Caring for patients with neuromuscular disease (NMD) is challenging. Respiratory care is of the utmost importance because it is a major determinant of quality of life and survival. Noninvasive ventilation (NIV) is one of the few modalities that has shown survival benefit in the NMD patient population. Newer modes with smart technologies are being developed to assist in better ventilation. Some noninvasive methods have shown success in the management of sialorrhea, which is of paramount importance in the initiation of NIV. This review will summarize the management of respiratory symptomatology in patients with NMD with recent advances made in NIV.
PerrinC, UnterbornJN, AmbrosioCD, HillNS. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve, 2004; 29(1):5–27.
2.
GargN, ParkSB, VucicS, YiannikasC, SpiesJ, HowellsJ, et al. Differentiating lower motor neuron syndromes. J Neurol Neurosurg Psychiatry, 2017; 88(6):474–483.
3.
BanfiP, TicozziN, LaxA, GuidugliGA, NicoliniA, SilaniV. A review of options for treating sialorrhea in amyotrophic lateral sclerosis. Respir Care, 2015; 60(3):446–454.
4.
OdachiK, NaritaY, MachinoY, YamadaT, NishimuraY, OtaY, et al. Efficacy of transdermal scopolamine for sialorrhea in patients with amyotrophic lateral sclerosis. Cogent Med, 2017; 4:1365401.
5.
SquiresN, HumberstoneM, WillsA, ArthurA. The use of botulinum toxin injections to manage drooling in amyotrophic lateral sclerosis/motor neurone disease: A systematic review. Dysphagia, 2014; 29:500–508.
6.
ContarinoMF, PompiliM, TittotoP, VanacoreN, SabatelliM, CedroneA, et al. Botulinum toxin B ultrasound-guided injections for sialorrhea in amyotrophic lateral sclerosis and Parkinson's disease. Parkinsonism Relat Disord, 2007; 13(5):299–303.
7.
Shariat-MadarB, ChunRH, SulmanCG, ConleySF. Safety of ultrasound-guided botulinum toxin injections for sialorrhea as performed by pediatric otolaryngologists. Otolaryngol Head Neck Surg, 2016; 154(5):924–927.
8.
HawkeyNM, ZaorskyNG, GallowayTJ. The role of radiation therapy in the management of sialorrhea: a systematic review. Laryngoscope, 2016; 126(1):80–85.
9.
AssoulineA, LevyA, Abdelnour-MalletM, Gonzalez- BermejoJ, LengletT, ForestierNL, et al. Therapy for hypersalivation: A prospective study in 50 amyotrophic lateral sclerosis patients. Int J Radiat Oncol Biol Phys, 2014; 88(3):589–595.
ChaushuG, DoriS, SelaBA, TaicherS, KronenbergJ, TalmiYP. Salivary flow dynamics after parotid surgery: A preliminary report. Otolaryngol Head Neck Surg, 2001; 124(3):270–273.
12.
GreensmithAL, JohnstoneBR, ReidSM, HazardCJ, JohnsonHM, ReddihoughDS. Prospective analysis of the outcome of surgical management of drooling in the pediatric population: A 10-year experience. Plast Reconstr Surg, 2005; 116(5):1233–1242.
13.
OzturkK, ErdurO, GulO, OlmezA. Feasibility of endoscopic submandibular ganglion neurectomy for drooling. Laryngoscope, 2017; 127(7):1604–1607.
14.
HillelA, MillerR. Bulbar amyotrophic lateral sclerosis: Patterns of progression and clinical management. Head Neck, 1989; 11(1):51–59.
15.
NonakaM, ImaiT, ShintaniT, KawamataM, ChibaS, MatsumotoH. Non-invasive positive pressure ventilation for laryngeal contraction disorder during sleep in multiple system atrophy. J Neurol Sci, 2006; 247(1):53–58.
16.
