Patients’ survival
| Variable | Value | HR (95% CI) | P value |
| Age at onset (years) | Per each year of age at onset | 1.030 (1.024 to 1.037) | 0.0001 |
| Diagnostic delay (months) | Per each month of delay | 0.953 (0.945 to 0.962) | 0.0001 |
| Site of onset | Spinal Bulbar | 1 (reference) 1.45 (1.27–1.67) | 0.0001 |
| ΔALSFRS-R | Per each point loss/month | 1.25 (1.20 to 1.29) | 0.0001 |
| ΔKing’s | Per each point loss/month | 1.56 (1.16 to 2.09) | 0.003 |
| ATXN2 polyQ | <31 ≥31 | 1 (reference) 1.58 (1.14 to 2.19) | 0.006 |
Cox multivariable analysis.