Baseline data of 4 cohorts of MND phenotypes and healthy controlsa
| PLS (n = 12) | ALS-L (n = 12) | ALS-B (n = 12) | PMA (n = 12) | Controls (n= 12) | |
|---|---|---|---|---|---|
| Male/female | 7:5 | 10:2 | 5:7 | 11:1 | 7:5 |
| Age (yr) (median/range) | 59.0 (48–74) | 58.0 (41–78) | 56.5 (42–72) | 60.5 (44–72) | 56.5 (46–80) |
| Duration symptoms (mo) | 50.2 ± 24.8 | 10.5 ± 2.5 | 9.8 ± 3.2 | 10.8 ± 2.0 | NA |
| Diagnostic delay (mo) | 24.4 ± 16.0 | 6.6 ± 2.5 | 6.7 ± 2.7 | 6.6 ± 2.9 | NA |
| Disease progression rateb | 0.010 ± 0.005 | 0.027 ± 0.016 | 0.026 ± 0.018 | 0.020 ± 0.010 | NA |
| VC (%) | 91.5 ± 23.3.028 | 101.3 ± 23.8 | 98.5 ± 17.9.043 | 102.5 ± 14.9 | 114.6 ± 18.5 |
| ALSFRS-R | 35.0 ± 4.6<.001 | 40.1 ± 3.9<.001 | 40.1 ± 4.7<.001. | 41.8 ± 3.7<.001 | 48.0 ± 0 |
| Finger tapping/sc | 2.3 ± 1.2<.001 | 3.3 ± 1.3.002 | 4.0 ± 1.3.013 | 4.8 ± 0.7 | 5.1 ± 0.6 |
| Foot tapping/sc | 1.4 ± 0.9<.001 | 2.4 ± 1.1.19 | 2.9 ± 1.3 | 2.4 ± 1.4 | 3.4 ± 0.7 |
| NAAx | 7.46 ± 0.80<.001 | 8.56 ± 1.16 | 8.78 ± 1.12 | 9.58 ± 0.78 | 9.32 ± 0.84 |
| Glx | 8.06 ± 1.34 | 8.75 ± 1.68 | 7.99 ± 1.85 | 7.68 ± 1.66 | 8.42 ± 1.47 |
| mIns | 4.78 ± 0.80.024 | 4.56 ± 0.94 | 4.28 ± 0.81 | 4.26 ± 0.91 | 3.93 ± 0.74 |
| NAAx/mIns | 1.62 ± 0.38.001 | 1.97 ± 0.59.024 | 2.12 ± 0.49 | 2.35 ± 0.55 | 2.46 ± 0.56 |
a Data are given as mean ± SD, unless otherwise indicated. Statistical significance compared with controls was set at P < .05 (P values in superscript).
b Disease progression rate = (48 − ALSFRS-R score at baseline) / disease duration (days).
c Finger and foot tapping: (L + R) / 2.