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Objective. To describe clinical characteristics, genetic details and management of patients of congenital myasthenic syndrome. Methods. A retrospective study was carried out from 2020 to 2023. Patient who presented with ptosis, ophthalmoplegia, proximal limb weakness with genetically confirmed mutations for CMS were included in the study. Patients who were positive for Acetylcholine receptor antibody (AChRAb) or Muscle specific kinase (MuSK) antibody or any other antibodies suggestive of acquired myasthenia were excluded from the study. Outcome. 6 genetically proven patients were identified out of which 4 patients had CHRNE mutation, 1 patient had CHRNA1 mutation and 1 patient had Dok7 mutation. Mean delay in diagnosis was 15 years. Patient with Dok 7 mutation responded to salbutamol, 1 patient of CHRNE mutation required combination of pyridostigmine, fluoxetine and salbutamol, rest all patient responded to pyridostigmine. It took 4 weeks for improvement after starting salbutamol in patient with Dok7 mutation. Conclusion. Although rare, CMS is potentially treatable group of disease. Patient presenting with early onset muscular weakness should be evaluated thoroughly and CMS should be considered in presence of eyelid ptosis, ophthalmoplegia and proximal weakness with diurnal fluctuations. RNS and genetic testing are paramount in achieving an early diagnosis.

PURPOSETo share our clinical experience with the diagnosis and management of children with hematolymphoid malignancies presenting with epilepsia partialis continua (EPC) as a sequelae of measles infection.MATERIALS AND METHODSIn December 2022, a series of children in our hemato-oncology unit presented with focal status epilepticus with no conclusive evidence pointing toward any underlying etiology. One such child had a typical measles rash a few weeks before the onset of this focal status epilepticus. After a series of cases with a similar presentation, a clinical pattern suspicious for measles became evident. cerebrospinal fluid polymerase chain reaction was positive for measles virus with measles immunoglobin M detected in the serum. This led to the diagnosis of measles inclusion-body encephalitis in a series of children who presented with EPC over a period of 3 months. EPC is a rare manifestation of measles that is seen only in immunocompromised patients.RESULTSAmong the 18 children reported in this series, only 10 had a history of rashes. The rash was mostly transient and elicited only on retrospective history taking. Five of the 18 children who did not lose consciousness during the prolonged seizure episode survived the disease but had residual neurologic sequelae. Among the 18 children, two were unimmunized and immunization status could not be confirmed in three other children.CONCLUSIONThis case series highlights the threats posed by measles infection in children with cancer who are immunosuppressed because of the underlying disease and ongoing chemotherapy. Loss of herd immunity because of declining measles immunization rates secondary to vaccine hesitancy and COVID-19 lockdown pose a greater risk of measles infection and its complications for patients with deficient immune systems.

Novel wind technologies, in particular airborne wind energy (AWE) and floating offshore wind turbines, have the potential to unlock untapped wind resources and contribute to power system stability in unique ways. So far, the techno-economic potential of both technologies has only been investigated at a small scale, whereas the most significant benefits will likely play out on a system scale. Given the urgency of the energy transition, the possible contribution of these novel technologies should be addressed. Therefore, we investigate the main system-level trade-offs in integrating AWE systems and floating wind turbines into a highly renewable future energy system. To do so, we develop a modelling workflow that integrates wind resource assessment and future cost and performance estimations into a large-scale energy system model, which finds cost-optimal system designs that are operationally feasible with hourly temporal resolution across ten countries in the North Sea region. Acknowledging the uncertainty on AWE systems’ future costs and performance and floating wind turbines, we examine a broad range of cost and technology development scenarios and identify which insights are consistent across different possible futures. We find that onshore AWE outperforms conventional onshore wind regarding system-wide benefits due to higher wind resource availability and distinctive hourly generation profiles, which are sometimes complementary to conventional onshore turbines. The achievable power density per ground surface area is the main limiting factor in large-scale onshore AWE deployment. Offshore AWE, in contrast, provides system benefits similar to those of offshore wind alternatives. Therefore, deployment is primarily driven by cost competitiveness. Floating wind turbines achieve higher performance than conventional wind turbines, so they can cost more and remain competitive. AWE, in particular, might be able to play a significant role in a climate-neutral European energy supply and thus warrants further study.

