The role of facemask spirometry in motor neuron disease

Thorax

Respiratory failure is the most frequent cause of death in people with motor neuron disease (MND). Vital capacity and maximal inspiratory and expiratory mouth pressures are the methods most commonly used to assess respiratory muscle impairment. Forced vital capacity (FVC) at diagnosis, and the rate of decline, are predictors of survival. An FVC of <50% predicted is proposed by the National Institute of Clinical Excellence (NICE, UK) as an indication of the need for evaluation for non-invasive ventilation.1

For many patients with MND who have facial or bulbar muscle weakness, standard spirometry with a mouthpiece or tube is inaccurate due to mouth leaks as they are unable to effectively seal their lips around the tube/mouthpiece. Sniff nasal inspiratory pressure (SNIP), and maximal inspiratory mouth pressure (PiMax) may be preferable, but are less widely available and not always successful.2 Mask spirometry has been used but not...