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Diaphragm neurostimulation consists of placing electrodes directly on or in proximity to the phrenic nerve(s) to elicit diaphragmatic contractions. Since its initial description in the 18th century, indications have shifted from cardiopulmonary resuscitation to long-term ventilatory support. Recently, the technical development of devices for temporary diaphragm neurostimulation has opened up the possibility of a new era for the management of mechanically ventilated patients. Combining positive pressure ventilation with diaphragm neurostimulation offers a potentially promising new approach to the delivery of mechanical ventilation which may benefit multiple organ systems. Maintaining diaphragm contractions during ventilation may attenuate diaphragm atrophy and accelerate weaning from mechanical ventilation. Preventing atelectasis and preserving lung volume can reduce lung stress and strain and improve homogeneity of ventilation, potentially mitigating ventilator-induced lung injury. Furthermore, restoring the thoracoabdominal pressure gradient generated by diaphragm contractions may attenuate the drop in cardiac output induced by positive pressure ventilation. Experimental evidence suggests diaphragm neurostimulation may prevent neuroinflammation associated with mechanical ventilation. This review describes the historical development and evolving approaches to diaphragm neurostimulation during mechanical ventilation and surveys the potential mechanisms of benefit. The review proposes a research agenda and offers perspectives for the future of diaphragm neurostimulation assisted mechanical ventilation for critically ill patients. [ABSTRACT FROM AUTHOR]

Non-invasive ventilation (NIV) is commonly used at home for patient with nocturnal hypoventilation caused by a chronic respiratory failure. Monitoring NIV is required to optimize the ventilator settings when the lung condition changes over time, and to detect common problems such as unintentional leaks, upper airway obstructions, and patient–ventilator asynchronies. This review describes the accuracy and limitations of the data recorded by the ventilator. To efficiently interpret this huge amount of data, clinician assess the daily use and regularity of NIV utilization, the unintentional leaks and their repartition along the NIV session, the apnea–hypopnea index and the flow waveform, and the patient–ventilator synchrony. Nocturnal recordings of gas exchanges are also required to detect nocturnal alveolar hypoventilation. This review describes the indication, validity criteria, and interpretation of nocturnal oximetry and transcutaneous capnography. Polygraphy and polysomnography are indicated in specific cases to characterize upper airway obstruction. Telemonitoring of the ventilator is a useful tool that should be integrated in the monitoring strategy. The technical solution, information, and limitations are discussed. In conclusion, a basic monitoring package is recommended for all patients complemented by advanced monitoring for specific cases. [ABSTRACT FROM AUTHOR]

Background: More than a symptom, dyspnoea is an existential experience shaping the lives of those afflicted, particularly when its persistence despite maximal pathophysiological treatments makes it pervasive. It is, however, insufficiently appreciated by concerned people themselves, family members, healthcare professionals and the public (dyspnoea invisibility), limiting access to appropriate care and support. Aim: To provide a better understanding of dyspnoea experiences and its invisibility. Design: Interpretative phenomenological analysis of data collected prospectively through in-depth semi-structured interviews. Setting/Participants: Pulmonary rehabilitation facility of a tertiary care university hospital; 11 people (six men, five women) with severe chronic obstructive pulmonary disease (stages 3 and 4 of the 4-stage international GOLD classification) admitted for immediate post-exacerbation rehabilitation. Results: We identified several types of dyspnoea invisibility depending on temporality and interlocutors: (1) invisibility as a symptom to oneself; (2) invisibility as a symptom to others; (3) invisibility as an experience that cannot be shared; (4) invisibility as an experience detached from objective measurements; (5) invisibility as an experience that does not generate empathic concern. The notion of invisibility was present in all the identified experiential dimensions of dyspnoea. It was seen as worsening the burden of the disease and as self-aggravating through self-isolation and self-censorship. Conclusions: The study confirmed that dyspnoea invisibility is a reality for people with advanced chronic obstructive pulmonary disease. It shows dyspnoea invisibility to be a multifaceted burden. Future research should aim at identifying individual and collective measures to overcome dyspnoea invisibility. [ABSTRACT FROM AUTHOR]

