Table 3 Summary of Australian ALS clinical information for each of the five SOD1 clusters.
Cluster | SOD1 mutation | No. of mutation carriers | % Male | % Spinal [n] | Mean age (years) at onset ± SD (range) [n] | Mean age (years) at death ± SD (range) [n] | Mean disease duration (months) ± SD (range) [n] |
|---|---|---|---|---|---|---|---|
C1 | p.I114T | 41 | 41% | 94% [16] | 57.1 ± 14.5 (29.5–76.0) [16] | 61.5 ± 12.6 (35.1–78.2) [18] | 64.1 ± 45.7 (28.5–177.1) [11] |
C4 | p.I114T | 3 | 33% | 100% [3] | 40.8 ± 12.5 (30.2–54.5) [3] | NA | NA |
C2 | p.V149G | 33 | 60% | 75% [16] | 43.0 ± 8.7 (25.1–57.6) [17] | 44.8 ± 7.8 (25.8–53.8) [21] | 29.5 ± 34.3 (7.8–145.0) [16] |
C3 | p.E101G | 6 | 50% | 100% [4] | 48.6 ± 9.9 (36.2–60.2) [4] | 64.2 ± 15.2 (45.9–83.0) [4] | 119.8 ± 59.1 (62.8–180.9) [3] |
C5 | p.E101G | 3 | 67% | 50% [2] | 42.6 [1] | 51.7 ± 11.7 (43.6–65.1) [3] | 46.0 [1] |
