Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: Survival rates in a controlled trial

Pinto, Anabela; Evangelista, Teresinha; Carvalho, Mamede de; Alves, Marta; Luís, M.L. Sales

doi:10.1016/0022-510x(95)00052-4

Cited by 257 publications

(169 citation statements)

References 11 publications

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“…Early NIV has been shown to have positive effects on chronic hypoventilation, quality of life and survival [12][13][14][15][16][17][18][19] in studies comparing ALS NIV users with patients who refuse or are not able to tolerate NIV. Bourke et al performed the only randomized, unblinded, controlled trial of NIV in ALS showing that NIV improved quality of life and survival in a group of ALS patients, particularly those with preserved bulbar function.…”

Section: Discussionmentioning

confidence: 99%

Effects of Noninvasive Ventilation on Sleep Outcomes in Amyotrophic Lateral Sclerosis

Katzberg

Selegiman

Guion³

et al. 2013

Journal of Clinical Sleep Medicine

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ing respiratory dysfunction, the goal of this study is not to fi nd a new indication for NIV in ALS, but rather to better understand its effects on sleep outcomes and sleep disordered breathing. The specifi c study aims are to evaluate the effects of NIV on oxygenation, overall sleep quality and other parameters such as apnea index, sleep latency, arousals, and total sleep time. METHODS Study DesignWe performed a pilot, self-controlled study in patients with a confi rmed diagnosis of ALS who were starting noninvasive ventilation (NIV) for impaired respiratory or sleep function. Study Objectives:The objective was to study the effects on noninvasive ventilation on sleep outcomes in patient with ALS, specifi cally oxygenation and overall sleep quality. Methods: Patients with ALS who met criteria for initiation of NIV were studied with a series of 2 home PSG studies, one without NIV and a follow-up study while using NIV. Primary outcome was a change in the maximum overnight oxygen saturation; secondary outcomes included change in mean overnight oxygen saturation, apnea and hypopnea indexes, sleep latency, sleep effi ciency, sleep arousals, and sleep architecture. Results: A total of 94 patients with ALS were screened for eligibility; 15 were enrolled; and 12 completed study procedures. Maximum overnight oxygen saturation improved by 7.0% (p = 0.01) and by 6.7% during REM sleep (p = 0.02) with NIV. Time spent below 90% oxygen saturation was also signifi cantly better with NIV (30% vs 19%, p < 0.01), and there was trend for improvement in mean overnight saturation (1.5%, p = 0.06). Apnea index (3.7 to 0.7), hypopnea index (6.2 to 5.7), and apnea hypopnea index (9.8 to 6.3) did not signifi cantly improve after introducing NIV. NIV had no effect on sleep effi ciency (mean change 10%), arousal index (7 to 12), or sleep stage distribution (Friedman chi-squared = 0.40). Conclusions: NIV improved oxygenation but showed no signifi cant effects on sleep effi ciency, sleep arousals, restful sleep, or sleep architecture. The net impact of these changes for patients deserves further study in a larger group of ALS patients. S C I E N T I F I C I N V E S T I G A T I O N SS leep dysfunction is commonly seen in patients with amyotrophic lateral sclerosis (ALS) through a number of nonrespiratory (psychological stress, pain, cramps) and respiratory (sleep apnea, hypoventilation) mechanisms.1 Most sleep dysfunction occurs later in the course of the illness, particularly when diaphragmatic dysfunction occurs and respiratory compromise becomes evident.2 The mechanisms underlying respiratory dysfunction during sleep in ALS depend on multiple factors, including the disease stage and whether or not the diaphragm is involved.The most effective interventions for respiratory and sleep dysfunction in ALS include noninvasive ventilation (NIV), which has been shown to improve respiratory parameters, survival and quality of life.3-6 The 2009 American Academy of Neurology Practice Parameter on ALS treatment recommended the use of NIV for patients ...

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Section: Discussionmentioning

confidence: 99%

Effects of Noninvasive Ventilation on Sleep Outcomes in Amyotrophic Lateral Sclerosis

Katzberg

Selegiman

Guion³

et al. 2013

Journal of Clinical Sleep Medicine

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“…Inexsufflation and use of high frequency chest wall oscillation are increasingly common and have been shown to improve ventilation [126][127][128][129]. Physical techniques such as breath stacking and assisted cough, as well as medication management (bronchodilators, anticholinergic agents), have become increasingly common in aggressive multidisciplinary treatment settings.…”

Section: Respiratory Therapiesmentioning

confidence: 99%

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Rosenfeld¹,

Strong

2015

Neurotherapeutics

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With the acceleration in our understanding of ALS and the related motor neuron disease has come even greater challenges in reconciling all of the proposed pathogenic mechanisms and how this will translate into impactful treatments. Fundamental issues such as diagnostic definition(s) of the disease spectrum, relevant biomarkers, the impact of multiple novel genetic mutations and the significant effect of symptomatic treatments on disease progression are all areas of active investigation. In this review, we will focus on these key issues and highlight the challenges that confront both clinicians and basic science researchers.

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“…In a controlled study of NIV in ALS/MND, PINTO et al [13] showed a significant increase in survival in patients with respiratory insufficiency using bilevel ventilatory support compared to a nonventilated control group. However, the quality-of-life tool used in this study showed no improvement.…”

Section: Motor Neurone Disease/amyotrophic Lateral Sclerosismentioning

confidence: 99%

Home ventilation: Table 1—

Simonds¹

2003

Eur Respir J

127

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Home ventilation is a growth area. Rapid expansion during the 1990s was stimulated by the development of noninvasive ventilation (NIV)viaa mask and the recognition that an increased number of patient groups can benefit. Although patients receiving NIV in the home outnumber those receiving invasive ventilationviatracheostomy, there is substantial variation in practice between European countries.Evidence that individuals who develop ventilatory failure as a consequence of chest wall disease or stable neuromuscular disease such as old poliomyelitis benefit from nocturnal NIV is overwhelming. Patients with progressive neuromuscular disease such as Duchenne muscular dystrophy and amyotrophic lateral sclerosis can also derive prolongation of life, palliation of symptoms and an improvement in quality of life.Home ventilation in chronic obstructive pulmonary disease (COPD) patients remains controversial. Multicentric randomised controlled trials of long-term oxygen therapy (LTOT)versusNIV plus LTOT in COPD have produced mixed results, although certain subgroups,e.g.those with recurrent infective exacerbations requiring short-term NIV, patients aged >65 yrs, and those with uncontrolled hypercapnia on LTOT or symptomatic nocturnal hypoventilation, may benefit.At the other end of the age spectrum, children as young as a few months can be successfully treated with noninvasive ventilation. Most work on paediatric home ventilation centres on children with congenital neuromuscular disease. Pressure preset bilevel ventilators are now the dominant form of ventilator in adults and children. Discharge planning is vital for the home ventilator patient and a sensible risk management strategy should be in place.

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Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: Survival rates in a controlled trial

Cited by 257 publications

References 11 publications

Effects of Noninvasive Ventilation on Sleep Outcomes in Amyotrophic Lateral Sclerosis

Effects of Noninvasive Ventilation on Sleep Outcomes in Amyotrophic Lateral Sclerosis

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Home ventilation: Table 1—

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