701 research outputs found

    High-fat diets and seizure control in myoclonic-astatic epilepsy: A single center's experience

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    AbstractPurposeTo determine the efficacy of the Modified Atkins Diet (MAD) and Ketogenic Diet (KD) in seizure control within a population of myoclonic-astatic epilepsy (MAE) patients.MethodsThis was a retrospective, single center study evaluating the seizure control by high fat diets. Seizure diaries kept by the parents performed seizure counts. All patients met the clinical criteria for MAE.ResultsNine patients met the clinical criteria. We found that both the MAD and KD were efficacious in complete seizure control and allowed other medications to be stopped in seven patients. Two patients had greater than 90% seizure control without medications, one on the KD and the other on the MAD. Seizure freedom has ranged from 13 to 36 months, and during this time four patients have been fully weaned off of diet management. One patient was found to have a mutation in SLC2A1.ConclusionOur results suggest that strictly defined MAE patients respond to the MAD with prolonged seizure control. Some patients may require the KD for seizure freedom, suggesting a common pathway of increased requirement for fats. Once controlled, those fully responsive to the Diet(s) could be weaned off traditional seizure medications and in many, subsequently off the MAD or KD

    Characterization of SuperCDMS 1-inch Ge Detectors

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    The newly commissioned SuperCDMS Soudan experiment aims to search for WIMP dark matter with a sensitivity to cross sections of 5×10^(−45)cm^2 and larger (90% CL upper limit). This goal is facilitated by a new set of germanium detectors, 2.5 times more massive than the ones used in the CDMS-II experiment, and with a different athermal phonon sensor layout that eliminates radial degeneracy in position reconstruction of high radius events. We present characterization data on these detectors, as well as improved techniques for correcting position-dependent variations in pulse shape across the detector. These improvements provide surface-event discrimination sufficient for a reach of 5×10^(−45)cm^2

    Pahs, Ionized Gas, and Molecular Hydrogen in Brightest Cluster Galaxies of Cool Core Clusters of Galaxies

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    We present measurements of 5-25 {\mu}m emission features of brightest cluster galaxies (BCGs) with strong optical emission lines in a sample of 9 cool-core clusters of galaxies observed with the Infrared Spectrograph on board the Spitzer Space Telescope. These systems provide a view of dusty molecular gas and star formation, surrounded by dense, X-ray emitting intracluster gas. Past work has shown that BCGs in cool-core clusters may host powerful radio sources, luminous optical emission line systems, and excess UV, while BCGs in other clusters never show this activity. In this sample, we detect polycyclic aromatic hydrocarbons (PAHs), extremely luminous, rotationally-excited molecular hydrogen line emission, forbidden line emission from ionized gas ([Ne II] and [Ne III]), and infrared continuum emission from warm dust and cool stars. We show here that these BCGs exhibit more luminous forbidden neon and H2 rotational line emission than star-forming galaxies with similar total infrared luminosities, as well as somewhat higher ratios of 70 {\mu}m / 24 {\mu}m luminosities. Our analysis suggests that while star formation processes dominate the heating of the dust and PAHs, a heating process consistent with suprathermal electron heating from the hot gas, distinct from star formation, is heating the molecular gas and contributing to the heating of the ionized gas in the galaxies. The survival of PAHs and dust suggests that dusty gas is somehow shielded from significant interaction with the X-ray gas.Comment: 27 preprint pages, 18 figures, accepted by Astrophysical Journa

    Live Coding, Live Notation, Live Performance

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    This paper/demonstration explores relationships between code, notation including representation, visualisation and performance. Performative aspects of live coding activities are increasingly being investigated as the live coding movement continues to grow and develop. Although live instrumental performance is sometimes included as an accompaniment to live coding, it is often not a fully integrated part of the performance, relying on improvisation and/or basic indicative forms of notation with varying levels of sophistication and universality. Technologies are developing which enable the use of fully explicit music notations as well as more graphic ones, allowing more fully integrated systems of code in and as performance which can also include notations of arbitrary complexity. This itself allows the full skills of instrumental musicians to be utilised and synchronised in the process. This presentation/demonstration presents work and performances already undertaken with these technologies, including technologies for body sensing and data acquisition in the translation of the movements of dancers and musicians into synchronously performable notation, integrated by live and prepared coding. The author together with clarinetist Ian Mitchell present a short live performance utilising these techniques, discuss methods for the dissemination and interpretation of live generated notations and investigate how they take advantage of instrumental musicians’ training-related neuroplasticity skills

