Reappraisal of the anatomical spreading and propagation hypothesis about TDP-43 aggregation in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.

Neuronal inclusion of transactivation response DNA-binding protein 43 kDa (TDP-43) is known to be a pathologic hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). TDP-43, which is physiologically a nuclear protein, is mislocalized from the nucleus and aggregated within the cytoplasm of affected neurons in ALS and FTLD patients. Neuropathologic or experimental studies have addressed mechanisms underlying spreading of TDP-43 inclusions in the central nervous system of ALS and FTLD patients.

Topical rapamycin versus betamethasone dipropionate ointment for treating oral erosive lichen planus: a randomized, double-blind, controlled study.

Background: Although superpotent topical corticosteroids are the first-line treatment for oral erosive lichen planus (OELP), topical rapamycin was found efficient in a previous case series.

Objectives: To compare the efficacy and safety of topical rapamycin and betamethasone dipropionate ointment for OELP in a randomized, double-blind trial.

Methods: Patients were randomized to receive treatment with betamethasone dipropionate ointment 0.

Gas Exchange and Ventilatory Efficiency During Exercise in Pulmonary Vascular Diseases.

Background And Objective: Ventilatory inefficiency (high V'/V'CO) and resting hypocapnia are common in pulmonary vascular disease and are associated with poor prognosis. Low resting PaCO suggests increased chemosensitivity or an altered PaCO set-point. We aimed to determine the relationships between exercise gas exchange variables reflecting the PaCO set-point, exercise capacity, hemodynamics and V'/V'CO.

Association of Low Baseline Diaphragm Muscle Mass With Prolonged Mechanical Ventilation and Mortality Among Critically Ill Adults.

Importance: Low diaphragm muscle mass at the outset of mechanical ventilation may predispose critically ill patients to poor clinical outcomes.

Objective: To determine whether lower baseline diaphragm thickness (Tdi) is associated with delayed liberation from mechanical ventilation and complications of acute respiratory failure (reintubation, tracheostomy, prolonged ventilation >14 days, or death in the hospital).

Design, Setting, And Participants: Secondary analysis (July 2018 to June 2019) of a prospective cohort study (data collected May 2013 to January 2016).

18F-FDOPA PET/CT Findings in a Patient With Primary Cerebral Amyloidoma.

Amino acid PET, including F-FDOPA, is recommended for initial characterization, delineation of tumor extent, and follow-up of gliomas because of its high diagnostic performances. F-FDOPA accumulates inside tumor cells via the L-type amino acid transporter 1 (LAT1) whose expression is increased in gliomas. We report here a case of a histopathologically proven brain amyloidoma that was first addressed for a suspected glioma.

Dose distribution of the brain tissue associated with cognitive functions in high-grade glioma patients.

Purpose: The purpose of this prospective dosimetric study was to assess the dose distribution regarding the brain areas implied in cognitive functions using two approaches: volumetric modulated arc therapy (VMAT) and helical tomotherapy (HT).

Patients And Methods: Thirty-seven patients were treated using a dual-arc VMAT approach for supratentorial glioblastoma between 2016 and 2018. The total dose of 60Gy in 30 daily fractions was administered to the planning target volume (PTV).

Monitoring diaphragm function in the ICU.

Purpose Of Review: To review the clinical problem of diaphragm function in critically ill patients and describes recent advances in bedside monitoring of diaphragm function.

Recent Findings: Diaphragm weakness, a consequence of diaphragm dysfunction and atrophy, is common in the ICU and associated with serious clinical consequences. The use of ultrasound to assess diaphragm structure (thickness, thickening) and mobility (caudal displacement) appears to be feasible and reproducible, but no large-scale 'real-life' study is available.

ERS statement on standardisation of cardiopulmonary exercise testing in chronic lung diseases.

The objective of this document was to standardise published cardiopulmonary exercise testing (CPET) protocols for improved interpretation in clinical settings and multicentre research projects. This document: 1) summarises the protocols and procedures used in published studies focusing on incremental CPET in chronic lung conditions; 2) presents standard incremental protocols for CPET on a stationary cycle ergometer and a treadmill; and 3) provides patients' perspectives on CPET obtained through an online survey supported by the European Lung Foundation. We systematically reviewed published studies obtained from EMBASE, Medline, Scopus, Web of Science and the Cochrane Library from inception to January 2017.

Evening sock marks as an adjunct to the clinical prediction of obstructive sleep apnea.

Study Objectives: Fluid overload shifting from the legs to the upper airway during sleep promotes obstructive sleep apnea (OSA) and interventions targeting fluid attenuate OSA. Fluid shift has been previously measured by bioelectrical impedance, a complex and time-consuming technique not applicable in the daily clinical settings. The aim of this study is to evaluate the presence of clinically detectable fluid overload and shift and its association with OSA.

Impact of Sleep Deprivation on Respiratory Motor Output and Endurance. A Physiological Study.

