Miracle-dz/UniFAR-FTUs huggingface dataset:
Familial Creutzfeldt-Jakob disease with an R208H-129V haplotype and Kuru plaques.

OBJECTIVE To report the clinical and neuropathological features in the first patient seen, to our knowledge, with familial Creutzfeldt-Jakob disease and an R208H mutation associated with a Val/Val homozygosity at codon 129 in the prion protein gene (PRNP) and a type 2 protease-resistant prion protein. PATIENT AND RESULTS A 61-year-old man with a long-standing history of memory loss and emotional disorders had an obvious behavioral change. Then he developed cerebellar ataxia, followed by cognitive decline. He had no myoclonus. Electroencephalography showed slow activity, and 14-3-3 protein detection was negative. Finally, the patient developed akinetic mutism and died 7 months after the onset of ataxia. Neuropathological examination showed severe spongiform changes in the frontal cortex and striatum and gliosis in the striatum and thalamus. Kuru plaques were noted in the cerebellum, notably in the molecular layer. Immunohistochemical findings showed granular, synaptic, perineuronal, and perivacuolar staining with antiprion antibodies. Kuru plaques were also stained. CONCLUSION This study strengthens the linkage of the R208H mutation to Creutzfeldt-Jakob disease and points to some particular features such as Kuru plaques and long-standing psychiatric signs.
Muscular adaptation to concentric and eccentric exercise at equal power levels.

The effect of training with concentric and eccentric contractions on fiber hypertrophy and isometric torque production was investigated in 20 healthy subjects. One group (eight female and two male subjects) performed concentric contractions of their quadriceps femoris muscles at an intensity of 90% of their maximal concentric power. The other group (six female and four male subjects) performed eccentric contractions at the same relative power level. Both groups exercised three times per week for 4 wk at a constant speed of 60 degrees.s-1 on a Kin-Com dynamometer. Needle biopsies were obtained from the vastus lateralis before and after the exercise program. Fiber-type differentiation was performed using a myosin ATPase stain at a preincubation of 10.5. Maximal isometric knee extension torque was also measured before and after the exercise program. An analysis of covariance was used to determine whether there were significant differences between the exercise groups in: 1) the post-exercise fiber areas and 2) maximal isometric torque (MIso), while controlling for initial differences. Results showed a significant difference between the Type II fiber areas (P < 0.01) and the MIso (P = 0.01). These data indicate that, when exercising at the same relative power level, a subject performing concentric contractions will show greater muscle hypertrophy and improve in MIso production more than a subject training with eccentric contractions.
ecutives en UEROS : la BADS comme indicateur du handicap ?

Le traumatisme crânien est la principale cause de mortalite et de handicap chez les jeunes europeens. Le syndrome dysexecutif, tres invalidant, en est l'une des sequelles les plus repandues. L'evaluation de l'efficience des fonctions executives, comme un facteur explicatif du handicap qui peut en decouler, est donc un enjeu majeur pour le neuropsychologue intervenant aupres de personnes victimes de lesions cerebrales d'origine traumatique. Si le bilan neuropsychologique classique a un reel interet pour definir precisement la nature des deficits, il est en revanche assez largement admis aujourd'hui que les tests executifs classiques sont parfois trop peu sensibles pour detecter des troubles fins ayant pourtant des repercussions dramatiques en termes d'insertion sociale et professionnelle sur la vie des personnes traumatisees. L'association de tests classiques et ecologiques semble donc indiquee en vue d'obtenir des informations complementaires et permettre le reperage de tels troubles. Notre etude a donc tente d'evaluer l'interet et les limites de l'administration d'une batterie d'evaluation ecologique des fonctions executives : la BADS, dans un contexte de reinsertion professionnelle a distance du traumatisme initial. Les resultats de notre travail, base sur trois etudes de cas, montrent que la BADS semble permettre une evaluation pertinente des fonctions executives. Si elle n'a pas paru suffisamment sensible pour differencier les resultats d'une personne victime de TC leger d'un echantillon controle, elle semble efficace pour reperer les troubles de personnes victimes de TC severes et appelle a de nouvelles etudes sur des echantillons plus consequents.
Classification of EEG signal during gaze on the different rotating vanes

