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Updated: May 21, 2026

Repeated Measurement of Respiratory Muscle Activity and Ventilation in Mouse Models of Neuromuscular Disease
Published on: April 17, 2017
Respiratory management of motor neurone disease: a review of current practice and new developments
Muhammad Khizar Rafiq1, Alison Ruth Proctor, Christopher J McDermott
1Academic Neurology Unit, Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK. m.k.rafi q@sheffi eld.ac.uk
Abstract:
Motor neurone disease is a neurodegenerative condition with a significant morbidity and shortened life expectancy. Hypoventilatory respiratory failure is the most common cause of death and respiratory function significantly predicts both survival and quality of life in patients with motor neurone disease. Accordingly, supporting and maintaining respiratory function is important in caring for these patients. The most significant advance in motor neurone disease care of recent years has been the domiciliary provision of non-invasive ventilation for treating respiratory failure. Neuromuscular respiratory weakness also leads to ineffective cough and retained airways secretions, predisposing to recurrent chest infections. In this review, we discuss current practice and recent developments in the respiratory management of motor neurone disease, in terms of ventilatory support and cough augmentation.
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