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Flammable liquids typically adopted in chemical and industrial processes can be subjected to intense heat radiation due to nearby hot surfaces or fires. A second cascading event is then likely because the flash point can be easily reached and the surrounding environment can provide sufficient energy for ignition. Nevertheless, the flaming condition is not necessarily reached instantaneously if no direct (e.g., spark) ignition is present. Hence, a time to flaming, i.e., in the absence of an ignition source, is extremely relevant for the active and passive fire protection of flammable liquid, as the minimum time for intervention can be defined before developing a pool fire and for the correct evaluation of domino effects, in analogy with gas fires. In addition, a complete characterization of liquid behaviour under radiant heating is essential to guarantee safe operations, prevention measures, robust databases and optimized design. Therefore, this work reports the results for three liquid solvent representative classes of substances: i) hexane, ii) ethyl acetate, and iii) ethanol. A bench scale facility was adopted for the characterization of mass and energy aspects involving a pool fire of the investigated substances. Among the others, the ignitability and the intensity of the generated pool fires were evaluated experimentally in terms of mass burning rate, heat release rate, and combustion efficiency. Different boundary conditions were tested to identify the optimized set of conditions for a robust evaluation of the safety aspects of liquid. Results showed the most relevant trends in terms of ignitability of liquid species and combustion intensity during the pseudo-steady state regime of a pool fire. In addition, indications on the boundary conditions to be considered for a robust and consistent dataset were provided in this work.

Background and objectivesBRAT1 encephalopathy is an ultra-rare autosomal recessive neonatal encephalopathy. We delineate the neonatal electroclinical phenotype at presentation and provide insights for early diagnosis.MethodsThrough a multinational collaborative, we studied a cohort of neonates with encephalopathy associated with biallelic pathogenic variants in BRAT1 for whom detailed clinical, neurophysiologic, and neuroimaging information was available from the onset of symptoms. Neuropathologic changes were also analyzed.ResultsWe included 19 neonates. Most neonates were born at term (16/19) from nonconsanguineous parents. 15/19 (79%) were admitted soon after birth to a neonatal intensive care unit, exhibiting multifocal myoclonus, both spontaneous and exacerbated by stimulation. 7/19 (37%) had arthrogryposis at birth, and all except 1 progressively developed hypertonia in the first week of life. Multifocal myoclonus, which was present in all but 1 infant, was the most prominent manifestation and did not show any EEG correlate in 16/19 (84%). Video-EEG at onset was unremarkable in 14/19 (74%) infants, and 6 (33%) had initially been misdiagnosed with hyperekplexia. Multifocal seizures were observed at a median age of 14 days (range: 1-29). During the first months of life, all infants developed progressive encephalopathy, acquired microcephaly, prolonged bouts of apnea, and bradycardia, leading to cardiac arrest and death at a median age of 3.5 months (range: 20 days to 30 months). Only 7 infants (37%) received a definite diagnosis before death, at a median age of 34 days (range: 25-126), and almost two-thirds (12/19, 63%) were diagnosed 8 days to 12 years postmortem (median: 6.5 years). Neuropathology examination, performed in 3 patients, revealed severely delayed myelination and diffuse astrogliosis, sparing the upper cortical layers.DiscussionBRAT1 encephalopathy is a neonatal-onset, rapidly progressive neurologic disorder. Neonates are often misdiagnosed as having hyperekplexia, and many die undiagnosed. The key phenotypic features are multifocal myoclonus, an organized EEG, progressive, persistent, and diffuse hypertonia, and an evolution into refractory multifocal seizures, prolonged bouts of apnea, bradycardia, and early death. Early recognition of BRAT1 encephalopathy allows for prompt workup, appropriate management, and genetic counseling.