Objective: Covid-19 is a highly infectious viral disease, and our understanding of the impact of this virus on the nervous system is limited. Therefore, we aimed to do a systematic analysis of the neurological manifestations. Methods: We retrospectively studied the clinical, laboratory, and radiological findings of patients with major neurological syndromes (MNS) in Covid-19 over 6 months. Results: We had 39 patients with major neurological syndromes (MNS). The most common MNS was cerebrovascular disease (CVD) (61.53%), in which ischemic stroke (83.33%), cortical sinus thrombosis (12.50%), and haemorrhagic stroke (4.16%) were seen. Among ischemic stroke patients, 50% had a large vessel occlusion, and 66.66% of patients with CVD had a significant residual disability. Cranial neuropathy (15.38%), GBS (10.26%), encephalitis (7.26%), and myelitis (5.12%) were the other MNS. Among the three encephalitis cases, two had CSF-Covid-19 PCR positivity and had severe manifestations and a poor outcome. Associated comorbidities included hypertension (30.76%), diabetes mellitus (12.82%), chronic kidney diseases (7.69%), and polycythaemia vera (2.56%). Lung involvement was seen in 64.1% of patients. Mortality was 17.94% in MNS with Covid-19. Conclusions: The most common major neurological syndrome associated with Covid-19 is CVD with increased frequency of large vessel occlusion causing significant morbidity and mortality. Simultaneous lung and other systemic involvement in MNS results in a deleterious outcome. Doi: 10.28991/SciMedJ-2022-0401-02 Full Text: PDF

The majority of remote locations not connected to the main electricity grid rely on diesel generators to provide electrical power. High fuel transportation costs and significant carbon emissions have motivated the development and installation of hybrid power systems using renewable energy such these locations. Because wind and solar energy is intermittent, such sources are usually combined with energy storage for a more stable power supply. This paper presents a modelling and sizing framework for off-grid hybrid power systems using airborne wind energy, solar PV, batteries and diesel generators. The framework is based on hourly time-series data of wind resources from the ERA5 reanalysis dataset and solar resources from the National Solar Radiation Database maintained by NREL. The load data also include hourly time series generated using a combination of modelled and real-life data from the ENTSO-E platform maintained by the European Network of Transmission System Operators for Electricity. The backbone of the framework is a strategy for the sizing of hybrid power system components, which aims to minimise the levelised cost of electricity. A soft-wing ground-generation-based AWE system was modelled based on the specifications provided by Kitepower B.V. The power curve was computed by optimising the operation of the system using a quasi-steady model. The solar PV modules, battery systems and diesel generator models were based on the specifications from publicly available off-the-shelf solutions. The source code of the framework in the MATLAB environment was made available through a GitHub repository. For the representation of results, a hypothetical case study of an off-grid military training camp located in Marseille, France, was described. The results show that significant reductions in the cost of electricity were possible by shifting from purely diesel-based electricity generation to an hybrid power system comprising airborne wind energy, solar PV, batteries and diesel.

In the current auction-based electricity market, the design of utility-scale renewable energy systems has traditionally been driven by the levelised cost of energy (LCoE). However, the market is gradually moving towards a subsidy-free era, which will expose the power plant owners to the fluctuating prices of electricity. This paper presents a computational approach to account for the influence of time-varying electricity prices on the design of airborne wind energy (AWE) systems. The framework combines an analytical performance model, providing the power curve of the system, with a wind resource characterisation based on ERA5 reanalysis data. The resulting annual energy production (AEP) model is coupled with a parametric cost model based on reference prototype data from Ampyx Power B.V. extended by scaling laws. Ultimately, an energy price model using real-life data from the ENTSO-E platform maintained by the association of EU transmission system operators was used to estimate the revenue profile. This framework was then used to compare the performance of systems based on multiple economic metrics within a chosen design space. The simulation results confirmed the expected behaviour that the electricity produced at lower wind speeds has a higher value than that produced at higher wind speeds. To account for this electricity price dependency on wind speeds in the design process, we propose an economic metric defined as the levelised profit of energy (LPoE). This approach determines the trade-offs between designing a system that minimises cost and designing a system that maximises value.