Background: Neuromuscular diseases cover a wide range of pathologies, which are slowly or rapidly progressive. Besides the reduced capacity of the inspiratory muscles there is a limitation of expiration which compromises coughing. Ventilatory respiratory insufficiency (VRI) develops in stages, beginning during sleep with hypercapnia manifesting later on. Several retrospective studies have shown an improved alveolar ventilation, survival and quality of life for most NMD, even in non-bulbar ALS. The initiation of NIV is seen as apropriate when VRI in sleep develops. But patient acceptance to use NIV for more than 4 hours must be taken into account. NIV is limited by the interface which may cause skin abrasions or leakage due to pressure. NIV may be used day and night in stable conditions and with assistance experienced in mechanically-assisted coughing (MAC). The discussion of whether to perform tracheostomy remains a preeminent topic especially in ALS.
Conclusion: NIV is effective in NMD and improves and prolongs life in slowly progressive NMD. Mask problems, leakage and bulbar dysfunction can limit the effectiveness. MAC is a tool that is very useful for elimination of bronchial secretions.