Table I.
Clinical features and functional scores of the study participants.
| Diagnosis | Age at scan | Sex | Symptom onset | Duration (months) | MRI interval (months) | ALSFRS-R baseline, follow-up | UMN score baseline, follow-up | Baseline rate of progression | TMT B – A (seconds | ACE (max 100) | Verbal fluency (max 7) |
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|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Letter | Category | |||||||||||||
| ALS01 | 65 | M | BO | 30 | 7.4 | 43 | 43 | 13 | 11 | 0.17 | 41 | 98 | 6 | 7 |
| ALS02 | 67 | M | RUL | 70 | 5.7 | 35 | 35 | 3 | 3 | 0.19 | – | 96 | 4 | 7 |
| ALS03 | 70 | M | RUL | 46 | 7.8 | 27 | 27 | 6 | 5 | 0.46 | – | 90 | 4 | 7 |
| ALS04 | 61 | M | LLL | 34 | 8.0 | 28 | 21 | 7 | 3 | 0.59 | 50 | 91 | 6 | 6 |
| ALS06 | 67 | F | BO | 63 | 6.3 | 33 | – | 13 | – | 0.24 | 9 | – | – | – |
| ALS07 | 77 | M | BO | 57 | 6.5 | 27 | 26 | 10 | 10 | 0.37 | 73 | 73 | – | – |
| ALS08 | 64 | M | LLL | 88 | 6.7 | 28 | 28 | 1 | 1 | 0.23 | 68 | 87 | 6 | 4 |
| ALS09 | 55 | F | RLL | 50 | – | 23 | – | 9 | – | 0.49 | 31 | 95 | 5 | 7 |
| ALS11 | 49 | F | RUL | 80 | 6.0 | 29 | 29 | 5 | 5 | 0.24 | – | 97 | 5 | 7 |
| ALS12 | 41 | M | LUL | 41 | 6.9 | 26 | 23 | 15 | 15 | 0.54 | 27 | 99 | 6 | 7 |
| ALS13 | 69 | M | LUL | 33 | 5.3 | 39 | 34 | 4 | 4 | 0.27 | 29 | 93 | 5 | 6 |
| ALS14 | 48 | F | RLL | 135 | 9.7 | 30 | 30 | 10 | 10 | 0.13 | 41 | 92 | 5 | 6 |
| ALS15 | 61 | M | RUL | 141 | – | 23 | – | 1 | – | 0.18 | – | 93 | 6 | 7 |
| ALS16 | 56 | F | LLs | 65 | 7.4 | 27 | 24 | 3 | 3 | 0.32 | – | 99 | 7 | 7 |
| ALS18 | 46 | F | RLL | 21 | 7.3 | 32 | 18 | 12 | 12 | 0.77 | 10 | 97 | 6 | 7 |
| ALS19 | 56 | F | LLL | 98 | 7.9 | 31 | 30 | 3 | 4 | 0.17 | 21 | 100 | 7 | 7 |
| ALS20 | 49 | M | LLL | 28 | 6.2 | 35 | 31 | 15 | 15 | 0.46 | – | – | 5 | 5 |
| ALS21 | 76 | M | RLL | 14 | 6.0 | 34 | 28 | 7 | 11 | 1.00 | 63 | 85 | 5 | 6 |
| ALS22 | 76 | M | Trunk | 61 | – | 43 | – | 3 | – | 0.08 | 54 | 98 | 6 | 6 |
| ALS24 | 55 | M | RLL | 15 | – | 42 | – | 12 | – | 0.41 | 23 | 97 | 6 | 7 |
| ALS25 | 75 | M | LLL | 81 | – | 35 | – | 13 | – | 0.16 | 46 | 76 | 6 | 5 |
| ALS26 | 52 | F | RLL | 20 | – | 38 | – | 11 | – | 0.51 | 19 | 93 | 4 | 6 |
| ALS27 | 64 | F | RUL | 8 | – | 18 | – | 14 | – | 3.94 | – | – | – | – |
| ALS mean (SD) | 61 (11) | 9F:14M | 56 (36) | 7 (1) | 32 (7) | 28 (6) | 8 (5) | 8 (5) | 0.52 (0.78) | 38 (20) | 92 (7) | 5 (1) | 6 (1) | |
| PLS01 | 63 | F | LLL | 92 | 7.3 | 32 | 27 | 15 | 15 | 0.17 | 30 | 98 | 6 | 7 |
| PLS02 | 45 | F | LLs | 180 | – | 35 | – | 15 | – | 0.07 | 27 | 77 | 5 | 6 |
| PLS03 | 77 | F | LLs | 62 | 7.2 | 34 | 32 | 10 | 10 | 0.22 | 70 | 96 | 7 | 5 |
| PLS04 | 77 | F | BO | 155 | – | 30 | – | 15 | – | 0.12 | 18 | 92 | 5 | 5 |
| PLS05 | 64 | M | RLL | 184 | 6.3 | 20 | 18 | 15 | 15 | 0.15 | – | 98 | – | – |
| PLS06 | 70 | M | RLL | 366 | 7.4 | 29 | 27 | 13 | 15 | 0.05 | 45 | 94 | 5 | 5 |
| PLS07 | 65 | F | LLL | 260 | – | 28 | – | 15 | – | 0.08 | 42 | – | 4 | 3 |
| PLS mean (SD) | 66 (11) | 5F:2M | 186 (103) | 7 (1) | 30 (5) | 26 (14) | 14 (2) | 14 (8) | 0.12 (0.06) | 39 (18) | 93 (8) | 5 (1) | 5 (1) | |
| Control | 66 | M | – | – | – | – | – | – | – | – | 24 | 100 | 7 | 7 |
| Control | 38 | F | – | – | – | – | – | – | – | – | 23 | 93 | 7 | 7 |
| Control | 46 | M | – | – | – | – | – | – | – | – | 36 | 97 | 7 | 7 |
| Control | 68 | F | – | – | – | – | – | – | – | – | 14 | 99 | 7 | 7 |
| Control | 38 | F | – | – | – | – | – | – | – | – | 15 | 98 | 5 | 7 |
| Control | 56 | F | – | – | – | – | – | – | – | – | 12 | 100 | 7 | 7 |
| Control | 46 | M | – | – | – | – | – | – | – | – | 20 | 99 | 7 | 7 |
| Control | 65 | F | – | – | – | – | – | – | – | – | 17 | 98 | 5 | 7 |
| Control | 66 | M | – | – | – | – | – | – | – | – | 23 | 100 | 7 | 7 |
| Control | 72 | M | – | – | – | – | – | – | – | – | 16 | 99 | 7 | 7 |
| Control | 45 | F | – | – | – | – | – | – | – | – | 10 | 98 | 7 | 7 |
| Control | 64 | F | – | – | – | – | – | – | – | – | 19 | 99 | 7 | 7 |
| Control mean (SD) | 56 (12) | 7F:5M | 19 (7) | 98 (2) | 7 (1) | 7 (0) | ||||||||
ACE: Addenbrookes Cognitive Examination; BO: bulbar onset; LUL: left upper limb; LLL: left lower limb; LLs: bilateral lower limb onset; RUL: right upper limb; RLL: right lower limb; TMT: Trail-Making Test; UMN: upper motor neuron. Bold type: p < 0.05.