Conséquences respiratoires chroniques des maladies neuromusculaires

Reviewing respiration pathophysiology allows understanding how a patient affected by a neuromuscular disease may present respiratory symptoms and which treatment can be effective. Affection of inspiration muscles, especially the diaphragm, generates a restrictive syndrome and early sleep disorders. Precise and simple investigations allow evaluating the extent of respiratory involvement and the indication for ventilatory support. The impairment of expiratory muscles has consequences, often neglected, such as impaired cough capacity and speech. Again, evaluation is useful in determining which treatment can be initiated. Finally, swallowing impairment should not be underestimated as it may compromise pulmonary function. Likewise, a thromboembolic disease does not be underestimated in patients with multiple risk factors. We exhaustively reviewed the different tools available for evaluation and treatment of the respiratory consequences of neuromuscular diseases. Their use should be considered for every patient presenting with a neuromuscular disease; multidisciplinary management should be implemented, involving a pneumologist.