Table 3.
Baseline Penn Comprehensive ALS Center Cohort Characteristics by Most Likely Trajectory Group
| Variable | Stable Low (n = 397) | Rapid Progressor (n = 329) | Slow Progressor (n = 111) | P Value |
|---|---|---|---|---|
| Age at diagnosis, yr | 65 ± 12*† | 62 ± 12* | 60 ± 13† | <0.001 |
| Sex, M, n (%) | 199 (50)* | 199 (61)* | 67 (60) | 0.01 |
| Race, n (%) | <0.001 | |||
| White | 312 (79)*† | 283 (86)* | 105 (94)† | |
| African American | 54 (13) | 19 (6) | 3 (3) | |
| Other | 31 (8) | 27 (8) | 3 (3) | |
| BMI class, n (%) | <0.001 | |||
| <18.5 kg/m2 | 30 (7)*† | 7 (2)* | 0 (0)† | |
| 18.5–24.9 kg/m2 | 173 (44) | 131 (40) | 44 (40) | |
| 25–29.9 kg/m2 | 123 (31) | 122 (37) | 40 (36) | |
| >30 kg/m2 | 71 (18) | 69 (21) | 27 (24) | |
| Diagnosis delay, yr | 1.0 (0.6–1.4) | 1.0 (0.5–1.5)‡ | 1.4 (0.6–2.7)‡ | <0.001 |
| Symptom onset to first visit, yr | 1.1 (0.7–2.0)† | 1.2 (0.8–2.0)‡ | 2.0 (1.0–3.3)†‡ | <0.001 |
| El Escorial criteria, n (%) | <0.001 | |||
| Definite ALS | 117 (29)*† | 54 (16)*‡ | 5 (5)†‡ | |
| Possible ALS | 103 (26) | 84 (26) | 29 (26) | |
| Probable ALS | 106 (27) | 117 (36) | 30 (27) | |
| Suspected ALS | 71 (18) | 74 (22) | 47 (42) | |
| Symptom onset site, n (%) | <0.001 | |||
| Limb | 271 (68)*† | 255 (78)* | 98 (88)† | |
| Bulbar | 126 (32) | 74 (22) | 13 (12) | |
| FVC seated, % predicted | 56 ± 18*† | 88 ± 14*‡ | 99 ± 13†‡ | <0.001 |
| ALSFRS-R total score | 33 ± 7*† | 38 ± 5*‡ | 41 ± 4†‡ | <0.001 |
| ALSFRS-R dyspnea, n (%) | <0.001 | |||
| 4 | 177 (45)*† | 218 (66)* | 78 (70)† | |
| 3 | 103 (26) | 90 (27) | 27 (24) | |
| 2 | 68 (17) | 15 (5) | 6 (6) | |
| 1 | 48 (12) | 6 (2) | 0 (0) | |
| 0 | 1 (<1) | 0 (0) | 0 (0) | |
| ALSFRS-R orthopnea, n (%) | <0.001 | |||
| 4 | 252 (64)*† | 312 (95)* | 109 (98)† | |
| 3 | 49 (12) | 9 (3) | 1 (1) | |
| 2 | 55 (14) | 7 (2) | 1 (1) | |
| 1 | 6 (1) | 0 (0) | 0 (0) | |
| 0 | 35 (9) | 1 (<1) | 0 (0) | |
| Smoking history, n (%) | 0.02 | |||
| Never | 191 (48)† | 175 (53) | 73 (66)† | |
| Previous | 165 (42) | 119 (36) | 32 (29) | |
| Current | 41 (10) | 35 (11) | 6 (5) | |
| Coronary artery disease, n (%) | 46 (12)† | 25 (8) | 3 (3)† | 0.009 |
| Diabetes mellitus, n (%) | 56 (14)† | 37 (11)‡ | 3 (3)†‡ | 0.004 |
| Hypertension, n (%) | 176 (44) | 118 (36) | 37 (33) | 0.02 |
| Survival, mo | 15 (9–25)*† | 25 (17–37)*‡ | 46 (28–69)†‡ | <0.001 |
| Survival from symptom onset, mo | 28 (19–46)*† | 39 (28–57)*‡ | 66 (49–97)†‡ | <0.001 |
For definition of abbreviations, see Table 1.
Survival is indicated by time between diagnosis and last follow-up date or date of death. Data are mean ± SD or median (25th–75th percentiles) unless otherwise indicated. Groups were compared using one-way ANOVA, Kruskal-Wallis, or Pearson chi-squared test, as appropriate.
*
For items in the same row, pairwise significant differences with Bonferroni correction.
†
For items in the same row, pairwise significant differences with Bonferroni correction.
‡
For items in the same row, pairwise significant differences with Bonferroni correction.