Utility of maximum inspiratory and expiratory pressures as a screening method for respiratory insufficiency in slowly progressive neuromuscular disorders

Wenninger, Stephan; Ståhl, Kristina; Wirner, Corinna; Einvag, Krisztina; Thiele, Simone; Walter, Maggie C.; Schöser, Benedikt

doi:10.1016/j.nmd.2020.06.009

Cited by 12 publications

(15 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Twenty-three studies assessed respiratory muscle strength. Eighteen used manometry [88][89][90][91][92][93][94][95][96][97][98][99][100]102,103,[108][109][110] (supplementary table 1).…”

Section: Respiratory Muscle Strengthmentioning

confidence: 99%

“…Fifteen studies of maximal expiratory pressure [88][89][90][91][92][93][96][97][98][99][100]102,103,108,110] 2D).…”

Section: Maximal Inspiratory and Expiratory Pressuresmentioning

confidence: 99%

“…and seventeen studies of maximal inspiratory pressure[88][89][90][91][92][93][95][96][97][98][99][100]102,103,108,109,110] were reported. Maximal expiratory pressure mean values were between 35.5 cmH 2 O to 71 cmH 2 O in patients with DM1 and 133.8±28 cmH 2 O in controls [88,90-92,97-100,102,103,108,110].…”

mentioning

confidence: 99%

“…Data of the different methods and outcome measures used to assess respiratory muscle strength in patients with DM1[88][89][90][91][92][93][94][95][96][97][98][99][100][101][102][103][107][108][109][110][111][112][113].…”

mentioning

confidence: 99%

See 3 more Smart Citations

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

Mateus

Costa

Viegas

et al. 2022

Neuromuscular Disorders

View full text Add to dashboard Cite

This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

show abstract

“…Twenty-three studies assessed respiratory muscle strength. Eighteen used manometry [88][89][90][91][92][93][94][95][96][97][98][99][100]102,103,[108][109][110] (supplementary table 1).…”

Section: Respiratory Muscle Strengthmentioning

confidence: 99%

“…Fifteen studies of maximal expiratory pressure [88][89][90][91][92][93][96][97][98][99][100]102,103,108,110] 2D).…”

Section: Maximal Inspiratory and Expiratory Pressuresmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

Mateus

Costa

Viegas

et al. 2022

Neuromuscular Disorders

View full text Add to dashboard Cite

show abstract

“…Forced Vital Capacity (FVC)). Longitudinal studies on the decline of respiratory muscle strength have been performed in other NMDs but not in SMA (14). Therefore, we studied the natural history of respiratory muscle strength and assessed differences between SMA types in a large, population-based, treatment-naïve cohort of SMA patients .…”

Section: Introductionmentioning

confidence: 99%

Natural History of Respiratory Muscle Strength in Spinal Muscular Atrophy: A Prospective National Cohort Study

Veldhoen

Wijngaarde

Hulzebos

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Respiratory complications are the most important cause of morbidity and mortality in Spinal Muscular Atrophy (SMA). Respiratory muscle weakness results in impaired cough, recurrent respiratory tract infections and finally respiratory failure. We assessed longitudinal patterns of measurements of respiratory muscle strength in a national cohort of treatment-naïve children and adults with SMA, hypothesizing a continued decline of respiratory muscle strength parameters throughout life. Methods: We measured Maximal Expiratory and Inspiratory Pressure (PEmax and PImax), Sniff Nasal Inspiratory Pressure (SNIP), Peak Expiratory Flow (PEF), and Peak Cough Flow (PCF) in treatment-naïve patients. We used both cross-sectional and longitudinal data in mixed-models to analyze natural history patterns. Results: We included 2756 measurements of respiratory muscle function from 80 treatment-naïve patients with SMA types 1c-3b. Both PEmax and PImax were significantly lower in more severe phenotypes. SNIP was low in nearly all patients, most pronounced in more severely affected patients. PEmax was low from early childhood onwards in patients with SMA type 1c–3a. PEF decline below 80% of predicted values was observed in early childhood in SMA types 1c-2 and in adolescence in patients with type 3a. Annual decline was linear (1-2%/year). All but type 3b patients had lowered PCF. Patients with types 2b and 3a had PCF levels between 160 and 270L/min, those with type 2a around 160L/min and patients with type 1c well below 160L/min. Conclusions: There are clear differences in respiratory muscle strength and its progressive decline between SMA types. We observed lower outcomes in more severe SMA types. Particularly PEmax and PEF may be suitable outcome measures for follow-up of patients with SMA. PEmax had the highest discriminative capacity and PEF declined in a rather linear pattern in all SMA types. PEmax was even low in patients with normal lung volumes. These natural history data may serve as a reference for longer-term treatment efficacy assessments.

show abstract

Respiratory Management in Acute Neuromuscular Disease

Damian

Wijdicks

2022

Emergencies in Neuromuscular Disorders

View full text Add to dashboard Cite

Utility of maximum inspiratory and expiratory pressures as a screening method for respiratory insufficiency in slowly progressive neuromuscular disorders

Cited by 12 publications

References 26 publications

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

Natural History of Respiratory Muscle Strength in Spinal Muscular Atrophy: A Prospective National Cohort Study

Respiratory Management in Acute Neuromuscular Disease

Contact Info

Product

Resources

About