In this paper a novel brain-computer interface based on the gaze on rotating vane using five channels of EEG signal is proposed. Classification of EEG signal is done in three sessions: 1-when vane rotates fast and slow in an anti-clockwise manner, 2-when vane rotates slow in a clockwise and rotates fast in an anti-clockwise manner, 3-when vane rotates slow in a clockwise and rotates slow in an anti-clockwise manner. The signals were obtained from seven healthy human subjects in age groups between 25 and 32 years old. Discrete Wavelet transform (DWT) were used to extracted feature vectors. The features classified by two classifiers, k-nearest neighbor (k-NN) algorithm and Linear Discriminant Classifier (LDC). Our results demonstrated that LDC was more accurate compared to k-NN. Analysis was carried out using MATLAB Software.
Online trajectory prediction and planning for social robot navigation

This paper addresses the safe and legible navigation of mobile robots in multi-agent encounters. A novel motion model provides the basis to predict, plan and coordinate agent trajectories in intersection scenarios. The approach establishes an implicit, non-overt cooperation between the robot and humans by linking the prediction and planning of agent trajectories within a unified representation in terms of timed elastic bands. The planning process maintains multiple topological alternatives to resolve the encounter in a manner compliant with the implicit rules and objectives of human proxemics. The trajectory is obtained by optimizing the timed elastic band considering multiple conflicting objectives such as fastest path and minimal spatial separation among agents but also global proxemic aspects such as motion coherence within a group. Cooperation is achieved by coupling predicted and planned agent trajectories to eventually reach an implicit agreement of the agents on how to circumnavigate each other. The parameters of the cost functions of the underlying motion model are identified by inverse optimal control from a dataset of 73 recorded encounters with up to five humans and a total of 283 individual trajectories. Playback simulations of recorded encounters and experiments with a robot traversing a group of oncoming humans demonstrate the feasibility of the approach to resolve general proxemic encounters.
Endoplasmic Reticulum Stress in the Kidney as a Novel Mediator of Kidney Injury

The endoplasmic reticulum (ER) plays an important role in the maintenance of protein homeostasis through its control of the concentration, conformation, folding, and trafficking of client proteins. Disturbances such as hypoxia, glucose depletion, and oxidative stress may lead to ER dysfunction, which can induce ER stress and the subsequent unfolded protein response (UPR). The UPR initially serves as an adaptive response, but will also induce apoptosis in cells under severe or prolonged ER stress. Oxidative stress and inflammation are compounded by ER stress via the UPR, suggesting the potential pathophysiological significance of this response across a wide range of diseases. Accumulating evidence indicates that ER stress contributes to glomerular and tubular damage in patients with acute and chronic kidney disease. In glomeruli, podocyte or mesangial dysfunction tends to induce the adaptive UPR, which involves ER chaperone expression and the attenuation of protein translation, to maintain ER homeostasis and ensure cell survival. In tubules, apoptosis resulting from epithelial cell damage is caused, at least in part, by the proapoptotic UPR. These findings emphasize the possibility of the development of novel renoprotective drugs which target ER stress.
Target-tracking deliveries using conventional multileaf collimators planned with 4D direct-aperture optimization

