When we defined survival by death, tracheostomy, or permanent assisted ventilation, we observed similar results to those using death only; individuals with C9orf72 expansions had an increased risk for developing subsequent features compared to those without (HR = 3.77 [95% CI 1.86–7.65], P < 0.001), and, in combination with C9orf72 carrier status, an initial ALS clinical syndrome was associated with a decreased hazard for developing subsequent features (HR = 0.49 [95% CI 0.25–0.97], P = 0.04). The gene discussed is C9orf72; the disease is amyotrophic lateral sclerosis.