Table 3.
Survival Hazard Risk of Predictors at First Visit (N=1442)
| Category | No. | Univariate model |
Multivariate model |
||||||
|---|---|---|---|---|---|---|---|---|---|
| HR (95% CI) | P | HR (95% CI) | Pe | ||||||
| Sex | |||||||||
| Male | 771 |  |
1.00 |  |
1.00 | ||||
| Female | 671 | 1.16 (1.05-1.29) | 4.4e-3 | 1.085 (0.86-1.36) | ns | ||||
| Age at first visit, y | |||||||||
| <50 | 178 |  |
1.00 |  |
1.00 | ||||
| 50-59 | 309 | 1.49 (1.23-1.80) | <1e-4 | 1.29 (0.83-2.06) | ns | ||||
| 60-69 | 511 | 1.87 (1.57-2.22) | <1e-4 | 1.89 (1.26-2.94) | 3.1e-3 | ||||
| 70-79 | 350 | 2.52 (2.10-3.03) | <1e-4 | 3.12 (2.00-4.99) | <1e-4 | ||||
| ≥80 | 94 | 2.85 (2.21-3.66) | <1e-4 | 1.71 (0.88-3.21) | ns | ||||
| Site of onset | |||||||||
| Spinal | 1007 |  |
1.00 |  |
1.00 | ||||
| Bulbar | 435 | 1.28 (1.15-1.44) | <1e-4 | 1.043 (0.82-1.34) | Ns | ||||
| C9orf72 status | |||||||||
| Noncarriers | 568 |  |
1.00 |  |
1.00 | ||||
| Carriers | 76 | 1.38 (1.08-1.75) | 8.9e-3 | 1.271 (0.90-1.77) | Ns | ||||
| Diagnostic delay, mo | |||||||||
| <6.0 | 335 |  |
1.00 | ||||||
| 6.0-11.9 | 503 | 0.88 (0.76-1.01) | ns | ||||||
| 12.0-18.0 | 245 | 0.84 (0.71-0.99) | 3.7e-2 | ||||||
| >18.0 | 359 | 0.64 (0.55-0.75) | <1e-4 | ||||||
| BMI | |||||||||
| <18.5 | 54 |  |
1.31 (0.98-1.73) | ns |  |
0.72 (0.41-1.19) | Ns | ||
| 18.5-24.9 | 423 | 1.00 | 1.00 | ||||||
| 25.0-29.9 | 359 | 0.85 (0.73-0.97) | 2.1e-2 | 0.72 (0.55-0.93) | 1.2e-2 | ||||
| ≥30.0 | 198 | 0.82 (0.69-0.97) | 2.4e-2 | 0.76 (0.56-1.05) | Ns | ||||
| FVC% | |||||||||
| >80 | 355 |  |
1.00 |  |
1.00 | ||||
| 50-80 | 392 | 1.34 (1.16-1.54) | <1e-4 | 1.40 (1.09-1.80) | 7.7e-3 | ||||
| <50 | 184 | 1.81 (1.51-2.16) | <1e-4 | 1.31 (0.91-1.88) | Ns | ||||
| ALS-CBS | |||||||||
| >15 | 264 |  |
1.00 |  |
1.00 | ||||
| ≤15 | 232 | 1.33 (1.11-1.59) | 1.7e-3 | 1.34 (1.07-1.69) | 1.4e-2 | ||||
| ALSFRS-R | |||||||||
| ≥44 | 44 |  |
1.00 | ||||||
| 40-43 | 124 | 1.24 (0.89-1.77) | ns | ||||||
| 33-39 | 299 | 1.49 (1.10-2.08) | 1.4e-2 | ||||||
| <33 | 337 | 1.74 (1.28-2.41) | 6e-4 | ||||||
| ΔFS | |||||||||
| <0.5 | 145 |  |
1.00 |  |
1.00 | ||||
| 0.5-0.9 | 207 | 1.43 (1.16-1.78) | 1.1e-3 | 1.09 (0.77-1.56) | ns | ||||
| 1.0-1.9 | 231 | 1.84 (1.49-2.28) | <1e-4 | 1.70 (1.20-2.44) | 3.4e-3 | ||||
| ≥2.0 | 221 | 2.14 (1.73-2.65) | <1e-4 | 1.63 (1.12-2.39) | 1.1e-2 | ||||
Abbreviations: ALS-CBS, amyotrophic lateral sclerosis cognitive behavioral screen scores; ALSFRS-R, amyotrophic lateral sclerosis functional rating scale-revised; BMI, body mass index; C9orf72, hexanucleotide repeat expansion; ΔFS, progression rate; FVC%, forced vital capacity percentage; HR, hazard ratio; ns, not significant.