Respiratory motion-induced degradation of intensity-modulated radiotherapy can be corrected by the dynamic target-tracking motion of multileaf collimator equipment on a conventional linear accelerator. This paper presents a new system by which the motion of the tissue and the delivery equipment can be incorporated into the treatment optimization using a 4D direct-aperture optimization method. The program can optimize a static or dynamic delivery with respect to a 4D patient model. The individualized patient model consists of a series of discrete phases and describes changes in tissue: deformation, geometry, attenuation and scatter properties over the breathing cycle. A set of treatment apertures is matched to the respiratory phases of the motion model, and motion of the apertures between phases is constrained by the maximum leaf velocity. Plans with dynamic and static deliveries optimized on 4D patient models were compared to static plans optimized on a single phase. This investigation was carried out on a 4D digital motion phantom and repeated on a 4D patient model. The effect of motion of the static plan on the 4D phantom was evaluated by recalculating dose from all phases of the 4D model. The plan cost was evaluated as a combination of the rms spread in tumour dose from the prescribed dose and the volume of normal lung receiving doses above 10 Gy with relative weightings of 5 and 1 respectively. The motion was found to degrade the static plan by 30 ± 4% with respect to the 3D cost function value. In contrast, the motion did not cause significant degradation to a treatment if the treatment was optimized on the 4D phantom and the cost was improved by 16 ± 3% by optimizing with dynamic leaf tracking motion. All results are relative to the static single-phase plan. In the 4D patient model the observed tissue motion was considerably less and the measured benefit of 4D planning was consequently reduced. For the 4D patient the plan cost was not significantly changed by the tissue motion. Optimizing on 4D patient conferred an improvement of 7.5 ± 0.3%, and the 4D plan with dynamic leaf motion improved the plan cost by 8.3 ± 0.1%.
Seasonal dependence of WRF model biases and sensitivity to PBL schemes over Europe

The seasonal dependence of Weather Research and Forecasting (WRF) model surface temperature biases and sensitivity to planetary boundary layer (PBL) schemes are jointly explored. For this purpose, the year 2001 was simulated using three different PBL schemes in a domain covering all Europe. The simulations were compared with gridded observations, upper‐air data and high‐frequency station data. Seasonal and daily cycles were analysed, aimed at providing a link between long‐term biases and restricted case studies. The results show that the model mean bias significantly depends on the season, being warm in winter and cold in summer. The winter warm bias is related to misrepresented cold extremes, while a systematic cold bias dominates the whole temperature range in summer. Regarding PBL schemes, an overall underestimation of the entrainment is found, with the non‐local Yonsei University scheme producing systematically warmer temperatures. It is shown that the opposite seasonal biases and systematic behaviour of the PBL schemes during the year lead to a different best‐performing scheme in winter and summer. Moreover, the best‐performing PBL scheme in an average sense is a result of the compensation of errors. The average summer results can be partially explained by a detailed case study. It is concluded that short‐term studies should be used with caution to decide on the parametrizations to be used in long‐term simulations. Copyright © 2012 Royal Meteorological Society
The Housing Security Consequences of Underemployment

This Positioning Paper introduces a research project that aims to provide an Australia-wide analysis of the consequences of underemployment for housing security. It explores the connection between an increasingly important but problematic feature of contemporary labour markets (underemployment) and a crucial dimension of housing research and policy (housing security). Underemployment is defined as those working less than 35 hours in a given week who would prefer to work more paid hours. In Australia, underemployment is well above the OECD average (OECD Employment Outlook, 2010). Underemployment, like unemployment, raises several housing policy concerns about the capacity of households to meet ongoing housing costs during a period of reduced or inadequate income. Small changes to incomes for some households pose a significant threat to housing security and stability. The research will make use of several quantitative secondary data sources including ABS labour force statistics and the Household Income and Labour Dynamics in Australia (HILDA) survey.
Don't hold your breath: anoxic convulsions from coupled hyperventilation–underwater breath‐holding