Sleep deprivation can alter endurance of skeletal muscles, but its impact on respiratory command is unknown. We aimed to assess the effect of sleep deprivation on respiratory motor output and inspiratory endurance. Inspiratory endurance was investigated twice in random order, following a normal sleep night and a sleepless night.

"I had the feeling that I was trapped": a bedside qualitative study of cognitive and affective attitudes toward noninvasive ventilation in patients with acute respiratory failure.

Background: Noninvasive ventilation (NIV) is the application of mechanical ventilation through a mask. It is used to treat certain forms of acute respiratory failure in intensive care units (ICU). NIV has clinical benefits but can be anxiogenic for the patients.

Intensity and quality of exertional dyspnoea in patients with stable pulmonary hypertension.

Dynamic hyperinflation is observed during exercise in 60% of patients with clinically stable pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), intensifying exertional dyspnoea. The impact of dynamic changes in respiratory mechanics during exercise on qualitative dimensions of dyspnoea in these patients has not been evaluated.26 patients (PAH n=17; CTEPH n=9) performed an incremental symptom-limited cycle exercise test.

Respective contribution of intensive care unit-acquired limb muscle and severe diaphragm weakness on weaning outcome and mortality: a post hoc analysis of two cohorts.

Background: Intensive care unit (ICU)-acquired weakness (ICU-AW) and ICU-acquired diaphragm dysfunction (ICU-DD) occur frequently in mechanically ventilated (MV) patients. It is unknown whether they have different risk factors and different impacts on outcome. This study was designed to (1) describe the respective risk factors associated with ICU-AW and severe ICU-DD and (2) evaluate the respective impact of ICU-AW and severe ICU-DD on outcome.

Direct transport vs secondary transfer to level I trauma centers in a French exclusive trauma system: Impact on mortality and determinants of triage on road-traffic victims.

Background: Transporting a severely injured patient directly to a trauma center (TC) is consensually considered optimal. Nevertheless, disagreement persists regarding the association between secondary transfer status and outcome. The aim of the study was to compare adjusted mortality between road traffic trauma patients directly or secondarily transported to a level 1 trauma center (TC) in an exclusive French trauma system with a physician staffed prehospital emergency medical system (EMS).

ZTE MR-based attenuation correction in brain FDG-PET/MR: performance in patients with cognitive impairment.

Objective: One of the main challenges of integrated PET/MR is to achieve an accurate PET attenuation correction (AC), especially in brain acquisition. Here, we evaluated an AC method based on zero echo time (ZTE) MRI, comparing it with the single-atlas AC method and CT-based AC, set as reference.

Methods: Fifty patients (70 ± 11 years old, 28 men) underwent FDG-PET/MR examination (SIGNA PET/MR 3.

Postural preinspiratory cortical activity, genioglossus activity and fluid shift in awake obstructive sleep apnoea patients.

New Finding: What is the central question of this study? Transition to supine posture induces an inspiratory load associated with cortical activation in awake healthy subjects. Some obstructive sleep apnoea patients exhibit this cortical activity in the sitting position contributing to the arousal-dependent compensation of their upper airway abnormalities. Does a transition to the supine posture in awake obstructive sleep apnoea patients increase this cortical activity? What is the main finding and its importance? The transition to supine posture induces a reduction in the cortical activity despite evidence of an increase in genioglossus activity, suggesting that genioglossus activation is not driven by cortical activity.

The level of activity of the alternative lengthening of telomeres correlates with patient age in IDH-mutant ATRX-loss-of-expression anaplastic astrocytomas.

All cancer cells need to maintain functional telomeres to sustain continuous cell division and proliferation. In human diffuse gliomas, functional telomeres are maintained due either to reactivation of telomerase expression, the main pathway in most cancer types, or to activation of a mechanism called the alternative lengthening of telomeres (ALT). The presence of IDH1/2 mutations (IDH-mutant) together with loss of ATRX expression (ATRX-lost) are frequently associated with ALT in diffuse gliomas.

Precision medicine is coming to town: personalising home ventilatory equipment in COPD patients with chronic hypercapnic respiratory failure.

Discharging a chronic critically ill patient is a risky procedure if the clinician does not have full control of his prescription. This is even more important when applying a machine to replace a failing organ, as is the case for home ventilation. Even if modern home ventilators fulfil quality and safety criteria and, 'on paper', ventilators and masks look very similar, performance and scenarios of applicability are not always equivalent.

Multidisciplinary advisory teams to manage multidrug-resistant tuberculosis: the example of the French Consilium.

The World Health Organization (WHO) recommends that multidrug-resistant tuberculosis (MDR-TB) treatment should be managed in collaboration with multidisciplinary advisory committees (consilia). A formal national Consilium has been established in France since 2005 to provide a centralised advisory service for clinicians managing MDR-TB and extensively drug-resistant (XDR-TB) cases. Review the activity of the French TB Consilium since its establishment.