364 research outputs found

    IDIOPATHIC PARTIAL EPILEPSY WITH AUDITORY FEATURES (IPEAF): A CLINICAL AND GENETIC STUDY OF 53 SPORADIC CASES

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    The purpose of our study was to describe the clinical characteristics of sporadic (S) cases of partial epilepsy with auditory features (PEAF) and pinpoint clinical, prognostic and genetic differences with respect to previously reported familial (F) cases of autosomal dominant partial epilepsy with auditory features (ADPEAF). We analysed 53 patients (24 females and 29 males) with PEAF diagnosed according to the following criteria: partial epilepsy with auditory symptoms, negative family history for epilepsy and absence of cerebral lesions on NMR study. All patients underwent a full clinical, neuroradiological and neurophysiological examination. Forty patients were screened for mutations in LGI1/epitempin, which is involved in ADPEAF. Age at onset ranged from 6 to 39 years (average 19 years). Secondarily generalized seizures were the most common type of seizures at onset (79%). Auditory auras occurred either in isolation (53%) or associated with visual, psychic or aphasic symptoms. Low seizure frequency at onset and good drug responsiveness were common, with 51% of patients seizure-free. Seizures tended to recur after drug withdrawal. Clinically, no major differences were found between S and F patients with respect to age at onset, seizure frequency and response to therapy. Analysis of LGI1/epitempin exons failed to disclose mutations. Our data support the existence of a peculiar form of non-lesional temporal lobe epilepsy closely related to ADPEAF but without a positive family history. This syndrome, here named IPEAF, has a benign course in the majority of patients and could be diagnosed by the presence of auditory aura. Although LGI1 mutations have been excluded, genetic factors may play an aetiopathogenetic role in at least some of these S cases

    SOXS: a wide band spectrograph to follow up transients

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    SOXS (Son Of X-Shooter) will be a spectrograph for the ESO NTT telescope capable to cover the optical and NIR bands, based on the heritage of the X-Shooter at the ESO-VLT. SOXS will be built and run by an international consortium, carrying out rapid and longer term Target of Opportunity requests on a variety of astronomical objects. SOXS will observe all kind of transient and variable sources from different surveys. These will be a mixture of fast alerts (e.g. gamma-ray bursts, gravitational waves, neutrino events), mid-term alerts (e.g. supernovae, X-ray transients), fixed time events (e.g. close-by passage of minor bodies). While the focus is on transients and variables, still there is a wide range of other astrophysical targets and science topics that will benefit from SOXS. The design foresees a spectrograph with a Resolution-Slit product ~ 4500, capable of simultaneously observing over the entire band the complete spectral range from the U- to the H-band. The limiting magnitude of R~20 (1 hr at S/N~10) is suited to study transients identified from on-going imaging surveys. Light imaging capabilities in the optical band (grizy) are also envisaged to allow for multi-band photometry of the faintest transients. This paper outlines the status of the project, now in Final Design Phase.Comment: 12 pages, 14 figures, to be published in SPIE Proceedings 1070

    An ultra-long new onset refractory status epilepticus: Winning the battle but losing the war?

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    New onset refractory status epilepticus (NORSE), is a rare and challenging condition occurring in previously healthy people. The etiology often remains undiscovered and is frequently associated with an unfavorable outcome. We report the electroclinical and neuroradiological evolution of an ultra-long case of NORSE of unknown etiology. A 38-year-old woman with a prodrome of fever, vomiting and diarrhea was admitted to our Intensive Care Unit for refractory convulsive status epilepticus (SE). Her past medical history was unremarkable. Extensive examinations were negative for potential viral, autoimmune and metabolic etiologies. Despite multiple therapeutical attempts with antiseizures medications, anesthetics and immunotherapy, seizures persisted. After nearly 6 months of enduring seizures, SE finally ceased and the patient gradually recovered to a minimum state of awareness. She was then able to communicate through one-word utterances and to understand simple tasks. At a three-years follow-up, she developed multifocal drug-resistant epilepsy, subcortical myoclonus and severe spastic quadraparesis, becoming completely dependent for activities of daily living. To our knowledge, this represents one of the longest cases of NORSE with final status resolution at this time. However, ultra-long SE in this case led to severe and disabling neurological sequelae. Future studies focused on disease modifying treatments for refractory SE are needed

