Ventilation In Amyotrophic Lateral Sclerosis Research Articles

Factors associated with adherence to noninvasive positive pressure ventilation in amyotrophic lateral sclerosis.

This cohort study aimed to investigate the factors associated with noninvasive positive pressure ventilation adherence and assess the long-term effects of noninvasive positive pressure ventilation adherence in patients with amyotrophic lateral sclerosis (ALS). The medical records of patients with ALS admitted to a tertiary hospital for noninvasive positive pressure ventilation initiation were retrospectively reviewed. Pulmonary function parameters, variables of blood gas analysis, the site of symptom onset, the time from onset and diagnosis to noninvasive positive pressure ventilation application, ALS Functional Rating Scale-Revised, neurophysiological index, and the length of hospital stay were evaluated. The adherence to noninvasive positive pressure ventilation was defined as the use of noninvasive positive pressure ventilation for ≥ 2 h/day or ≥ 4 h/day. The correlations between noninvasive positive pressure ventilation adherence or length of hospital stay and other clinical parameters were analyzed. Fifty-one patients with ALS were included in the study. The time from onset and diagnosis to NIPPV application was reduced by 16 months in the adherent group than that in the non-adherent group; however, the parameters of blood gas analysis and pulmonary function tests did not differ significantly between the groups. Furthermore, the neurophysiological index of the abductor digiti minimi muscle was higher by 4.05 in the adherent group than that in the non-adherent group. The adherence to noninvasive positive pressure ventilation prolonged tracheostomy-free survival compared to that of non-adherence. Desaturation events, lower forced vital capacity, last pCO2, bicarbonate, and base excess, and higher differences in pCO2, were associated with an increase in the length of hospital stay. Noninvasive positive pressure ventilation application shortly after symptom onset and ALS diagnosis in patients with CO2 retention and reduced forced vital capacity can be considered for successful adherence. Adherence to noninvasive positive pressure ventilation may result in reduced tracheostomy conversion rates and prolonged tracheostomy-free survival.

Learning prognostic models using a mixture of biclustering and triclustering: Predicting the need for non-invasive ventilation in Amyotrophic Lateral Sclerosis

Longitudinal cohort studies to study disease progression generally combine temporal features produced under periodic assessments (clinical follow-up) with static features associated with single-time assessments, genetic, psychophysiological, and demographic profiles. Subspace clustering, including biclustering and triclustering stances, enables the discovery of local and discriminative patterns from such multidimensional cohort data. These patterns, highly interpretable, are relevant to identifying groups of patients with similar traits or progression patterns. Despite their potential, their use for improving predictive tasks in clinical domains remains unexplored.In this work, we propose to learn predictive models from static and temporal data using discriminative patterns, obtained via biclustering and triclustering, as features within a state-of-the-art classifier, thus enhancing model interpretation. triCluster is extended to find time-contiguous triclusters in temporal data (temporal patterns) and a biclustering algorithm to discover coherent patterns in static data. The transformed data space, composed of bicluster and tricluster features, capture local and cross-variable associations with discriminative power, yielding unique statistical properties of interest.As a case study, we applied our methodology to follow-up data from Portuguese patients with Amyotrophic Lateral Sclerosis (ALS) to predict the need for non-invasive ventilation (NIV) since the last appointment. The results showed that, in general, our methodology outperformed baseline results using the original features. Furthermore, the bicluster/tricluster-based patterns used by the classifier can be used by clinicians to understand the models by highlighting relevant prognostic patterns.

Alveolar Ventilation-Targeted Versus Spontaneous/Timed Mode for Home Noninvasive Ventilation in Amyotrophic Lateral Sclerosis.