Abstract Proprioception plays a key role in moving our body dexterously and effortlessly. Nevertheless, the majority of investigations evaluating the benefits of providing supplemental feedback to prosthetics users focus on delivering touch restitution. These studies evaluate the influence of touch sensation in an attempt to improve the controllability of current robotic devices. Contrarily, investigations evaluating the capabilities of proprioceptive supplemental feedback have yet to be comprehensively analyzed to the same extent, marking a major gap in knowledge within the current research climate. The non-invasive strategies employed so far to restitute proprioception are reviewed in this work. In the absence of a clearly superior strategy, approaches employing vibrotactile, electrotactile and skin-stretch stimulation achieved better and more consistent results, considering both kinesthetic and grip force information, compared with other strategies or any incidental feedback. Although emulating the richness of the physiological sensory return through artificial feedback is the primary hurdle, measuring its effects to eventually support the integration of cumbersome and energy intensive hardware into commercial prosthetic devices could represent an even greater challenge. Thus, we analyze the strengths and limitations of previous studies and discuss the possible benefits of coupling objective measures, like neurophysiological parameters, as well as measures of prosthesis embodiment and cognitive load with behavioral measures of performance. Such insights aim to provide additional and collateral outcomes to be considered in the experimental design of future investigations of proprioception restitution that could, in the end, allow researchers to gain a more detailed understanding of possibly similar behavioral results and, thus, support one strategy over another.

Background and purposeKetogenic diet (KD) is an emerging treatment option for super-refractory status epilepticus (SRSE). We evaluated the effectiveness of KD in patients presenting SRSE including NORSE (and its subcategory FIRES).MethodsA retrospective review of the medical records was performed at the Necker Enfants Malades Hospital. All children with SRSE in whom KD was started during the last 10 years were included. A systematic search was carried out for all study designs, including at least one patient of any age with SRSE in whom KD was started. The primary outcome was the responder rate and Kaplan–Meier survival curves were generated for the time-to-KD response. As secondary outcomes, Cox proportional hazard models were created to assess the impact of NORSE-related factors on KD efficacy.ResultsSixteen children received KD for treatment of SRSE, and three had NORSE presentation (one infectious etiology, two FIRES). In medical literature, 1,613 records were initially identified, and 75 were selected for review. We selected 276 patients receiving KD during SRSE. The most common etiology of SRSE was acute symptomatic (21.3%), among these patients, 67.7% presented with NORSE of immune and infectious etiologies. Other etiologies were remote symptomatic (6.8%), progressive symptomatic (6.1%), and SE in defined electroclinical syndromes (14.8%), including two patients with genetic etiology and NORSE presentation. The etiology was unknown in 50.7% of the patients presenting with cryptogenic NORSE, of which 102 presented with FIRES. Overall, most patients with NORSE benefit from KD (p < 0.004), but they needed a longer time to achieve RSE resolution after starting KD compared with other non-NORSE SRSE (p = 0.001). The response to KD in the NORSE group with identified etiology compared to the cryptogenic NORSE was significantly higher (p = 0.01), and the time to achieve SE resolution after starting KD was shorter (p = 0.04).ConclusionsThe search for underlying etiology should help to a better-targeted therapy. KD can have good efficacy in NORSE; however, the time to achieve SE resolution seems to be longer in cryptogenic cases. These findings highlight the therapeutic role of KD in NORSE, even though this favorable response needs to be better confirmed in prospective controlled studies.

BackgroundDespite the latest advances in prenatal diagnosis and postnatal embolization procedures, intracranial arteriovenous shunts (AVSs) are still associated with high mortality and morbidity rates. Our aim was to evaluate the presentation and clinical course, the neurodevelopmental outcome, and the genetic findings of neonates with AVSs.MethodsIn this retrospective observational study, medical records of neonates with cerebral AVSs admitted to our hospital from January 2020 to July 2022 were revised. In particular, we evaluated neuroimaging characteristics, endovascular treatment, neurophysiological features, neurodevelopmental outcomes, and genetic findings.ResultsWe described the characteristics of 11 patients with AVSs. Ten infants (90.9%) required embolization during the first three months of life. In 5/9 infants, pathological electroencephalography findings were observed; of them, two patients presented seizures. Eight patients performed Median Nerve Somatosensory Evoked Potentials (MN-SEPs): of them, six had an impaired response. We found normal responses at Visual Evoked Potentials and Brainstem Auditory Evoked Potentials. Eight patients survived (72.7%) and were enrolled in our multidisciplinary follow-up program. Of them, 7/8 completed the Bayley-III Scales at 6 months of corrected age: none of them had cognitive and language delays; conversely, a patient had a moderate delay on the Motor scale. The remaining survivor patient developed cerebral palsy and could not undergo Bayley-III evaluation because of the severe psychomotor delay. From the genetic point of view, we found a novel pathogenic variant in the NOTCH3 gene and three additional genomic defects of uncertain pathogenicity.ConclusionWe propose SEPs as an ancillary test to discern the most vulnerable infants at the bedside, particularly to identify possible future motor impairment in follow-up. The early identification of a cognitive or motor delay is critical to intervene with personalized rehabilitation treatment and minimize future impairment promptly. Furthermore, the correct interpretation of identified genetic variants could provide useful information, but further studies are needed to investigate the role of these variants in the pathogenesis of AVSs.

