A population based study of respiratory function in motor neuron disease patients living in Tayside and North East Fife, Scotland

Respiratory failure is a major cause of morbidity and the principal cause of death in motor neuron disease; non-invasive ventilation is increasingly used worldwide to palliate the respiratory symptoms. This observational study was designed to evaluate the prevalence of respiratory insufficiency within the motor neuron disease population of Tayside and North East Fife, Scotland. Twenty-six patients were identified, their diagnosis confirmed according to agreed criteria and subjected to the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, the Epworth Sleepiness questionnaire; spirometry, sniff nasal inspiratory pressure and nocturnal pulse oximetry measurements.

Twenty-two (84.6%) patients reported one or more symptoms of respiratory insufficiency, 19 patients (73%) had forced vital capacity <80% of predicted in the sitting position and 10 (38.5%) had oxygen saturation <90% for >5% of night. On this basis a potential 10 patients required consideration for ventilation. As well as probable improvement in quality of life and survival for those patients this potential increase in workload has major educational, management and resource implications for health care providers.