Table 1.
Demographics and clinical characteristics of patients with ALS with different severities of decrements of CMAP-12 amplitude.
| Normal | Mild | Moderate | Severe | Total | p-value | |
|---|---|---|---|---|---|---|
| Total n (%) | 36 (24.2) | 33 (22.1) | 23 (15.4) | 57 (38.3) | 149 | |
| Age at onset | 51 ± 10.3 | 51.4 ± 13.0 | 50.5 ± 9.9 | 50.3 ± 10.0 | 0.964 | |
| Sex ratio (M:F) | 1.4:1 | 1.4:1 | 0.9:1 | 2.2:1 | 1.5:1 | 0.367 |
| Onset site, n (%) | <0.0001 | |||||
| Bulbar | 9 (25.0) | 6 (18.2) | 1 (4.3) | 0 | 16 (10.7) | |
| Upper limbs | 14 (38.9) | 23 (69.7) | 16 (69.6) | 32 (56.1) | 85 (57.1) | |
| Lower limbs | 13 (36.1) | 4 (12.1) | 6 (26.1) | 25 (43.9) | 48 (32.2) | |
| Phenotype, n (%) | 0.066 | |||||
| Limb-onset ALS | 24 (66.7) | 25 (75.8) | 19 (82.6) | 52 (91.2) | 120 (80.5) | |
| Bulbar-onset ALS | 8 (22.2) | 5 (15.1) | 1 (4.3) | 0 | 14 (9.4) | |
| FAS-type ALS | 1 (2.8) | 2 (6.1) | 2 (8.7) | 3 (5.3) | 8 (5.4) | |
| PMA | 2 (5.5) | 0 | 1 (4.3) | 1 (1.8) | 4 (2.7) | |
| Suspected PLS | 1 (2.8) | 1 (3.0) | 0 | 1 (1.8) | 3 (2.0) | |
| Airlie house category (%) | 0.399 | |||||
| Clinically definite | 7 (19.4) | 7 (21.2) | 7 (30.4) | 16 (28.1) | 37 (24.8) | |
| Clinically probable | 6 (16.7) | 7 (21.2) | 9 (39.1) | 17 (29.8) | 39 (26.2) | |
| Laboratory-supported probable | 15 (41.7) | 9 (23.3) | 3 (13.1) | 16 (28.1) | 43 (28.9) | |
| Clinically possible | 6 (16.7) | 9 (27.3) | 3 (13.1) | 7 (12.3) | 25 (16.8) | |
| Suspected | 2 (5.5) | 1 (3.0) | 1 (4.3) | 1 (1.7) | 5 (3.3) | |
| FRS score | 34.6 ± 4.6 | 35.3 ± 3.6 | 33.6 ± 4.0 | 31.7 ± 5.1 | 0.001 | |
| FRS-R score | 42.3 ± 5.0 | 43.2 ± 3.7 | 41.7 ± 4.0 | 39.1 ± 5.4 | 0.001 |