Table 1.
Demographic and clinical characteristics of the patients with ALS examined in this study.
| Demographic and clinical characteristics | |
|---|---|
| Phenotype (Total n% from 499 cases) | |
| Limb-onset ALS | 377 (75.56%) |
| Bular-onset ALS | 88 (17.64%) |
| Flail-arm syndrome (FAS) | 30 (6.01%) |
| Progressive muscular atrophy (PMA) | 1 (0.20%) |
| Primary lateral sclerosis (PLS) | 3 (0.60%) |
| Male (Total n% from 499 cases) | 321 (64.32%) |
| Age at onset (years ± SD) | 51.29 ± 11.87 |
| BMI(kg/m2) | 23.21 ± 3.28 |
| Smoking | 124 (24.84%) |
| Alcohol abusing | 94 (18.84%) |
| Pesticides | 76 (15.23%) |
| Riluzole using | 139 (27.85%) |
| Treated with non-invasive positive pressure ventilation | 44 (8.81%) |
| Disease progression ΔFS Median [25th, 75th percentiles] | 0.72 [0.29–0.89] |
ΔFS: progression rate of ALSFRS-R: (48-ALSFRS-R score at first visit)/(time in months between the first symptoms and the first examination).