Advertisement

Neuromuscular Patients (Amyotrophic Lateral Sclerosis and Other Conditions)

  • Giuseppe FiorentinoEmail author
  • Antonio M. Esquinas
  • Anna Annunziata
Chapter

Abstract

Many progressive neuromuscular disorders involve muscles of the respiratory system which are involved in breathing and coughing. Ventilatory assistance becomes an important part of management for patients with advanced neuromuscular disease. The combination of NIV with cough-assist techniques decreases pulmonary morbidity and hospital admissions.

Technological advancements in NIV have led to a significant improvement in ability to treatment these patients.

Abbreviations

ALS

Amyotrophic lateral sclerosis

COPD

Chronic obstructive pulmonary disease

DM1

Myotonic dystrophy type 1

DMD

Duchenne muscular dystrophy

NIV

Non-invasive ventilation

NMD

Neuromuscular disorders

NPPV

Non-invasive positive pressure ventilation

OSA

Obstructive sleep apnoea syndrome

PEEP

Positive end-expiratory pressure

PSV

Pressure support ventilation

QoL

Quality of life

SDB

Sleep disorder breathing

SMA

Spinal muscular atrophy

References

  1. 1.
    Ambrosino N, Carpenè N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J. 2009;34(2):444–51.CrossRefGoogle Scholar
  2. 2.
    de Visser M, Oliver DJ. Palliative care in neuromuscular diseases. Curr Opin Neurol. 2017;30(6):686–91.CrossRefGoogle Scholar
  3. 3.
    Bach JR. Noninvasive respiratory management of patients with neuromuscular disease. Ann Rehabil Med. 2017;41(4):519–38.CrossRefGoogle Scholar
  4. 4.
    Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest. 1998;112:1024–8.CrossRefGoogle Scholar
  5. 5.
    Raphael JC, Chevret S, Chastang C, et al. Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. Lancet. 1994;343:1600–4.CrossRefGoogle Scholar
  6. 6.
    Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5(2):140–7.CrossRefGoogle Scholar
  7. 7.
    Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest. 1993;103:174–82.CrossRefGoogle Scholar
  8. 8.
    Rabinstein AA. Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid. Curr Opin Crit Care. 2016;22(2):94–9.PubMedGoogle Scholar
  9. 9.
    Burns TM, Graham CD, Rose MR, et al. Quality of life and measures of quality of life in patients with neuromuscular disorders. Muscle Nerve. 2012;46(1):9–25.CrossRefGoogle Scholar
  10. 10.
    Lacombe M, Del Amo Castrillo L, 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):215–22.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Giuseppe Fiorentino
    • 1
    Email author
  • Antonio M. Esquinas
    • 2
  • Anna Annunziata
    • 1
  1. 1.UOC di Fisiopatologia e Riabilitazione RespiratoriaMonaldi HospitalNaplesItaly
  2. 2.Intensive Care Unit and Non Invasive Ventilatory UnitHospital Morales MeseguerMurciaSpain

Personalised recommendations