Effective airway clearance is essential for persons with neuromuscular disease. Airway clearance prevents infection and optimizes respiratory function and ventilation. The use of mechanical insufflation-exsufflation (MI-E) to promote airway clearance in persons with neuromuscular disorders is well documented. The aim of this scoping review was to identify and map the available evidence relating to physiological effects of MI-E in patients with neuromuscular disease. Electronic databases, including MEDLINE, CINAHL, EMBASE, EMCARE, SCOPUS, COCHRANE, and Web of Science, were searched from inception to November 2024. Inclusion criteria were studies involving spontaneous breathing, adult or pediatric participants with a neuromuscular diagnosis, English/translated to English articles, full-text articles, and studies involving MI-E with clear strategies. Data were extracted by 2 authors using a bespoke extraction form. A Mixed Methods Appraisal Tool was used to assess quality. Of 1,176 abstracts identified, 25 records were included. The review data suggested MI-E was well tolerated in persons with neuromuscular disease and proposed improved cough compared with participants’ baseline. Studies predominately used cough peak flow to assess MI-E effectiveness, with emerging evidence looking at upper airway dynamics. Studies specifically focused on a pediatric neuromuscular cohort are vital, as this remains an under-researched area.
KhanA, Frazer-GreenL, AminR, et al.Respiratory management of patients with neuromuscular weakness: an American college of chest physicians clinical practice guideline and expert panel report. Chest, 2023; 164(2):394–413; doi: 10.1016/j.chest.2023.03.011
2.
IrfanM, SelimB, RabinsteinAA, et al.Neuromuscular disorders and sleep in critically ill patients. Crit Care Clin, 2015; 31(3):533–550; doi: 10.1016/j.ccc.2015.03.007
3.
VolskoTA. Airway clearance therapy: finding the evidence. Respir Care, 2013; 58(10):1669–1678; doi: 10.4187/respcare.02590
4.
Fernandez-CarmonaA, Olivencia-PenaL, Yuste-OssorioME, et al.; Grupo de Trabajo de Unidad de Ventilación Mecánica Domiciliaria de Granada. Ineffective cough and mechanical mucociliary clearance techniques. Med Intensiva (Engl Ed), 2018; 42(1):50–59; doi: 10.1016/j.medin.2017.05.003
5.
EdelL, GrimeC, RobinsonV, et al.A new respiratory scoring system for evaluation of respiratory outcomes in children with spinal muscular atrophy type1 (SMA1) on SMN enhancing drugs. Neuromuscul Disord, 2021; 31(4):300–309; doi: 10.1016/j.nmd.2021.01.008
PollockDK, KhalilH, EvansC, et al.The role of scoping reviews in guideline development. J Clin Epidemiol, 2024; 169:111301; doi: 10.1016/j.jclinepi.2024.111301
9.
OuzzaniM, HammadyH, FedorowiczZ, et al.Rayyan-a web and mobile app for systematic reviews. Syst Rev, 2016; 5(1):210; doi: 10.1186/s13643-016-0384-4
10.
HongQN, Gonzalez-ReyesA, PluyeP. Improving the usefulness of a tool for appraising the quality of qualitative, quantitative and mixed methods studies, the Mixed Methods Appraisal Tool (MMAT). J Eval Clin Pract, 2018; 24(3):459–467; doi: 10.1111/jep.12884
11.
ChatwinM, RossE, HartN, et al.Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J, 2003; 21(3):502–508; doi: 10.1183/09031936.03.00048102
12.
ChatwinM, SimondsAK. The addition of mechanical insufflation/exsufflation shortens airway-clearance sessions in neuromuscular patients with chest infection. Respir Care, 2009; 54(11):1473–1479.
13.
HovB, AndersenT, ToussaintM, et al.Mechanically assisted cough strategies: user perspectives and cough flows in children with neurodisability. ERJ Open Res, 2024; 10(1):e00274–e02023; doi: 10.1183/23120541.00274-2023
14.
ShahNM, AppsC, KaltsakasG, et al.The effect of pressure changes during mechanical insufflation-exsufflation on respiratory and airway physiology. Chest, 2024; 165(4):929–941; doi: 10.1016/j.chest.2023.10.015
15.
LacombeM, Del Amo CastrilloL, BoreA, et al.Comparison of three cough-augmentation techniques in neuromuscular patients: mechanical insufflation combined with manually assisted cough, insufflation-exsufflation alone and insufflation-exsufflation combined with manually assisted cough. Respiration, 2014; 88(3):215–222; doi: 10.1159/000364911
16.
