AbstractBackgroundThe efficacy and safety of diaphragm pacing (DP) for amyotrophic lateral sclerosis (ALS) patients has not been elucidated.AimTo investigate the efficacy and safety of DP for ALS in Japan.Materials and MethodsWe implanted the NeuRx RA/4 Diaphragm Pacing SystemR (NeuRx) in 5 ALS patients without tracheostomy mechanical ventilation and conditioned subsequently. Participants could combine NeuRx with non‐invasive ventilation such as biphasic positive airway pressure (BIPAP). The primary endpoint was overall survival, defined as the period from implantation to the administration of mechanical ventilation with tracheostomy or death.ResultsThree men and 2 women (59.6 ± 9.6 years old, 44 to 70 years old) underwent laparoscopic implantation of NeuRx. The survival differed considerably among individuals (20.7 ± 18.6 months, 5.0‐48.7 months). However, 2 patients with BIPAP showed longer survival. Breakage of the external cord connector occurred in 3 patients and focal epidermal infection occurred in two patients: however, these complications did not lead to serious conditions. An autopsy of one patient revealed no severe damage to the adjacent diaphragm tissue.ConclusionThe efficacy of NeuRx varied between individuals. The combination use of NeuRx with BIPAP might lead to longer survival than NeuRx alone. No serious safety issues were observed.