    Optimization of paper characterization procedures for cultural heritage

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    La conservazione dei Beni Culturali è di importanza fondamentale per sostenere l’identità nazionale. Trattamenti con le radiazioni ionizzanti vengono utilizzati in tutto il mondo per la conservazione di opere d’arte costituite da materiali di origine naturale (carta, legno, pergamena) ma in Italia non sono ancora accettati del tutto, probabilmente a causa della non corretta conoscenza degli effetti indotti dalla radiazione sul manufatto. Da questo punto di vista, lo studio approfondito di questi effetti secondari e delle condizioni di irraggiamento che permettono di minimizzarli riveste un ruolo centrale. In questo report sono descritte procedure convalidate di caratterizzazione fisico chimica della carta, prima e dopo irraggiamento, con diverse tecniche sperimentali.Conservation and preservation of Cultural Heritage is of paramount importance to maintain the national identity. Ionizing radiation treatments are worldwide applied for conservation of artworks made from materials of organic origin (paper, wood, parchment, etc.) but in Italy they are not fully accepted probably due to the incorrect knowledge of the radiation induced effects on the artifacts. In this regard, the investigation of these secondary effects and of their minimization plays a key role. In this report, established procedures for the physico-chemical characterization of paper, before

    The effect of quarantine due to Covid-19 pandemic on seizure frequency in 102 adult people with epilepsy from Apulia and Basilicata regions, Southern Italy

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    Objective: following the COVID-19 pandemic, a quarantine was imposed to all of regions Italy by 9th March until 3rd May 2020. We investigated the effect of COVID-19 infection and quarantine on seizure frequency in adult people with epilepsy (PwE) of Apulia and Basilicata regions, Southern Italy. Methods: This is an observational, retrospective study based on prospective data collection of 102 successive PWE. The frequency of seizures was evaluated during pre-quarantine (January- February), quarantine (March-April), and post-quarantine period (May-June), while PwE were divided into A) cases responding to treatment with ≤ 1 seizure per year; B) cases responding to treatment with 2-5 seizure per year; C) cases with drug-resistant epilepsy with ≤ 4 seizures per month; D) cases with drug-resistant epilepsy with 5-10 seizures per month. PwE underwent several self-report questionnaires regarding therapeutic compliance, mood, stress and sleep during quarantine period. Results: Approximately 50 % of PwE showed seizure frequency changes (22.55 % an increase and 27.45 % a reduction) during quarantine. Seizure frequency significantly (p < 0.05) increased in PwE responding to treatment with ≤ 1 seizure per year, while significantly (p < 0.05) reduced in PwE with drug-resistant epilepsy with 5-10 seizures per month. The data was not influenced by therapeutic adherence, sleep and depression. The analysis of anxiety showed a moderate level of anxiety in PwE responding to treatment with < 1 seizure per year, while moderate stress was perceived by all PwE. Seizure frequency changes were related to quarantine, but not to COVID-19 infection. In fact, unlike other regions of Italy, particularly Northern Italy, Apulia and Basilicata regions were less affected by COVID-19 infection, and almost all PwE recognized the quarantine as a stressful event. Emotional distress and anxiety due to social isolation, but also the relative reduction of triggers for epileptic seizures were the most important factors for changes in seizure frequency. Conclusions: Our study adds to the growing concern that the indirect effects of COVID-19 pandemic will far outstrip the direct consequences of the infection

    Gamma irradiation for Cultural Heritage conservation: Comparison of the side effects on new and old paper

