Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis

Lyall, Rebecca; Donaldson, Nora; Polkey, Michael I.; Leigh, P. Nigel; Moxham, John

doi:10.1093/brain/124.10.2000

Cited by 341 publications

(262 citation statements)

References 48 publications

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“…However, patients with neuromuscular diseases characterised by facial or bulbar muscle weakness, e.g. amyotrophic lateral sclerosis, myasthenia gravis, myotonic dystrophy and facioscapulohumeral muscular dystrophy, often cannot effectively seal the lips around the mouthpiece, especially during forced manoeuvres such as spirometric and maximal respiratory pressure measurements [9,10]. In these cases, the conventional equipment with a tube or flanged type of mouthpiece is not adequate to evaluate respiratory muscle function.…”

mentioning

confidence: 99%

Face mask spirometry and respiratory pressures in normal subjects

Wohlgemuth¹,

Kooi²,

Hendriks³

et al. 2003

Eur Respir J

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Spirometry and maximal respiratory pressures are pulmonary function parameters commonly used to evaluate respiratory function. Prediction values are available for conventional lung function devices using a standard tube or flanged type of mouthpiece connection. This equipment is not suitable for patients with facial or buccal muscle weakness, because of air leakage around the mouthpiece. A face mask was used for the portable lung function devices used in the neuromuscular department.The aim of this study was to compare the face mask and the conventional mouthpiece for the measurement of spirometry and of respiratory pressures in 22 healthy subjects.Values obtained with the conventional mouthpiece differed significantly from values obtained with the face mask. With the mask, forced vital capacity and forced expiratory volume in one second were 200 mL lower, and maximal expiratory pressure was 3.2 kPa lower than with the mouthpiece. Subsequently, new prediction values for face mask spirometry and maximal respiratory pressures were obtained from 252 other healthy subjects, from which new prediction equations were derived.It was concluded that the face mask connection to the lung function device is a valid alternative, is easy to use and is most useful to monitor changes in patients. This study confirms the importance of appropriate prediction equations, depending on subjectinstrument interfaces. Measurement of vital capacity (VC) and maximal respiratory pressures is commonly used to evaluate respiratory function and respiratory muscle strength in patients with chronic obstructive pulmonary disease or neuromuscular diseases [1][2][3]. Portable devices are useful for rapid and simple assessment of respiratory function in the office and at the bedside. Prediction values of spirometry and maximal respiratory pressures are available for portable and nonportable devices with a standard tube or a flanged type of mouthpiece connection [4][5][6][7][8]. This equipment is only suitable for subjects capable of making a good seal with their lips around the mouthpiece. However, patients with neuromuscular diseases characterised by facial or bulbar muscle weakness, e.g. amyotrophic lateral sclerosis, myasthenia gravis, myotonic dystrophy and facioscapulohumeral muscular dystrophy, often cannot effectively seal the lips around the mouthpiece, especially during forced manoeuvres such as spirometric and maximal respiratory pressure measurements [9,10]. In these cases, the conventional equipment with a tube or flanged type of mouthpiece is not adequate to evaluate respiratory muscle function. Therefore, a face mask adjusted to a portable spirometer and manometer was used to minimise air leakage in neuromuscular patients.The objective of this study was two-fold. First, possible differences in measurements between the original devices with the conventional mouthpiece and the face mask-adjusted devices were studied in healthy subjects. Secondly, a study to render new prediction equations for face mask spirometry and maximal re...

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mentioning

confidence: 99%

Face mask spirometry and respiratory pressures in normal subjects

Wohlgemuth¹,

Kooi²,

Hendriks³

et al. 2003

Eur Respir J

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“…This finding is of great clinical significance given the rapidly degenerating nature of ALS. Maintaining MEP, maximum inspiratory pressure, and FVC have been shown to be highly predictive of 1‐year survival and are associated with tracheostomy‐free survival in ALS 18. Additionally, expiratory muscle weakness has been indicated as a causative factor in the inability to generate adequate cough flow rates and has been associated with concomitant inspiratory muscle weakness in ALS 19.…”

Section: Discussionmentioning

confidence: 99%

Respiratory training in an individual with amyotrophic lateral sclerosis

Tabor

Rosado

Robison

et al. 2016

Ann Clin Transl Neurol

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We examined the impact of expiratory muscle strength training on maximum expiratory pressure, cough spirometry, and disease progression in a 71‐year‐old male with amyotrophic lateral sclerosis. Maximum expiratory pressure declined 9% over an 8‐week sham training period, but subsequently improved by 102% following 8 weeks of expiratory muscle strength training. Improvements in cough spirometry and mitigated disease progression were also observed post expiratory muscle strength training. Improvements in maximum expiratory pressures were maintained 6 months following expiratory muscle strength training and were 79% higher than baseline data obtained 301 days prior. In this spinal‐onset amyotrophic lateral sclerosis patient, respiratory training improved subglottic air pressure generation and sequential cough generation.

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“…During an effective cough, the expiratory flow rate increases and secretions in the airway are moved towards the buccal cavity 13,18,21) . Alone, isometric training of the pelvis elevator muscles is unlikely to improve cough force, since in addition to expiratory muscle strength, factors that determine cough effectiveness include: the ability to instantaneously increase intrathoracic pressure 22) ; contraction of the expiratory muscles to increase this pressure 13) ; timing of glottis opening 23) ; and bulbar function 6,20,24) .…”

Section: Discussionmentioning

confidence: 99%

The Effects of Strength Training of Pelvis Elevator Muscles on Exhalation Capacity in Healthy Men

2007

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Abstract. The effects on expiratory muscle strength and exhalation capacity of increased pelvis elevator muscles strength caused by strength training are not known. Twenty-six male students were randomized to either a training group or a control group. The training group had pelvis elevator muscles strength training twice daily for 4 weeks. At the beginning and end of the study, pelvis elevator muscle strength, maximum expiratory muscle strength (PE max ), and peak cough flow (PCF) were measured, and pulmonary function testing (forced vital capacity (FVC), forced expiratory volume in 1 s (FEV 1.0 ), peak expiratory flow rate (PFR)) was performed. In the training group, significant increases were seen in pelvis elevator muscles strength, PE max , and FEV 1.0 ; no changes were seen in FVC, PFR, or PCF. No significant correlation was seen between change in pelvis elevator muscles strength and changes in exhalation capacity parameters in the training group. Strength training of the pelvis elevator muscles increases respiratory muscle strength, which would suggest that such training could increase exhalation capacity. However, the increase in pelvis elevator muscles strength that occurred as a result of training could not fully account for the changes in expiratory muscle strength or other parameters of exhalation capacity noted after training.

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Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis

Cited by 341 publications

References 48 publications

Face mask spirometry and respiratory pressures in normal subjects

Face mask spirometry and respiratory pressures in normal subjects

Respiratory training in an individual with amyotrophic lateral sclerosis

The Effects of Strength Training of Pelvis Elevator Muscles on Exhalation Capacity in Healthy Men

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