Clinical record We report anoxic convulsions occurring in two medical students competing in a breath-hold dive competition in shallow water. The seizure-like activity occurred during a competition called the "Dolphin Dive", which was part of a university medical school swimming function. The goal set for the competitors was to swim as far as possible underwater in a swimming pool without taking a breath. Three students participated in the event and two students had convulsions. In both cases, the students hyperventilated before the dive. Student 1 A 27-year-old man, with a past medical history of asthma, had been involved in underwater hockey and spear fishing, and was previously able to underwater breath-hold for 2 minutes while keeping still. During the competition, he swam about 60 metres and spent 40 seconds underwater. After the dive, he stood up in the water, lost consciousness and was witnessed to have multifocal myoclonic jerks lasting about 1 minute. He recalled some jerking of the limbs when asked afterward. Student 2 A 27-year-old man, with no significant past medical history, had previously swum at national championship level and had worked as a surf lifesaver. He reported swimming about 85 metres for the competition and spending 90 seconds underwater. At the end of his dive, he became unconscious, floated to the surface of the pool and had multifocal myoclonic jerks lasting less than 1 minute. He had no recollection of the convulsive movements. Many people were observing the race and so the students were rapidly retrieved from the pool; they regained consciousness and did not experience any long-term consequences.
B cell activation through CD40 and IL4R ligation modulates the response of chronic lymphocytic leukaemia cells to BAFF and APRIL

The two tumour necrosis factor family proteins BAFF (TNFSF13B) and APRIL (TNFSF13) and their receptors [BAFF‐R (TNFRSF13C), TACI (TNFRSF13B), BCMA (TNFRSF17)] play a critical role in the survival of normal B cells. The sensitivity of normal B cells to BAFF and APRIL can be modulated by signals regulated by their receptors. This modulation, however, has not been extensively investigated in chronic lymphocytic leukaemia (CLL) cells. We evaluated the expression, regulation and signalling of BAFF and APRIL receptors in normal and in CLL cells upon stimulation through CD40+IL4R and BCR. We further analysed the prognostic value of BAFF and APRIL receptors expression in patients with CLL. BCMA expression was significantly higher on CLL cells than on normal B cells. BCR and CD40+IL4R stimulation promoted an increase in TACI and BCMA expression, cell viability and activation in normal B cells. A similar effect was observed in CLL cells after CD40+IL4R but not BCR stimulation. BCMA expression correlated with unmutated IGHV genes, poor‐risk cytogenetics, and short progression‐free survival. These findings further characterize the link between CD40+IL4R regulatory signals, BAFF, APRIL and their receptors and the survival of leukaemic cells and clinical features of CLL.
THE LANGUAGE TEACHER AND STUDENTS' MOTIVATION: LET'S USE DIFFERENT TYPES OF ACTIVITIES IN THE CLASSROOM!

The following article aims to encourage reflection on the teaching and learning of a foreign language. The paper seeks to emphasize the importance of one of the most fundamental elements when learning a language: motivation. This essay reminds teachers the significant role they play in students’ learning process as well as in the learners’ level of motivation. This paper attempts to raise awareness about the need to motivate language learners, which can be achieved by using different types of activities. It is suggested to consider students’ age, interests, needs and skills so that they can learn meaningfully, but also in an enjoyable way. The suggestions given in this article can be useful for language teachers in general.
Childhood adversity and midlife suicidal ideation

Background Childhood adversity predicts adolescent suicidal ideation but there are few studies examining whether the risk of childhood adversity extends to suicidal ideation in midlife. We hypothesized that childhood adversity predicts midlife suicidal ideation and this is partially mediated by adolescent internalizing disorders, externalizing disorders and adult exposure to life events and interpersonal difficulties. Method At 45 years, 9377 women and men from the UK 1958 British Birth Cohort Study participated in a clinical survey. Childhood adversity was prospectively assessed at the ages of 7, 11 and 16 years. Suicidal ideation at midlife was assessed by the depressive ideas subscale of the Revised Clinical Interview Schedule. Internalizing and externalizing disorders were measured by the Rutter scales at 16 years. Life events, periods of unemployment, partnership separations and alcohol dependence were measured through adulthood. Results Illness in the household, paternal absence, institutional care, parental divorce and retrospective reports of parental physical and sexual abuse predicted suicidal ideation at 45 years. Three or more childhood adversities were associated with suicidal ideation at 45 years [odds ratio (OR) 4.31, 95% confidence interval (CI) 2.67–6.94]. Psychological distress at 16 years partially mediated the associations of physical abuse (OR 3.41, 95% CI 2.29–5.75), sexual abuse (OR 4.99, 95% CI 2.90–11.16) with suicidal ideation. Adult life events partially mediated the association of parental divorce (OR 6.34, 95% CI −7.16 to 36.75) and physical (OR 9.59, 95% CI 4.97–27.88) and sexual abuse (OR 6.59, 95% CI 2.40–38.36) with suicidal ideation at 45 years. Conclusions Adversity in childhood predicts suicidal ideation in midlife, partially mediated by adolescent internalizing and externalizing disorders, adult life events and interpersonal difficulties. Understanding the pathways from adversity to suicidal ideation can inform suicide prevention and the targeting of preventive interventions.
Imagining organization through metaphor and metonymy: Unpacking the process-entity paradox

