INTRODUCTION: Kaczyńska et al. reported a family with myoclonus-dystonia (M-D) caused by a truncating SGCE mutation, in which two members had epilepsy. Further, patients had mild psychiatric and developmental deficits. CLINICAL REFLECTIONS: Characteristic motor features of M-D include myoclonus, dystonia and tremor. A wide range of additional disease manifestations are known. A few patients with M

We argue that the neoliberal tradition and new public management reforms of the public sector effectively erode the core (liberal) democratic values of the rule of law and transparency. The tension between public law and managerially-influenced governmental policy is in practice resolved by the emergence of what we call “shadow management” in public administration, whereby managerial decisions tha

A number of men with malignant lymphoma of the histiocytic type and previous exposure to phenoxy acids or chlorophenols were observed and reported in 1979. A matched case-control study has therefore been performed with cases of malignant lymphoma (Hodgkin's disease and non-Hodgkin lymphoma). This study included 169 cases and 338 controls. The results indicate that exposure to phenoxy acids, chloro

The debate on militant democracy is very much consumed by the question of whether legal restrictions against antidemocratic parties are justified. By contrast, this paper gestures towards a social conception of democratic self-defence. It does so by examining Hermann Heller’s ideas on the origins and solutions to antidemocratic threats. Arguing against Schmitt’s ‘authoritarian liberalism’, Heller

The strategic direction of the European Union (EU) over the past twenty years has focused on increasing resource productivity and innovation in the economy, aiming at the efficient use and secured supplies of resources, economic growth and job creation, with fewer environmental impacts. One of the basic premises of the new ‘Green Deal’ for Europe is the promotion of the circular economy. The aim o

Background: Symptoms of depression and anxiety are common in children with obsessive-compulsive disorder (OCD) and associated with more severe OCD, greater impairment, and worse treatment outcome. Beyond twin studies showing that genetic factors contribute to the high co-occurrence, few studies have examined how OCD, depression, and anxiety are linked in youth, and current studies often fail to ac

We discuss the associated cc¯ and bb¯ quark pairs production in the double-parton scattering (DPS) process in ultraperipheral (UPCs) AA collisions. We derive an analogue of the inclusive DPS pocket formula and the photon-energy dependent effective cross section considering an overlap between the hard SPS scatterings. We provide numerical predictions for the DPS cross sections for the cc¯ bb¯ produ

East African landscapes today are the result of the cumulative effects of climate and land-use change over millennial timescales. In this review, we compile archaeological and palaeoenvironmental data from East Africa to document land-cover change, and environmental, subsistence and land-use transitions, over the past 6000 years. Throughout East Africa there have been a series of relatively rapid

The semi-arid Amboseli landscape, southern Kenya, is characterised by intermittent groundwater-fed wetlands that form sedimentary geoarchives recording past ecosystem changes. We present a 5000-year environmental history of a radiocarbon dated sediment core from Esambu Swamp adjacent to Amboseli National Park. Although radiocarbon dates suggest an unconformity or sedimentary gap that spans between

The ESS linac is currently under construction in Lund, Sweden, and once completed it will deliver an unprecedented 5 MW of average power. The ion source and LEBT commissioning starts in 2018 and will continue with the RFQ, MEBT and the frst DTL tank next year and up to the end of the fourth DTL tank in 2020. This paper will summarize the commissioning plans for the normal conducting linac with foc

BACKGROUND: Resection of the primary tumour is a prerequisite for cure in patients with colorectal cancer, but hepatic metastasectomy has been used increasingly with curative intent. This national registry study examined prognostic factors for radically treated primary tumours, including the subgroup of patients undergoing liver metastasectomy. METHODS: Patients who had radical resection of primar

BACKGROUND: Individual randomized controlled trials (RCTs) of periprocedural anticoagulation with bivalirudin versus heparin during percutaneous coronary intervention (PCI) have reported conflicting results. Study-level meta-analyses lack granularity to adjust for confounders, explore heterogeneity, or identify subgroups that may particularly benefit or be harmed. OBJECTIVE: To overcome these limi

Bispecific antibodies come in many different formats, including the particularly interesting two-in-one antibodies, where one conventional IgG binds two different antigens. The IgG format allows these antibodies to mediate Fc-related functionality, and their wild-type structure ensures low immunogenicity and enables standard methods to be used for development. It is however difficult, time-consumi

We demonstrate for the first time that seeded harmonic generation on electron storage rings can produce coherent optical pulses in the vacuum ultraviolet spectral range. The experiment is performed at Elettra, where coherent pulses are generated at 132nm, with a duration of about 100fs. The light source has a repetition rate of 1kHz and adjustable polarization; it is very bright, with a peak power

Taking advantage of the storage ring free electron laser beamline at Elettra, we have implemented an experimental setup for the generation of sub-picosecond (ps) coherent optical pulses in the VUV range. The setup is based on the frequency up-conversion of a high-power external signal (provided by a Ti:Sapphire laser) and makes use of a relativistic electron bunch as resonating medium. The produce