Respiratory management of motor neurone disease: A review of current practice and new developments

Abstract

Motor neurone disease is a neurodegenerative condition with a signifi cant morbidity and shortened life expectancy. Hypoventilatory respiratory failure is the most common cause of death and respiratory function signifi cantly 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 signifi cant 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.