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Background: Axial myopathy is a rare neuromuscular disorder of variable etiology characterised by preferential involvement of the paraspinal muscles. We reviewed clinical features of patients with axial myopathies and the diagnostic yield of myositis-associated antibodies and targeted next generation sequencing panels. Methods: We performed a retrospective review of patients presenting with axial myopathy at the Montreal Neurological Hospital from 2011-2018. Data collection included clinical presentation, disease course, results of electromyography, imaging, laboratory and genetic testing, and histopathology on muscle biopsy. Results: Twenty-five patients were identified. Initial manifestation of axial weakness was head drop (15), camptocormia (8), and rigid spine (2). Autoimmune myositis was diagnosed in 9 patients, seropositive in 7 out of 7 tested for myositis-associated antibodies. Genetic testing was consistent with oculopharyngeal muscular dystrophy in one patient and RYR-1 (ryanodine receptor 1) related core myopathy in another. Local radiotherapy or spine surgery preceded the onset of axial weakness in 1 and 6 patients, respectively. Muscle biopsies were available in 17 patients and revealed myopathic changes (16), inflammatory changes (6), and myopathy with vacuoles (3). Conclusions: Recent advancements in genetic and antibody testing, combined with paraspinal muscle biopsy, allow for more precise classification and identification of potentially treatable axial myopathies.
We present here the results of a search for new microquasars at low galactic latitudes, based on a cross-identification between the ROSAT all sky Bright Source Catalog (RBSC) and the NRAO VLA Sky Survey (NVSS) and follow-up observations. The results obtained up to now suggest that persistent/silent microquasars such as LS 5039 are rare objects in our Galaxy, and indicate that future deeper surveys, and harder than the RBSC in X-rays, will play a fundamental role in order to discover them.
UX Arietis is an active binary system with an orbital period of 6.44 days. The presence of long lasting large spots on the surface of the more active star has been deduced from optical observations (Vogt & Hatzes 1991).
The observations presented here have been performed from December 1992 until October 1994 using the Effelsberg 100 m telescope. The instrument was available for this program in the gaps between previously scheduled observations. For this reason our observations span a frequency interval between 1.4 GHz (21 cm) and 43 GHz (7 mm), depending on the scheduled receiver at each observing time. These data, which are an extension of those published by Neidhöfer et al. (1993), are shown in Fig. 1, plotted in terms of the known orbital period (6.44 days). The search for different periodicities (by using the Phase Dispersion Minimization method) has shown the existence of two main other periods: at 156 days and at 24 days (Massi et al., in preparation).
The importance of chronic low-grade inflammation in the pathology of numerous age-related chronic conditions is now clear. An unresolved inflammatory response is likely to be involved from the early stages of disease development. The present position paper is the most recent in a series produced by the International Life Sciences Institute's European Branch (ILSI Europe). It is co-authored by the speakers from a 2013 workshop led by the Obesity and Diabetes Task Force entitled ‘Low-grade inflammation, a high-grade challenge: biomarkers and modulation by dietary strategies’. The latest research in the areas of acute and chronic inflammation and cardiometabolic, gut and cognitive health is presented along with the cellular and molecular mechanisms underlying inflammation–health/disease associations. The evidence relating diet composition and early-life nutrition to inflammatory status is reviewed. Human epidemiological and intervention data are thus far heavily reliant on the measurement of inflammatory markers in the circulation, and in particular cytokines in the fasting state, which are recognised as an insensitive and highly variable index of tissue inflammation. Potential novel kinetic and integrated approaches to capture inflammatory status in humans are discussed. Such approaches are likely to provide a more discriminating means of quantifying inflammation–health/disease associations, and the ability of diet to positively modulate inflammation and provide the much needed evidence to develop research portfolios that will inform new product development and associated health claims.
As essay after essay in this series has reminded us, the term “neomedievalism” is too multivalent and maddeningly complex to define with any satisfaction: any attempt to create a definition invariably oversimplifies the concept or distorts it to fit current needs. In the case of neomedievalism, rather than attempt another iteration of an Ur-definition, Carol R. Robinson and Pamela Clements have done invaluable work in creating a field guide to understanding the characteristics of neomedievalism. In brief, we can call a text neomedieval when it does one or more of the following:
It is playful or ironic in nature.
It calls attention to its own construction, often as a work of bricolage.
It deliberately shatters any possibility for a “sealed world” of the text.
It refuses the nostalgic fantasy of being able to retrieve the medieval past.
Its task is to create a conscious vision of an alternative universe.
This last item holds the most promise as a way of reading George R. R. Martin's multi-volume A Song of Ice and Fire as a text concerned with particular ethical issues surrounding disability: the damage ableist discourses and narratives inflict on the disabled.