Objective: The coronavirus disease pandemic (COVID-19) increased the risk of shortage in intensive care devices, including fittings with intentional leaks. 3D-printing has been used worldwide to produce missing devices. Here we provide key elements towards better quality control of 3D-printed ventilation fittings in a context of sanitary crisis. Material and methods: Five 3D-printed designs were assessed for non-intentional (junctional and parietal) and intentional leaks: 4 fittings 3D-printed in-house using FDeposition Modelling (FDM), 1 FDM 3D-printed fitting provided by an independent maker, and 2 fittings 3D-printed in-house using Polyjet technology. Five industrial models were included as controls. Two values of wall thickness and the use of coating were tested for in-house FDM-printed devices. Results: Industrial and Polyjet-printed fittings had no parietal and junctional leaks, and satisfactory intentional leaks. In-house FDM-printed fittings had constant parietal leaks without coating, but this post-treatment method was efficient in controlling parietal sealing, even in devices with thinner walls (0.7 mm vs 2.3 mm). Nevertheless, the use of coating systematically induced absent or insufficient intentional leaks. Junctional leaks were constant with FDM-printed fittings but could be controlled using rubber junctions rather than usual rigid junctions. The properties of Polyjet-printed and FDM-printed fittings were stable over a period of 18 months. Conclusions: 3D-printing is a valid technology to produce ventilation devices but requires care in the choice of printing methods, raw materials, and post-treatment procedures. Even in a context of sanitary crisis, devices produced outside hospitals should be used only after professional quality control, with precise data available on printing protocols. The mechanical properties of ventilation devices are crucial for efficient ventilation, avoiding rebreathing of CO2, and preventing the dispersion of viral particles that can contaminate health professionals. Specific norms are still required to formalise quality control procedures for ventilation fittings, with the rise of 3D-printing initiatives and the perspective of new pandemics. [ABSTRACT FROM AUTHOR]

The article presents an analysis of early diaphragm pacing in patients with amyotrophic lateral sclerosis (ALS) to use histopathological and electrophysiological data to test the hypothesis that diaphragm pacing. It mentions decrease in survival observed with active stimulation was associated with an accelerated deterioration of lung function. it also mentions muscle electrical activation after experimental nerve injury can impair reinnervation and exacerbate atrophy.

Background: Combined effects of leaks, mechanical property of respiratory system and upper airway (UA) patency on patient-ventilator synchrony (PVA) and the level of clinically "tolerable" leaks are not well established in home ventilators.Methods: We comparatively assessed on a bench model, the highest leak level tolerated without inducing significant asynchrony ("critical leak") in three home ventilators (Astral 150, Trilogy 100 and Vivo 60; noted as A150, T100 and V60 respectively) subjected to three simulated diseased respiratory conditions: chronic obstructive pulmonary disease (COPD), obesity hypoventilation (OHS) and neuromuscular disorders (NMD), with both open and closed UA. Also, total leak values in the device reports were compared to the bench-measured values.Results: With open UA, all ventilators were able to avoid asynchrony up to a 30 L/min leak and even to 55 L/min in some cases. UA closure and respiratory diseases especially OHS influenced PVA. With closed UA, the critical leak of A150 and T100 remained higher than 55 L/min in COPD and OHS, while for V60 decreased to 41 and 33 L/min respectively. In NMD with closed UA, only T100 reached a high critical leak of 69 L/min. Besides, inspiratory trigger sensitivity change was often necessary to avoid PVA.Conclusions: Home ventilators were able to avoid PVA in high-level leak conditions. However, asynchrony appeared in cases of abnormal mechanical properties of respiratory system or closed UA. In case of closed UA, the EPAP should be adjusted prior to the inspiratory trigger.Trial Registration: Not applicable. [ABSTRACT FROM AUTHOR]

Respiratory work is physiologically increased during sleep and leads to severe alterations in COPD patients, especially by raising sleep hypoventilation. The diurnal impact of these nocturnal events may have been underestimated in COPD patients. Impaired sleep and the increase of respiratory work may be one of the major trigger of diurnal events like hypoventilation, exacerbation and even mortality. One of the most commonly used nocturnal treatments at the present time is noninvasive ventilation (NIV). However, there is an on-going debate concerning the indications and objectives of NIV in COPD patients. In most studies, NIV initiation and monitoring depend on diurnal tools like PaCO2, and the nocturnal efficacy of this treatment has not yet been adequately determined. In other respiratory diseases, sleep events have a predominant role in NIV therapy. Such nocturnal events drive NIV initiation and setting adaptation. Monitoring of sleep events is associated with an increase in health related to quality of life and a decrease in mortality. The monitoring may be the solution to solve the debate of NIV in COPD patients. This article reviews the impact of sleep in COPD patients and the value of long-term NIV. [ABSTRACT FROM AUTHOR]