Flammable liquids typically adopted in chemical and industrial processes can be subjected to intense heat radiation due to nearby hot surfaces or fires. A second cascading event is then likely because the flash point can be easily reached and the surrounding environment can provide sufficient energy for ignition. Nevertheless, the flaming condition is not necessarily reached instantaneously if no direct (e.g., spark) ignition is present. Hence, a time to flaming, i.e., in the absence of an ignition source, is extremely relevant for the active and passive fire protection of flammable liquid, as the minimum time for intervention can be defined before developing a pool fire and for the correct evaluation of domino effects, in analogy with gas fires. In addition, a complete characterization of liquid behaviour under radiant heating is essential to guarantee safe operations, prevention measures, robust databases and optimized design. Therefore, this work reports the results for three liquid solvent representative classes of substances: i) hexane, ii) ethyl acetate, and iii) ethanol. A bench scale facility was adopted for the characterization of mass and energy aspects involving a pool fire of the investigated substances. Among the others, the ignitability and the intensity of the generated pool fires were evaluated experimentally in terms of mass burning rate, heat release rate, and combustion efficiency. Different boundary conditions were tested to identify the optimized set of conditions for a robust evaluation of the safety aspects of liquid. Results showed the most relevant trends in terms of ignitability of liquid species and combustion intensity during the pseudo-steady state regime of a pool fire. In addition, indications on the boundary conditions to be considered for a robust and consistent dataset were provided in this work. [ABSTRACT FROM AUTHOR]

Background: Using machine learning to combine wrist accelerometer (ACM) and electrodermal activity (EDA) has been shown effective to detect primarily and secondarily generalized tonic-clonic seizures, here termed as convulsive seizures (CS). A prospective study was conducted for the FDA clearance of an ACM and EDA-based CS-detection device based on a predefined machine learning algorithm. Here we present its performance on pediatric and adult patients in epilepsy monitoring units (EMUs).Methods: Patients diagnosed with epilepsy participated in a prospective multi-center clinical study. Three board-certified neurologists independently labeled CS from video-EEG. The Detection Algorithm was evaluated in terms of Sensitivity and false alarm rate per 24 h-worn (FAR) on all the data and on only periods of rest. Performance were analyzed also applying the Detection Algorithm offline, with a less sensitive but more specific parameters configuration (“Active mode”).Results: Data from 152 patients (429 days) were used for performance evaluation (85 pediatric aged 6–20 years, and 67 adult aged 21–63 years). Thirty-six patients (18 pediatric) experienced a total of 66 CS (35 pediatric). The Sensitivity (corrected for clustered data) was 0.92, with a 95% confidence interval (CI) of [0.85-1.00] for the pediatric population, not significantly different (p > 0.05) from the adult population's Sensitivity (0.94, CI: [0.89–1.00]). The FAR on the pediatric population was 1.26 (CI: [0.87–1.73]), higher (p < 0.001) than in the adult population (0.57, CI: [0.36–0.81]). Using the Active mode, the FAR decreased by 68% while reducing Sensitivity to 0.95 across the population. During rest periods, the FAR's were 0 for all patients, lower than during activity periods (p < 0.001).Conclusions: Performance complies with FDA's requirements of a lower bound of CI for Sensitivity higher than 0.7 and of a FAR lower than 2, for both age groups. The pediatric FAR was higher than the adult FAR, likely due to higher pediatric activity. The high Sensitivity and precision (having no false alarms) during sleep might help mitigate SUDEP risk by summoning caregiver intervention. The Active mode may be advantageous for some patients, reducing the impact of the FAR on daily life. Future work will examine the performance and usability outside of EMUs.

Rheumatoid meningitis (RM) is a rare but often aggressive neurological complication of rheumatoid arthritis. The diagnosis of RM, besides the clinical, radiological, and laboratory criteria, usually requires a cerebral biopsy. Based on the two cases presented in this paper, we propose a new laboratory marker. Cerebrospinal fluid and serum anti-cyclic citrullinated peptide (CCP) IgG were measured, and the intrathecal synthesis of anti-CCP antibodies (anti-CCP antibody index) was calculated using the hyperbolic function. The anti-CCP antibody index was positive in both cases at first diagnosis and progressively decreased after treatments. Together with clinical and radiological criteria, the calculation of the anti-CCP intrathecal synthesis, more than the simple measurement of serum or cerebrospinal fluid anti-CCP antibody titers, may represent a useful tool for RM diagnosis and, possibly, for treatment response.

