Cognitive impairment in ALS patients and validation of the Spanish version of the ALS-CBS test

Our aim was to develop and validate the Spanish version of the Amyotrophic Lateral Sclerosis Cognitive Behavioural Screen (ALS-CBS) and investigate behavioural/cognitive impairment in our ALS patients. We enrolled 50 patients with definite or probable ALS, evaluated by the Motor Neuron Disease Unit (using El Escorial criteria) and Dementia Unit, and assessed with the Spanish ALS-CBS. The patients' cognitive/behavioural status was classified according to current criteria. Patients were classified into each diagnostic category: ALS-no impairment, 36%; ALS-mild cognitive impairment, 34%; ALS-mild behavioural impairment, 6%; ALS-mild cognitive/behavioural impairment, 12%; ALS-frontotemporal dementia, 12%. Cognitive impairment was more common in bulbar (90.9%) than spinal (48.7%) forms (p < 0.012). The Spanish ALS-CBS was validated. Performance to differentiate normal vs. impaired individuals was: 1) cognition (cut-off 15; AUC, 84.7%): sensitivity 86.2%, specificity 62%, positive predictive value 75.8%, negative predictive value 76.5%; 2) behaviour (cut-off 36; AUC, 83.3%): sensitivity 93.3%, specificity 74.3%, positive predictive value 61%, negative predictive value 96.3%. Performance to differentiate between patients with and without dementia: 1) cognition (cut-off 8; AUC, 87.3%): sensitivity 83.3%, specificity 75%, positive predictive value 31.3%, negative predictive value 97.1%; 2) behaviour (cut-off 35; AUC, 80.9%): sensitivity 83.3%, specificity 69%, positive predictive value 25%, negative predictive value 96.7%. In conclusion, cognitive impairment is common in ALS patients, particularly in bulbar forms. The Spanish version of the ALS-CBS is useful for screening cognitive/behavioural impairment in this population

Survival benefit of multidisciplinary care in amyotrophic lateral sclerosis in Spain: association with noninvasive mechanical ventilation

Purpose: Multidisciplinary care has become the preferred model of care for patients with amyotrophic lateral sclerosis (ALS). It is assumed that the sum of interventions associated with this approach has a positive effect on survival. The objective of the study was to evaluate the impact of a multidisciplinary care approach on the survival of patients with ALS. Patients and methods: We performed a retrospective review of prospectively collected data in a tertiary referral center in Spain. Participants were patients with definite or probable ALS managed in a multidisciplinary care program. We compared demographic and survival data of patients with definite or probable ALS treated in a referral center without and with implementation of a multidisciplinary care program. We performed time-dependent multivariate survival analysis of the use of noninvasive mechanical ventilation (NIMV) and gastrostomy. Results: We evaluated 398 consecutive patients, of whom 54 were treated by a general neurologist and 344 were treated in the multidisciplinary care clinic. Patients receiving multidisciplinary care were older (62 vs 58 years), tended to have bulbar onset disease (30% vs 17.7%), and were more likely to receive riluzole (88.7% vs 29.6%, p0.001), and nutrition via gastrostomy (32.3% vs 3.7%, p<0.01). Kaplan-Meier analysis showed a 6-month increase in survival (log-rank, 16.03, p<0.001). Application of the Andersen-Gill model showed that the variables associated with reduced mortality were reduced time to NIMV and gastrostomy and the duration of both, thus reflecting compliance. Conclusions: Multidisciplinary care increased the survival of ALS patients in our study population. Timely use of respiratory support and gastrostomy are fundamental aspects of this benefit

Review of Financing Mechanisms to Promote Decarbonization Alternatives in Rail and Inland Waterway Transport

The financial market has a strong influence on supporting the transition to a zero-emission transport system, as this sector requires large investments to implement low-emission technologies and infrastructure and to promote intermodality. This article presents the results obtained from a literature review on the financing mechanisms that have been used in different countries to finance alternatives to decarbonization in rail and inland waterway modes. For this purpose, databases such as Google Scholar, Scopus, MDPI, websites of governments and other related organizations were reviewed. Over 240 sources of information from articles and documents focusing on green finance in rail and inland waterway modes were reviewed. Our study identified many gaps in the literature on green transport financing. We also identified that in the databases consulted, it was possible to obtain more academic sources related to rail transport than inland waterway transport. Finally, an analysis is made of how implementation projects in the rail and inland waterway modes have been financed in Colombia

Industry 4.0 Technologies Applied to Inland Waterway Transport: Systematic Literature Review

