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Background Motoneuron disease (MND) is a rare, progressive neuromuscular condition with usually guarded prognosis. Long-term non-invasive ventilation (LT-NIV) can prolong overall survival; however, due to disease heterogeneity it is unclear which patient might benefit from LT-NIV. The aim of this project was to analyse the overall survival (OS) of patients with MND referred to our tertiary long-term ventilation service.Methods We studied 73 patients with established MND (68±12 years, 73% men). Overall survival from the referral to our service was analysed with Cox-regression and Log-rank tests.Results Amyotrophic Lateral Sclerosis (ALS) was the most common MND (n=63). Patients with ALS tended to have lower OS compared to non-ALS MND (median survival 25 vs. 51 months, p=0.06). In patients with ALS, whilst bulbar onset was not associated with reduced overall survival (p=0.18), the 5 year survival of patients with bulbar ALS was 28% compared to non-bulbar patients (42%). In non-hypercapnic patients with ALS (pCO2<6 kPa, n=42), LT-NIV (n=13) did not significantly prolong OS (p=0.99). In patients with daytime hypercapnia, 2 patients refused LT-NIV. Their median survival was 5 months versus 22 months who accepted LT-NIV. Age, sex, riluzole use or comorbidities did not affect OS (all p>0.05).Discussion It is important to distinguish patients with ALS and non-ALS MND, as the former is associated with worse prognosis, especially in bulbar-onset subtype. LT-NIV is associated with survival benefit in patients with hypercapnia.