Multivariate analyses, with all factors analysed in the same Cox models
| Category | Survival, years (HR (95% CI)) | p | Disease progression, years (HR (95% CI)) | p | ||||
|---|---|---|---|---|---|---|---|---|
| In most patients who received riluzole, therapy was initiated at the time of the first examination and diagnosis, prior to a documented 20 point progression. In contrast, NIV initiation and PEG placement were generally performed in patients who had reached an advanced degree of disability. Thus, the Cox model for disease progression did not include NIV and PEG use. | ||||||||
| Baseline FVC <75% | 2.07 (1.73 to 2.48) | <0.001 | 1.56 (1.28 to 1.90) | <0.001 | ||||
| Age at onset, years | 1.04 (1.03 to 1.05) | <0.001 | 1.01 (0.99 to 1.01) | 0.01 | ||||
| Limb vs. bulbar onset | 1.09 (0.87 to 1.37) | 0.4 | 1.34 (1.07 to 1.68) | 0.009 | ||||
| FS to FE time, months | 0.95 (0.94 to 0.96) | <0.001 | 0.98 (0.97 to 0.98) | <0.001 | ||||
| Women versus men | 1.10 (0.92 to 1.33) | 0.3 | 1.20 (0.99 to 1.43) | 0.05 | ||||
| Riluzole (ever versus never) | 1.14 (0.94 to 1.38) | 0.2 | 1.12 (0.95 to 1.32) | 0.2 | ||||
| NIV (ever versus never) | 0.77 (0.62 to 0.95) | 0.02 | ND | ND | ||||
| PEG (ever versus never) | 0.74 (0.62 to 0.88) | 0.001 | ND | ND | ||||