[Current approach to respiratory problems in neuromuscular diseases]

Neuromuscular diseases lead to respiratory failure as they cause progressive weakness of the muscle of respiration. Studies show that noninvasive mechanical ventilation improves symptoms, gas exchange, quality of life, and survival in these patients. Noninvasive mechanical ventilation should be use at the onset of nocturnal hypoventilation. Airway clearance therapy is very important in these patients. The combination of noninvasive mechanical ventilation with cough-assist techniques decreases pulmonary morbidity and hospital admissions. Invasive mechanical ventilation is indicated for patients who require continuous mechanical ventilation for longer than a few days, or those who have contraindications to noninvasive mechanical ventilation. Diaphragmatic pacemakers may be a treatment option in patients with high spinal cord injuries. Episodes of acute respiratory failure in neuromuscular diseases are precipitated by respiratory infections. Management of episodes of acute respiratory failure may require endotracheal intubation.