Use of the Intermittent Abdominal Pressure Ventilation to guarantee speech in a tracheostomized Amyotrophic Lateral Sclerosis patient

n/

Anti-calcitonin gene-related peptide monoclonal antibodies for the treatment of vestibular migraine: A prospective observational cohort study

Background: Vestibular migraine is considered the most common cause of recurrent vertigo for which specific treatments are missing. Monoclonal antibodies against calcitonin gene-related peptide,, are effective in preventing migraine. Since CGRP is also detected in human cochlear and vestibular organs it may also play a role in vestibular physiology. Methods: This is a prospective observational cohort study, aiming at evaluating the efficacy of erenumab, fremanezumab or galcanezumab for the treatment of fifty vestibular migraine patients. We assessed mean monthly days with headache and dizziness/vestibular symptoms, pain intensity and migraine-related clinical burden occurring for 18 months. Results: Response to treatment was excellent as 45 (90%) patients had at least a 50% reduction in vertigo frequency, 43 (86%) had at least a 50% reduction in headache frequency, and 40 (80%) a MIDAS reduction of at least 50%. Overall, 39 (78%) patients had a concomitant reduction of all three parameters. Mean monthly days with dizziness/vestibular symptoms showed an overall significant decrease from a mean of 10.3 ± 1.9 at baseline to 0.8 ± 0.3 days, difference 9.5 (CI 95% 3.6, 15.4; p < 0.001) after twelve months. Conclusion: We show that anti-CGRP mAbs may be effective in the treatment of Vestibular Migraine. Their use should be encouraged early in the disease course to allow for a better symptom control and quality of life improvement

Burning Mouth Syndrome and Hypertension: Prevalence, Gender Differences and Association with Pain and Psycho-Social Characteristics—A Case Control Study

Background: To assess the prevalence of hypertension (HTN) in burning mouth syndrome (BMS) patients and to investigate its relationship with sociodemographic factors, pain and the psychological profile. Methods: A case-control study was conducted by enrolling 242 BMS patients and 242 controls matched for age and gender. Sociodemographic and clinical characteristics were recorded, and all participants completed numeric rating scale (NRS), the short-form of the McGill pain questionnaire (SF-MPQ), the Hamilton rating scale for anxiety and depression (HAM-A, HAM-D), the Pittsburgh sleep quality index (PSQI) and the Epworth sleepiness scale (ESS). Results: The BMS patients presented with a statistically significant higher prevalence of HTN compared to that in the controls (55% versus 33.5%; p-value: <0.001) and higher median scores of the NRS, SF-MPQ, HAM-A, HAM-D, PSQI and ESS (p < 0.001). Multivariate regression analysis in the BMS patients indicated positive correlations between HTN and age, systemic diseases, drug consumption and anxiety (p-value: <0.001) and these predictors were responsible for 11.3% of the HTN variance in the BMS patients, when considered together. Conclusions: The prevalence of HTN was significantly higher in the BMS patients, since ageing, the presence of comorbidities, drug consumption and anxiety were potential predictors. Further studies are needed to better investigate the relationship between BMS and HTN

Hydrophilic 1,10-phenanthroline derivatives for selective Am(III) stripping into aqueous solutions

The novel and fully combustible hydrophilic 1,10-phenanthroline-2,9-dicarboxamide (1) was synthesized and investigated as Am(III) stripping agent in a simulated advanced hydrometallurgical process, in comparison with two other 1,10-phenanthroline-based ligands 2 and 3. The stripping efficiency and the Am(III)/lanthanides(III) selectivity of the TODGA (org)/phen-derivative (aq) extracting system were studied under several experimental conditions by liquid–liquid extraction tests. The results obtained clarify the main limitations of these ligands in the scope of the hydrometallurgical reprocessing but also enable to get indications to steer future investigations in the domain of the selective An(III) recovery for the advanced reprocessing of Spent Nuclear Fuel by hydrophilic ligands

Consensus-Based Care Recommendations for Pulmonologists Treating Adults with Myotonic Dystrophy Type 1

Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract 2018;8(6):507-20]. In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death in this patient population, followed by cardiac complications [de Die-Smulders et al.: Brain 1998;121(Pt 8):1557-63], [Mathieu et al.: Neurology 1999;52(8):1658-62], [Groh et al.: Muscle Nerve 2011;43(5):648-51]. This paper provides a more detailed outline on the diagnostic and management protocols, which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists discussed respiratory testing and management at baseline and during follow-up visits, based on their clinical experience with patients with DM1. The details are presented in this report. Recent Findings: Myotonic recruited 66 international clinicians experienced in the treatment of people living with DM1 to develop and publish consensus-based care recommendations targeting all body systems affected by this disease [Ashizawa et al.: Neurol Clin Pract. 2018;8(6):507-20]. Myotonic then worked with 12 international respiratory therapists, pulmonologists and neurologists with long-standing experience in DM respiratory care to develop consensus-based care recommendations for pulmonologists using a methodology called the Single Text Procedure. This process generated a 7-page document that provides detailed respiratory care recommendations for the management of patients living with DM1. This consensus is completely based on expert opinion and not backed up by empirical evidence due to limited clinical care data available for respiratory care management in DM patients. Nevertheless, we believe it is of relevance for professionals treating adults with myotonic dystrophy because it addresses practical issues related to respiratory management and care, which have been adapted to meet the specific issues in patients with DM1. Summary: The resulting recommendations are intended to improve respiratory care for the most vulnerable of DM1 patients and lower the risk of untoward respiratory complications and mortality by providing pulmonologist who are less experienced with DM1 with practical indications on which tests and when to perform them, adapting the general respiratory knowledge to specific issues related to this multiorgan disease

