Embodied responses to musical experience detected by human bio-feedback brain features in a geminoid augmented architecture

This paper presents the conceptual framework for a study of musical experience and the associated architecture centred on Human-Humanoid Interaction (HHI). On the grounds of the theoretical and experimental literature on the biological foundation of music, the grammar of music perception and the perception and feeling of emotions in music hearing, we argue that music cognition is specific and that it is realized by a cognitive capacity for music that consists of conceptual and affective constituents. We discuss the relationship between such constituents that enables understanding, that is extracting meaning from music at the different levels of the organization of sounds that are felt as bearers of affects and emotions. To account for the way such cognitive mechanisms are realized in music hearing and extended to movements and gestures we bring in the construct of tensions and of music experience as a cognitive frame. Finally, we describe the principled approach to the design and the architecture of a BCI-controlled robotic system that can be employed to map and specify the constituents of the cognitive capacity for music as well as to simulate their contribution to music meaning understanding in the context of music experience by displaying it through the Geminoid robot movements

Innate immunity in myasthenia gravis thymus: Pathogenic effects of Toll-like receptor 4 signaling on autoimmunity

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Inflammation and Epstein-Barr Virus Infection Are Common Features of Myasthenia Gravis Thymus: Possible Roles in Pathogenesis

The thymus plays a major role in myasthenia gravis (MG). Our recent finding of a persistent Epstein-Barr (EBV) virus infection in some MG thymuses, combined with data showing that the thymus is in a proinflammatory state in most patients, supports a viral contribution to the pathogenesis of MG. Aim of this study was to gain further evidence for intrathymic chronic inflammation and EBV infection in MG patients. Transcriptional profiling by low density array and real-time PCR showed overexpression of genes involved in inflammatory and immune response in MG thymuses. Real-time PCR for EBV genome, latent (EBER1, EBNA1, LMP1) and lytic (BZLF1) transcripts, and immunohistochemistry for LMP1 and BZLF1 proteins confirmed an active intrathymic EBV infection, further supporting the hypothesis that EBV might contribute to onset or perpetuation of the autoimmune response in MG. Altogether, our results support a role of inflammation and EBV infection as pathogenic features of MG thymus

A Large Family with p.Arg554His Mutation in ABCD1: Clinical Features and Genotype/Phenotype Correlation in Female Carriers

X-linked adrenoleukodystrophy (X-ALD, OMIM #300100) is the most common peroxisomal disorder clinically characterized by two main phenotypes: adrenomyeloneuropathy (AMN) and the cerebral demyelinating form of X-ALD (cerebral ALD). The disease is caused by defects in the gene for the adenosine triphosphate (ATP)-binding cassette protein, subfamily D (ABCD1) that encodes the peroxisomal transporter of very-long-chain fatty acids (VLCFAs). The defective function of ABCD1 protein prevents β-oxidation of VLCFAs, which thus accumulate in tissues and plasma, to represent the hallmark of the disease. As in many X-linked diseases, it has been routinely expected that female carriers are asymptomatic. Nonetheless, recent findings indicate that most ABCD1 female carriers become symptomatic, with a motor disability that typically appears between the fourth and fifth decade. In this paper, we report a large family in which affected males died during the first decade, while affected females develop, during the fourth decade, progressive lower limb weakness with spastic or ataxic-spastic gait, tetra-hyperreflexia with sensory alterations. Clinical and genetic evaluations were performed in nine subjects, eight females (five affected and three healthy) and one healthy male. All affected females were carriers of the c.1661G>A (p.Arg554His, rs201568579) mutation. This study strengthens the relevance of clinical symptoms in female carriers of ABCD1 mutations, which leads to a better understanding of the role of the genetic background and the genotype-phenotype correlation. This indicates the relevance to include ABCD1 genes in genetic panels for gait disturbance in women

Bremsstrahlung emission during α\alpha-decay of 226Ra^{226}{\rm Ra}

We obtained the spectrum of probability of the bremsstrahlung emission accompanying the $\alpha$-decay of $^{226}{\rm Ra}$ (E$_{\alpha}$=4.8 MeV) by measuring the $\alpha$-$\gamma$ coincidences and using the model presented in our previous study on the $\alpha-$decay of $^{214}{\rm Po}$ (E$_{\alpha}$=7.7 MeV). We compare the experimental data with the quantum mechanical calculation and find a good agreement between theory and experiment. We discuss the differences between the photon spectra connected with the $\alpha$-decay of the $^{226}{\rm Ra}$ and $^{214}{\rm Po}$ nuclei. For the two mentioned nuclei we analyze the bremsstrahlung emission contributions from the tunneling and external regions of the nucleus barrier into the total spectrum, and we find the destructive interference between these contributions. We also find that the emission of photons during tunneling of the $\alpha$-particle gives an important contribution to the bremsstrahlung spectrum in the whole E$_{\gamma}$ energy range of the studied $^{226}$Ra nucleus

