Clinical data
| ALSFR-S | Muscular testing | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Patient | Onset | Duration (months) | El Escorial criteria | Atrophy | Total (/48) | Hand (/4) | Total (/150) | UL (/45) | Hand (/10) |
| 1.M.68 | UL | 74 | Probable | + | 42 | 1 | 148 | 43 | 8 |
| 2.M.50 | UL | 28 | Probable | + | 42 | 1 | 145 | 40 | 5 |
| 3.M.54 | LL | 28 | Probable | + | 41 | 4 | 120 | 41 | 8 |
| 4.M.59 | LL | 24 | Probable | + | 34 | 3 | 102 | 39 | 7 |
| 5.M.62 | B | 12 | Probable | + | 43 | 3 | 147 | 44 | 9 |
| 6.M.48 | UL | 23 | Probable | + | 39 | 2 | 145 | 42 | 8 |
| 7.M.61 | LL | 33 | Probable | + | 39 | 4 | 105 | 43 | 8 |
| 8.M.44 | LL | 21 | Probable | − | 40 | 4 | 131 | 44 | 9 |
| 9.F.76 | LL | 26 | Lab-supported | − | 34 | 4 | 105 | 41 | 8 |
| 10.M.66 | UL | 34 | Probable | + | 37 | 2 | 144 | 41 | 6 |
| 11.M.45 | B | 15 | Definite | + | 31 | 1 | 137 | 41 | 8 |
| 12.M.55 | LL | 58 | Probable | + | 30 | 2 | 79 | 38 | 6 |
| 13.F.37 | UL | 39 | Probable | + | 41 | 3 | 140 | 39 | 6 |
| 14.M.51 | UL | 10 | Probable | + | 45 | 4 | 134 | 43 | 8 |
| 15.M.52 | UL | 15 | Lab-supported | + | 46 | 4 | 145 | 40 | 8 |
| 16.F.50 | UL | 14 | Probable | + | 44 | 4 | 145 | 44 | 9 |
| 17.F.56 | UL | 15 | Probable | + | 40 | 3 | 144 | 42 | 7 |
| 18.M.58 | LL | 43 | Probable | + | 33 | 3 | 114 | 42 | 8 |
| 19.F.62 | LL | 8 | Probable | − | 40 | 3 | 145 | 42 | 8 |
| 20.M.65 | UL | 17 | Probable | + | 45 | 3 | 145 | 40 | 8 |
| 21.M.63 | LL | 19 | Lab-supported | + | 40 | 3 | 129 | 43 | 8 |
Patient: rank.gender (F, female; M, male).age (years old); Onset: first clinical signs manifested in upper limb (UL) or lower limb (LL) or bulbar (B); Duration of the disease in months; El Escorial criteria for amyotrophic lateral sclerosis (ALS): definite ALS (Definite), clinically probable ALS (Probable), Clinically probable—laboratory supported ALS (Lab-supported); Atrophy observed (+) or not (−) on the side explored during the electrophysiological investigations; ALSFR-S: total score of the revised ALS functional resting scale (Total/maximal score 48) and for handwriting (Hand/4); Muscular testing: in upper and lower limbs and in the neck (Total/150), on the side explored during the electrophysiological investigations (UL/45) and in intrinsic hand muscles (Hand/10).