Table 1.
Clinical and demographic characteristics of ALS patients.
| Age at symptoms onset (years) (median; IQR) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) |
60 (52–67) 50 years; 26 66 years; 34 |
| Sex (Male/Female) Male (%) |
28/32 (47%) |
| Age at diagnosis (years) (median; IQR) |
61 (53–68) |
| Onset to diagnosis interval (months) (median; IQR) |
10.5 (5.95–16.62) |
| Site of onset: Bulbar/Spinal (n. of patients) Bulbar (%) |
18/42 (30%) |
| ALS phenotypes: Classic/Bulbar/Flail arm/Flail leg/Pyramidal/Respiratory/PLMN/PUMN (n. of patients) Classic (%) |
50/4/0/0/1/0/5/0 (83%) |
| ALSFRS-r at diagnosis (median; IQR) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) |
41 (33–44) 42, 44 27, 16 |
| ALSFRS-r-B subscore at diagnosis (median; IQR) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) |
11 (9–12) 11; 50 7; 10 |
| ALSFRS-r-S subscore at diagnosis (median; IQR) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) |
19 (12–22) 20; 43 9; 17 |
| Midbrain volume (mm3) (Estimated marginal mean*) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) Cluster III (centroid; n. of patients) |
5649 5897 mm3; 28 5526 mm3; 8 5400 mm3; 24 |
| Pons volume (mm3) (Estimated marginal mean*) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) Cluster III (centroid; n. of patients) |
13807 14611 mm3; 20 13869 mm3; 16 13097 mm3; 24 |
| Medulla oblongata volume (mm3) (Estimated marginal mean*) Cluster I (centroid; n. of patients) Cluster II (centroid; n. of patients) Cluster III (centroid; n. of patients) |
3418 3628 mm3; 20 3401 mm3; 20 3227 mm3; 20 |
| Tracheostomy, number of patients (yes/no) yes (%) |
24/36 (40.7%) |
| Death, number of patients (yes/no) yes (%) |
38/22 (63.3%) |
| Composite outcome, number of patients (yes/no) yes (%) |
47/13 (78.3%) |
| Survival time from symptoms onset to composite outcome (months) (estimated median; 95% C.I.) |
41 (40.12 – 43.7) |
Centroid and n. patients were calculated for age at symptoms onset, total ALSFRS-r, bulbar subscores of ALSFRS-r (ALSFRS-r-B), spinal subscores of ALSFRS-r (ALSFRS-r-S) and volume of each brainstem structure.
* Estimated marginal means were calculated using age at first neurological evaluation, gender and total intracranial volume (TIV) as covariates. (Age at first neurological evaluation = 60.44, gender = 1.47, TIV = 1443686.89).
PLMN: pure lower motor neuron phenotype.
PUMN: pure upper motor neuron phenotype.