Table 1.
demographic/clinical parameter | |
Age [in years, mean ± SD, range] | 60± 11.5 (27.3 - 78.8) |
Gender [male/female, n, %] | 49/36, 57.6%/42.4% |
Riluzole [yes/no, n, %] | 65/20, 76.4%/23.5% |
Disease duration [in months, mean ± SD, range] | 20 ± 21.6 (4 −136) |
Onset-type [limb/bulbar, n, %] | 61/24, 71.8%/28,2% |
ALSFRS-R total score [mean ± SD, range] | 38.1 ± 6.9 (15 - 47) |
PR [points lost per month, mean ± SD, range] | 0.69 ± 0.55 (0.06 – 2.71) |
rD50 [mean ± SD, range] | 0.27 ± 0.13 (0.05 – 0.7) |
Phase I (0 ≤ rD50 < 0.25) [n per phase, %]; | I: 34, 40% |
Phase II (0.25 ≤ rD50 < 0.5) [n per phase, %]; | II: 48, 56.4% |
Phase III/IV (rD50 ≥ 0.5) [n per phase, %] | III: 3, 3.5% |
D50 [months, mean ± SD, range] | 36.3 ± 28.7 (8.07 – 175.55) |
cFS [points, mean ± SD, range] | 38 ± 6.4 (15.1 – 48.6) |
cFL [points lost per month, mean ± SD, range] | 0.89 ± 0.57 (0.11 – 2.85) |
King's Stage [n per stage I-V] | I: 28 |
II: 28 | |
III: 20 | |
IV: 9 | |
V: 0 | |
MiToS Stage [n per stage 0-V] | 0: 59 |
I: 21 | |
II: 4 | |
III: 1 | |
IV/V: 0 |
Abbreviations: ALSFRS-R revised ALS functional rating scale (assessed within 100 days prior to or after MRI acquisition); D50 estimated time in months for an individual to lose 50% of functionality; PR Progression Rate, calculated as (48 – current ALSFRS-R)/months since symptom onset; cFS calculated functional state, cFL calculated functional loss, rD50 relative D50, each at the time of examination; MiToS Milano-Torino functional stage; SD standard deviation.