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    Ionizing radiations, commonly applied as diagnostic tools in Cultural Heritage (CH) field, are also proving effective for eliminating biodeteriogens (insects, fungi, bacteria and molds) responsible for the degradation of CH artifacts and often harmful for restorers, archivists and librarians. The use of ionizing radiations, such as gamma rays, for CH treatments is spreading in many countries. However, some CH operators remain resistant due to insufficient knowledge about the potential physico-chemical modifications (secondary effects) induced by radiation. This work aims to investigate and compare the effects of irradiation parameters (such as absorbed dose and dose rate) on old paper samples and new pure-cellulose paper, chosen as a reference model material. Absorbed doses up to 8 kGy have been used, as these values are commonly agreed upon for the preservation treatment of CH artifacts and are generally effective for biodeteriogens removal. Optimizing irradiation conditions helps to minimize secondary effects (such as oxidation, depolymerization or color changes), thereby increasing the reliability of the process and boosting confidence among CH operators. The secondary effects were analyzed using various physico-chemical characterizations (Fourier Transform Infrared spectroscopy, Raman microscopy, viscosimetric and colorimetric analysis) on old and new paper samples. The results indicate varying behaviors, correlated with paper composition, sample age and irradiation parameters, towards gamma radiation. This groundbreaking study not only confirms the efficacy of gamma irradiation treatments but also provides essential data that will aid in the development of optimized best practice protocols and guidelines for non-destructive and minimally destructive methods applied to real case studies and treatments

    Gamma irradiation for Cultural Heritage conservation: Comparison of the side effects on new and old paper

    Get PDF
    Ionizing radiations, commonly applied as diagnostic tools in Cultural Heritage (CH) field, are also proving effective for eliminating biodeteriogens (insects, fungi, bacteria and molds) responsible for the degradation of CH artifacts and often harmful for restorers, archivists and librarians. The use of ionizing radiations, such as gamma rays, for CH treatments is spreading in many countries. However, some CH operators remain resistant due to insufficient knowledge about the potential physico-chemical modifications (secondary effects) induced by radiation. This work aims to investigate and compare the effects of irradiation parameters (such as absorbed dose and dose rate) on old paper samples and new pure-cellulose paper, chosen as a reference model material. Absorbed doses up to 8 kGy have been used, as these values are commonly agreed upon for the preservation treatment of CH artifacts and are generally effective for biodeteriogens removal. Optimizing irradiation conditions helps to minimize secondary effects (such as oxidation, depolymerization or color changes), thereby increasing the reliability of the process and boosting confidence among CH operators. The secondary effects were analyzed using various physico-chemical characterizations (Fourier Transform Infrared spectroscopy, Raman microscopy, viscosimetric and colorimetric analysis) on old and new paper samples. The results indicate varying behaviors, correlated with paper composition, sample age and irradiation parameters, towards gamma radiation. This groundbreaking study not only confirms the efficacy of gamma irradiation treatments but also provides essential data that will aid in the development of optimized best practice protocols and guidelines for non-destructive and minimally destructive methods applied to real case studies and treatments

    Incidence trends of colorectal cancer in the early 2000s in Italy. Figures from the IMPATTO study on colorectal cancer screening

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    We utilised the IMPATTO study's archives to describe the 2000-2008 colorectal cancer (CRC) incidence rate trends in Italy, once screening programmes based on the faecal immunochemical test were implemented in different areas. Data on CRCs diagnosed in Italy from 2000 to 2008 in subjects aged 40-79 years were collected by 23 cancer registries. Incidence rate trends were evaluated as a whole and by macro-area (North-Centre and South-Islands), presence of a screening programme, sex, ten-year age class, anatomic site, stage at diagnosis, and pattern of diagnosis (screen-detected, non-screen-detected). The annual percent change (APC) of incidence rate trends, with 95% confidence intervals (95%CI), were computed. The study included 46,857 CRCs diagnosed in subjects aged 40-79 years, of which 2,806 were screendetected. The incidence rates in the North-Centre were higher than in the South and on the Islands. During the study period, screening programmes had been implemented only in the North-Centre and had a significant effect on incidence rates, with an initial sharp increase in incidence, followed by a decrease that started in the 3rd-4th years of screening. These incidence rate trends were exclusively due to modifications in the rates of stage I cases. After screening programmes started, incidence increased in all anatomic sites, particularly in the distal colon. The differential figures introduced by the implementation of screening programmes warrant a continuous surveillance of CRC incidence and mortality trends to monitor the impact of screening at a national level
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