283 research outputs found

    Serum neurofilament light as biomarker of seizure-related neuronal injury in status epilepticus

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    Biomarkers of neuronal damage in status epilepticus (SE) would be of great relevance for clinical and research purposes. In a retrospective cross-sectional study, serum neurofilament light chain (NfL) levels were measured in patients with SE (30 subjects), patients with drug-resistant epilepsy (30 subjects), and healthy controls (30 subjects). Serum NfL levels were higher in patients with SE (median = 26.15 pg/ml) compared to both epilepsy patients (median = 7.35 pg/ml) and healthy controls (median = 6.81 pg/ml; p <.001). In patients with SE, serum NfL levels showed a high correlation with cerebrospinal fluid (CSF) NfL (τ =.68, p <.001) as well as with CSF total tau (t-tau) levels (τ =.627, p <.001); they were higher in SE lasting >24 h (p =.013), in refractory/superrefractory SE (p =.004), and in patients who died within 30 days or who presented a worsening of clinical conditions (p =.001). Values of >28.8 pg/ml predicted 30-day clinical worsening or death (odds ratio [OR] = 10.83, 95% confidence interval [CI] = 1.96–59.83, p =.006) and SE refractoriness (OR = 9.33, 95% CI = 1.51–57.65, p =.016). In conclusion, serum NfL levels are increased in SE and correlate with SE treatment response, duration, and outcomes, therefore representing a promising biomarker of seizure-related neuronal damage

    Can Disruption of Basal Ganglia-Thalamocortical Circuit in Wilson Disease Be Associated with Juvenile Myoclonic Epilepsy Phenotype?

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    In this paper, we describe the multimodal MRI findings in a patient with Wilson disease and a seizure disorder, characterized by an electroclinical picture resembling juvenile myoclonic epilepsy. The brain structural MRI showed a deposition of ferromagnetic materials in the basal ganglia, with marked hypointensities in T2-weighted images of globus pallidus internus bilaterally. A resting-state fMRI study revealed increased functional connectivity in the patient, compared to control subjects, in the following networks: (1) between the primary motor cortex and several cortical regions, including the secondary somatosensory cortex and (2) between the globus pallidus and the thalamo-frontal network. These findings suggest that globus pallidus alterations, due to metal accumulation, can lead to a reduction in the normal globus pallidus inhibitory tone on the thalamo-(motor)-cortical pathway. This, in turn, can result in hyperconnectivity in the motor cortex circuitry, leading to myoclonus and tonic-clonic seizures. We suppose that, in this patient, Wilson disease generated a ‘lesion model’ of myoclonic epilepsy

    Interseismic ground velocities in Central Apennines from GPS and InSAR measurements: new contributions for seismic hazard models by preliminary results of ESA CHARMING project

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    The contribution of space geodetic techniques to interseismic velocity estimation, and thus seismic hazard modelling, has been recognized since two decades and made possible in more recent years by the increased availability and accuracy of geodetic measurements. We present the preliminary results of a feasibility study performed within the CHARMING project (Constraining Seismic Hazard Models with InSAR and GPS), funded by the European Space Agency (ESA). For a 200 km x 200 km study area, covering the Abruzzi region (central Italy) we measure the mean surface deformation rates from Synthetic Aperture Radar and GPS, finding several local to regional deformation gradients consistent with the tectonic context. We then use a kinematic finite element model to derive the long-term strain rates, as well as earthquake recurrence relations. In turn these are input to state-of-the-art probabilistic seismic hazard models, the output of which is validated statistically using data from the Italian national accelerometric and macroseismic intensity databases.Published373-3773T. Pericolosità sismica e contributo alla definizione del rischioN/A or not JCRope

    Mid-term review results of the ESA STSE Pathfinder CHARMING project (Constraining Seismic Hazard Models with InSAR and GPS)

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    We probe the feasibility of integrating GPS and Synthetic Aperture Radar deformation rates within the seismic hazard models of the central Apennines (Italy), exploiting data from over 100 GPS stations and the ~20- year long ERS and ENVISAT SAR image archive. We then use a kinematic finite element model to derive the long-term strain rates, as well as earthquake recurrence relations. In turn these are input to state-of-the-art probabilistic seismic hazard models, the output of which is validated statistically using data from the Italian national accelerometric and macroseismic intensity databases.Published23-273T. Pericolosità sismica e contributo alla definizione del rischioN/A or not JCRrestricte

    Presurgical language fMRI: Clinical practices and patient outcomes in epilepsy surgical planning

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    The goal of this study was to document current clinical practice and report patient outcomes in presurgical language functional MRI (fMRI) for epilepsy surgery. Epilepsy surgical programs worldwide were surveyed as to the utility, implementation, and efficacy of language fMRI in the clinic; 82 programs responded. Respondents were predominantly US (61%) academic programs (85%), and evaluated adults (44%), adults and children (40%), or children only (16%). Nearly all (96%) reported using language fMRI. Surprisingly, fMRI is used to guide surgical margins (44% of programs) as well as lateralize language (100%). Sites using fMRI for localization most often use a distance margin around activation of 10mm. While considered useful, 56% of programs reported at least one instance of disagreement with other measures. Direct brain stimulation typically confirmed fMRI findings (74%) when guiding margins, but instances of unpredicted decline were reported by 17% of programs and 54% reported unexpected preservation of function. Programs reporting unexpected decline did not clearly differ from those which did not. Clinicians using fMRI to guide surgical margins do not typically map known language-critical areas beyond Broca's and Wernicke's. This initial data shows many clinical teams are confident using fMRI not only for language lateralization but also to guide surgical margins. Reported cases of unexpected language preservation when fMRI activation is resected, and cases of language decline when it is not, emphasize a critical need for further validation. Comprehensive studies comparing commonly-used fMRI paradigms to predict stimulation mapping and post-surgical language decline remain of high importance

