13 research outputs found

    Patterns of Retinal Damage Facilitate Differential Diagnosis between Susac Syndrome and MS

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    Susac syndrome, a rare but probably underdiagnosed combination of encephalopathy, hearing loss, and visual deficits due to branch retinal artery occlusion of unknown aetiology has to be considered as differential diagnosis in various conditions. Particularly, differentiation from multiple sclerosis is often challenging since both clinical presentation and diagnostic findings may overlap. Optical coherence tomography is a powerful and easy to perform diagnostic tool to analyse the morphological integrity of retinal structures and is increasingly established to depict characteristic patterns of retinal pathology in multiple sclerosis. Against this background we hypothesised that differential patterns of retinal pathology facilitate a reliable differentiation between Susac syndrome and multiple sclerosis. In this multicenter cross-sectional observational study optical coherence tomography was performed in nine patients with a definite diagnosis of Susac syndrome. Data were compared with age-, sex-, and disease duration-matched relapsing remitting multiple sclerosis patients with and without a history of optic neuritis, and with healthy controls. Using generalised estimating equation models, Susac patients showed a significant reduction in either or both retinal nerve fibre layer thickness and total macular volume in comparison to both healthy controls and relapsing remitting multiple sclerosis patients. However, in contrast to the multiple sclerosis patients this reduction was not distributed over the entire scanning area but showed a distinct sectorial loss especially in the macular measurements. We therefore conclude that patients with Susac syndrome show distinct abnormalities in optical coherence tomography in comparison to multiple sclerosis patients. These findings recommend optical coherence tomography as a promising tool for differentiating Susac syndrome from MS

    Regulators of AWC-Mediated Olfactory Plasticity in Caenorhabditis elegans

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    While most sensory neurons will adapt to prolonged stimulation by down-regulating their responsiveness to the signal, it is not clear which events initiate long-lasting sensory adaptation. Likewise, we are just beginning to understand how the physiology of the adapted cell is altered. Caenorhabditis elegans is inherently attracted to specific odors that are sensed by the paired AWC olfactory sensory neurons. The attraction diminishes if the animal experiences these odors for a prolonged period of time in the absence of food. The AWC neuron responds acutely to odor-exposure by closing calcium channels. While odortaxis requires a Gα subunit protein, cGMP-gated channels, and guanylyl cyclases, adaptation to prolonged odor exposure requires nuclear entry of the cGMP-dependent protein kinase, EGL-4. We asked which candidate members of the olfactory signal transduction pathway promote nuclear entry of EGL-4 and which molecules might induce long-term adaptation downstream of EGL-4 nuclear entry. We found that initiation of long-term adaptation, as assessed by nuclear entry of EGL-4, is dependent on G-protein mediated signaling but is independent of fluxes in calcium levels. We show that long-term adaptation requires polyunsaturated fatty acids (PUFAs) that may act on the transient receptor potential (TRP) channel type V OSM-9 downstream of EGL-4 nuclear entry. We also present evidence that high diacylglycerol (DAG) levels block long-term adaptation without affecting EGL-4 nuclear entry. Our analysis provides a model for the process of long-term adaptation that occurs within the AWC neuron of C. elegans: G-protein signaling initiates long-lasting olfactory adaptation by promoting the nuclear entry of EGL-4, and once EGL-4 has entered the nucleus, processes such as PUFA activation of the TRP channel OSM-9 may dampen the output of the AWC neuron

    Herschel unveils a puzzling uniformity of distant dusty galaxies

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    The Herschel Space Observatory enables us to accurately measure the bolometric output of starburst galaxies and active galactic nuclei (AGN) by directly sampling the peak of their far-infrared (IR) emission. Here we examine whether the spectral energy distribution (SED) and dust temperature of galaxies have strongly evolved over the last 80% of the age of the Universe. We discuss possible consequences for the determination of starformation rates (SFR) and any evidence for a major change in their star-formation properties. We use Herschel deep extragalactic surveys from 100 to 500 μ m to compute total IR luminosities in galaxies down to the faintest levels, using PACS and SPIRE in the GOODS-North field (PEP and HerMES key programs). An extension to fainter luminosities is done by stacking images on 24 μ m prior positions. We show that measurements in the SPIRE bands can be used below the statistical confusion limit if information at higher spatial resolution is used, e.g. at 24 μ m, to identify "isolated" galaxies whose flux is not boosted by bright neighbors. Below z ∼ 1.5, mid-IR extrapolations are correct for star-forming galaxies with a dispersion of only 40% (0.15 dex), therefore similar to z ∼ 0 galaxies, over three decades in luminosity below the regime of ultra-luminous IR galaxies (ULIRGs, LIR ≥ 1012 L⊙). This narrow distribution is puzzling when considering the range of physical processes that could have affected the SED of these galaxies. Extrapolations from only one of the 160 μ m, 250 μ m or 350 μ m bands alone tend to overestimate the total IR luminosity. This may be explained by the lack of far-IR constraints around and above ∼150 μ m (rest-frame) before Herschel on those templates. We also note that the dust temperature of luminous IR galaxies (LIRGs, LIR ≥ 1011 L⊙) around z ∼ 1 is mildly colder by 10-15% than their local analogs and up to 20% for ULIRGs at z ∼ 1.6 (using a single modified blackbody-fit to the peak far-IR emission with an emissivity index of β = 1.5). Above z = 1.5, distant galaxies are found to exhibit a substantially larger mid- over far-IR ratio, which could either result from stronger broad emission lines or warm dust continuum heated by a hidden AGN. Two thirds of the AGNs identified in the field with a measured redshift exhibit the same behavior as purely star-forming galaxies. Hence a large fraction of AGNs harbor coeval star formation at very high SFR and in conditions similar to purely star-forming galaxies. © 2010 ESO
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