In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness leads to respiratory failure. Non-invasive ventilation (NIV) maintains adequate ventilation in ALS patients. NIV alleviates symptoms and improves survival. In 2006, French guidelines established criteria for NIV initiation based on limited evidence. Their impact on clinical practice remains unknown. Our objective was to describe NIV initiation practices of the main French ALS tertiary referral centre with respect to guidelines. In this retrospective descriptive study, 624 patients followed in a single national reference centre began NIV between 2005 and 2013. We analysed criteria used to initiate NIV, including symptoms, PaCO2, forced vital capacity, maximal inspiratory pressures and time spent with SpO2<90% at night. At NIV initiation, 90% of patients were symptomatic. Median PaCO2was 48 mmHg. The main criterion to initiate NIV was ‘symptoms’ followed by ‘hypercapnia’ in 42% and 34% of cases, respectively. NIV was initiated on functional parameters in only 5% of cases. Guidelines were followed in 81% of cases. In conclusion, despite compliance with French guidelines, the majority of patients are treated at the stage of symptomatic daytime hypoventilation, which suggests that NIV is initiated late in the course of ALS. Whether this practice could be improved by changing guidelines or increasing respiratory-dedicated resources remains to be determined. [ABSTRACT FROM AUTHOR]

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure. Non-invasive ventilation alleviates respiratory symptoms and prolongs life, but is a palliative intervention. Slowing the deterioration of diaphragm function before respiratory failure would be desirable. We aimed to assess whether early diaphragm pacing could slow down diaphragm deterioration and would therefore delay the need for non-invasive ventilation.Methods: We did a multicentre, randomised, controlled, triple-blind trial in patients with probable or definite ALS in 12 ALS centres in France. The main inclusion criterion was moderate respiratory involvement (forced vital capacity 60-80% predicted). Other key eligibility criteria were age older than 18 years and bilateral responses of the diaphragm to diagnostic phrenic stimulation. All patients were operated laparoscopically and received phrenic stimulators. Clinicians randomly assigned patients (1:1) to receive either active or sham stimulation with a central web-based randomisation system (computer-generated list). Investigators, patients, and an external outcome allocation committee were masked to treatment. The primary outcome was non-invasive ventilation-free survival, analysed in the intention-to-treat population. Safety outcomes were also assessed in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT01583088.Findings: Between Sept 27, 2012, and July 8, 2015, 74 participants were randomly assigned to receive either active (n=37) or sham (n=37) stimulation. On July 16, 2015, an unplanned masked analysis was done after another trial showed excess mortality with diaphragm pacing in patients with hypoventilation (DiPALS, ISRCTN 53817913). In view of this finding, we analysed mortality in our study and found excess mortality (death from any cause) in our active stimulation group. We therefore terminated the study on July, 16, 2015. Median non-invasive ventilation-free survival was 6·0 months (95% CI 3·6-8·7) in the active stimulation group versus 8·8 months (4·2-not reached) in the control (sham stimulation) group (hazard ratio 1·96 [95% CI 1·08-3·56], p=0·02). Serious adverse events (mainly capnothorax or pneumothorax, acute respiratory failure, venous thromboembolism, and gastrostomy) were frequent (24 [65%] patients in the active stimulation group vs 22 [59%] patients in the control group). No treatment-related death was reported.Interpretation: Early diaphragm pacing in patients with ALS and incipient respiratory involvement did not delay non-invasive ventilation and was associated with decreased survival. Diaphragm pacing is not indicated at the early stage of the ALS-related respiratory involvement.Funding: Hospital Program for Clinical Research, French Ministry of Health; French Patients' Association for ALS Research (Association pour la Recherche sur la Sclérose Latérale Amyotrophique); and Thierry de Latran Foundation. [ABSTRACT FROM AUTHOR]

Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disease due to PHOX2B mutations. CCHS patients suffer from many autonomic disorders, dominated clinically by defective ventilatory automatisms. From birth, the life of CCHS patients depends on ventilatory support during sleep, involving a high burden of care. Whether or not this impairs the quality of life of these patients during adulthood remains unknown. Methods: We applied the medical outcome study short form-36 (SF-36) to 12 CCHS patients aged 15-33 (9 women) at the time of their passage from pediatric to adult care. Scores for the SF-36 dimensions were compared to the age- and gender-matched French reference population after transformation into standardized Z-scores. The SF-36 physical component summary score (PCS) and mental component summary score (MCS) were compared to American reference values. Results: Median Z-scores were significantly different from zerofor PF (physical functioning, p = 0.020) and GH (general health perception, p = 0.0342) and for PCS (p = 0.020). The other physical dimensions (RP, role limitation due to physical function; BP, bodily pain) and the mental dimensions (VT, vitality; SF, social functioning; RE, role limitation due to emotional function; MH, mental health) and MCS were not altered. Conclusions: We conclude that, despite the physical constraints imposed by CCHS and its anxiogenic nature, this disease is associated with an impairment of health-related quality of life in young adults that remains moderate. Whatever the underlying explanations, these results convey hope to parents with a child diagnosed with CCHS and for patients themselves. [ABSTRACT FROM AUTHOR]

Diaphragm pacing allows certain quadriplegic patients to be weaned from mechanical ventilation. Pacing failure can result from device dysfunction, neurotransmission failure, or degraded lung mechanics (such as atelectasis). We report two cases where progressive pacing failure was attributed to deteriorated chest wall mechanics. The first patient suffered from cervical spinal cord injury at age 45, was implanted with a phrenic stimulator (intrathoracic), successfully weaned from ventilation, and permanently paced for 7 years. Pacing effectiveness then slowly declined, finally attributed to rib cage stiffening due to ankylosing spondylitis. The second patient became quadriplegic after meningitis at age 15, was implanted with a phrenic stimulator (intradiaphragmatic) and weaned. After a year hypoventilation developed without obvious cause. In relationship with complex endocrine disorders, the patient had gained 31 kg. Pacing failure was attributed to excessive mechanical inspiratory load. Rib cage mechanics abnormalities should be listed among causes of diaphragm pacing failure and it should be kept in mind that a “good diaphragm” is not sufficient to produce a “good inspiration”. [ABSTRACT FROM AUTHOR]

NIV adherence ('quantity' of ventilation) has a prognostic impact in amyotrophic lateral sclerosis (ALS). We hypothesized that NIV effectiveness ('quality') could also have a similar impact. NIV effectiveness was evaluated in 82 patients within the first month (M1) and every three months (symptoms, arterial blood bases, and nocturnal pulsed oxygen saturation − SpO2). Kaplan-Meier survival and risk factors for mortality one year after NIV initiation were evaluated. Forty patients were considered 'correctly ventilated' at M1 (Group 1, less than 5% of nocturnal oximetry time with an SpO2<90% − TS90) while 42 were not (Group 2). Both groups were comparable in terms of respiratory and neurological baseline characteristics. Survival was better in Group 1 (75% survival at 12 months) than in Group 2 (43% survival at 12 months, p = 0.002). In 12 Group 2 patients corrective measures were efficient in correcting TS90 at six months. In this subgroup, one-year mortality was not different from that in Group 1. Multivariate analysis identified independent mortality risk factors expectedly including bulbar involvement (HR = 4.31 (1.73 − 10.76), p = 0.002), 'rapid respiratory decline' (HR = 3.55 (1.29 − 9.75), p = 0.014) and vital capacity (HR = 0.97 (0.95 − 0.99), p = 0.010), but also inadequate ventilation in the first month (HR = 2.32 (1.09 − 4.94), p = 0.029). In conclusion, in ALS patients NIV effectiveness to correct nocturnal desaturations is an independent prognostic factor. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Determining the basis for a taxonomy of mechanical ventilation" by R.L Chatburn and colleagues in the April 2012 issue.

Background: The diaphragm is difficult to biopsy because of its anatomic location. We describe a new laparoscopic diaphragm biopsy technique. MATERIAL AND METHODS: Fifty one patients with amyotrophic lateral sclerosis gave their consent to diaphragm biopsy in the context of an implanted phrenic nerve stimulation protocol (NCT01583088). The biopsy was taken from the costal diaphragm, after opening the parietal peritoneum with scissors, and by grasping the diaphragmatic muscle over the rib with toothed laparoscopy forceps. Results: The first four electrocoagulation biopsies were unsuitable for morphologic examination. The following 47 biopsies were therefore performed without electrocoagulation. The mean size of the biopsy fragments obtained after preparation was 3 ± 1 × 2 ± 1 × 1 ± 1 mm (maximum: 4 × 3 × 2 mm). A diaphragmatic injury occurred during the section in three cases requiring immediate suture without causing pneumothorax. A small pleural effusion was observed on the postoperative chest x-ray in one patient with a spontaneously favorable outcome. Numerous stains were able to be performed on the fragments obtained. Conclusions: Diaphragm biopsy can be safely performed by laparoscopy and yields tissue suitable for our future histologic evaluation. [ABSTRACT FROM AUTHOR]

