21 research outputs found

    ILAE Neuroimaging Task Force Highlight: harnessing optimized imaging protocols for drug-resistant childhood epilepsy

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    The ILAE Neuroimaging Task Force aims to publish educational case reports highlighting basic aspects related to neuroimaging in epilepsy consistent with the educational mission of the ILAE. Previous quantitative MRI studies have established important imaging markers of epilepsy-related pathology, including features sensitive to hippocampal cell loss and reactive astrogliosis. Here, we review the case of a female with pediatric drug-resistant epilepsy. Throughout her course of treatment, she had seven MRI investigations at several centers; the first three did not follow optimized epilepsy imaging protocols whereas the remaining four adhered to HARNESS-MRI protocols (harmonized neuroimaging of epilepsy structural sequences). Visual inspection of a set of HARNESS-MR images revealed conspicuous left hippocampal hyperintensity which may have been initially overlooked on non-optimized MR images. Quantitative analysis of these multimodal imaging data along hippocampal subfields provided clear evidence of hippocampal sclerosis, with increased atrophy, increased mean diffusivity, increased T2-FLAIR signal, and lower qT1 values observed in the anterior portions of the left, compared to the right hippocampus. The patient underwent a left anterior temporal lobectomy with amygdalohippocampectomy at age 16 years. Histopathology of the resected specimen also confirmed hippocampal sclerosis with widespread gliosis and focal neuronal loss in the hippocampal subfields overlapping with regions of multimodal quantitative alterations. The patient remains seizure-free one year after surgery. Collectively, this case highlights the need for optimized data acquisition protocols early in the treatment of epilepsy and supports quantitative analysis of MRI contrasts to enhance personalized diagnosis and prognosis of drug-resistant patients with epilepsy

    Future emerging technologies in the wind power sector: A European perspective

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    This paper represents an expert view from Europe of future emerging technologies within the wind energy sector considering their potential, challenges, applications and technology readiness and how they might evolve in the coming years. These technologies were identified as originating primarily from the academic sector, some start-up companies and a few larger industrial entities. The following areas were considered: airborne wind energy, offshore floating concepts, smart rotors, wind-induced energy harvesting devices, blade tip-mounted rotors, unconventional power transmission systems, multi-rotor turbines, alternative support structures, modular high voltage direct current generators, innovative blade manufacturing techniques, diffuser-augmented turbines and small turbine technologies. The future role of advanced multiscale modelling and data availability is also considered. This expert review has highlighted that more research will be required to realise many of these emerging technologies. However, there is a need to identify synergies between fundamental and industrial research by correctly targeting public and private funding in these emerging technology areas as industrial development may outpace more fundamental research faster than anticipated

    Workshop on identification of future emerging technologies in the ocean energy sector. 27th March 2018 Ispra, Italy

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    As part of the European Commission's internal Low Carbon Energy Observatory (LCEO) project, the Joint Research Centre (JRC) is developing an inventory of Future Emerging Technologies (FET) relevant to energy supply. A key part of the LCEO initiative is the consultation of external experts, addressing both those with in-depth experience in specific fields and those with a broad perspective on relevant science and engineering aspects. In this context, on March 27, 2018 the JRC organised a Workshop on Identification of Future Emerging Technologies for Ocean Energy, on it premises in Ispra. The workshop was organised on the idea of a colloquium between international experts to discuss about future emerging technologies considering different aspects such as their technology readiness level (TRL), the potential advantages and challenges affecting their development, and evaluating the possible speed of development

    The Americans with Disabilities Act and the Constitution of the United States (Dissertation)

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    Deficits in social behavioral tests in a mouse model of alternating hemiplegia of childhood

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    Social behavioral deficits have been observed in patients diagnosed with alternating hemiplegia of childhood (AHC), rapid-onset dystonia-parkinsonism and CAPOS syndrome, in which specific missense mutations in ATP1A3, encoding the Na+, K+-ATPase α3 subunit, have been identified. To test the hypothesis that social behavioral deficits represent part of the phenotype of Na+, K+-ATPase α3 mutations, we assessed the social behavior of the Myshkin mouse model of AHC, which has an I810N mutation identical to that found in an AHC patient with co-morbid autism. Myshkin mice displayed deficits in three tests of social behavior: nest building, pup retrieval and the three-chamber social approach test. Chronic treatment with the mood stabilizer lithium enhanced nest building in wild-type but not Myshkin mice. In light of previous studies revealing a broad profile of neurobehavioral deficits in the Myshkin model – consistent with the complex clinical profile of AHC – our results suggest that Na+, K+-ATPase α3 dysfunction has a deleterious, but nonspecific, effect on social behavior. By better defining the behavioral profile of Myshkin mice, we identify additional ATP1A3-related symptoms for which the Myshkin model could be used as a tool to advance understanding of the underlying neural mechanisms and develop novel therapeutic strategies
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