Pathogenesis of respiratory failure in neuromuscular diseases: diagnosis and treatment

In reviewing the pathogenesis of respiratory failure in neuromuscular diseases, with special reference to ventilatory pump, we propose specific tests of the respiratory muscle pump alongside the standard function tests for assessing the respiratory system. Accurate diagnosis must be followed by target therapy, which must be given as often and for as long as needed. Early evaluation and treatment of the respiratory problems are essential to prognosis, whether of survival or of quality of life, in neuromuscular diseases.