Table 1.
# | Range of age | ALSFRS-R [0 48] | MUNIX left | MUNIX right | Time span since onset | Onset | Progression rate |
---|---|---|---|---|---|---|---|
1 | 21–25 | 30 | 6.63 | 10.43 | 49 | Arm | 0.37 |
2 | 26–30 | 35 | 8.20 | 29.21 | 28 | Arm | 0.46 |
3 | 31–35 | 24 | 6.94 | 2.83 | 28 | Leg | 0.86 |
4 | 41–45 | 24 | * | * | 28 | Leg | 0.86 |
5 | 41–45 | 41 | 54.12 | 38.00 | 19 | Leg | 0.37 |
6 | 41–45 | 28 | 4.85 | 0.70 | 38 | Arm | 0.53 |
7 | 41–45 | 46 | * | * | 16 | Arm | 0.13 |
8 | 46–50 | 39 | 138.40 | 41.88 | 56 | Leg | 0.16 |
9 | 46–50 | 48 | 170.10 | 187.30 | 19 | Leg | 0.00 |
10 | 46–50 | 24 | 48.37 | 18.51 | 24 | Arm | 1.00 |
11 | 46–50 | 46 | 213.60 | 193.00 | 3 | Leg | 0.67 |
12 | 46–50 | 40 | 114.50 | 97.18 | 21 | Leg | 0.38 |
13 | 46–50 | 38 | * | * | 25 | Leg | 0.40 |
14 | 51–55 | 42 | 27.25 | 21.62 | 7 | Arm | 0.86 |
15 | 51–55 | 35 | * | * | 33 | Leg | 0.39 |
16 | 56–60 | 38 | 123.90 | 101.60 | 29 | Leg | 0.34 |
17 | 56–60 | 44 | 126.90 | 0.00 | 13 | Leg | 0.31 |
18 | 61–65 | 42 | 83.10 | 119.00 | 10 | Leg | 0.60 |
19 | 61–65 | 24 | 12.34 | 9.66 | 33 | Leg | 0.73 |
20 | 66–70 | 21 | 6.94 | 2.83 | 36 | Leg | 0.75 |
Summary of baseline characteristics of all 20 limb-onset ALS patients including age (in ranges of years), ALSFRS-R sum score upon initial MRI scan (48 in clinical non affected), neurophysiological MUNIX scores for left and right ADM upon initial MRI scan, the length of time span between symptom onset and initial MRI scan in months, onset of disease (arm, leg), and progression rate. In four out of 20 ALS patients (marked with *), neurophysiological assessment was still conducted using MUNE technique (patient 4: left MUNE = 2, right MUNE = 1; patient 7: left MUNE = 3, MUNE right = 77; patient 13: left MUNE = 275, right MUNE = 85; patient 15: left MUNE = 240, right MUNE = 120). No MUNIX scores were obtained in these four patients. Progression rates were calculated by [48-ALSFRS-R sum score/months since symptom onset; see (8)]. Neuropsychological assessment using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was conducted only in patient 3 (129/136 score points) and patient 9 (100/136 score points).