Table 1.
Participant characteristics (all participants).
| Control | At‐risk | Converter | ALS | |
|---|---|---|---|---|
| n | 16 | 55 | 7 | 12 |
| Baseline age, years; mean ± SD 1 | 46.0 ± 11.3 | 47.4 ± 11.4 | 55.0 ± 14.5 | 57.5 ± 7.2 |
| Male, n (%) 2 | 7 (43.8) | 17 (30.9) | 5 (71.4) | 6 (50.0) |
| Gene, n | ||||
| SOD1 A4V | – | 18 | 4 | 2 |
| SOD1 non‐A4V | – | 15 | 0 | 1 |
| C9ORF72 | – | 18 | 2 | 2 |
| Other | – | 4 | 1 | 0 |
| Unknown | – | 0 | 0 | 7 |
| Onset site, n | ||||
| Bulbar | – | – | 0 | 2 |
| Limb | – | – | 5 | 9 |
| Other | – | – | 1 | 1 |
| Unknown | – | – | 1 | 0 |
| Baseline time from onset, months; median [IQR] | – | – |
−12.1 [−22.9 to −9.3] |
38.30 [24.8 to 60.3] |
| Baseline time from diagnosis, months; median [IQR] | – | – |
−13.3 [−25.0 to −9.9] |
13.0 [7.8 to 22.2] |
| Baseline ALSFRS‐R, median [IQR] | – | – | – |
39.0 [35.5 to 44.5] |
| Baseline ∆FRS, median [IQR] | – | – | – |
0.31 [0.21 to 0.57] |
ALS, amyotrophic lateral sclerosis; SD, standard deviation; IQR, interquartile range; ALSFRS‐R, revised ALS functional rating scale; ∆FRS, estimated ALSFRS‐R progression based on baseline ALSFRS‐R, points per month.
1
Kruskal‐Wallis H‐test P = 0.024 (Dunn’s post hoc ALS vs control P = 0.0178, ALS vs. at‐risk P = 0.025).
2
Fisher exact test P = 0.150.