Real and perceived feet orientation under fatiguing and non-fatiguing conditions in an immersive virtual reality environment

Lower limbs position sense is a complex yet poorly understood mechanism, influenced by many factors. Hence, we investigated the position sense of lower limbs through feet orientation with the use of Immersive Virtual Reality (IVR). Participants had to indicate how they perceived the real orientation of their feet by orientating a virtual representation of the feet that was shown in an IVR scenario. We calculated the angle between the two virtual feet (α-VR) after a high-knee step-in-place task. Simultaneously, we recorded the real angle between the two feet (α-R) (T1). Hence, we assessed whether the acute fatigue impacted the position sense. The same procedure was repeated after inducing muscle fatigue (T2) and after 10 min from T2 (T3). Finally, we also recorded the time needed to confirm the perceived position before and after the acute fatigue protocol. Thirty healthy adults (27.5 ± 3.8: 57% women, 43% men) were immersed in an IVR scenario with a representation of two feet. We found a mean difference between α-VR and α-R of 20.89° [95% CI: 14.67°, 27.10°] in T1, 16.76° [9.57°, 23.94°] in T2, and 16.34° [10.00°, 22.68°] in T3. Participants spent 12.59, 17.50 and 17.95 s confirming the perceived position of their feet at T1, T2, T3, respectively. Participants indicated their feet as forwarding parallel though divergent, showing a mismatch in the perceived position of feet. Fatigue seemed not to have an impact on position sense but delayed the time to accomplish this task

Consensus-based care recommendations for adults with myotonic dystrophy type 1

Purpose of review Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. Summary The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments. Described as “one of the more variable diseases found in medicine,” myotonic dystrophy type 1 (DM1) is an autosomal dominant, triplet-repeat expansion disorder that affects somewhere between 1:3,000 and 1:8,000 individuals worldwide.1 There is a modest association between increased repeat expansion and disease severity, as evidenced by the average age of onset and overall morbidity of the condition. An expansion of over 35 repeats typically indicates an unstable and expanding mutation. An expansion of 50 repeats or higher is consistent with a diagnosis of DM1. DM1 is a multisystem and heterogeneous disease characterized by distal weakness, atrophy, and myotonia, as well as symptoms in the heart, brain, gastrointestinal tract, endocrine, and respiratory systems. Symptoms may occur at any age. The severity of the condition varies widely among affected individuals, even among members of the same family. Comprehensive evidence-based guidelines do not currently exist to guide the treatment of DM1 patients. As a result, the international patient community reports varied levels of care and care quality, and difficulty accessing care adequate to manage their symptoms, unless they have access to multidisciplinary neuromuscular clinics. Consensus-based care recommendations can help standardize and improve the quality of care received by DM1 patients and assist clinicians who may not be familiar with the significant variability, range of symptoms, and severity of the disease. Care recommendations can also improve the landscape for clinical trial success by eliminating some of the inconsistencies in patient care to allow more accurate understanding of the benefit of potential therapies

An Intelligent Conversational Agent for the Legal Domain

An intelligent conversational agent for the legal domain is an AI-powered system that can communicate with users in natural language and provide legal advice or assistance. In this paper, we present CREA2, an agent designed to process legal concepts and be able to guide users on legal matters. The conversational agent can help users navigate legal procedures, understand legal jargon, and provide recommendations for legal action. The agent can also give suggestions helpful in drafting legal documents, such as contracts, leases, and notices. Additionally, conversational agents can help reduce the workload of legal professionals by handling routine legal tasks. CREA2, in particular, will guide the user in resolving disputes between people residing within the European Union, proposing solutions in controversies between two or more people who are contending over assets in a divorce, an inheritance, or the division of a company. The conversational agent can later be accessed through various channels, including messaging platforms, websites, and mobile applications. This paper presents a retrieval system that evaluates the similarity between a user’s query and a given question. The system uses natural language processing (NLP) algorithms to interpret user input and associate responses by addressing the problem as a semantic search similar question retrieval. Although a common approach to question and answer (Q&A) retrieval is to create labelled Q&A pairs for training, we exploit an unsupervised information retrieval system in order to evaluate the similarity degree between a given query and a set of questions contained in the knowledge base. We used the recently proposed SBERT model for the evaluation of relevance. In the paper, we illustrate the effective design principles, the implemented details and the results of the conversational system and describe the experimental campaign carried out on it