Effects of Alterations in Pulmonary Function and Sleep Variables on Survival in Patients With Amyotrophic Lateral Sclerosis

Gay, Peter C.; Westbrook, Philip R.; Daube, Jasper R.; Litchy, William J.; Windebank, Anthony J.; Iverson, Rita

doi:10.1016/s0025-6196(12)62080-1

Cited by 114 publications

(99 citation statements)

References 22 publications

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“…The commonest form of sleep-disordered breathing in patients with respiratory muscle weakness is hypoventilation due to reduced tidal volume, particularly during REM sleep [3,4]. Similar REM-related hypoventilation has been described in several other situations (e.g.…”

Section: Sleep Breathing Disorders In Neuromuscular Diseasementioning

confidence: 64%

“…In some studies predominantly obstructive events have been reported, either in a substantial proportion of subjects [7,10,38] or overall [4,8,40]. Obstructive events are more likely in subjects with a high body mass index (BMI) [40], who snore [7,40], or have anatomical abnormalities such as macroglossia (e.g.…”

Section: Sleep Breathing Disorders In Neuromuscular Diseasementioning

confidence: 99%

“…Respiratory muscle weakness and sleep-disordered breathing are both common in neuromuscular diseases such as amyotrophic lateral sclerosis (ALS) [1][2][3][4][5], muscular dystrophies (e.g. Duchenne9s muscular dystrophy (DMD) [6][7][8][9][10], and myotonic dystrophy (MD) [11][12][13][14], myopathies (e.g.…”

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confidence: 99%

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Sleep and breathing in neuromuscular disease

Bourke¹,

Gibson²

2002

Eur Respir J

151

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Respiratory muscle weakness in neuromuscular disease causes significant morbidity and mortality. The published data on respiratory muscle activity and breathing during sleep in normal subjects, the impact of respiratory muscle weakness on sleep and breathing and the relations to daytime respiratory function in neuromuscular disease are reviewed here. In normal subjects during sleep upper airway resistance increases, chemosensitivity is reduced and the wakefulness drive to breathe is lost, resulting in a fall in ventilation. During rapid eye movement (REM) sleep, ribcage and accessory breathing muscles are suppressed, particularly during bursts of eye movements, and breathing is more irregular, rapid and shallow, with a further fall in ventilation.In subjects with respiratory muscle weakness sleep is fragmented, with shorter total sleep time, frequent arousals, an increase in stage 1 sleep and a reduction in, or complete suppression of, REM sleep. Sleep-disordered breathing and nocturnal desaturation are common and most severe during REM sleep. Correlations between daytime respiratory function and nocturnal desaturation are moderate or weak, but daytime respiratory function has greater prognostic value than nocturnal measurements.Noninvasive ventilation improves sleep quality and breathing in subjects with respiratory muscle weakness. However, the optimal criteria for initiation of ventilation and its role in rapidly progressive neuromuscular diseases are unclear.

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Section: Sleep Breathing Disorders In Neuromuscular Diseasementioning

confidence: 64%

Section: Sleep Breathing Disorders In Neuromuscular Diseasementioning

confidence: 99%

See 1 more Smart Citation

Sleep and breathing in neuromuscular disease

Bourke¹,

Gibson²

2002

Eur Respir J

151

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“…29 On occasion, episodes of obstructive apnea may occur in patients with ALS, particularly in patients with bulbar symptoms, where weakness of pharyngeal muscle dilators may cause collapse of the pharyngeal wall and increased upper airway resistance. This was consistent with our study, which found that less than 20% of apneas were obstructive, which is not surprising given that none of our patients were predominantly bulbar or had bulbar onset ALS, and also that patients did not have increased BMI or neck circumference (which are associated with obstructive sleep apnea).…”

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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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“…However, a sniff nasal pressure test < 40% of predicted value (or < 60 cmH 2 O) is a significant predictor of sleep disordered breathing, nocturnal hypoxemia, hypercapnia and mortality (Fitting et al, 1999;Lyall et al, 2001b;Carrat et al, 2011). Finally, nocturnal hypoventilation and sleep-disordered breathing are common problems in ALS with the progression of the disease, and can occur even when respiratory muscle function is only mildly affected and in the presence of normal daytime gas exchange (Gay et al, 1991;Ferguson et al, 1996;Arnulf et al, 2000). Nocturnal hypoventilation is particularly severe during rapid eye movements (REM) sleep, when all postural and accessory muscles are physiologically atonic, and only the diaphragm, which may itself be impaired, is left to sustain ventilation and overcome any upper airway resistance (Ferguson et al, 1996).…”

Section: Wwwintechopencommentioning

confidence: 99%