AndersenTM, SandnesA, FondenesO, et al.Laryngeal responses to mechanically assisted cough in progressing amyotrophic lateral sclerosis. Respir Care, 2018; 63(5):538–549; doi: 10.4187/respcare.05924
17.
JungJH, OhHJ, LeeJW, et al.Improvement of peak cough flow after the application of a mechanical in-exsufflator in patients with neuromuscular disease and pneumonia: a pilot study. Ann Rehabil Med, 2018; 42(6):833–837; doi: 10.5535/arm.2018.42.6.833
18.
CesareoA, LoMauroA, SantiM, et al.Acute effects of mechanical insufflation-exsufflation on the breathing pattern in stable subjects with Duchenne muscular dystrophy. Respir Care, 2018; 63(8):955–965; doi: 10.4187/respcare.05895
19.
SantosDB, BoreA, CastrilloLDA, et al.Assisted vital capacity to assess recruitment level in neuromuscular diseases. Respir Physiol Neurobiol, 2017; 243:32–38; doi: 10.1016/j.resp.2017.05.001
20.
SteindorM, PichlerA, HeitschmidtL, et al.Multiple breath washout lung function reveals ventilation inhomogeneity unresponsive to mechanical assisted cough in patients with neuromuscular disease. BMC Pulm Med, 2022; 22(1):217; doi: 10.1186/s12890-022-02012-z
21.
BachJR. Mechanical insufflation-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest, 1993; 104(5):1553–1562; doi: 10.1378/chest.104.5.1553
22.
SanchoJ, ServeraE, DiazJ, et al.Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest, 2004; 125(4):1400–1405; doi: 10.1378/chest.125.4.1400
23.
SenentC, GolmardJL, SalachasF, et al.A comparison of assisted cough techniques in stable patients with severe respiratory insufficiency due to amyotrophic lateral sclerosis. Amyotroph Lateral Scler, 2011; 12(1):26–32; doi: 10.3109/17482968.2010.535541
24.
WinckJC, GoncalvesMR, LourencoC, et al.Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest, 2004; 126(3):774–780; doi: 10.1378/chest.126.3.774
25.
SanchoJ, BuresE, de La AsuncionS, et al.Effect of high-frequency oscillations on cough peak flows generated by mechanical in-exsufflation in medically stable subjects with amyotrophic lateral sclerosis. Respir Care, 2016; 61(8):1051–1058; doi: 10.4187/respcare.04552
26.
AndersenT, SandnesA, BrekkaAK, et al.Laryngeal response patterns influence the efficacy of mechanical assisted cough in amyotrophic lateral sclerosis. Thorax, 2017; 72(3):221–229; doi: 10.1136/thoraxjnl-2015-207555
27.
KikuchiK, SatakeM, KimotoY, et al.Approaches to cough peak flow measurement with Duchenne muscular dystrophy. Respir Care, 2018; 63(12):1514–1519; doi: 10.4187/respcare.06124
28.
LalmoldaC, PradosH, MateuG, et al.Titration of mechanical insufflation-exsufflation optimal pressure combinations in neuromuscular diseases by flow/pressure waveform analysis. Arch Bronconeumol (Engl Ed), 2019; 55(5):246–251; doi: 10.1016/j.arbres.2018.10.011
29.
LacombeM, BoreA, Amo CastrilloLD, et al.Peak cough flow fails to detect upper airway collapse during negative pressure titration for cough-assist. Arch Phys Med Rehabil, 2019; 100(12):2346–2353; doi: 10.1016/j.apmr.2019.06.012
30.
PigattoAV, KaoTJ, MuellerJL, et al.Electrical impedance tomography detects changes in ventilation after airway clearance in spinal muscular atrophy type I. Respir Physiol Neurobiol, 2021; 294:103773; doi: 10.1016/j.resp.2021.103773
31.
SanchoJ, FerrerS, BuresE, et al.Waveforms analysis in patients with amyotrophic lateral sclerosis for enhanced efficacy of mechanically assisted coughing. Respir Care, 2022; 67(10):1226–1235; doi: 10.4187/respcare.09978
32.
CasaultaC, MesserliF, RodriguezR, et al.Changes in ventilation distribution in children with neuromuscular disease using the insufflator/exsufflator technique: an observational study. Sci Rep, 2022; 12(1):7009; doi: 10.1038/s41598-022-11190-z
33.
