Table 2.
Baseline Penn cohort characteristics by composite outcome (including death)
| Characteristic | Composite outcome | p-value | |
|---|---|---|---|
| No outcome (n=465) | Outcome achieved (n=300) | ||
| Age at diagnosis years | 61±12 | 65±11 | <0.001 |
| Male sex | 272 (59) | 168 (56) | 0.50 |
| Ethnicity | 0.25 | ||
| Caucasian | 410 (88) | 252 (84) | |
| African-american | 28 (6) | 25 (8) | |
| Other | 27 (6) | 23 (8) | |
| BMI class kg-m−2 | <0.001 | ||
| <18.5 | 9 (2) | 21 (7) | |
| 18.5−24.9 | 186 (40) | 132 (44) | |
| 25−29.9 | 177 (38) | 93 (31) | |
| ≥30 | 93 (20) | 54 (18) | |
| Diagnosis delay years | 1.0 (0.6−2.0) | 0.8 (0.5−1.3) | <0.001 |
| El Escorial criteria | <0.001 | ||
| Definite ALS | 62 (13) | 90 (30) | |
| Possible ALS | 125 (27) | 72 (24) | |
| Probable ALS | 154 (33) | 85 (28) | |
| Suspected ALS | 124 (27) | 53 (18) | |
| Symptom onset site | 0.002 | ||
| Limb | 380 (82) | 216 (72) | |
| Bulbar | 85 (18) | 84 (28) | |
| FVC % predicted | 89±15 | 69±14 | <0.001 |
| ALSFRS-R total score | 38±5 | 34±6 | <0.001 |
| ALSFRS-R dyspnoea score | <0.001 | ||
| 4 | 316 (68) | 138 (46) | |
| 3 | 118 (25) | 87 (29) | |
| 2 | 26 (6) | 48 (16) | |
| 1 | 4 (1) | 27 (9) | |
| 0 | 1 (<1) | 0 (0) | |
| ALSFRS-R orthopnoea score | <0.001 | ||
| 4 | 432 (93) | 225 (75) | |
| 3 | 20 (4) | 23 (7) | |
| 2 | 10 (2) | 32 (11) | |
| 1 | 0 (0) | 2 (1) | |
| 0 | 3 (1) | 18 (6) | |
| Smoking history | 0.11 | ||
| Never | 256 (55) | 150 (50) | |
| Previous | 160 (34) | 125 (42) | |
| Current | 49 (11) | 25 (8) | |
| Coronary artery disease | 34 (7) | 36 (12) | 0.028 |
| Diabetes mellitus | 31 (7) | 50 (17) | <0.001 |
| Hypertension | 161 (35) | 136 (45) | 0.003 |
Data are presented as n (%), mean±SD, or median (interquartile range). Comparisons between data are made using the t-test, the Chi-squared test, or the Wilcoxon–Mann–Whitney test. BMI: body mass index; ALS: amyotrophic lateral sclerosis; FVC: forced vital capacity; ALSFRS-R: amyotrophic lateral sclerosis functional rating scale-revised.