Overnight polysomnography should be the first-line diagnostic test in patients with severe respiratory disease. However, if access to polysomnography is limited, overnight polygraphy can be used as an alternative first-line diagnostic. https://bit.ly/3KlmFED., Competing Interests: Conflict of interest: L. Grassion reports payment or honoraria from ASTEN Santé, Air Liquide Medical System, SOS Oxygène, SANOFI Genzyme, AstraZeneca, and Boehringer Ingelheim, outside the submitted work; support for attending meetings and/or travel from SOS Oxygene, and Asten Santé, outside the submitted work; participation on a Data Safety Monitoring Board or Advisory Board for VIVISOL, outside the submitted work. Conflict of interest: J. Gonzalez-Bermejo reports grants from Breas, L3medical, and Air Liquide, outside the submitted work; consulting fees from Breas, outside the submitted work; and is President of the French respiratory society, outside the submitted work. Conflict of interest: I. Arnulf has nothing to disclose. Conflict of interest: M. Patout reports grants or contracts from Fisher & Paykel, Resmed, and Asten Santé, outside the submitted work; consulting fees from Philips Respironics, Resmed, Asten Santé, and GSK, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Philips Respironics, Asten Santé, Resmed, Air Liquide Medical, SOS Oxygène, Antadir, Chiesi, and Jazz Pharmaceutical, outside the submitted work; support for attending meetings and/or travel from Asten Santé, outside the submitted work; participation on a Data Safety Monitoring Board or Advisory Board for Resmed, Philips Respironics, and Asten Santé, outside the submitted work; stock or stock options for Kernel Biomedical, outside the submitted work; receipt of equipment, materials, drugs, medical writing, gifts or other services from Philips Respironics, Resmed, and Fisher & Paykel, outside the submitted work., (Copyright ©ERS 2023.)

In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV. MPV represents a difficult task for home ventilators due to rapidly changing load conditions resulting from intermittent connections and disconnections from MPV circuit. The 252nd ENMC International Expert Workshop, held March 6th to 8th 2020 in Amsterdam, reported general guidelines for management of daytime MPV in NMDs. This report could not present all the detail regarding the technical issues important for clinical success of MPV. Based on the expert workshop discussions and the evidence from existing studies, the current narrative review aims to identify the technical issues of MPV and offers guidance via a decisional algorithm and educational figures providing relevant information that is important for successful implementation of MPV., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Amyotrophic lateral sclerosis (ALS) patients show progressive respiratory muscle weakness leading to death from respiratory failure. However, there are no data on diaphragm histological changes in ALS patients and how they correlate with routine respiratory measurements.We collected 39 diaphragm biopsies concomitantly with laparoscopic insertion of intradiaphragmatic electrodes during a randomised controlled trial evaluating early diaphragm pacing in ALS (https://clinicaltrials.gov; NCT01583088). Myofibre type, size and distribution were evaluated by immunofluorescence microscopy and correlated with spirometry, respiratory muscle strength and phrenic nerve conduction parameters. The relationship between these variables and diaphragm atrophy was assessed using multivariate regression models.All patients exhibited significant slow- and fast-twitch diaphragmatic atrophy. Vital capacity (VC), maximal inspiratory pressure, sniff nasal inspiratory pressure (SNIP) and twitch transdiaphragmatic pressure did not correlate with the severity of diaphragm atrophy. Inspiratory capacity (IC) correlated modestly with slow-twitch myofibre atrophy. Supine fall in VC correlated weakly with fast-twitch myofibre atrophy. Multivariate analysis showed that IC, SNIP and functional residual capacity were independent predictors of slow-twitch diaphragmatic atrophy, but not fast-twitch atrophy.Routine respiratory tests are poor predictors of diaphragm structural changes. Improved detection of diaphragm atrophy is essential for clinical practice and for management of trials specifically targeting diaphragm muscle function., Competing Interests: Conflict of interest: R. Guimarães-Costa has nothing to disclose. Conflict of interest: T. Similowski reports personal fees from AstraZeneca, Boerhinger Ingelheim France, GSK Lungpacer Inc., TEVA, Chiesi, Pierre Fabre and Invacare, and personal fees and non-financial support from Novartis, outside the submitted work. In addition, he received honoraria from Synapse Biomedical to translate the DPS/NeurRx4 user manual from English into French in 2007 and, from 2012 to 2016, Synapse Biomedical contributed to a fundraiser organised by T. Similowski to promote respiratory research. T. Similowski is also engaged in scientific collaborations with two other companies manufacturing phrenic stimulation devices. With Lungpacer Inc (Canada) he acts as a paid consultant and investigator in clinical trials, while with Atrotech-Neuroresp (France) he acts as a probono consultant (no fees or other support). Conflict of interest: I. Rivals has nothing to disclose. Conflict of interest: C. Morélot-Panzini has nothing to disclose. Conflict of interest: M-C. Nierat has nothing to disclose Conflict of interest: M.T. Bui has nothing to disclose. Conflict of interest: D. Akbar has nothing to disclose. Conflict of interest: C. Straus has nothing to disclose. Conflict of interest: N.B. Romero has nothing to disclose. Conflict of interest: P.P. Michel has nothing to disclose. Conflict of interest: F. Menegaux has nothing to disclose. Conflict of interest: F. Salachas has nothing to disclose. Conflict of interest: J. Gonzalez-Bermejo has nothing to disclose. Conflict of interest: G. Bruneteau has nothing to disclose., (Copyright ©ERS 2019.)

