Postural breathing pattern changes in patients with myotonic dystrophy

Flow and airway pressure analysis for detecting ineffective effort during mechanical ventilation in neuromuscular patients

“…Marked respiratory effort instability is a well-known feature of MD1 16,17 and this population frequently presents irregular breathing during wakefulness. 18 This may increase the difficulty in ventilator settings adaptations in order to avoid IE, especially the inspiratory trigger sensitivity and the inspiratory pressure level which are usually adjusted with the patient's usual and relatively stable inspiratory effort. This foreseeable difficulty of ventilator settings adaptations in this subpopulation due to the instability of the ventilator control, may partly explain the important nonadherence to NIV which in turn may jeopardize the survival of DM1 patients.…”

“…These patients had neither severe inspiratory dysfunction nor severe leaks. However, as MD1 patients present respiratory effort instability, [16][17][18] the lower sensitivity in these two patients is ascribable to a small number of inspiratory efforts detectable only by Pes measurement. Despite these false negatives, the overall good performance of the noninvasive method translated into a high NPV.…”

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

“…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).…”

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

“…Three studies analysed peak cough flow [89,92,93] and one study evaluated cough Pgas [92] (supplementary table 1). Mean results obtained for peak cough flow were between 280 L/min to 284.7 L/min in patients with DM1 and 547.4±156.6 L/min in controls [92] (table 3).…”