MathewA, MathewMC, ThomasM, AntonisamyB. The efficacy of diazepam in enhancing motor function in children with spastic cerebral palsy. J Trop Pediatr, 2005; 51(2):109–113.
17.
LechtzinN, WienerCM, ShadeDM, ClawsonL, DietteGB. Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest, 2002; 121(2):436–442.
18.
JacksonCE, RosenfeldJ, MooreDH, BryanWW, BarohnRJ, WrenchM, et al. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci, 2001; 191(1-2):75–78.
National Institute for Health and Care Excellence. Motor neurone disease: Assessment and management (NICE guideline NG42). http://nice.org.uk/guidance/ng42. Accessed April 4, 2018.
21.
FinderJD, BirnkrantD, CarlJ, FarberH, GozalD, IannacconeST, et al. Respiratory care of the patient with Duchenne muscular dystrophy: An official ATS consensus statement. Am J Respir Crit Care Med, 2004; 170:456–465.
22.
AndrewsJ, SoimA, PandyaS, WestfieldC, CiafaloniE, FoxD, et al. Respiratory care received by individuals with Duchenne muscular dystrophy from 2000 to 2011. Respir Care, 2016; 61(10):1349–1359.
23.
LechtzinN, ScottY, BusseA, ClawsonL, KimballR, WienerC. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler, 2007; 8:185–188.
24.
AndersenPM, AbrahamsS, BorasioGD, de CarvalhoM, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)–revised report of an EFNS task force. Eur J Neurol, 2012; 19(3):360–375.
25.
SelimBJ, WolfeL, ColemanJM3rd, DewanNA. Initiation of noninvasive ventilation for sleep related hypoventilation disorders: advanced modes and devices. Chest, 2018; 153(1):251–265.
26.
SchrothMK. Special considerations in the respiratory management of spinal muscular atrophy. Pediatrics, 2009; 123(Suppl 4):S245–S249.
27.
SchorrF, GentaPR, GregorioMG, Danzi-SoaresNJ, Lorenzi-FilhoG. Continuous positive airway pressure delivered by oronasal mask may not be effective for obstructive sleep apnoea. Eur Respir J, 2012; 40:503–505.
28.
NgJR, AiyappanV, MercerJ, CatchesidePG, Chai-CoetzerCL, McEvoyRD, et al. Choosing an oronasal mask to deliver continuous positive airway pressure may cause more upper airway obstruction or lead to higher continuous positive airway pressure requirements than a nasal mask in some patients: A case series. Clin Sleep Med, 2016; 12(9):1227–1232.
29.
SharmaR, WolfeL. Use of non-invasive ventilation in neuromuscular disease. Curr Sleep Med Rep, 2017; 3:290.
30.
SheersN, BerlowitzD, RautelaL, BatchelderI, HopkinsonK, HowardM. Improved survival with an ambulatory model of non-invasive ventilation implementation in motor neuron disease. Amyotrophic Lateral Scler Frontotemporal Degener, 2014; 5(3-4):180–184.
31.
NicholsonTT, SmithSB, SiddiqueT, SufitR, Ajroud-DrissS, ColemanJM3rd, et al. Respiratory pattern and tidal volumes differ for pressure support and volume-assured pressure support in amyotrophic lateral sclerosis. Ann Am Thorac Soc, 2017; 14(7):1139–1146.
32.
SchwabR, BadrS, EpsteinL, GayP, GozalD, MalcolmK, et al. An official American Thoracic Society Statement: Continuous positive airway pressure adherence tracking systems. 2013; 188(5):613–620.
33.
BerryRB, ChediakA, BrownLK, FinderJ, GozalD, IberC, et al. Best clinical practices for the sleep center adjustment of noninvasive positive pressure ventilation (NPPV) in stable chronic alveolar hypoventilation syndromes. J Clin Sleep Med, 2010; 6(5):491–509.
34.