In the landmark case Arline vs. Nassau County, Justice William J. Brennan, Jr., summarizing the need for an inclusive definition of disability, noted the problems that narratives of disability posed for the disabled: “society's accumulated myths and fears about disability and disease are as handicapping as are the physical limitations that flow from actual impairment.”
This work presents an optical characterization by luminescence and reflectivity of GaN layers grown on sapphire using MOVPE, HVPE and GSMBE. Well resolved optical spectra are obtained for each growth technique. The luminescence of Mg doped MOVPE grown GaN is also studied. A Mg acceptor optical depth of ~ 260 meV is obtained.
Ethics in post-medieval responses to the Middle Ages form the main focus of this volume. The six opening essays tackle such issues as the legitimacy of reinventing medieval customs and ideas, at what point the production and enjoyment of caricaturizing the Middle Ages become inappropriate, how medievalists treat disadvantaged communities, and the tension between political action and ethics in medievalism. The eight subsequent articles then build on this foundation as they concentrate on capitalist motives for melding superficially incompatible narratives in medievalist video games, Dan Brown's use of Dante's Inferno to promote a positivist, transhumanist agenda, disjunctures from medieval literature to medievalist film in portrayals of human sacrifice, the influence of Beowulf on horror films and vice versa, portrayals of war in Beowulf films, socialism in William Morris's translation of Beowulf, bias in Charles Alfred Stothard's Monumental Effigies of Great Britain, and a medieval source for death in the Harry Potter novels. The volume as a whole invites and informs a much larger discussion on such vital issues as the ethical choices medievalists make, the implications of those choices for their makers, and the impact of those choices on the world around us. Karl Fugelso is Professor of Art History at Towson University in Baltimore, Maryland. Contributors: Mary R. Bowman, Harry Brown, Louise D'Arcens, Alison Gulley, Nickolas Haydock, Lisa Hicks, Lesley E. Jacobs, Michael R. Kightley, Phillip Lindley, Pascal J. Massie, Lauryn S. Mayer, Brent Moberley, Kevin Moberley, Daniel-Raymond Nadon, Jason Pitruzello, Nancy M. Resh, Carol L. Robinson, Christopher Roman, M.J. Toswell.
To describe a relatively unknown clinical entity – inflammatory cast of the tympanic membrane after acute otitis media – and its simple out-patient treatment.
Retrospective review of case series.
Subspecialty practice at a tertiary hospital.
Seven patients diagnosed previously with acute otitis media with perforation or otitis externa, and with persistent ear discomfort.
Retrospective chart review.
The patients presented with weeks to months of persistent hearing loss after acute otitis media with perforation or acute otitis externa. Visits to their primary care physicians had been uninformative. After comparison of the affected and unaffected tympanic membranes, a thin, hard cast was identified and removed from the affected tympanic membrane. Improvement in hearing was documented in the three patients who underwent audiometric testing; the remainder had subjective improvement without audiometric evaluation.
Otolaryngologists should be aware of the possibility of an inflammatory cast of the tympanic membrane following acute otitis media with perforation or otitis externa, and should carefully compare the unaffected and affected ears in such cases. Treatment – removal of the rigid cast – is both simple and effective.
In this paper we review studies aiming at elucidation of the mechanisms responsible for anomalously low pressure coefficients of the light emission energy, dEE/dP, observed in quantum structures of InGaN/GaN and GaN/AlGaN. We have established that in hexagonal InGaN/GaN and GaN/AlGaN structures the main mechanism involved is related to the pressure induced increase of the piezoelectric field which determines also the strong red shift of the emission energy with thickness of the quantum well. To reproduce the experimental findings in InGaN/GaN case, it is necessary to take into account the dependence of the piezoelectric constants on the volume-conserving strain. Whereas the experimental results on a decrease of dEE/dP in GaN/AlGaNstructures can be fully accounted for within the linear elasticity theory. In contrast to these findings, dEE/dP magnitude measured in cubic InGaN/GaN quantum structures shows value close to changes of the InGaN bangap with pressure obtained from first principle calculations. The latter result is consistent with the absence of the built-in electric fields in the cubic nitride structures.
We present the results of the experimental and theoretical studies of the low field mobility of two-dimensional electrons in the homoepitaxial AlGaN/GaN heterostructures and in the AlGaN/GaN heterostructures grown on SiC. We show that, at cryogenic temperatures, the temperature dependence of the mobility is primarily determined by the deformation potential scattering and that most of other important scattering mechanisms are temperature independent. We show also that two-dimensional (2D) and three-dimensional (3D) mobility models yield very close results. The analysis of the mobility dependence on the electron sheet density ns shows two possible explanations of the non monotonic mobility versus carrier density dependence: i) the alloy/interface scattering and ii) transfer of the 2D electrons into 3D states in GaN. We present experimental data suggesting that for high 2D gas densities in the investigated structure grown on SiC the 2D-3D transition should take place and might be responsible for the mobility decrease at high electron sheet densities.