    The 2013 European Seismic Hazard Model: key components and results

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    The 2013 European Seismic Hazard Model (ESHM13) results from a community-based probabilistic seismic hazard assessment supported by the EU-FP7 project “Seismic Hazard Harmonization in Europe” (SHARE, 2009–2013). The ESHM13 is a consistent seismic hazard model for Europe and Turkey which overcomes the limitation of national borders and includes a through quantification of the uncertainties. It is the first completed regional effort contributing to the “Global Earthquake Model” initiative. It might serve as a reference model for various applications, from earthquake preparedness to earthquake risk mitigation strategies, including the update of the European seismic regulations for building design (Eurocode 8), and thus it is useful for future safety assessment and improvement of private and public buildings. Although its results constitute a reference for Europe, they do not replace the existing national design regulations that are in place for seismic design and construction of buildings. The ESHM13 represents a significant improvement compared to previous efforts as it is based on (1) the compilation of updated and harmonised versions of the databases required for probabilistic seismic hazard assessment, (2) the adoption of standard procedures and robust methods, especially for expert elicitation and consensus building among hundreds of European experts, (3) the multi-disciplinary input from all branches of earthquake science and engineering, (4) the direct involvement of the CEN/TC250/SC8 committee in defining output specifications relevant for Eurocode 8 and (5) the accounting for epistemic uncertainties of model components and hazard results. Furthermore, enormous effort was devoted to transparently document and ensure open availability of all data, results and methods through the European Facility for Earthquake Hazard and Risk (www.​efehr.​org)

    The spectrum of epilepsy with eyelid myoclonia: delineation of disease subtypes from a large multicenter study

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    Objective Epilepsy with eyelid myoclonia (EEM) has been associated with marked clinical heterogeneity. Early epilepsy onset has been recently linked to lower chances of achieving sustained remission and to a less favorable neuropsychiatric outcome. However, much work is still needed to better delineate this epilepsy syndrome. Methods In this multicenter retrospective cohort study, we included 267 EEM patients from nine countries. Data on electroclinical and demographic features, intellectual functioning, migraine with or without aura, family history of epilepsy, and epilepsy syndromes in relatives were collected in each patient. The impact of age at epilepsy onset (AEO) on EEM clinical features was investigated, along with the distinctive clinical characteristics of patients showing sporadic myoclonia involving body regions other than eyelids (body-MYO). Results Kernel density estimation revealed a trimodal distribution of AEO, and Fisher-Jenks optimization disclosed three EEM subgroups: early onset (EO-EEM), intermediate onset (IO-EEM), and late onset (LO-EEM). EO-EEM was associated with the highest rate of intellectual disability, antiseizure medication refractoriness, and psychiatric comorbidities and with the lowest rate of family history of epilepsy. LO-EEM was associated with the highest proportion of body-MYO and generalized tonic-clonic seizures (GTCS), whereas IO-EEM had the lowest observed rate of additional findings. A family history of EEM was significantly more frequent in IO-EEM and LO-EEM compared with EO-EEM. In the subset of patients with body-MYO (58/267), we observed a significantly higher rate of migraine and GTCS but no relevant differences in other electroclinical features and seizure outcome. Significance Based on AEO, we identified consistent EEM subtypes characterized by distinct electroclinical and familial features. Our observations shed new light on the spectrum of clinical features of this generalized epilepsy syndrome and may help clinicians toward a more accurate classification and prognostic profiling of EEM patients

    International consensus recommendations for management of new onset refractory status epilepticus including febrile infection-related epilepsy syndrome: Statements and supporting evidence

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    Objective: This study was undertaken to develop consensus-based recommendations for the management of adult and pediatric patients with new onset refractory status epilepticus (NORSE)/febrile infection-related epilepsy syndrome (FIRES) based on best evidence and experience. Methods: The Delphi methodology was followed. A facilitator group of nine experts was established, who defined the scope, users, and suggestions for recommendations. Following a review of the current literature, recommendation statements concerning diagnosis, treatment, and research directions were generated, which were then rated on a scale of 1 (strongly disagree) to 9 (strongly agree) by a panel of 48 experts in the field. Consensus that a statement was appropriate was reached if the median score was ≥7 and inappropriate if the median score was ≤3. The analysis of evidence was mapped to the results of each statement included in the Delphi survey. Results: Overall, 85 recommendation statements achieved consensus. The recommendations are divided into five sections: (1) disease characteristics; (2) diagnostic testing and sampling; (3) acute treatment; (4) treatment in the postacute phase; and (5) research, registries, and future directions in NORSE/FIRES. The detailed results and discussion of all 85 statements are outlined herein. A corresponding summary of findings and practical flowsheets are presented in a companion article. Significance: This detailed analysis offers insight into the supporting evidence and the current gaps in the literature that are associated with expert consensus statements related to NORSE/FIRES. The recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research

    Exigências minerais comparadas de dois cultivares de soja (Glycine max (L.) Merrill): Santa Rosa e UFV-1

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    Macro and micronutrients were determined in the various organs of two soybean cultivars grown in nutrient solution until the period of pod filling. The main conclusions were as follows: (1) there are striking differences between the two cultivars with respect of composition, total absorption and proportion of elements in the pods; (2) Santa Rosa showed higher efficiency for utilization of N, P and K for yield formation.Plantas de soja, cv. Santa Rosa e cv. UFV-1 foram cultivadas em solução nutritiva até a produção de vagens quando foram colhidas e analisadas. A variedade Santa Rosa mostrou-se menos exigente em N, P, K, Ca, Mg, Cu, Fe, Mn e Zn; a exigência de S foi igual e a de Cl maior que a da cv. UFV-1
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