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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