Lo CocoD, MarcheseS, PescoMC, La BellaV, PiccoliF, Lo CocoA. Noninvasive positive-pressure ventilation in ALS: Predictors of tolerance and survival. Neurology, 2006; 67(5):761–765.
35.
BermejoJG, PanziniCM, ArnolN, MeiningerV, KraouaS, SalachasF, et al. Prognostic value of efficiently correcting nocturnal desaturations after one month of non-invasive ventilation in amyotrophic lateral sclerosis: A retrospective monocentre observational cohort study. Amyotroph Lateral Scler Frontotemporal Degener,, 2013; 14:373–379.
36.
DME MAC LCDs: CIGNA Government Services (L11518) dated Feb 4, 2011; National Government Services (L27230), dated Oct 1, 2011; National Heritage Insurance Company (L11504), dated Feb 4, 2011; and Noridian Administrative Services (L171), dated Oct 1, 2011.
37.
RokadiaHK, AdamsJR, McCarthyK, AboussouanLS, Mireles-CabodevilaE. Cough augmentation in a patient with neuromuscular disease. Annals Am Thorac Soc, 2015; 12(12):1888–1891.
38.
SchmittJK, StiensS, TrincherR, LamM, SarkaratiM, LinderS. Survey of use of the insufflator-exsufflator in patients with spinal cord injury. J Spinal Cord Med, 2007; 30(2):127–130.
39.
VianelloA, CorradoA, ArcaroG, GallanF, OriC, MinuzzoM, et al. Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil. 2005; 84(2):83–88.
40.
AndersenT, SandnesA, FondenesO, NilsenR, TysnesOB, HeimdalJH, et al. Laryngeal responses to mechanically assisted cough in deteriorating amyotrophic lateral sclerosis. Respir Care, 2018; 63(5):538–549.
41.
AugerC, HernandoV, GalmicheH. Use of mechanical insufflations-exsufflation devices for airway clearance in subjects with neuromuscular disease. Respir Care, 2017; 62(2):236–245.
42.
KimSM, ChoiWA, WonYH, KangSW. A comparison of cough assistance techniques in patients with respiratory muscle weakness. Yonsei Med J, 2016; 57(6):1488–1493.
43.
RafiqM, BradburnM, ProctorA, BillingsC, BianchiS, McdermottC, et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener, 2015; 16:448–455.
44.
SiriwatR, DeerojanawongJ, SritippayawanS, HantragoolS, CheanprapaiP. Mechanical insufflation-exsufflation versus conventional chest physiotherapy in children with cerebral palsy. Respir Care, 2018; 63(2):187–193.
45.
LangeDJ, LechtzinN, DaveyC, DavidW, Heiman-PattersonT, GelinasD, et al. High-frequency chest wall oscillation in ALS: An exploratory randomized, controlled trial. Neurology, 2006; 67(6):991–997.
46.
LechtzinN, WolfeLF, FrickKD. The impact of high-frequency chest wall oscillation on healthcare use in patients with neuromuscular diseases. Ann Am Thorac Soc, 2016; 13(6):904–909.
47.
McKimD, GrillerN, LeBlancC, WoolnoughA, KingJ. Twenty-four-hour noninvasive ventilation in Duchenne muscular dystrophy: A safe alternative to tracheostomy. Can Respir J, 2013; 20(1):e5–e9.
48.
ToussaintM, SteensM, WasteelsG, SoudonP. Diurnal ventilation via mouthpiece: Survival in end-stage Duchenne patients. Eur Respir J, 2006; 28:549–555.
49.
ClemensKE, KlaschikE. Morphine in the management of dyspnoea in ALS. A pilot study. Eur J Neurol, 2008; 15:445–450.
50.
AbernethyAP, McDonaldCF, FrithPA, ClarkK, HerndonJE2nd, MarcelloJ, et al. Effect of palliative oxygen versus room air in relief of breathlessness in patients with refractory dyspnoea: A double-blind, randomised controlled trial. Lancet, 2010; 376:784–793.
