Table 1.
Clinical characteristics of Chinese and German ALS patients with SOD1 mutations
| Total (data available) | China | Germany | P | Adjusted Pa | |
|---|---|---|---|---|---|
| Nominal variables, n (%) | |||||
| Numbers of subjects | 150 | 66 | 84 | ||
| Sex, male | 80 (55.6%) (144) | 35 (53.8%) | 45 (57.0%) | 0.70 | |
|
Young-onset ALS (25–45 years) |
63 (47.5%) (139) | 40 (62.5%) | 23 (30.7%) | < 0.001 | |
| Site of onset, spinal | 116 (94.3%) (123) | 57 (91.9%) | 59 (96.7%) | 0.25 | |
| Pure LMN | 16 (20.0%) (80) | 9 (17.3%) | 7 (25.0%) | 0.41 | |
| Riluzole prescription | 54 (53.5%) (101) | 15 (28.3%) | 39 (81.3%) | < 0.001 | |
| Continuous variables, median (IQR) | |||||
| Age of onset (years) | 46.0 (40.0–54.0) (139) | 43.0 (38.3–50.0) | 50.0 (41.0–58.0) | 0.002 | 0.07 |
| BMI at diagnosis | 23.5 (21.6–26.3) (91) | 22.6 (20.9–24.9) | 25.9 (23.1–28.7) | < 0.001 | 0.03 |
| Diagnostic delay (months) | 12.0 (6.0–35.0) (107) | 14.5 (6.0–36.5) | 11.0 (6.0–32.0) | 0.59 | |
| ALSFRS-R at diagnosis | 41.0 (35.0–45.0) (116) | 42.0 (35.5–46.0) | 40.0 (31.0–44.0) | 0.04 | 0.70 |
| Early progression rate | 0.42 (0.14–0.90) (116) | 0.33 (0.15–0.90) | 0.46 (0.13–0.93) | 0.79 | 0.29 |
| Late progression rate | 0.26 (0.09–0.79) (69) | 0.28 (0.08–0.80) | 0.17 (0.11–0.77) | 0.89 | |
| Survival (months) | 141.0 (21.0–364.0) (140) | NA | 198.0 (22.0–364.0) | 0.90 | |
| Follow-up period | 24.0 (7.3–40.8) (88) | 30.0 (10.0–42.0) | 15.0 (6.0–40.0) | 0.06 | |
Bold P-values are significant as P < 0.05
LMN, Lower motor neuron
aAdjusting for the corresponding data reported in general ALS cohorts of both countries[6]