Table 2.
Survival Based on Clinical Characteristics at First Visit (N=1442)
| Category | No. (%) | Survival from diagnosis, median (95% CI), mo | P value |
|---|---|---|---|
| Sex | 5.7e-3 | ||
| Female | 671 (46.5) | 18.33 (16.97-20.03) | |
| Male | 771 (53.5) | 16.27 (15.23-17.27) | |
| Ethnoracial | 4.1e-2 | ||
| Non-Hispanic White | 1327 (93.6) | 17.0 (8.9-30.8) | |
| African American | 80 (5.5) | 21.3 (10.4-35.0) | |
| Hispanic | 28 (1.9) | 12.6 (10.1-21.9) | |
| Other | 7 (0.4) | 15.7 (5.1-51.3) | |
| Age range, y | <1e-4 | ||
| <50 | 178 (12.3) | 30.84 (26.17-35.67) | |
| 50-59 | 309 (21.4) | 22.70 (19.87-25.13) | |
| 60-69 | 511 (35.4) | 16.77 (15.67-18.50) | |
| 70-79 | 350 (24.3) | 12.53 (10.93-13.33) | |
| ≥80 | 94 (6.5) | 8.42 (6.83-12.57) | |
| Site of onset/sex | <1e-4 | ||
| Bulbar | |||
| Female | 252 (17.5) | 15.64 (13.33-17.03) | |
| Male | 183 (12.7) | 16.30 (13.60-18.43) | |
| Total | 435 (30.2) | 15.87 (14.43-17.03) | |
| Spinal | |||
| Female | 419 (29.1) | 16.83 (15.50-18.50) | |
| Male | 588 (40.8) | 19.75 (17.37-22.17) | |
| Total | 1,007 (69.8) | 18.00 (16.93-19.77) | |
| Diagnostic delay, mo | <1e-4 | ||
| <6.0 | 335 (23.2) | 15.97 (14.40-16.97) | |
| 6.0-11.9 | 503 (34.9) | 16.77 (15.37-17.70) | |
| 12.0-18.0 | 245 (17.0) | 17.03 (15.53-19.67) | |
| >18.0 | 359 (24.9) | 22.03 (19.33-25.20) | |
| Disease duration, mo | <1e-4 | ||
| <12 | 122 (8.5) | 3.89 (3.43-4.30) | |
| 12-24 | 392 (27.2) | 10.05 (9.27-10.50) | |
| 24-60 | 682 (47.3) | 22.37 (21.17-23.70) | |
| >60 | 246 (17.1) | 56.05 (53.17-59.03) | |
| BMI | 2.4e-3 | ||
| <18.5 | 54 (5.2) | 10.17 (5.43-14.00) | |
| 18.5-24.9 | 423 (40.9) | 16.47 (14.40-17.60) | |
| 25.0-29.9 | 359 (34.7) | 17.77 (15.90-17.60) | |
| ≥30.0 | 198 (19.2) | 17.82 (16.13-21.60) | |
| Missing | 408 (28.3) | 18.73 (17.23-21.50) | |
| FVC% | <1e-4 | ||
| >80 | 355 (38.1) | 22.43 (20.10-24.30) | |
| 50-80 | 392 (42.1) | 14.64 (13.10-16.47) | |
| <50 | 184 (19.8) | 11.14 (8.53-13.40) | |
| Missing | 511 (35.4) | 18.43 (16.73-20.03) | |
| C9orf72 status | 7.3e-3 | ||
| Carriers | 76 (11.8) | 16.95 (12.47-20.57) | |
| Noncarriers | 568 (88.2) | 19.89 (18.00-21.97) | |
| Missing | 798 (55.3) | 15.97 (14.77-16.93) | |
| ALS-CBS | 2.3e-3 | ||
| >15 | 264 (53.2) | 18.50 (16.93-21.83) | |
| ≤15 | 232 (46.8) | 15.97 (13.10-18.13) | |
| Missing | 946 (65.6) | 17.23 (16.13-18.43) | |
| ALSFRS-R | 4e-4 | ||
| >44 | 71 (5.5) | 23.85 (19.60-33.87) | |
| 40-44 | 135 (15.4) | 23.23 (19.67-27.43) | |
| 33-39 | 299 (37.9) | 18.13 (16.83-20.37) | |
| <33 | 299 (41.9) | 15.23 (12.97-17.40) | |
| Missing | 638 (44.2) | 16.2 (15.13-17.27) | |
| ΔFS | <1e-4 | ||
| <0.5 | 145 (18.0) | 28.4 (22.53-33.83) | |
| 0.5-0.9 | 207 (25.8) | 21.6 (19.23-24.40) | |
| 1.0-1.9 | 231 (28.7) | 16.93 (15.23-19.30) | |
| ≥2.0 | 221 (27.5) | 13.1 (11.53-15.67) | |
| Missing | 638 (44.2) | 16.2 (15.13-17.27) |
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 mutation; ΔFS, progression rate; FVC%, forced vital capacity percentage.