Decipher non-canonical SPAST splicing mutations with the help of functional assays in patients affected by spastic paraplegia 4 (SPG4)

Flowchart showing the molecular approach used to decipher the non-canonical splicing mutations

Effectiveness and safety of filgotinib in rheumatoid arthritis: a real-life multicentre experience

Objectives: We investigated the effectiveness and safety of filgotinib in a real-life multicentre cohort of rheumatoid arthritis (RA) patients. Methods: RA patients were evaluated at baseline and after 12 and 24 weeks and were stratified based on previous treatments as biologic disease-modifying anti-rheumatic drug (bDMARD)-naive and bDMARD-insufficient responders (IR). Concomitant usage of methotrexate (MTX) and oral glucocorticoids (GC) was recorded. At each timepoint we recorded disease activity, laboratory parameters and adverse events. Results: 126 patients were enrolled. 15.8% were bDMARD-naive (G0), while 84% were bDMARD-IR (G1). In G0, 45% of patients were in monotherapy (G2) and 55% were taken MTX (G3). In G1, 50% of patients were in monotherapy (G4) and 50% used MTX (G5).A significant reduction in all parameters at 12 weeks was observed; in the extension to 24 weeks the significant reduction was maintained for patient global assessment (PGA), examiner global assessment (EGA), visual analogue scale (VAS) pain, VAS fatigue, disease activity score (DAS)28- C-reactive protein (CRP) and CRP values. Filgotinib in monotherapy showed better outcomes in bDMARD-naive patients, with significant differences for patient reported outcomes (PROs) and DAS28-CRP. At 12 weeks, low disease activity (LDA) and remission were achieved in a percentage of 37.2 % and 10.7 % by simplified disease activity index (SDAI), 42.6 % and 5.7 % by clinical disease activity index (CDAI), 26.8 % and 25.2 % by DAS28-CRP, respectively. A significant decrease in steroid dose was evidenced in all patients. We observed a major adverse cardiovascular event in one patient and an increase in transaminase in another. No infections from Herpes Zoster were reported. Conclusions: Our real-world data confirm the effectiveness and safety of filgotinib in the management of RA, especially in bDMARD-naive patients

Telenoid android robot as an embodied perceptual social regulation medium engaging natural human–humanoid interaction

The present paper aims to validate our research on human–humanoid interaction (HHI) using the minimalist humanoid robot Telenoid. We conducted the human–robot interaction test with 142 young people who had no prior interaction experience with this robot. The main goal is the analysis of the two social dimensions (“Perception” and “Believability”) useful for increasing the natural behaviour between users and Telenoid. We administered our custom questionnaire to human subjects in association with a well defined experimental setting (“ordinary and goal-guided task”). A thorough analysis of the questionnaires has been carried out and reliability and internal consistency in correlation between the multiple items has been calculated. Our experimental results show that the perceptual behaviour and believability, as implicit social competences, could improve the meaningfulness and the natural-like sense of human–humanoid interaction in everyday life task-driven activities. Telenoid is perceived as an autonomous cooperative agent for a shared environment by human being

Investigation of the role of the projectile-target orientation angles on the evaporation residue production

The measured yield of evaporation residues in reactions with massive nuclei have been well reproduced by using the partial fusion and quasifission cross sections obtained in the dinuclear-system model. The influence of the orientation angles of the projectile- and target-nucleus symmetry axes relative to the beam direction on the production of the evaporation residues is investigated for the 48Ca + 154Sm reaction as a function of the beam energy. At the low beam energies only the orientation angles close to αP = 30° (projectile) and αP = 0°-15° (target) can contribute to the formation of evaporation residues. At large beam energies (about Ec.m. = 140-180 MeV) the collisions at all values of orientation angles αP and αT of reactants can contribute to the evaporation residue cross section which ranges between 10-100 mb, while at Ec.m. > 185 MeV the evaporation residue cross section ranges between 0.1-1 mb because the fission barrier for the compound nucleus decreases by increasing its excitation energy and angular momentum. © Pleiades Publishing, Ltd. 2009.SCOPUS: ar.jinfo:eu-repo/semantics/publishe