Background: Combined effects of leaks, mechanical property of respiratory system and upper airway (UA) patency on patient-ventilator synchrony (PVA) and the level of clinically "tolerable" leaks are not well established in home ventilators., Methods: We comparatively assessed on a bench model, the highest leak level tolerated without inducing significant asynchrony ("critical leak") in three home ventilators (Astral 150, Trilogy 100 and Vivo 60; noted as A150, T100 and V60 respectively) subjected to three simulated diseased respiratory conditions: chronic obstructive pulmonary disease (COPD), obesity hypoventilation (OHS) and neuromuscular disorders (NMD), with both open and closed UA. Also, total leak values in the device reports were compared to the bench-measured values., Results: With open UA, all ventilators were able to avoid asynchrony up to a 30 L/min leak and even to 55 L/min in some cases. UA closure and respiratory diseases especially OHS influenced PVA. With closed UA, the critical leak of A150 and T100 remained higher than 55 L/min in COPD and OHS, while for V60 decreased to 41 and 33 L/min respectively. In NMD with closed UA, only T100 reached a high critical leak of 69 L/min. Besides, inspiratory trigger sensitivity change was often necessary to avoid PVA., Conclusions: Home ventilators were able to avoid PVA in high-level leak conditions. However, asynchrony appeared in cases of abnormal mechanical properties of respiratory system or closed UA. In case of closed UA, the EPAP should be adjusted prior to the inspiratory trigger., Trial Registration: Not applicable.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing diaphragm weakness that can be partially compensated by inspiratory neck muscle recruitment. This disappears during sleep, which is compatible with a cortical contribution to the drive to breathe. We hypothesised that ALS patients with respiratory failure exhibit respiratory-related cortical activity, relieved by noninvasive ventilation (NIV) and related to dyspnoea.We studied 14 ALS patients with respiratory failure. Electroencephalographic recordings (EEGs) and electromyographic recordings of inspiratory neck muscles were performed during spontaneous breathing and NIV. Dyspnoea was evaluated using the Multidimensional Dyspnea Profile.Eight patients exhibited slow EEG negativities preceding inspiration (pre-inspiratory potentials) during spontaneous breathing. Pre-inspiratory potentials were attenuated during NIV (p=0.04). Patients without pre-inspiratory potentials presented more advanced forms of ALS and more severe respiratory impairment, but less severe dyspnoea. Patients with pre-inspiratory potentials had stronger inspiratory neck muscle activation and more severe dyspnoea during spontaneous breathing.ALS-related diaphragm weakness can engage cortical resources to augment the neural drive to breathe. This might reflect a compensatory mechanism, with the intensity of dyspnoea a negative consequence. Disease progression and the corresponding neural loss could abolish this phenomenon. A putative cognitive cost should be investigated., (Copyright ©ERS 2016.)