Ophthalmoplegic Migraine and Infundibular Dilatation of a Cerebral Artery

Ophthalmoplegic migraine (OM) is a childhood disorder of uncertain etiology manifesting recurrent unilateral headache associated with a transitory oculomotor (usually IIIrd nerve) palsy. Recent publications emphasize the finding on MRI of contrast enhancement in the IIIrd nerve suggesting that OM may be a recurrent inflammatory neuropathy. We report the case of a 7-year-old boy with typical symptoms of this disorder. Angio MR and Angio CT revealed the presence of an infundibular dilatation of a perforating branch of the posterior cerebral artery adjacent to the symptomatic IIIrd nerve. We speculate that this and perhaps other cases of OM may have a different pathophysiology related to compression of the IIIrd nerve by an adjacent vascular structure that could activate the trigeminovascular system and produce migrainous pain

Glucosylsphingosine Is a Highly Sensitive and Specific Biomarker for Primary Diagnostic and Follow-Up Monitoring in Gaucher Disease in a Non-Jewish, Caucasian Cohort of Gaucher Disease Patients

Gaucher disease (GD) is the most common lysosomal storage disorder (LSD). Based on a deficient β-glucocerebrosidase it leads to an accumulation of glucosylceramide. Standard diagnostic procedures include measurement of enzyme activity, genetic testing as well as analysis of chitotriosidase and CCL18/PARC as biomarkers. Even though chitotriosidase is the most well-established biomarker in GD, it is not specific for GD. Furthermore, it may be false negative in a significant percentage of GD patients due to mutation. Additionally, chitotriosidase reflects the changes in the course of the disease belatedly. This further enhances the need for a reliable biomarker, especially for the monitoring of the disease and the impact of potential treatments.Here, we evaluated the sensitivity and specificity of the previously reported biomarker Glucosylsphingosine with regard to different control groups (healthy control vs. GD carriers vs. other LSDs).Only GD patients displayed elevated levels of Glucosylsphingosine higher than 12 ng/ml whereas the comparison controls groups revealed concentrations below the pathological cut-off, verifying the specificity of Glucosylsphingosine as a biomarker for GD. In addition, we evaluated the biomarker before and during enzyme replacement therapy (ERT) in 19 patients, demonstrating a decrease in Glucosylsphingosine over time with the most pronounced reduction within the first 6 months of ERT. Furthermore, our data reveals a correlation between the medical consequence of specific mutations and Glucosylsphingosine.In summary, Glucosylsphingosine is a very promising, reliable and specific biomarker for GD

Single‐Column Model Simulations of Subtropical Marine Boundary‐Layer Cloud Transitions Under Weakening Inversions

Results are presented of the GASS/EUCLIPSE single‐column model intercomparison study on the subtropical marine low‐level cloud transition. A central goal is to establish the performance of state‐of‐the‐art boundary‐layer schemes for weather and climate models for this cloud regime, using large‐eddy simulations of the same scenes as a reference. A novelty is that the comparison covers four different cases instead of one, in order to broaden the covered parameter space. Three cases are situated in the North‐Eastern Pacific, while one reflects conditions in the North‐Eastern Atlantic. A set of variables is considered that reflects key aspects of the transition process, making use of simple metrics to establish the model performance. Using this method, some longstanding problems in low‐level cloud representation are identified. Considerable spread exists among models concerning the cloud amount, its vertical structure, and the associated impact on radiative transfer. The sign and amplitude of these biases differ somewhat per case, depending on how far the transition has progressed. After cloud breakup the ensemble median exhibits the well‐known “too few too bright” problem. The boundary‐layer deepening rate and its state of decoupling are both underestimated, while the representation of the thin capping cloud layer appears complicated by a lack of vertical resolution. Encouragingly, some models are successful in representing the full set of variables, in particular, the vertical structure and diurnal cycle of the cloud layer in transition. An intriguing result is that the median of the model ensemble performs best, inspiring a new approach in subgrid parameterization

Europe After Wyclif

This volume brings together scholarship that discusses late-medieval religious controversy on a pan-European scale, with particular attention to developments in England, Bohemia, and at the general councils of the fifteenth century. Controversies such as those that developed in England and Bohemia have received ample attention for decades, and recent scholarship has introduced valuable perspectives and findings to our knowledge of these aspects of European religion, literature, history, and thought. Yet until recently, scholars working on these controversies have tended to work in regional isolation, a practice that has given rise to the impression that the controversies were more or less insular, their significance measured in terms of their local or regional influence

Select strengths and biases of models in representing the Arctic winter boundary layer over sea ice: the Larcform 1 single column model intercomparison

Weather and climate models struggle to represent lower tropospheric temperature and moisture profiles and surface fluxes in Arctic winter, partly because they lack or misrepresent physical processes that are specific to high latitudes. Observations have revealed two preferred states of the Arctic winter boundary layer. In the cloudy state, cloud liquid water limits surface radiative cooling, and temperature inversions are weak and elevated. In the radiatively clear state, strong surface radiative cooling leads to the build-up of surface-based temperature inversions. Many large-scale models lack the cloudy state, and some substantially underestimate inversion strength in the clear state. Here, the transformation from a moist to a cold dry air mass is modeled using an idealized Lagrangian perspective. The trajectory includes both boundary layer states, and the single-column experiment is the first Lagrangian Arctic air formation experiment (Larcform 1) organized within GEWEX GASS (Global atmospheric system studies). The intercomparison reproduces the typical biases of large-scale models: some models lack the cloudy state of the boundary layer due to the representation of mixed-phase microphysics or to the interaction between micro- and macrophysics. In some models, high emissivities of ice clouds or the lack of an insulating snow layer prevent the build-up of surface-based inversions in the radiatively clear state. Models substantially disagree on the amount of cloud liquid water in the cloudy state and on turbulent heat fluxes under clear skies. Observations of air mass transformations including both boundary layer states would allow for a tighter constraint of model behavior

Factors Controlling Rapid Stratocumulus Cloud Thinning

Abstract The relationship between the inversion stability and the liquid water path (LWP) tendency of a vertically well-mixed, adiabatic stratocumulus cloud layer is investigated in this study through the analysis of the budget equation for the LWP. The LWP budget is mainly determined by the turbulent fluxes of heat and moisture at the top and the base of the cloud layer, as well as by the source terms due to radiation and precipitation. Through substitution of the inversion stability parameter κ into the budget equation, it immediately follows that the LWP tendency will become negative for increasing values of κ due to the entrainment of increasingly dry air. Large κ values are therefore associated with strong cloud thinning. Using the steady-state solution for the LWP, an equilibrium value κeq is formulated, beyond which the stratocumulus cloud will thin. The Second Dynamics and Chemistry of Marine Stratocumulus field study (DYCOMS-II) is used to illustrate that, depending mainly on the magnitude of the moisture flux at cloud base, stratocumulus clouds can persist well within the buoyancy reversal regime.</jats:p