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Ventilatory failure in chronic neuromuscular disease
  1. Gabriella Heloise Long1,
  2. Andrew Bentley1,2,
  3. Mark Roberts1,3,
  4. James B Lilleker3,4
  1. 1 North West Ventilation Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK
  2. 2 Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK
  3. 3 Muscle Diseases Unit, Salford Royal Hospital Manchester Centre for Clinical Neurosciences, Salford, UK
  4. 4 Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, University of Manchester, Manchester, UK
  1. Correspondence to Dr Gabriella Heloise Long, North West Ventilation Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, M23 9LT, UK; gabriella.long1{at}


Patients with neuromuscular diseases (NMD) can present to the neurologist with symptoms and signs of respiratory failure, either acutely or as an insidious process in the outpatient setting. Since the advent of non-invasive ventilation, the outcomes of patients with ventilatory failure due to NMD have dramatically improved. However, the natural history of different NMDs requires a nuanced approach to respiratory investigation and management. Respiratory failure dictates the prognosis of many NMDs and timing the most appropriate investigation and referral to ventilation services is crucial in optimising care.


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  • Contributors GHL, AB and JBL were involved in the design and conception of the paper. GL wrote the first draft. GHL, AB, MR and JBL edited, critically revised and approved the final manuscript for submission.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally reviewed by Jon Walters, Swansea, UK.