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Table 1 Clinical characteristics of Chinese and German ALS patients with SOD1 mutations

From: A natural history comparison of SOD1-mutant patients with amyotrophic lateral sclerosis between Chinese and German populations

  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  
  1. Bold P-values are significant as P < 0.05
  2. LMN, Lower motor neuron
  3. aAdjusting for the corresponding data reported in general ALS cohorts of both countries[6