Evidence base: Motor neurone disease

A summary of the key trials, guidelines, key texts and online resources to help in the management of motor neurone disease.

Patient with motor neurone disease on a ventilator (Photograph: SPL)
Patient with motor neurone disease on a ventilator (Photograph: SPL)


This Cochrane review of riluzole treatment of ALS concludes that riluzole 50mg twice daily does provide a modest survival benefit in ALS.

  • Bourke SC, Tomlinson, M, Wiliams 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: 140-7.

This trial of NIV in ALS gives good evidence that NIV confers a modest survival benefit and improves quality of life.

This Cochrane review of the evidence supporting enteral feeding in ALS concluded that there may be a survival benefit.



  • Talbot K, Turner MR, Marsden R et al. Motor Neuron Disease. A Practical Manual. Oxford University Press, Oxford. 2009.

This book is a good and concise guide to the care of MND patients.

  • Lindquist UC. Rowing without oars. John Murray, London, 2005.

This is a moving patient account of living with MND.


The RCGP covers this topic in statement 15.7 Neurological problems.


  • The website of the MND Association provides information about MND for healthcare professionals and patients. www.mnda.org
  • The ALS Association, which is based in the US, is another very well respected resource. www.alsa.org

Contributed by Dr Clare Wood-Allum, lecturer in neurology and honorary registrar, and Dr Christopher McDermott, lecturer and consultant neurologist for University of Sheffield and Sheffield Teaching Hospitals NHS Trust.

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