Table 1.
Subject characteristics including disease duration, site of onset, ALS-FRS-R, disease progression rate (slope of ALS-FRS-R), UMN burden score,27 and cognitive profile measured by the ECAS.28.
| Gender (m/f) | Age (years) | Disease duration (months median) | Site of onset bulbar/spinal | ALS-FRS-R median | ALS-FRS-R slope median | UMN burden score | ECAS total score | |
|---|---|---|---|---|---|---|---|---|
| C9orf72-associated ALS | 17/8 | 63 ± 11 (range 35–79) | 12, range 3–37 | 10/15 | 41 (range 27–48) | −13/year range (−1 to −31)/year | 7 ± 6 range (2–14) | 30 ± 8 |
| Sporadic ALS | 14/11 | 64 ± 11 (range 31–83) | 11, range 5–28 | 7/18 | 43 (range 30–48) | −8/year range (−1 to −28)/year | 7 ± 4 range (2–14) | Not available |
| Controls | 18/7 | 63 ± 16 (range 26–80) | – | – | – | – | – | – |
| p | Kruskal–Wallis 0.62 | Kruskal–Wallis 0.81 | t test 0.98 | – | t test 0.80 | t test 0.64 | t test 0.16 |
ALS, amyotrophic lateral sclerosis ; ALS-FRS-R, revised ALS functional rating scale; ECAS, Edinburgh cognitive and behavioral ALS screen; f, female; m, male; UMN, upper motor neuron.