Table 1.
Baseline characteristics of the Penn cohort (n=765)
| Characteristic | Result |
|---|---|
| Age at diagnosis years | 63±12 |
| Male sex | 440 (58) |
| Ethnicity | |
| Caucasian | 662 (87) |
| African-american | 53 (7) |
| Other | 50 (6) |
| BMI class kg-m−2 | |
| <18.5 | 30 (4) |
| 18.5−24.9 | 318 (42) |
| 25−29.9 | 270 (35) |
| ≥30 | 147 (19) |
| Diagnosis delay years | 1.0 (0.6−1.7) |
| El Escorial criteria | |
| Definite ALS | 152 (20) |
| Possible ALS | 197 (26) |
| Probable ALS | 239 (31) |
| Suspected ALS | 177 (23) |
| Symptom onset site | |
| Limb | 596 (78) |
| Bulbar | 169 (22) |
| FVC % predicted | 81±18 |
| ALSFRS-R total score | 37±6 |
| ALSFRS-R dyspnoea score | |
| 4 | 454 (59) |
| 3 | 205 (27) |
| 2 | 74 (10) |
| 1 | 31 (4) |
| 0 | 1 (<1) |
| ALSFRS-R orthopnoea score | |
| 4 | 657 (86) |
| 3 | 43 (5) |
| 2 | 42 (5) |
| 1 | 2 (<1) |
| 0 | 21 (3) |
| Smoking history | |
| Current | 74 (10) |
| Previous | 285 (37) |
| Never | 406 (53) |
| Coronary artery disease | 70 (9) |
| Diabetes mellitus | 81 (11) |
| Hypertension | 297 (39) |
Data are presented as n (%), mean±SD or median (interquartile range). BMI: body mass index; ALS: amyotrophic lateral sclerosis; FVC: forced vital capacity; ALSFRS-R: amyotrophic lateral sclerosis functional rating scale-revised.