Univariable survival analysis disclosed that a higher rate of disease progression (ΔFS), along with lower Amyotrophic Laterals Sclerosis Rating Scale-Revised (ALSFRS-R) score, older age of onset (≥70 years), bulbar onset disease and shorter disease duration were predictors of survival
| Predictor variable | Number | Median survival time from initial visit (years) | HR | 95% CI | p Value |
|---|---|---|---|---|---|
| Gender | |||||
| Male | 93 | 1.4 | 1 | ||
| Female | 71 | 1.4 | 0.92 | 0.64 to 1.30 | 0.626 |
| Age | |||||
| <70 | 104 | 1.6 | 1 | ||
| ≥70 | 60 | 1 | 1.77 | 1.23 to 2.55 | 0.002 |
| Site of onset | |||||
| Limb | 95 | 1.6 | 1 | ||
| Bulbar | 69 | 0.9 | 1.76 | 1.23 to 2.51 | 0.002 |
| Symptom duration (years) | |||||
| 0.7 | 41 | 1.3 | 2.52 | 1.45 to 4.39 | <0.001 |
| 0.7–1 | 42 | 1.4 | 2.74 | 1.61 to 4.68 | <0.001 |
| 1.1–2 | 40 | 1.2 | 2.81 | 1.62 to 4.89 | <0.001 |
| >2 | 41 | 5 | 1 | <0.001 | |
| Riluzole | |||||
| Yes | 127 | 1.5 | 0.7 | 0.46 to 1.05 | 0.087 |
| No | 37 | 0.7 | 1 | ||
| PEG tube | |||||
| Yes | 81 | 1.4 | 1.36 | 0.96 to 1.94 | 0.09 |
| No | 83 | 1.5 | 1 | ||
| NIV | |||||
| Yes | 34 | 1.3 | 1.24 | 0.83 to 1.85 | 0.304 |
| No | 130 | 1.4 | 1 | ||
| ΔFS tertiles | |||||
| <0.47 | 56 | 2.4 | 1 | ||
| 0.47–1.11 | 54 | 1.6 | 1.77 | 1.12 to 2.82 | 0.015 |
| >1.11 | 54 | 0.7 | 3.74 | 2.40 to 5.82 | <0.001 |
| ALSFRS-R quartiles | |||||
| ≤33 | 48 | 0.6 | 2.37 | 1.40 to 4.04 | 0.002 |
| 34–39 | 42 | 1.5 | 1.65 | 0.94 to 2.92 | 0.084 |
| 40–43 | 42 | 1.5 | 1.72 | 0.99 to 3.01 | 0.056 |
| ≥44 | 32 | 2.6 | 1 | ||
The presence of a percutaneous endoscopic gastrostomy (PEG) tube and non-invasive ventilation did not significantly impact on survival in the present cohort.