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Chronic neuromuscular diseases affect the respiratory muscles in varying patterns and degrees. As a result, patients with these disorders develop restrictive pulmonary disease, ineffective cough, atelectasis and pneumonia, and chronic respiratory insufficiency leading to respiratory failure. Therapeutic strategies are evolving to augment cough and airway clearance, improve lung volumes, and support the patient with progressive ventilatory failure. These techniques have improved longevity and quality of life for many patients with neuromuscular disease.