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

Neuromuscular Disorders : NMD
Stephan WenningerBenedikt Schoser

Abstract

The aim of this study was to assess whether different cut-offs of maximum inspiratory and/or expiratory pressure (MIP/MEP) are valuable screening parameters to detect restrictive respiratory insufficiency. Spirometry, MIP, MEP and capillary blood gas analysis were obtained from patients with confirmed neuromuscular disorders. We calculated regression analysis, sensitivity, specificity and predictive values. We enrolled 29 patients with myotonic dystrophy type 1 (DM1), 19 with late-onset Pompe disease (LOPD), and 24 with spinal muscular atrophy type 3. Moderate to high reduction in manometry was exclusively found in LOPD and DM1 patients. Significant associations were found between manometry and spirometry. Highest adjusted r2 was found for MIP % predicted and forced vital capacity (FVC) % predicted. Manometry predicted abnormal FVC and forced expiratory volume 1 s (FEV1). MEP > 80 cmH2O predicted normal FVC and FEV1, regardless of cut-off values. MIP and MEP did not positively predict alterations in capillary blood gas analysis. Disease-specific cut-offs of manometry did not increase the prediction rate of patients with abnormal FVC and FEV1. Predicted values should be calculated for a more comprehensive interpretation of manom...Continue Reading

Citations

Feb 25, 2021·Journal of Neurology·Stephan WenningerBenedikt Schoser

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