The focus of this article is inland waterway transport. Different problems in this domain have been studied due to the increase in waterway traffic globally. Industry 4.0 technologies have become an alternative for the possible solution of these problems. For this reason, this paper aims to answer the following research questions: (1) What are the main problems in transporting cargo by inland waterway? (2) What technological strategies are being studied to solve these problems? (3) What technologies from Industry 4.0 are used within the technological strategies to solve the exposed problems? This study adopts a Systematic Literature Review (SLR) approach. For this work, were recovered 645 articles, 88 of which were eligible, from which we could identify five domains corresponding to (1) traffic monitoring, (2) smart navigation, (3) emission reduction, (4) analytics with big data, and (5) cybersecurity. The strategies currently being considered combine navigation technologies, such as AIS (Automatic Identification System), which offers a large amount of data, with Industry 4.0 tools and mainly machine learning techniques, to take advantage of data collected over a long time. This study is, to our knowledge, one of the first to show how Industry 4.0 technologies are currently being used to tackle inland waterway transport problems and current application trends in the scientific community, which is a first step for the development of future studies and more advanced solutions

A Review of Economic Incentives to Promote Decarbonization Alternatives in Maritime and Inland Waterway Transport Modes

Public policies and economic incentives are widely used as a strategy to stimulate the use of green technologies and low-emission practices in the waterborne transport sector. Since the Paris Agreement, countries have been encouraged to implement more strategies to reduce greenhouse gas emissions and to build resilience against climate change impacts in developing countries. This article presents a literature review on policies, regulations, and programs that represent economic incentives to promote alternatives to decarbonize maritime and inland waterway transport in sixteen countries, including Colombia. More than one hundred thirty sources of information were reviewed, including official portals of governments, port authorities and organizations, and scientific articles; therefore, the incentives found were grouped into three categories: project financing, differentiated port tariffs, and incentives to cover onshore power service fees. As a result of this review, it was found that differentiated port tariffs were the most common type of incentive. Finally, the specific case of Colombia was analyzed, which provides a deeper perspective of current policies and measures aimed at encouraging the decarbonization of waterborne transport and compares them with the international panorama

A Review of Economic Incentives to Promote Decarbonization Alternatives in Maritime and Inland Waterway Transport Modes

Public policies and economic incentives are widely used as a strategy to stimulate the use of green technologies and low-emission practices in the waterborne transport sector. Since the Paris Agreement, countries have been encouraged to implement more strategies to reduce greenhouse gas emissions and to build resilience against climate change impacts in developing countries. This article presents a literature review on policies, regulations, and programs that represent economic incentives to promote alternatives to decarbonize maritime and inland waterway transport in sixteen countries, including Colombia. More than one hundred thirty sources of information were reviewed, including official portals of governments, port authorities and organizations, and scientific articles; therefore, the incentives found were grouped into three categories: project financing, differentiated port tariffs, and incentives to cover onshore power service fees. As a result of this review, it was found that differentiated port tariffs were the most common type of incentive. Finally, the specific case of Colombia was analyzed, which provides a deeper perspective of current policies and measures aimed at encouraging the decarbonization of waterborne transport and compares them with the international panorama

Masitinib as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomized clinical trial

Objective: To assess masitinib in the treatment of ALS. Methods: Double-blind study, randomly assigning 394 patients (1:1:1) to receive riluzole (100 mg/d) plus placebo or masitinib at 4.5 or 3.0 mg/kg/d. Following a blinded transition from phase 2 to phase 2/3, a prospectively defined two-tiered design was implemented based on ALSFRS-R progression rate from disease-onset to baseline (ΔFS). This approach selects a more homogeneous primary efficacy population (“Normal Progressors”, ΔFS &lt; 1.1 points/month) while concurrently permitting secondary assessment of the broader population. Primary endpoint was decline in ALSFRS-R at week-48 (ΔALSFRS-R), with the high-dose “Normal Progressor” cohort being the prospectively declared primary efficacy population. Missing data were imputed via last observation carried forward (LOCF) methodology with sensitivity analyses performed to test robustness. Results: For the primary efficacy population, masitinib (n = 99) showed significant benefit over placebo (n = 102) with a ΔALSFRS-R between-group difference (ΔLSM) of 3.4 (95% CI 0.65–6.13; p = 0.016), corresponding to a 27% slowing in rate of functional decline (LOCF methodology). Sensitivity analyses were all convergent, including the conservative multiple imputation technique of FCS-REGPMM with a ΔLSM of 3.4 (95% CI 0.53–6.33; p = 0.020). Secondary endpoints (ALSAQ-40, FVC, and time-to-event analysis) were also significant. Conversely, no significant treatment-effect according to ΔALSFRS-R was seen for the broader “Normal and Fast Progressor” masitinib 4.5 mg/kg/d cohort, or either of the low-dose (masitinib 3.0 mg/kg/d) cohorts. Rates of treatment-emergent adverse events (AEs) (regardless of causality or post-onset ΔFS) were 88% with masitinib 4.5 mg/kg/d, 85% with 3.0 mg/kg/d, and 79% with placebo. Likewise, rates of serious AE were 31, 23, and 18%, respectively. No distinct event contributed to the higher rate observed for masitinib and no deaths were related to masitinib. Conclusions: Results show that masitinib at 4.5 mg/kg/d can benefit patients with ALS. A confirmatory phase 3 study will be initiated to substantiate these data