Move Toward to Non Invasive Mechanical Ventilation in Amyotrophic Lateral Sclerosis: A Clinical Review

Subjects with amyotrophic lateral sclerosis (ALS) can have fast or slow evolution. Precocious diagnosis significantly impacts on natural history, even though the prognosis remains severe, indeed the mortality rate at 5 years is higher than 80%. Progressive weakness of bulbar, limb, and respiratory muscles is characteristic. Death generally occurs from 2 to 4 years following symptom onset, primarily owing to respiratory failure. Treatments for respiratory aspects of ALS are evolving. This manuscript that recapitulates the current indications is aimed to define a rational basis for a patientoriented approach to treatment of ALS, mainly focused on noninvasive mechanical ventilation (NIV).