Within organization studies, Morgan’s seminal book Images of Organization has laid the groundwork for an entire research tradition of studying organizational phenomena through metaphorical lenses. Within Morgan’s list of images, that of ‘organization as flux and transformation’ stands out in two important regards. First, it has a strong metonymic dimension, as it implies that organizations consist of and are constituted by processes. Second, the image invites scholars to comprehend organizations as a paradoxical relation between organization (an entity) and process (a non-entity). In this article, we build on Morgan’s work and argue that flux-based images of organization vary in their ability to deal with the process-entity paradox, depending on the degree to which its metaphorical and metonymic dimensions are intertwined. We also examine three offsprings of the flux image: Organization as Becoming, Organization as Practice, and Organization as Communication. We compare these images regarding their metaphor–metonymy dynamics, the directionality of their process of imagination, and their degree of concreteness. We contribute to Morgan’s work, and to organization studies more generally, by offering an analytical grid for unpacking different processes of imagining organization. Moreover, our grid helps explain why images of organization vary in their ability to comprehend organizations in dialectical and paradoxical ways.
Macro-model of Spin-Transfer Torque based Magnetic Tunnel Junction device for hybrid Magnetic-CMOS design

The development of hybrid magnetic-CMOS circuits such as MRAM (magnetic RAM) and magnetic logic circuit requires efficient simulation models for the magnetic devices. A macro-model of magnetic tunnel junction (MTJ) is presented in this paper. This device is the most commonly used magnetic components in CMOS circuits. This model is based on spin-transfer torque (STT) writing approach. This very promising approach should constitute the second generation of MRAM switching technology; it features small switching current (~120uA) and high programming speed (<1ns). The macro-model has been developed in Verilog-A language and implemented on Cadence Virtuoso platform with Spectre 5.0. 32 simulator. Many experimental parameters are integrated in this model to improve the simulation accuracy. So, the model can efficiently be used to design hybrid magnetic CMOS circuits
The Mathematical Theory of Communication.

T HE recent development of various methods of modulation such as PCM and PPM which exchange bandwidth for signal-to-noise ratio has intensified the interest in a general theory of communication. A basis for such a theory is contained in the important papers of Nyquist and Hartley on this subject. In the present paper we will extend the theory to include a number of new factors, in particular the effect of noise in the channel, and the savings possible due to the statistical structure of the original message and due to the nature of the final destination of the information. The fundamental problem of communication is that of reproducing at one point either exactly or approximately a message selected at another point. Frequently the messages have meaning; that is they refer to or are correlated according to some system with certain physical or conceptual entities. These semantic aspects of communication are irrelevant to the engineering problem. The significant aspect is that the actual message is one selected from a set of possible messages. The system must be designed to operate for each possible selection, not just the one which will actually be chosen since this is unknown at the time of design. If the number of messages in the set is finite then this number or any monotonic function of this number can be regarded as a measure of the information produced when one message is chosen from the set, all choices being equally likely. As was pointed out by Hartley the most natural choice is the logarithmic function. Although this definition must be generalized considerably when we consider the influence of the statistics of the message and when we have a continuous range of messages, we will in all cases use an essentially logarithmic measure. The logarithmic measure is more convenient for various reasons: