| Summary: | Motor neurone disease (MND) is a fatal neurodegenerative disease. It is characterised by the onset of symptoms and signs of degeneration of primarily the upper and lower motor neurones. This leads to progressive weakness of the bulbar, limb, thoracic and abdominal muscles. Respiratory muscle weakness resulting in respiratory impairment is a major feature of MND, and is a strong predictor of quality of life and survival. Non-invasive ventilation can improve symptoms and signs related to respiratory impairment and hence survival. There is currently no evidence-based guideline for use in England, Wales and Northern Ireland that addresses the use of non-invasive ventilation in patients with MND. This guideline considers the signs and symptoms that can be used for predicting respiratory impairment in patients with MND, the diagnostic accuracy of investigations for detecting and monitoring respiratory impairment, the clinical and cost effectiveness of non-invasive ventilation for treating respiratory impairment and the information and support needs of patients and their families and carers relating to the use of non-invasive ventilation
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