Home noninvasive ventilation (NIV) is increasingly used in amyotrophic lateral sclerosis (ALS) to improve symptoms and survival. Our primary objective was to compare intelligent volume-assured pressure support (iVAPS) versus spontaneous/timed (S/T) modes regarding time to first change in ventilator parameters and the number of interventions over 6 months in subjects with ALS in a respiratory therapist (RT)-led program. In this study, 30 subjects with ALS meeting criteria for NIV initiation were randomized to iVAPS or S/T. NIV was initiated using standardized protocols targeting optimal tidal volume and comfort in a daytime session. Download data were recorded at 1 week and 1 and 6 months. Any changes in ventilator parameters were recorded. Of the 30 subjects, 56.7% had bulbar onset ALS, 8 died, and 11 in each group completed the study. Median time to first parameter change was 33.5 (interquartile range [IQR] 7.7-96.0) d versus 41.0 (IQR 12.5-216.5) d for iVAPS versus S/T groups, respectively, (P = .48). The average number of RT interventions was similar between groups (1.1 ± 1.1 vs 0.9 ± 0.9 at 1 month, P = .72; 2.4 ± 2.1 vs 2.4 ± 2.3 at 6 months, P = .95, for iVAPS vs S/T, respectively). Adherence was significantly lower with iVAPS than S/T at 1 week but not at 1 or 6 months. Download parameters were similar between groups at 1 week and 6 months except for higher residual apnea-hypopnea index (AHI) and less spontaneously triggered breaths with iVAPS at 6 months. The time to first change of parameters and the number of interventions at 6 months from NIV initiation were similar for the iVAPS and S/T modes in subjects with ALS. With iVAPS, adherence was lower transiently at NIV initiation, and the residual AHI was higher at 6 months. Alveolar ventilation-targeted NIV may require a longer adaptation period and result in greater upper-airway instability predominantly in patients with bulbar ALS.

Learning Prognostic Models Using Disease Progression Patterns: Predicting the Need for Non-Invasive Ventilation in Amyotrophic Lateral Sclerosis.

Amyotrophic Lateral Sclerosis is a devastating neurodegenerative disease causing rapid degeneration of motor neurons and usually leading to death by respiratory failure. Since there is no cure, treatment's goal is to improve symptoms and prolong survival. Non-invasive Ventilation (NIV) is an effective treatment, leading to extended life expectancy and improved quality of life. In this scenario, it is paramount to predict its need in order to allow preventive or timely administration. In this work, we propose to use itemset mining together with sequential pattern mining to unravel disease presentation patterns together with disease progression patterns by analysing, respectively, static data collected at diagnosis and longitudinal data from patient follow-up. The goal is to use these static and temporal patterns as features in prognostic models, enabling to take disease progression into account in predictions and promoting model interpretability. As case study, we predict the need for NIV within 90, 180 and 365 days (short, mid and long-term predictions). The learnt prognostic models are promising. Pattern evaluation through growth rate suggests bulbar function and phrenic nerve response amplitude, additionally to respiratory function, are significant features towards determining patient evolution. This confirms clinical knowledge regarding relevant biomarkers of disease progression towards respiratory insufficiency.

Non-invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: Utilization and survival rates in a cohort study over 12 years in Germany.

The aim of this study was to investigate utilization rates, treatment pathways and survival prognosis in patients with amyotrophic lateral sclerosis (ALS) undergoing non-invasive (NIV) and tracheostomy invasive ventilation (TIV) in a real-world setting. A prospective cohort study using a single-centre register of 2702 ALS patients (2007 to 2019) was conducted. Utilization of NIV/TIV and survival data were analysed in three cohorts: (i) non-NIV; (ii) NIV (NIV without subsequent TIV); and (iii) TIV (including TIV preceded by NIV). A total of 1720 patients with available data were identified, 72.0% of whom (n=1238) did not receive ventilation therapy. NIV was performed in 20.8% of patients (n=358). TIV was performed in 9.5% of patients (n=164), encompassing both primary TIV (7.2%, n=124) and TIV with preceding NIV (2.3%, n=40). TIV was more often utilized without previous NIV (25.7% vs. 8.3% of all ventilated patients), demonstrating that primary TIV was the prevailing pathway for invasive ventilation. The median (range) survival was significantly longer in the NIV cohort (40.8[37.2-44.3] months) and the TIV cohort (82.1[68.7-95.6] months) as compared to the non-NIV cohort (33.6[31.6-35.7] months). Although NIV represents the standard of care, its utilization rate was low. TIV was mainly started without preceding NIV, suggesting that TIV may not be confined to NIV treatment escalation. However, TIV was pursued in a minority of patients who had previously undergone NIV. The survival benefit observed in the patients with NIV was equal to that reported in a controlled pivotal trial, but the prognosis with TIV is highly variable. The determinants of utilization of NIV/TIV and of survival (bulbar syndrome, availability of ventilation-related home nursing, cultural factors) warrant further investigation.

