Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

Stiff person spectrum disorders (SPSD) are paradigm autoimmune movement disorders characterized by stiffness, spasms and hyperekplexia. Though rare, SPSD represent a not-to-miss diagnosis because of the associated disease burden and treatment implications. After decades as an enigmatic orphan disease, major advances in our understanding of the evolving spectrum of diseases have been made along with the identification of multiple associated autoantibodies. However, the most important recent developments relate to the recognition of a wider affection, beyond the classic core motor symptoms, and to further insights into immunomodulatory and symptomatic therapies. In this review, we summarize the recent literature on the clinical and paraclinical spectrum, current pathophysiological understanding, as well as current and possibly future therapeutic strategies

Porosity and thickness characterization of porous Si and oxidized porous Si layers – an ultraviolet-visible-mid infrared ellipsometry study

International audienc

Measurements of air pollution emission factors for marine transportation in SECA

The chemical composition of the plumes of seagoing ships was investigated during a two weeks long measurement campaign in the port of Rotterdam, Hoek van Holland, The Netherlands, in September 2009. Altogether, 497 ships were monitored and a statistical evaluation of emission factors (g kg-1 fuel) was provided. The concerned main atmospheric components were SO2, NO2, NOX and the aerosol particle number. In addition, the elemental and water-soluble ionic composition of the emitted particulate matter was measured. Emission factors were expressed as a function of ship type, power and crankshaft rotational speed. The average SO2 emission factor was found to be roughly half of what is allowed in sulphur emission control areas (16 34 vs. 30 g kg-1 fuel), and exceedances of this limit were rarely registered. A significant linear relationship was observed between the SO2 and particle number emission factor. The slope of the regression line, 2x1018 (kg fuel)-1, provides the average number of sulphate particles from 1 kg sulphur burnt with the fuel, while the intercept, 0.5x1016 (kg fuel)-1, gives the average number of primary particles (mainly soot and ash) formed during the burning of 1 kg fuel. Water-soluble ionic composition analysis of the aerosol samples from the plumes showed that approx. 144 g of sulphate particles were emitted from 1 kg sulphur burnt with the fuel. The mass median diameter of sulphate particles estimated from the measurements was 42 nm.JRC.H.2-Air and Climat

Plurality in the Measurement of Social Media Use and Mental Health: An Exploratory Study Among Adolescents and Young Adults

On a daily basis, individuals between 12 and 25 years of age engage with their mobile devices for many hours. Social Media Use (SMU) has important implications for the social life of younger individuals in particular. However, measuring SMU and its effects often poses challenges to researchers. In this exploratory study, we focus on some of these challenges, by addressing how plurality in the measurement and age-specific characteristics of SMU can influence its relationship with measures of subjective mental health (MH). We conducted a survey among a nationally representative sample of Dutch adolescents and young adults (N=3,669). Using these data, we show that measures of SMU show little similarity with each other, and that age-group differences underlie SMU. Similar to the small associations previously shown in social media-effects research, we also find some evidence that greater SMU associates to drops and to increases in MH. Albeit nuanced, associations between SMU and MH were found to be characterized by both linear and quadratic functions. These findings bear implications for the level of association between different measures of SMU and its theorized relationship with other dependent variables of interest in media-effects research

Agriculture in the Face of Changing Markets, Institutions and Policies: Challenges and Strategies

Since the late 1980s, agriculture in Central and Eastern European Countries (CEECs) has been under considerable adjustment pressure due to changing political, economic and institutional environments. These changes have been linked to the transition process, as well as the ongoing integration into the European Union and the world market. Reduced subsidies, increased environmental and food quality demands, as well as structural changes in the supply, processing and food retailing sector call for major structural adjustments and the improvement of farmersâ managerial abilities. Though such changes always carry significant threats to farms, they also offer new opportunities for the farms' entrepreneurial engagement. Upcoming changes in the agricultural environment and their possible consequences for farm structures across Europe are thus still timely subjects. The objective of the IAMO Forum 2006 is to contribute to the success of agriculture in the CEECs, as well as their neighboring countries, in todayâs increasingly competitive environment. Concrete questions the conference focuses on are: What are the most suitable farm organizations, cooperative arrangements and contractual forms? How to improve efficiency and productivity? Where do market niches lie and what are the new product demands? This book contains 33 invited and selected contributions. These papers will be presented at the IAMO Forum 2006 in order to offer a platform for scientists, practitioners and policy-makers to discuss challenges and potential strategies at the farm, value chain, rural society and policy levels in order to cope with the upcoming challenges. IAMO Forum 2006, as well as this book, would not have been possible without the engagement of many people and institutions. We thank the authors of the submitted abstracts and papers, as well as the referees, for their evaluation of the abstracts from which the papers were selected. In particular, we would like to express our thanks to OLIVER JUNGKLAUS, GABRIELE MEWES, KLAUS REINSBERG and ANGELA SCHOLZ, who significantly contributed to the organization of the Forum. Furthermore, our thanks goes to SILKE SCHARF for her work on the layout and editing support of this book, and to JIM CURTISS, JAMIE BULLOCH, and DÃNALL Ã MEARÃIN for their English proof-reading. As experience from previous years documents, the course of the IAMO Forum continues to profit from the support and engagement of the IAMO administration, which we gratefully acknowledge. Last but not least, we are very grateful to the Robert Bosch Foundation, the Federal Ministry of Nutrition, Agriculture and Consumer Protection (BMELV), the German Research Foundation (DFG), the Haniel Foundation and the Leibniz Institute of Agricultural Development in Central and Eastern Europe (IAMO) for their respective financial support.Agribusiness, Community/Rural/Urban Development, Farm Management, Industrial Organization, International Development, Labor and Human Capital, Land Economics/Use, Productivity Analysis,

