Long-Term Effect of LIAM on Respiratory Performance in NIV Patients Suffering From Neuromuscular Disease

Inclusion Criteria: * o neuromuscular disease ( progressive muscular dystrophy, for example; Duchenne muscular dystrophy, spinal muscular atrophy) * age ≥ 18 years * indication to NIV (ongoing ventilation or new patients) * vital capacity ≤ 50% pred * peak cough flow \< 270 l/min * "LIAM Responder" = increased insufflation capacity (≥ + 50%) and Peak Cough Flow (PCF ≥+ 50%) with LIAM Exclusion Criteria: * o acute respiratory failure (respiratory acidosis) * home treatment by instrumental cough assistance in the preceding 12 months * ongoing medical treatment of the neuromuscular disease (for ex: corticosteroids in Duchenne disease, enzyme therapy in Pompe disease) * previous pneumothorax * plan of legal protection * pregnant or breastfeeding women * failure to cooperate * no affiliation to a social security scheme