Noninvasive Ventilation in Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease involving upper and lower motor neurons and has limited treatment options. The weakness progresses to involve the diaphragms, resulting in respiratory failure and death. Home noninvasive ventilation has been shown to improve survival and quality of life, especially in those with intact bulbar function. Once initiated, close monitoring with nocturnal oximetry, remote downloads from the home noninvasive ventilation machine, and measurement of serum bicarbonate should be conducted. Additionally, transcutaneous CO2 monitoring can be considered if available. This article discusses the indications, timing, initiation, and management of noninvasive ventilation in amyotrophic lateral sclerosis.

Gastrostomy and mechanical ventilation in amyotrophic lateral sclerosis: how best to support the decision-making process?

The unfavourable outcome of amyotrophic lateral sclerosis (ALS) confronts patients with challenging decisions regarding life-sustaining measures. The decision-making process is usually triggered by medical consultations and patient-dependent factors. This may largely depend on the physician's depth of knowledge and professional experience. This paper presents an overview of the life-sustaining methods used in ALS and their effects on disease progression, survival and quality of life of patients and their caregivers. It is intended to aid physicians in their discussions with patients. We interrogate all the positive and negative facets of life-sustaining measures that may allow for optimisation of the decision-making process and care provision.

Effects of noninvasive ventilation in amyotrophic lateral sclerosis: The complication of bulbar impairment

Amyotrophic lateral sclerosis is a neurodegenerative illness that causes gradual loss of muscle function. Patients eventually develop bulbar impairment, requiring extensive respiratory support. Noninvasive ventilation (NIV) has gained attention as an easily accessible method with promising benefits. We conducted this systematic review to outline the therapeutic effects of NIV, add to previous publications discussing this topic by providing updates on newly completed and ongoing studies, and identify limitations that must be addressed in future trials. A search of PubMed and Cochran for relevant primary studies yielded 26 publications. Studies indicate NIV use is associated with improvements in quality of life, regardless of the severity of bulbar impairment. However, NIV’s benefits on survival were limited to patients with less bulbar impairment. In addition, our review found several limitations that undermine the efforts to establish a definitive treatment regimen. Future studies will need to address these problems in order to provide patients with better respiratory care.

What is the right moment for noninvasive ventilation in amyotrophic lateral sclerosis?

The most common cause of death in patients with amyotrophic lateral sclerosis (ALS) is respiratory failure, often in the period of 2-5 years, with a small percentage of patients surviving up to 10 years or more. The aim of the study was to evaluate the significance of pulmonary function tests in prediction of mortality and definition of indications for noninvasive mechanical ventilation (NIMV). This retrospective-prospective study was performed at the Clinic of Pulmonology, Clinical Centre of Serbia in the period from January 2015 to December 2017. Patients with diagnosis of ALS established according to El Escorial criteria were included. The study included 76 patients with ALS, 50 (65.85%) with spinal and 26 (34.2%) with bulbar form of disease. Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) were higher in spinal form of ALS, and the difference was statistically significant when compared to bulbar form. Form of disease, FVC < 70%, maximum inspiratory pressure (PImax) < 50 and maximum expiratory pressure (PEmax) < 50 were significant factors for survival. The patients with bulbar form of disease had 2.174 (95.0% CI: 1.261-3.747) higher risk for death. Our study points to the significance of timely application and early start of NIMV in patients with ALS as an important approach to defer functional impairment, which would mean that the criteria, in our country, for application of these devices must be changed, not only regarding the value of current functional diagnostic tests used in everyday practice in patients with ALS but also in regard to the introduction of new diagnostic tests, such as sniff nasal inspiratory pressure and/or polysomnographic testing.

Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis

Weight loss is an independent predictor of survival in the early stages of amyotrophic lateral sclerosis (ALS). However, the effects of weight variations on the functional prognosis after tracheostomy and invasive ventilation (TIV) in ALS remain unknown. This prospective cohort study aimed to investigate the relationship between weight loss before TIV and disease progression after TIV in ALS patients. Sixty ALS patients with TIV were enrolled and classified into subgroups based on the rate of decline in body mass index, from onset to TIV utilization (ΔBMI). During follow-up, we assessed the patients for presence of communication impairments, ophthalmoplegia, total quadriplegia, mouth opening disability, and dysuria. We analyzed the relationship between ΔBMI and the communication stage or motor disabilities. The log-rank test showed that patients with a ΔBMI ≥ 1.7 kg/m2/year showed a shorter period of preserved communication ability (p = 0.0001), shorter time to develop ophthalmoplegia (p = 0.0001), total quadriplegia (p < 0.0001), mouth opening disability (p < 0.0001), and dysuria (p = 0.0455). Cox multivariate analyses showed that a larger ΔBMI was an independent prognostic factor for the early development of ophthalmoplegia (p = 0.0400) and total quadriplegia (p = 0.0445). Weight loss in the early stages of ALS predicts disease progression in patients with advanced stages of ALS using TIV.

Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis.

Upper airway obstruction with decreased central drive (ODCD) is one of the causes of ineffective noninvasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS). The aim of this study is to determine the mechanism responsible for ODCD in ALS patients using NIV.This is a prospective study that included ALS patients with home NIV. Severity of bulbar dysfunction was assessed with the Norris scale bulbar subscore; data on upper or lower bulbar motor neuron predominant dysfunction on physical examination were collected. Polysomnography was performed on every patient while using NIV and the ODCD index (ODCDI: number of ODCD events/total sleep time) was calculated. To determine the possible central origin of ODCD, controller gain was measured by inducing a hypocapnic hyperventilation apnoea. Sonography of the upper airway during NIV was performed to determine the location of the ODCD.30 patients were enrolled; three (10%) had ODCDI >5 h−1. The vast majority of ODCD events were produced during non-rapid eye movement sleep stages and were a consequence of an adduction of the vocal folds. Patients with ODCDI >5 h−1 had upper motor neuron predominant dysfunction at the bulbar level, and had greater controller gain (1.97±0.33 versus 0.91±0.36 L·min−1·mmHg−1; p<0.001) and lower carbon dioxide (CO2) reserve (4.00±0.00 versus 10.37±5.13 mmHg; p=0.043). ODCDI was correlated with the severity of bulbar dysfunction (r= −0.37; p=0.044), controller gain (r=0.59; p=0.001) and CO2 reserve (r= −0.35; p=0.037).ODCD events in ALS patients using NIV have a central origin, and are associated with instability in the control of breathing and an upper motor neuron predominant dysfunction at the bulbar level.

Non-invasive ventilation in amyotrophic lateral sclerosis.

Non-invasive ventilation (NIV) has become an important cornerstone of symptomatic treatment in amyotrophic lateral sclerosis (ALS), improving survival and quality of life.In this review, we summarize the most important recent developments and insights, including evidence of efficacy, indication criteria and time of initiation, ventilation parameters and adaptation strategies, treatment of complicating factors, transition from NIV to invasive ventilation, termination of NIV and end-of-life management. Recent publications have questioned former conventions and guideline recommendations, especially with regard to timing and prognostic factors; therefore, a fresh look and re-evaluation of current evidence is needed.

Practical implementation of noninvasive ventilation in Amyotrophic Lateral Sclerosis: lessons learned from a clinical case series.

PurposeNoninvasive ventilation (NIV) may improve survival and quality of life in Amyotrophic Lateral Sclerosis (ALS) patients. There is a surprising paucity of practical guidelines for office-based implementation and management of NIV outside of tertiary ALS centers. We saw the need for a clinical protocol to allow feasible and consistent NIV management in this patient population.MethodsWe created a clinical protocol for office-based initiation of NIV implemented on consecutive ALS patients referred from our regional ALS multidisciplinary clinic. The protocol provided initial empiric settings using a bilevel device in volume-assured pressure support mode. A respiratory therapist (RT) initiated NIV in an office setting and made adjustments according to patient tolerance and therapy targets outlined in the protocol. Later setting changes were performed at patient or provider request. We evaluated patient adherence and efficacy via device download at 30 days and 1 year.ResultsWe present data from a case series of the first 14 consecutive patients initiated on NIV over a 20-month period. Our protocol underwent iterative modification based on clinical experience and patient feedback. Early challenges included the significant time and resource burden required to coordinate device downloads and patient follow-up. Early 30-day NIV adherence was variable (median 20 out of 30 days), while 1-year NIV adherence was excellent (median 27.5 out of 30 days).ConclusionsOur RT-driven clinical NIV protocol was feasible but labor intensive. Achieving real-world adherence of NIV in our ALS patients required iterative protocol adjustment, significant RT provider time, and tele-based follow-up.