    Induction of Blood Brain Barrier Tight Junction Protein Alterations by CD8 T Cells

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    Disruption of the blood brain barrier (BBB) is a hallmark feature of immune-mediated neurological disorders as diverse as viral hemorrhagic fevers, cerebral malaria and acute hemorrhagic leukoencephalitis. Although current models hypothesize that immune cells promote vascular permeability in human disease, the role CD8 T cells play in BBB breakdown remains poorly defined. Our laboratory has developed a novel murine model of CD8 T cell mediated central nervous system (CNS) vascular permeability using a variation of the Theiler's virus model of multiple sclerosis. In previous studies, we observed that MHC class II−/− (CD4 T cell deficient), IFN-γR−/−, TNF-α−/−, TNFR1−/−, TNFR2−/−, and TNFR1/TNFR2 double knockout mice as well as those with inhibition of IL-1 and LTβ activity were susceptible to CNS vascular permeability. Therefore, the objective of this study was to determine the extent immune effector proteins utilized by CD8 T cells, perforin and FasL, contributed to CNS vascular permeability. Using techniques such as fluorescent activated cell sorting (FACS), T1 gadolinium-enhanced magnetic resonance imaging (MRI), FITC-albumin leakage assays, microvessel isolation, western blotting and immunofluorescent microscopy, we show that in vivo stimulation of CNS infiltrating antigen-specific CD8 T cells initiates astrocyte activation, alteration of BBB tight junction proteins and increased CNS vascular permeability in a non-apoptotic manner. Using the aforementioned techniques, we found that despite having similar expansion of CD8 T cells in the brain as wildtype and Fas Ligand deficient animals, perforin deficient mice were resistant to tight junction alterations and CNS vascular permeability. To our knowledge, this study is the first to demonstrate that CNS infiltrating antigen-specific CD8 T cells have the capacity to initiate BBB tight junction disruption through a non-apoptotic perforin dependent mechanism and our model is one of few that are useful for studies in this field. These novel findings are highly relevant to the development of therapies designed to control immune mediated CNS vascular permeability

    Uncovering the Genetic Landscape for Multiple Sleep-Wake Traits

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    Despite decades of research in defining sleep-wake properties in mammals, little is known about the nature or identity of genes that regulate sleep, a fundamental behaviour that in humans occupies about one-third of the entire lifespan. While genome-wide association studies in humans and quantitative trait loci (QTL) analyses in mice have identified candidate genes for an increasing number of complex traits and genetic diseases, the resources and time-consuming process necessary for obtaining detailed quantitative data have made sleep seemingly intractable to similar large-scale genomic approaches. Here we describe analysis of 20 sleep-wake traits from 269 mice from a genetically segregating population that reveals 52 significant QTL representing a minimum of 20 genomic loci. While many (28) QTL affected a particular sleep-wake trait (e.g., amount of wake) across the full 24-hr day, other loci only affected a trait in the light or dark period while some loci had opposite effects on the trait during the light vs. dark. Analysis of a dataset for multiple sleep-wake traits led to previously undetected interactions (including the differential genetic control of number and duration of REM bouts), as well as possible shared genetic regulatory mechanisms for seemingly different unrelated sleep-wake traits (e.g., number of arousals and REM latency). Construction of a Bayesian network for sleep-wake traits and loci led to the identification of sub-networks of linkage not detectable in smaller data sets or limited single-trait analyses. For example, the network analyses revealed a novel chain of causal relationships between the chromosome 17@29cM QTL, total amount of wake, and duration of wake bouts in both light and dark periods that implies a mechanism whereby overall sleep need, mediated by this locus, in turn determines the length of each wake bout. Taken together, the present results reveal a complex genetic landscape underlying multiple sleep-wake traits and emphasize the need for a systems biology approach for elucidating the full extent of the genetic regulatory mechanisms of this complex and universal behavior
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