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Table 3 Demographic and clinical features of familial and sporadic ALS patients with SOD1 mutations

From: Better survival in female SOD1-mutant patients with ALS: a study of SOD1-related natural history

  Case count, n (%) Bulbar site at onset, n (%) AAO, years, mean (SD) Delay, months, median (IQR) Disease progression, ΔFS, median (IQR) Survival time, months, mean (95% Cl)&
Total 66 5(8.1%) 43.92(9.24) 14.50(6.00–36.50) 0.33 (0.15–0.90) 97.08 (75.61–118.54)
Missing 4 2 2 9 5
fALS 47(71.2%) 3(7.0%) 43.49(7.50) 20.00(6.50–39.00) 0.27(0.15–0.87) 87.46(61.74–113.18)
sALS 19(28.8%) 2(10.5%) 44.95(12.63) 8.00(6.00–25.00) 0.58(0.14–0.98) 121.49(86.24–156.74)
p value 0.638 0.64 0.26 0.59 0.38
Male 35(53.8%) 4(11.8%) 44.62(10.38) 9.50(4.75–24.25) 0.55(0.24–0.94) 57.40(38.89–75.91)
Female 30(46.2%) 1(3.6%) 43.13(7.84) 24.00(9.50–47.50) 0.19(0.06–0.90) 125.64(99.83–151.45)
p value -# 0.366 0.525 0.009* 0.041* 0.006*
  1. AAO, age at onset. Delay, diagnostic delay from onset
  2. & Due to more than 50% of subjects being censored (lost to follow-up or still surviving) in the sALS and female groups, the mean (95% CI) survival time was calculated
  3. # There was one case without gender information
  4. *In bold with statistical significance at p < 0.05