Demographic and clinical characteristics of the patients with ALS examined in this study
| Phenotype | ||||||
|---|---|---|---|---|---|---|
| Limb-onset ALS | Bulbar-onset ALS | FAS | PMA | PLS | Total | |
| Total n (%) from 1624 cases | 1220 (75.1) | 227 (14.0) | 126 (7.8) | 43 (2.6) | 8 (0.5) | 1624 |
| Male | 748 (72.6) | 129 (12.5) | 112 (10.9) | 35 (3.4) | 6 (0.6) | 1030 |
| Female | 472 (79.5) | 98 (16.5) | 14 (2.4) | 8 (1.4) | 2 (0.3) | 594 |
| M:F ratio | 1.6:1 | 1.3:1 | 8.0:1 | 4.4:1 | 3.0:1 | 1.7:1 |
| Age at onset (years) | ||||||
| Mean (95% CI) | 48.7 (48.0 to 49.3) | 53.3 (51.8 to 54.8) | 55.0 (53.0 to 56.9) | 47.7 (44.1 to 51.3) | 50.0 (46.0 to 54.0) | 49.8 (49.2 to 50.3) |
| Median (range) | 49 (18–82) | 53 (22–79) | 55 (25–76) | 45 (28–74) | 51 (41–56) | 50 (18–82) |
| BMI (kg/m2) | ||||||
| Mean (95% CI) | 23.1 (22.8 to 23.3) | 22.2 (21.7 to 22.7) | 23.1 (22.4 to 23.8) | 23.0 (21.6 to 24.4) | 24.7 (22.1 to 27.3) | 22.9 (22.7 to 23.1) |
| Median (range) | 23.1 (13.7–35.0) | 21.7 (14.1–32.6) | 23.3 (17.3–31.6) | 22.8 (16.5–28.4) | 25.0 (19.2–29.4) | 22.9 (13.7–35.0) |
| Diagnostic delay from symptom onset (months) | ||||||
| Mean (95% CI) | 19.6 (18.4 to 20.8) | 15.6 (14.0 to 17.2) | 29.1 (23.2 to 34.9) | 28.5 (19.1 to 37.8) | 55.4 (42.6 to 68.2) | 20.2 (19.1 to 21.3) |
| Median (range) | 13 (0–228) | 12 (0–115) | 19 (3–222) | 20.5 (4–134) | 49 (42–84) | 14 (0–228) |
| Airlie House category at presentation, n (%) | ||||||
| Clinically definite | 344 (28.2) | 83 (36.6) | 9 (7.1) | 0 | 0 | 436 (26.8) |
| Clinically probable | 302 (24.8) | 53 (23.3) | 15 (11.9) | 0 | 0 | 370 (22.8) |
| Laboratory-supported probable | 157 (12.9) | 34 (15.0) | 13 (10.3) | 0 | 0 | 204 (12.6) |
| Clinically possible | 224 (18.4) | 46 (20.3) | 42 (33.3) | 0 | 8 (100.0) | 320 (19.7) |
| Suspected* | 12 (1.0) | 2 (0.9) | 7 (5.6) | 43 (100.0) | 0 | 64 (3.9) |
| Missing | 181 (14.8) | 9 (4.0) | 40 (31.7) | 0 | 0 | 230 (14.2) |
| Number of cases reaching the end point by 1 May 2013, n (%) | 447 (36.6) | 96 (42.3) | 48 (38.1) | 15 (34.9) | 0 | 606 (37.3) |
| 5-year survival rate, % | 49 | 37 | 58 | 64 | 100 | 49 |
| 10-year survival rate, % | 33 | 32 | 37 | 37 | 100 | 32 |
*Patients with pure lower motor syndromes were assigned to an additional category of suspected ALS because they could not be classified using the diagnostic criteria for ALS.
ALS, amyotrophic lateral sclerosis; BMI, body mass index; F, female; FAS, flail arm syndrome; M, male; PLS, primary lateral sclerosis; PMA, progressive muscular atrophy.