Dravet syndrome (DS) is a rare developmental and epileptic encephalopathy. Infants with DS are especially vulnerable to the detrimental effects of prolonged and frequent seizures on development. Fenfluramine (FFA) is approved for the treatment of DS in patients aged 2 years and older. This study aims to evaluate the safety and efficacy of FFA in patients with DS younger than 2 years. We analyzed safety, tolerability, seizure, and neuropsychological outcome in a real‐world setting. Developmental profile was investigated using Griffiths Mental Development Scales (GMDS). Five patients received FFA at a mean age of 14.9 months (9.6–18.6). Median follow‐up was 13 months (interquartile range [IQR] = 12.9–24.4). All patients showed good tolerance to FFA. No significant variation of body mass index or echocardiographic issue was observed. Monthly median convulsive seizure frequency (MCSF) was 1.71 (IQR = 1.56–3.27) at the 6‐month baseline period and.92 (IQR =.43–1.28) at last follow‐up, with a median 54.43 (IQR = 40.91–60.83) percentage reduction in MCSF. Two of five patients had a performance improvement on GMDS subscales. Overall, the use of FFA below the age of 2 years in our small sample of patients was safe and represents a promising opportunity for seizure control and for protection of the neurodevelopmental outcome. [ABSTRACT FROM AUTHOR]

Abstract Adherence to the thinness model, self-acceptance such as self-esteem is psychological dynamics influencing the young age and emerging adulthood of women life. The purpose of this study was to investigate the girls and young women’ ability to deal with the adherence to thinness model according to their self-body management thought daily self-perception of ownhabits and aptitude. We analysed their emotional patterns and body management to elucidate the Italian phenomenon. A cross-sectional study was conducted on 2287 Italian female distribute in range age 15–25 years old and distributed in girl and young women groups. We conducted a Survey study by snowball sampling technique. Our results showed that girls had higher emotional pattern scores when their weight and shape fit the thinness model: skinny girls felt positively about their body even if when they did not take adequate care of it. Italian girls consider the underweight body mass index an adherence model. Findings suggest the urgent need to plan prevention programme to model healthy behaviours about their daily good practice overcoming social and cultural models based on appearance.

Salvator Rosa (Napoli, 1615- Roma 1673) fu pittore e poeta tra i più interessanti del secolo diciassettesimo. Il saggio intende indagare il complesso rapporto artistico e personale che Rosa ebbe con la città di Napoli, in cui era nato e dalla quale era quasi fuggito quando era poco più che un ragazzo. Attraverso una lettura delle principali fonti biobibliografiche a lui contemporanee, di alcune delle sue tele, dei versi delle Satire e di stralci delle lettere che a Napoli dedicherà, s’intende sottolineare che della capitale del Meridione la scrittura e l’arte di Salvator Rosa sono in grado di restituirci un’immagine altra rispetto a quella topica di Napoli gentile, perla luminosa di quella Campania felix che proprio nel Seicento attrae pittori e poeti da ogni parte d’Europa. Nei versi di Rosa, come nella scrittura in prosa, Napoli è, infatti, croce e delizia, prigione da cui fuggire e porto di quell’agognato ritorno che, al di là delle leggendarie asserzioni di alcuni biografi romantici, mai avverrà. Salvator Rosa (Naples, 1615- Rome, 1673) was a painter and poet among the most interesting of the seventeenth century. The essay intends to investigate the complex artistic and personal relationship that Rosa had with the city of Naples, where she was born and from which she had fled when she was little more than a boy. Through a reading of the main biobibliographic sources to him contemporary, some of his canvases, the verses of the Satire and the excerpts of the letters that Naples will dedicate, it is meant to point out that in the capital of the South the writing and art of Salvator Rosa are Able to give us an image other than the topical one of Napoli's kind, bright pearl of the Campania felix that in the seventeenth century attracts painters and poets from all over Europe. In the lines of Rosa, as in prose writing, Naples is, in fact, a cross and delight, a prison from which to flee and bring about that auspicious return that, beyond the legendary assertions of some romantic biographers, will never happen.