FaurouxB, GuillemotN, AubertinG, et al.Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest, 2008; 133(1):161–168; doi: 10.1378/chest.07-1615
34.
StehlingF, BouikidisA, ScharaU, et al.Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders. Chron Respir Dis, 2015; 12(1):31–35; doi: 10.1177/1479972314562209
35.
VeldhoenES, VercoelenF, RosL, et al.Short-term effect of air stacking and mechanical insufflation-exsufflation on lung function in patients with neuromuscular diseases. Chron Respir Dis, 2022; 19:14799731221094619; doi: 10.1177/14799731221094619
36.
TroxellDA, BachJR, NilsestuenJO. Mechanical insufflation-exsufflation implementation and management, aided by graphics analysis. Chest, 2023; 164(6):1505–1511; doi: 10.1016/j.chest.2023.07.007
37.
SwingwoodEL, StilmaW, TumeLN, et al.The use of mechanical insufflation-exsufflation in invasively ventilated critically ill adults. Respir Care, 2022; 67(8):1043–1057; doi: 10.4187/respcare.09704
38.
TerziN, VaugierI, GuerinC, et al.Comparison of four mechanical insufflation-exsufflation devices: effect of simulated airway collapse on cough peak flow. Respir Care, 2023; 68(4):462–469; doi: 10.4187/respcare.10086
39.
LebretM, VuillermozA, FresnelE, et al.Discussion on comparison of four mechanical insufflation-exsufflation devices: effect of simulated airway collapse on cough peak flow. Respir Care, 2023; 69(1):145–146; doi: 10.4187/respcare.11539
40.
Martinez-AlejosR, FresnelE, VuillermozA, et al.Accuracy of real-time data provided by mechanical insufflation-exsufflation devices. Respir Care, 2025; 70(3):313–318; doi: 10.4187/respcare.12221
41.
AndersenT, FondenesO, RoksundOD, et al.From bedside to bench - In vivo and in vitro evaluation of mechanically assisted cough treatment in a patient with bulbar Amyotrophic Lateral Sclerosis. Respir Med Case Rep, 2022; 37:101649; doi: 10.1016/j.rmcr.2022.101649
42.
SubbaraoP, MillaC, AuroraP, et al.Multiple-breath washout as a lung function test in cystic fibrosis. a cystic fibrosis foundation workshop report. Ann Am Thorac Soc, 2015; 12(6):932–939; doi: 10.1513/AnnalsATS.201501-021FR
43.
GallagherJT, L IvesK, CheifetzIM. Electrical impedance tomography in mechanically ventilated children: will it impact clinical care? Respir Care, 2022; 67(4):494–495; doi: 10.4187/respcare.10077
44.
KimuraS, SatoM, SanoK, et al.Electrical impedance tomography for evaluating child respiratory rehabilitation. Pediatr Int, 2023; 65(1):e15572; doi: 10.1111/ped.15572
45.
VeldhoenES, Verweij-van den OudenrijnLP, RosLA, et al.Effect of mechanical insufflation-exsufflation in children with neuromuscular weakness. Pediatr Pulmonol, 2020; 55(2):510–513; doi: 10.1002/ppul.24614
46.
VollsæterM, SkjoldmoA, RøksundO, et al.Tailoring NIV by dynamic laryngoscopy in a child with spinal muscular atrophy type I. Clin Case Rep, 2021; 9(4):1925–1928; doi: 10.1002/ccr3.3905
47.
Herrero-CortinaB, Alcaraz-SerranoV, TorresA, et al.Reliability and minimum important difference of sputum weight in bronchiectasis. Respir Care, 2020; 65(10):1478–1487; doi: 10.4187/respcare.07175
48.
YasokawaN, TanakaH, KuroseK, et al.Mechanical insufflation-exsufflation-related bilateral pneumothorax. Respir Med Case Rep, 2020; 29:101017; doi: 10.1016/j.rmcr.2020.101017
49.
McDonaldLA, BerlowitzDJ, HowardME, et al.Pneumothorax in neuromuscular disease associated with lung volume recruitment and mechanical insufflation-exsufflation. Respirol Case Rep, 2019; 7(6):e00447; doi: 10.1002/rcr2.447
50.
SuriP, BurnsSP, BachJR. Pneumothorax associated with mechanical insufflation-exsufflation and related factors. Am J Phys Med Rehabil, 2008; 87(11):951–955; doi: 10.1097/PHM.0b013e31817c181e
