Table 1 Characteristics of the study participants (n = 188) and comparison between ALS bulbar onset (n = 67) and ALS limb onset (n = 121)a.
Variables | Total (n = 188) | ALS bulbar onset (n = 67) | ALS limb onset (n = 121) | p-value |
---|---|---|---|---|
Sex, n (%) | 0.982 | |||
Male | 90 (47.9) | 32 (47.8) | 32 (47.8) | |
Female | 98 (52.1) | 35 (52.2) | 63 (52.1) | |
Age at symptom onset (years, median (IQR)) | 57.1 (49.7–64.0) | 60.1 (53.8–67.7) | 55.8 (47.5–61.0) | < 0.001 |
Age at PEG placement (years, median (IQR)) | 60.0 (53.1–66.3) | 62.4 (55.9–69.5) | 58.3 (51.3–63.3) | < 0.001 |
Site of onset, n (%) | < 0.001 | |||
Bulbar | 67 (35.6) | 67 (100.0) | 0 (0.0) | |
Limb | 121 (64.4) | 0 (0.0) | 121 (100.0) | |
Time from symptom onset to PEG (months, median (IQR)) | 30 (20–44) | 23 (19–41) | 32 (23–47) | 0.012 |
Time from diagnosis to PEG (months, median (IQR)) | 19 (11–31) | 13 (8–24) | 23 (13–34) | < 0.001 |
Incidence of aspiration pneumonia, n (%) | 54 (28.7) | 14 (20.9) | 40 (33.1) | 0.078 |
Before PEG insertion, n (%) | 10 (5.3) | 2 (3.0) | 8 (6.6) | 0.289 |
After PEG insertion, n (%) | 49 (26.1) | 14 (20.9) | 35 (28.9) | 0.230 |
Tracheostomy, n (%) | 84 (44.7) | 22 (32.8) | 62 (51.2) | 0.015 |
Before PEG insertion, n (%) | 27 (14.4) | 5 (7.5) | 22 (18.2) | 0.045 |
After PEG insertion, n (%) | 57 (30.3) | 17 (25.4) | 40 (33.1) | 0.272 |
Death, n (%) | 61 (32.5) | 25 (37.3) | 36 (29.8) | 0.289 |
Time from symptom onset to death (months, median (IQR)) | 39 (28–55) | 34 (24–58) | 39.5 (30.3–53.3) | 0.395 |
Pharmacological treatment options received | ||||
Riluzole, n (%) | 144 (76.6) | 49 (73.1) | 95 (78.5) | 0.404 |
Edaravone, n (%) | 39 (20.7) | 15 (22.4) | 24 (19.8) | 0.679 |
Nuedexta, n (%) | 7 (3.7) | 2 (3.0) | 5 (4.1) | 0.691 |