Table 1.
Demographic and clinical characteristics of the patients with ALS.
| All patients with ALS | Patients compared with their spouses | Patients compared with healthy controls | |
|---|---|---|---|
| Total, n | 160 | 63 | 148 |
| Age, year [mean (95% CI)] | 53.98 (52.50–55.46) | 51.93 (49.87–54.00) | 53.76 (52.19–55.34) |
| Male to female ratio | 1.58:1 | 1.73:1 | 1.51:1 |
| Diagnostic delay, day [median (IQR)] | 385.5 (462) | 401.0 (349) | 388.5 (457.75) |
| FRS-R score [median (IQR)] | 41.0 (9) | 39.0 (10) | 41.0 (9) |
| Phenotype, n (%) | |||
| Limb-onset ALS | 98 (61.3) | 38 (60.3) | 91 (61.5) |
| Bulbar-onset ALS | 27 (16.9) | 11 (17.5) | 26 (17.6) |
| FAS | 24 (15.0) | 9 (14.3) | 20 (13.5) |
| PMA | 4 (2.5) | 1 (1.6) | 4 (2.7) |
| Familial ALS | 7 (4.4) | 4 (6.3) | 7 (4.7) |
| Category, n (%) | |||
| Definite | 65 (40.6) | 26 (41.3) | 60 (40.5) |
| Probable | 42 (26.3) | 17 (27.0) | 39 (26.4) |
| Probable supported by laboratory findings | 24 (15.0) | 8 (12.7) | 22 (14.9) |
| Possible | 27 (16.9) | 11 (17.5) | 25 (16.9) |
| Pure LMN impairment | 2 (1.3) | 1 (1.6) | 2 (1.4) |
| Riluzole, n (%) | 122 (76.3) | 46 (73.0) | 112 (75.7) |
| Smoking, n (%) | 53 (33.1) | 24 (38.1) | 49 (33.1) |
| Alcohol abuse, n (%) | 44 (37.5) | 22 (34.9) | 39 (26.4) |
| History of contact with pesticides, n (%) | 28 (17.5) | 10 (15.9) | 24 (17.0) |
ALS amyotrophic lateral sclerosis, FAS flail arm syndrome, PMA progressive muscular atrophy, LMN lower motor neuron.