Methods and prognosis of non-invasive ventilation in neuromuscular disease

Ventilatory failure is the commonest terminal event in many neuromuscular disorders. Increasingly, non-invasive methods of ventilation, employing both negative and positive pressure techniques, have been used, with the aim of prolonging life and reducing morbidity. The use of non-invasive ventilation is reviewed in three main groups of neurological diseases, and the degree of success assessed. It is concluded that non-invasive methods can be effective in reducing morbidity in selected patients, especially those with more slowly progressive disorders. A reduction in mortality is suggested by comparison with historical controls. The institution of ventilatory support is a major undertaking for the medical team, patient and family, and should only be initiated after a full and frank discussion of the overall prognosis, in terms of both mortality and quality of life. The patient and principal caregivers can then make an informed decision as to the desirability of home ventilation.