RESULTS: From initiation of NIPPV, median survival of tolerant patients was significantly higher than those who were intolerant of or not using NIPPV (413 days vs. 267 days; p=0.0036). Median survival of 15 patients with a diaphragm pacer was longer relative to those without but the difference was not statistically different (613 days vs. 293 days; p=0.113). Patients prescribed riluzole had a better survival than those not prescribed it (median survival of 335 days vs. 268 days, p=0.04). Patients with an ALSFRS-R bulbar subscore £8 (normal=12) had a lower median survival than those with a subscore >8 (291 days vs. 378 days; p=0.01). In patients with an ALSFRS-R bulbar subscore £8, tolerant ones had better survival compared to intolerant ones (576 days vs. 383 days, p=0.0009). All of the following were predictors of significantly better survival in a Cox proportional hazard model: tolerance to NIPPV, use of riluzole, age < 60 years, time from symptom onset to initiation of NIPPV being >2 years, and >50% predicted FVC at initiation of NIPPV.