Table 1.
Demographic and clinical features of the ALS patients, stratified by sex.
| Demographic and Clinical Features |
Women N (%), Mean [SD] | Men N (%), Mean [SD] | Total N (%), Mean [SD] | p-Value |
|---|---|---|---|---|
| Mean age at onset, years | 67.84 [11.52] | 66.32 [11.34] | 67.01 [11.44] | 0.009 |
| Mean age at diagnosis, years | 68.95 [11.47] | 67.33 [11.33] | 68.06 [11.42] | 0.004 |
| Mean diagnostic delay, months | 13.57 [12.67] | 13.74 [14.05] | 13.67 [13.44] | 0.807 |
| Site of onset | <0.001 | |||
| Bulbar | 239 (32.83) | 219 (24.75) | 458 (28.30) | <0.001 |
| Spinal, UL | 146 (20.05) | 268 (30.28) | 414 (25.67) | <0.001 |
| Spinal, LL | 182 (25.00) | 226 (25.54) | 408 (25.29) | 0.805 |
| Respiratory | 10 (1.37) | 20 (2.26) | 30 (1.86) | 0.189 |
| Phenotype | <0.001 | |||
| Bulbar | 237 (32.55) | 218 (24.63) | 455 (28.20) | <0.001 |
| Classic | 220 (30.21) | 347 (39.21) | 567 (35.15) | <0.001 |
| Flail arm | 24 (3.30) | 39 (4.41) | 63 (3.91) | 0.252 |
| Flail leg | 45 (6.18) | 75 (8.47) | 120 (7.44) | 0.081 |
| UMN-p | 42 (5.77) | 37 (4.18) | 79 (4.90) | 0.141 |
| Respiratory | 10 (1.37) | 20 (2.26) | 30 (1.86) | 0.190 |
| FTD | 49 (6.73) | 64 (7.23) | 113 (7.01) | 0.829 |
| Family history of ALS/FTD | 84 (11.53) | 117 (13.22) | 201 (12.46) | 0.433 |
| ALSFRS-R score at diagnosis | 37.39 [8.19] | 39.27 [7.09] | 38.45 [7.65] | <0.001 |
| Progression rate at diagnosis * | 1.13 [142] | 0.92 [1.00] | 1.01 [1.21] | 0.002 |
| Progression rate—first to last visit # | 1.38 [1.96] | 1.41 [1.73] | 1.39 [1.83] | 0.786 |
| FVC at diagnosis | 84.61 [27.36] | 84.66 [24.77] | 84.64 [25.85] | 0.979 |
| Monthly FVC decline § | 2.84 [4.35] | 2.53 [3.60] | 2.65 [3.92] | 0.344 |
| Riluzole treatment | 479 (65.80) | 643 (72.66) | 1122 (69.56) | 0.009 |
| Death | 566 (77.74) | 682 (77.06) | 1248 (77.37) | 0.638 |
| Total | 728 (45.13) | 885 (54.87) | 1613 (100) |
Table 1 shows differential distribution of main clinical features between sexes tested with a chi-square test or t-test as appropriate. Spinal UL: spinal upper limb; Spinal LL: spinal lower limb; UMN-p: Upper Motor Neuron predominant; FTD: frontotemporal dementia; FVC: forced vital capacity; SD: standard deviation. * Progression rate at diagnosis was calculated as monthly decline in ALSFRS-R score assuming a total score of 48 at onset; it was calculated for 1162 patients. # Progression rate from first to last visit was calculated as monthly decline in ALSFRS-R score from the first to the last available visit or from the first visit to the first time when a total score of 0 was recorded (whichever came first). It was calculated for 1049 patients in total. § Monthly decline in FVC was calculated for 600 patients in total, considering first FVC% value, the last available FVC% value and time between the first and last FVC.