臨床ゲノミクスジャーナル

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

Solimando AG, Ciccol S, Lernia GD, Leonel P, D’Errico E, Schneider A, Fritz G, Brienza N, Racanelli V and Vacca A

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).