Ultrasound Imaging in Psoriatic Arthritis: What Have We Learnt in the Last Five Years?

Psoriatic arthritis (PsA) is a complex heterogeneous disease with multiple inter-related pathologies such as synovitis, enthesitis, tendinopathy, and dactylitis. Clinical assessment is limited in its detail to assess pathology, thus in recent years, ultrasound (US) has become more popular, given its high sensitivity to detect inflammatory arthritis and ability to inform clinical decisions. Although a qualitative technique, US findings can be graded semi-quantitatively for grayscale (GS) and power Doppler (PD). Synovitis is frequently present in inflammatory arthritis pathologies, and in PsA, recent evidence shows a propensity for tendon and entheseal lesions. The presence of flexor tenosynovitis and flexor tendon insertional enthesopathy at accessory pulleys is supportive of the “Deep Koebner” concept. Peri-tendinous inflammation—mutual to PsA or rheumatoid arthritis (RA), is associated with soft tissue oedema with PD signal frequently at the flexor tendon compartments in PsA. Research on enthesitis in PsA/PsO has improved understanding in subclinical and clinical PsA, explored associations with progression to PsA, and investigated links to prognosis assessment. Dactylitis is a pathognomonic PsA lesion where US has enhanced knowledge of the disease course and pathology of lesions such as: flexor tenosynovitis; synovitis; and soft tissue oedema. Increased US sensitivity has also brought innovation including promising automated ultrasound scanning techniques. So, what have we learnt in recent years and what are the unmet needs to focus future research initiatives in this disabling disease? This narrative review article assesses the neoteric evidence, bringing into context the knowledge gained and highlighting potential areas of research

An intrinsically disordered proteins community for ELIXIR.

Intrinsically disordered proteins (IDPs) and intrinsically disordered regions (IDRs) are now recognised as major determinants in cellular regulation. This white paper presents a roadmap for future e-infrastructure developments in the field of IDP research within the ELIXIR framework. The goal of these developments is to drive the creation of high-quality tools and resources to support the identification, analysis and functional characterisation of IDPs. The roadmap is the result of a workshop titled "An intrinsically disordered protein user community proposal for ELIXIR" held at the University of Padua. The workshop, and further consultation with the members of the wider IDP community, identified the key priority areas for the roadmap including the development of standards for data annotation, storage and dissemination; integration of IDP data into the ELIXIR Core Data Resources; and the creation of benchmarking criteria for IDP-related software. Here, we discuss these areas of priority, how they can be implemented in cooperation with the ELIXIR platforms, and their connections to existing ELIXIR Communities and international consortia. The article provides a preliminary blueprint for an IDP Community in ELIXIR and is an appeal to identify and involve new stakeholders

Reclaiming the humanity in personality Disorder.

This paper provides a commentary upon the nursing care of individuals diagnosed with personality disorder and associated education courses. The discussion focuses upon recent policy trends in the UK as a point of departure. This policy discourse is critical of mainstream mental health services in previously operating to exclude such individuals. One of the consequences has been a recent growth in interest in relevant training courses, many of which devote significant attention to staff attitudes regarding this client group. Various previous researchers and commentators have remarked upon the implications for practice of a perceived negative attitude among care staff. We reflect upon our own anecdotal experience of developing and delivering new university-based courses for practitioners working in the field of personality disorder to offer a particular critique of the UK context, in which this policy, training, and practice is framed. Social constructionist theories are drawn on to offer insights into public and practitioner discourse and the possible effects on therapeutic relationships. The available discourse constructs individuals with a diagnosis of personality disorder as essentially different from other people. We argue that staff training and practice development initiatives are likely to be more successful if such discourse is challenged, and attempts are made in therapeutic encounters to recognize shared characteristics and positive attributes as much as perceived difference and negative attributes. We refer to this as a re-engagement with common humanity. Despite the singular national context, the discursive themes explored are not necessarily restricted to the UK