Table 1.
Clinical and D50 model parameters of the ALS cohort.
| High | Intermediate | Low | |
|---|---|---|---|
| (0 ≤ D50 < 20) | (20 ≤ D50 < 40) | (D50 ≥ 40) | |
| n = 64 | n = 92 | n = 66 | |
| M50 in months | 8.10 (5.81–11.4) | 14.6 (10.8–20.7) | 29.3 (19.8–43.2) |
| MUNIX APB | 38.9 (2.00–91.8) | 55.7 (14.9–100.3) | 50.5 (15.0–100.5) |
| MUNIX TA | 76.0 (37.1–108.6) | 47.3 (2.00–92.3) | 45.3 (2.00–99.1) |
| MUNIX ADM | 73.6 (31.7–111.0) | 71.3 (23.3–120.4) | 66.3 (10.0–108.3) |
| MUSIX200 in months | 9.04 (6.91–15.9) | 16.8 (11.7–24.2) | 33.1 (21.4–45.7) |
| MUSIX APB | 97.8 (67.3–250.0) | 89.3 (64.9–180.0) | 88.2 (68.1–174.2) |
| MUSIX TA | 49.3 (43.3–65.8) | 59.4 (47.4–250.0) | 73.2 (50.1–250.0) |
| MUSIX ADM | 93.7 (72.4–133.7) | 91.7 (72.0–130.9) | 103.7 (75.4–194.3) |
| CMAP50 in months* | 8.62 (6.37–14.2) | 17.8 (12.0–24.4) | 34.8 (21.3–58.6) |
| CMAP APB | 3.78 (0.50–6.71) | 4.66 (1.44–6.63) | 4.49 (2.06–6.96) |
| CMAP TA | 3.72 (1.96–5.22) | 2.74 (0.50–4.95) | 3.33 (0.50–5.38) |
| CMAP ADM | 6.16 (3.10–8.45) | 6.81 (3.09–9.12) | 6.38 (1.11–9.59) |
| n of set small values | 18/9/10 | 17/24/11 | 11/20/13 |
| D50 disease progression model parameters | |||
| rD50 at MUNIX | 0.36 (0.23–0.45) | 0.28 (0.18–0.41) | 0.22 (0.12–0.34) |
| rD50 phase (I/II/III-IV) | 21/33/10 | 35/46/11 | 36/28/2 |
| D50 in months | 13.8 (8.57–17.1) | 28.0 (23.3–32.4) | 61.5 (47.0–94.1) |
| Demographic and clinical parameters | |||
| Age at MUNIX measurement | 69.3 (58.9–75.5) | 65.7 (58.9–69.9) | 64.6 (57.6–70.6) |
| Gender (female/male) | 30/34 | 39/53 | 28/38 |
| Disease progression rate** | 1.41 (0.91–2.39) | 0.64 (0.47–0.80) | 0.25 (0.16–0.38) |
| ALSFRS-R at MUNIX measurement** | 36 (30–41) | 38 (32–43) | 40 (35–45) |
| n of ALSFRS-R observations | 3 (3–5) | 7 (4–10) | 11 (6–17) |
| Disease duration at MUNIX | 8.30 (6.09–12.4) | 15.7 (10.4–24.3) | 30.9 (19.6–45.1) |
| ALS phenotype | |||
| Classic | 33 | 53 | 42 |
| Bulbar | 30 | 34 | 15 |
| Flail Arm | 0 | 1 | 3 |
| Flail Leg | 0 | 0 | 2 |
| Pyramidal | 0 | 1 | 3 |
| PLMN | 1 | 3 | 1 |
| Riluzole intake yes/no | 56/8 | 84/8 | 58/8 |
| Revised El Escorial criteria | |||
| Definite | 26 | 40 | 18 |
| Probable | 17 | 24 | 17 |
| LSPR | 21 | 26 | 25 |
| Possible | 0 | 2 | 6 |
Values are given as median and interquartile range or numbers.
ALS amyotrophic lateral sclerosis, CMAP compound muscle action potential, MUNIX motor unit number index, MUSIX motor unit size index, LSPR laboratory-supported probable, PLMN pure lower motor neuron. *Related to n = 62 in the high, n = 91 in the intermediate and n = 63 in the low aggressiveness subgroup, because 6 patients had no loss of function in comparison to the mean of CMAP of healthy controls. **Related to 199 of 222 ALS patients in whom the range between MUNIX and ALSFRS-R was 0 ± 4 weeks. Phenotypes in accordance to Chio et al.25.