Ethical challenges in tracheostomy-assisted ventilation in amyotrophic lateral sclerosis

The special nature of amyotrophic lateral sclerosis (ALS) and tracheostomy with invasive ventilation (TIV) leads to challenges that can be difficult in two senses: not only to handle well, but also to discuss with patients and other involved stakeholders. Because of the delicate nature of interpersonal relations and communication in ALS, some of the downsides to TIV may almost take on a nature of taboo, making them difficult to raise for open discussion. Yet these ethical challenges are important to be aware of, not only for health professionals and managers but, arguably, also for patients and next of kin. They are important also for a wider professional and societal debate about whether and to whom TIV should be offered. In this paper we highlight and examine ethical challenges in TIV for ALS, with a special emphasis on those that are hard to discuss openly and that therefore might fail to be addressed. The analysis is structured by the four core principles of healthcare ethics: beneficence, nonmaleficence, respect for patient autonomy, and justice.

Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis.

We evaluated post-noninvasive ventilation survival and factors for the transition to tracheostomy in amyotrophic lateral sclerosis (ALS). We analyzed 197 patients using a prospectively collected database with 114 patients since 2000. Among 114 patients, 59 patients underwent noninvasive ventilation (NIV), which prolonged the total median survival time to 43 months compared with 32 months without treatment. The best post-NIV survival was associated with a lack of bulbar symptoms, higher measured pulmonary function, and a slower rate of progression at diagnosis. The transition rate from NIV to tracheostomy gradually decreased over the years. Patients using NIV for more than 6 months were more likely to refuse tracheostomy and to be women. This study confirmed a positive survival effect with NIV, which was less effective in patients with bulbar dysfunction. Additional studies are required to determine the best timing for using NIV with ALS in patients with bulbar dysfunction. Muscle Nerve 58:770-776 2018.

Patterns of Non-Invasive Ventilation in Amyotrophic Lateral Sclerosis.

Non-invasive ventilation (NIV) improves quality of life and survival in patients with amyotrophic lateral sclerosis (ALS) and respiratory symptoms. Little is known about the patterns of NIV use over time and the impact of NIV on end-of-life decision-making in ALS. This study assessed the pattern of NIV use over the course of the disease and the timing of end-of-life discussions in people living with ALS. A retrospective single-center cohort study was performed at London Health Sciences Centre. Daily NIV duration of use was evaluated at 3-month intervals. The timing of diagnosis, NIV initiation, discussions relating to do-not-attempt-resuscitation (DNAR) and death were examined. In total, 48 patients were included in the analysis. Duration of NIV use increased over time, and tolerance to NIV was observed to be better than expected in patients with bulbar-onset ALS. There was a high degree of variability in the timing of end-of-life discussions in patients with ALS (356±451 days from diagnosis). In this cohort, there was a strong association between the timing of discussions regarding code status and establishment of a DNAR order (r2=0.93). This retrospective cohort study suggests that the use of NIV in ALS increases over time and that there remains a great deal of variability in the timing of end-of-life discussions in people living with ALS. Future prospective studies exploring the use NIV over the disease trajectory and how NIV affects end-of-life decision-making in people with ALS are needed.

Patient Preferences for Treatments of Neuromuscular Diseases: A Systematic Literature Review.

Treatment decisions of neuromuscular diseases involve weighing clinical benefits and risks, as well as impact on patient social life, work status, other activities of daily living, and health-related quality of life. To conduct a systemic literature review of patient preferences for treatments of neuromuscular diseases. We searched Embase, Web of Science, and PubMed for full-text articles reporting results from studies of patient preferences for treatments of neuromuscular diseases. We excluded articles published before the year 2000, articles written in a language other than English, articles only reporting proxy-assessments of patient preferences, and studies reporting results for a sample comprising <5 patients. The search resulted in the identification of 305 unique publications. Of these, 275 were excluded following title and abstract screening and 23 following full-text review. Seven articles were included for data synthesis. Preference data were identified for a hypothetical treatment with pulmonary benefits of Duchenne muscular dystrophy and Becker muscular dystrophy, pathways for different routes of opioid drug administration in motor neuron disease, wheelchair features in amyotrophic lateral sclerosis (ALS), ankle foot orthoses in patients with Charcot Marie Tooth disease, and mechanical ventilation in ALS and a mixed cohort of patients with neuromuscular diseases. Despite considerable research into the development of new health technologies targeting neuromuscular diseases, little is known of patients' preferences for pharmacological interventions. More research is needed to help incorporate patient preferences in clinical decision-making to improve treatment satisfaction, medication compliance, and health outcomes.

