de prótesis en aorta ascendente. ¿Es necesario retirar siempre la prótesis? ¿Cuánto tiempo con tratamiento antibiótico?

IntroducciónEl seudoaneurisma aórtico con infección de prótesis en aorta ascendente (PIPAA) tras cirugía cardíaca es una entidad infrecuente (0,9-2%) pero grave (mortalidad intrahospitalaria > 40%). El tratamiento más extendido es la cirugía con recambio protésico y terapia antibiótica adecuada a antibiograma; pero el recambio protésico en ocasiones es técnicamente inviable e incluso puede aumentar la mortalidad perioperatoria. Existen casos en los que se ha preservado la prótesis infectada con éxito terapéutico, realizándose limpieza/reparación quirúrgica local apoyada con omentoplastia. No existe consenso en la duración de la terapia médica, y el tratamiento «supresor a largo plazo» en ocasiones se complica por efectos adversos de los antibióticos.ObjetivosAportar dos nuevos casos y evaluar el tratamiento realizado tras un seguimiento a largo plazo.MétodoAnálisis descriptivo de aspectos microbiológicos, farmacológicos y resultados de la terapia realizada, en dos casos de PIPAA de pacientes intervenidos por disección de aorta (prótesis de dacrón en posición supracoronariana) y por insuficiencia y anuloectasia aórtica (tubo valvulado). Se realiza tratamiento quirúrgico conservador de la prótesis aórtica (limpieza quirúrgica, reparación del seudoaneurisma y omentoplastia), asociándose terapia antibiótica prolongada ajustada a antibiograma.ConclusiónAmbos casos presentan, tras más de 1 año de seguimiento, según criterios clínicos, microbiológicos y pruebas de imagen, ausencia de signos de recidiva infecciosa, resultando la terapia adecuada. Aun sin poder establecer tiempo óptimo de tratamiento, serían razonables 6 semanas de tratamiento endovenoso seguidas de 24 semanas de terapia supresora, a ser posible oral, y valorar su retirada siempre que no existan signos de recidiva

Demersal and epibenthic communities of sedimentary habitats in the Avilés Canyon System, Cantabrian Sea (NE Atlantic)

The aim of this study was to describe the epibenthic and demersal communities of the Avilés Canyon System (ACS) in relation to the environmental variables that characterize their biotope. ACS (Cantabrian Sea, NE Atlantic) was recently included in the Natura 2000 network as a Site of Community Importance (SCI). Data of faunal biomass derived from 6 surveys carried out using beam trawl and otter trawls in 2009 and 2010 within INDEMARES and ERDEM projects. Data were divided into two groups to obtain information about the two ecological compartments: benthic and demersal. The total number of species used in this analysis ascended to 116 in the case of benthic organisms and 110 in the case of demersal. Hierarchical clustering analysis was applied to obtain groups of samples similar in terms of species composition for the two ecological components. Depth was the main discriminating factor for grouping hauls, showing high consistency of bathymetric range independently from the compartment examined. Six groups were identified by depth as follows: medium shelf (∼100–200 m), external shelf (∼150–300m), shelf break (∼300–400 m), upper-slope (∼500–700 m), upper middle-slope (∼700–1100 m), and lower-middle-slope (1200–1500 m). SIMPER analysis on biomass values was performed to determine the structure of the faunal assemblages observed for each group in both compartments. Using biomass values for the analysis allowed for the exploration of groups playing important roles in ecosystem functioning and energy fluxes taking place on the sedimentary bottom of this SCI. Finally, CCA analysis revealed that the main environmental drivers were depth, broad scale bathymetric position index (BPI), near-bottom salinity, sedimentary type, and dynamics related variables (Q50 phy and So). This study gives an inventory of the soft bottom assemblages along a very wide depth range (100–1500 m) inside a SCI, linking both epibenthic and demersal communities with the biotope preferences. This study contributes to fundamental knowledge on soft-bottom communities as a pre-requisite, necessary for the next steps in terms of management framework in the SCI

Synaptic, axonal damage and inflammatory cerebrospinal fluid biomarkers in neurodegenerative dementias

INTRODUCTION: Synaptic damage, axonal neurodegeneration, and neuroinflammation are common features in Alzheimer's disease (AD), frontotemporal dementia (FTD), and Creutzfeldt-Jakob disease (CJD). METHODS: Unicentric cohort of 353 participants included healthy control (HC) subjects, AD continuum stages, genetic AD and FTD, and FTD and CJD. We measured cerebrospinal fluid neurofilament light (NF-L), neurogranin (Ng), 14-3-3, and YKL-40 proteins. RESULTS: Biomarkers showed differences in HC subjects versus AD, FTD, and CJD. Disease groups differed between them except AD versus FTD for YKL-40. Only NF-L differed between all stages within the AD continuum. AD and FTD symptomatic mutation carriers presented differences with respect to HC subjects. Applying the AT(N) system, 96% subjects were positive for neurodegeneration if 14-3-3 was used, 94% if NF-L was used, 62% if Ng was used, and 53% if YKL-40 was used. DISCUSSION: Biomarkers of synapse and neurodegeneration differentiate HC subjects from neurodegenerative dementias and between AD, FTD, and CJD. NF-L and 14-3-3 performed similar to total tau when AT(N) system was applied

Effect of immunocastration on behaviour and blood parameters (cortisol and testosterone) of Holstein bulls

To evaluate the effect that immunocastration has on behaviour, testosterone and cortisol levels of feedlot Holstein bulls, 720 intact animals aged between 7 and 8 months, weighing 232±1.19 kg were randomly assigned to two treatments: immunocastration using the Bopriva vaccine and a placebo (360 animals per treatment). The bulls were slaughtered at day 239 of treatment. Animals were vaccinated on days 1, 21, 101, and 181, and on those same days testosterone levels were measured; while cortisol, glucose and creatin kinase measurements were done on day 181 and during exanguination at slaughter. Sexual, aggressive and social behaviours were evaluated and it was found that intact bulls showed a higher average of head butts, mounting, threats, flehmen sign and sniffing (P<0.05), no differences were found for vocalisations, lowering of the head and grooming (P>0.05). Testosterone levels in intact bulls remained at 0.47ng/mL throughout the study, however, by day 181 differences (P<0.05) were observed in immunised bulls, with values of 0.22ng/mL. At slaughter, testosterone levels were 0.21 ± 0.06 ng/mL in immunocastrated bulls and 0.54 ± 0.06 ng/mL in the placebo group. The use of immunocastration with Bopriva has shown to be effective to reduce testosterone, sexual and aggressive behaviours on Holstein bulls