Table 1 Detailed ALS patient and specimen data.
Patient (Number) | Sex | Age at onset (years) | Age at biopsy (years) | Site of onset | Familiar recurrence | Time from onset to biopsy (months) | Patients stratification based on duration of disease at biopsy | Site of biopsy | MRC score* | ALSFRS-R** | Disease duration (months) | Patients stratification based on evolution of the disease | Gene mutation | Gene expression | MicroRNAs expression | Cell Cultures | Protein expression | Histology |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | F | 50 | NA | Spinal | Sporadic | NA | NA | Deltoid | F = 0 | NA | 134 (T) | slow | Negative | x | x | x | ||
2 | F | 70 | 71 | Spinal | Sporadic | 13 | late | Quadriceps | F = 5 | 33/48 | 30 (D) | rapid | Negative | x | x | x | ||
3 | M | 69 | 71 | Spinal | Sporadic | 18 | late | Deltoid | NA | NA | 16 (T) | rapid | Negative | x | x | x | ||
4 | M | 63 | 63 | Spinal | Sporadic | 7 | early | Quadriceps | F = 5 | 36/48 | 28 (D) | rapid | Negative | x | x | |||
5 | M | 65 | 65 | Spinal | Sporadic | 6 | early | Deltoid | F = 3 | 42/48 | 59 (D) | slow | Negative | x | x | |||
6 | M | 43 | 43 | Spinal | Sporadic | 9 | early | NA | NA | 37/48 | 14 (LF) | NA | Negative | x | x | |||
7 | F | 59 | 59 | Spinal | Sporadic | 10 | early | Quadriceps | F = 5 | 42/48 | 58 (D) | slow | C9ORF72 | x | x | x | ||
8 | F | 54 | 55 | Bulbar | Sporadic | 19 | late | Deltoid | F = 5 | 43/48 | 39 (D) | rapid | Negative | x | x | x | ||
9 | M | 50 | 53 | Spinal | Familial | 28 | late | Quadriceps | F = 5 | 41/48 | 48 (A) | slow | Negative | x | x | x | x | x |
10 | F | 28 | 30 | Spinal | Sporadic | 26 | late | Quadriceps | F = 5 | 41/48 | 48 (A) | slow | Negative | x | x | x | x | |
11 | M | 69 | 70 | Bulbar | Sporadic | 13 | late | Quadriceps | F = 4 | 23/48 | 12 (T) | rapid | Negative | x | x | x | x | x |
12 | F | 51 | 54 | Spinal | Sporadic | 36 | late | Quadriceps | NA | NA | 52 (A) | slow | Negative | x | x | x | ||
13 | F | 55 | 56 | Spinal | Familial | 14 | late | Quadriceps | F = 3 | 43/48 | 25 (A) | NA | C9ORF72 | x | x | x | ||
14 | F | 57 | 57 | Spinal | Familial | 13 | late | Quadriceps | F = 5 | 46/48 | 18 (A) | NA | SOD1 L84F | x | x | x | x |
