Non-invasive ventilation in motor neuron disease: current UK practice

Amyotroph Lateral Scler Other Motor Neuron Disord. 2002 Sep;3(3):145-9. doi: 10.1080/146608202760834157.


Objective: To evaluate the clinical application of non-invasive ventilation (NIV) in motor neuron disease (MND) in the UK.

Method: We conducted a postal survey of the current clinical practice of all consultant neurologists in the UK, with a second mailing to non-responders after 6 weeks. The principal outcome measures assessed were: 1. the percentage of patients with MND receiving NIV, 2. access to a NIV service, 3. routine monitoring of respiratory function, and 4. influence of symptoms, respiratory function, rate of disease progression, level of disability and bulbar involvement on referral for NIV.

Results: The response rate was 76%, 1719 new patients had been diagnosed in the preceding 12 months and a total of 2280 patients were under review. Of these, 126 were currently receiving NIV (5.5% of patients under review, estimated 2.6-3.5% of all MND patients). Most neurologists (172/265) had referred no patients for NIV in the preceding year, while three neurologists made 30% of all referrals nationally. Referral was based primarily on symptoms, and was influenced by the number of MND patients under review, level of disability, rate of disease progression and availability of a NIV service. Bulbar involvement was considered a relative contra-indication to NIV by 51% of responders.

Conclusions: In the UK, few patients with MND are treated with NIV. There is marked variation in clinical practice. This may reflect uncertainty about the role of non-invasive ventilation in MND, and emphasizes the need for a randomized controlled trial to assess the impact of NIV on quality of life and survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Humans
  • Motor Neuron Disease / complications
  • Motor Neuron Disease / epidemiology
  • Motor Neuron Disease / therapy*
  • Neurology
  • Positive-Pressure Respiration / methods
  • Positive-Pressure Respiration / statistics & numerical data*
  • Professional Practice / statistics & numerical data
  • Professional Practice / trends*
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / therapy*
  • Surveys and Questionnaires
  • United Kingdom / epidemiology