The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis

BackgroundNon-invasive ventilation (NIV) improves survival and quality of life in amyotrophic lateral sclerosis (ALS) patients. The timing of referral to a home ventilation service (HVS), which is in part based on respiratory function tests, has shown room for improvement. It is currently unknown which respiratory function test predicts an appropriate timing of the initiation of NIV.MethodsWe analysed, retrospectively, serial data of five respiratory function tests: forced vital capacity (FVC), peak cough flow (PCF), maximum inspiratory and expiratory pressure (MIP and MEP) and sniff nasal inspiratory pressure (SNIP) in patients with ALS. Patients who had had at least one assessment of respiratory function and one visit at the HVS, were included. Our aim was to detect the test with the highest predictive value for the need for elective NIV in the following 3 months. We analysed time curves, currently used cut-off values for referral, and respiratory function test results between ‘NIV indication’ and ‘no-NIV indication’ patients.ResultsOne hundred ten patients with ALS were included of whom 87 received an NIV indication; 11.5% had one assessment before receiving an NIV indication, 88.5% had two or more assessments. The NIV indication was based on complaints of hypoventilation and/or proven (nocturnal) hypercapnia. The five respiratory function tests showed a descending trend during disease progression, where SNIP showed the greatest decline within the latest 3 months before NIV indication (mean = −22%). PCF at the time of referral to the HVS significantly discriminated between the groups ‘NIV-indication’ and ‘no NIV-indication yet’ patients at the first HVS visit: 259 (±92) vs. 348 (±137) L/min, p = 0.019. PCF and SNIP showed the best predictive characteristics in terms of sensitivity.ConclusionSNIP showed the greatest decline prior to NIV indication and PCF significantly differentiated ‘NIV-indication’ from ‘no NIV-indication yet’ patients with ALS. Currently used cut-off values might be adjusted and other respiratory function tests such as SNIP and PCF may become part of routine care in patients with ALS in order to avoid non-timely initiation of (non-invasive) ventilation.

Initiation of non-invasive ventilation in amyotrophic lateral sclerosis and clinical practice guidelines: Single-centre, retrospective, descriptive study in a national reference centre

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 PaCO2 was 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.

Noninvasive ventilation in amyotrophic lateral sclerosis: effects on sleep quality and quality of life

Objectives: Little is known about the effects of noninvasive ventilation (NIV) on sleep quality in amyotrophic lateral sclerosis (ALS). We aim to evaluate the long-term effects of NIV on sleep quality and quality of life in patients with ALS.Methods: In this prospective observational study, 13 ALS patients were followed for one year after initiating NIV. We evaluated sleep quality, quality of life and functional status with several questionnaires: Epworth sleepiness Scale (ESS), Pittsburg sleep quality index (PSQI), Short Form 36 Health Questionnaire (SF-36), McGill Quality of Life questionnaire (McGillQoL) and revised Amyotrophic Lateral Sclerosis Functional Rating Scale scores (ALSFRS-R).Results: Median and interquartile range (IQR) at the start of NIV was 59 (53–65) years. The ALSFRS-R at start was 30 (24–37) (median, IQR), with three patients having severe bulbar impairment (ALSFRS-R-bulbar ≤ 9). The PaCO2 at start of NIV treatment was 48 (43–52) mmHg (median, IQR). During the one-year follow-up period, a significant decrease in the ALSFRS-R was observed. The impact of NIV in a short term (1 month) revealed a statistically significant decrease in ESS, decrease in total PSQI and of four PSQI subscales and improvement of almost all subscales of the McGill questionnaire. Long-term analyses (9 months to 1 year) revealed that amelioration in ESS and total PSQI was sustained.Conclusion: We conclude that accurately titrated NIV in ALS patients can stabilize sleep quality and quality of life for at least one year, despite significant disease progression.