Table 1.
Demographic and clinical features of patients according to age group at diagnosis.
| Age at Diagnosis ≤80 years | Age at Diagnosis >80 years | Total | p Value | ||||
|---|---|---|---|---|---|---|---|
| N or Mean | % or SD | N or Mean | % or SD | N or Mean | % or SD | ||
| Male sex | 783 | 56.29 | 102 | 45.94 | 885 | 54.87 | 0.004 |
| Mean age at onset, years | 64.44 | 10.13 | 82.83 | 3.76 | 67.01 | 11.42 | <0.001 |
| Mean age at diagnosis, years | 65.53 | 10.12 | 83.97 | 3.66 | 68.06 | 11.42 | <0.001 |
| Mean diagnostic delay, months | 13.66 | 13.73 | 13.71 | 11.56 | 13.67 | 13.44 | 0.954 |
| Site of onset ° | <0.001 | ||||||
| Bulbar | 373 | 32.58 | 85 | 51.52 | 458 | 34.96 | <0.001 |
| Spinal UL | 381 | 33.28 | 33 | 20.00 | 414 | 31.60 | <0.001 |
| Spinal LL | 361 | 31.53 | 47 | 28.48 | 408 | 31.15 | 0.128 |
| Respiratory | 30 | 2.62 | 0 | 0.00 | 30 | 2.29 | 0.012 |
| Phenotype ° | <0.001 | ||||||
| Bulbar | 369 | 32.09 | 86 | 52.43 | 455 | 34.63 | <0.001 |
| Classic | 515 | 44.78 | 52 | 31.71 | 567 | 43.15 | <0.001 |
| Flail Arm | 58 | 5.04 | 5 | 3.05 | 63 | 4.79 | 0.171 |
| Flail Leg | 105 | 9.13 | 15 | 9.15 | 120 | 9.13 | 0.676 |
| UMNp | 74 | 6.43 | 5 | 3.05 | 79 | 6.01 | 0.049 |
| Respiratory | 29 | 2.52 | 1 | 0.61 | 30 | 2.28 | 0.094 |
| FTD ° | 94 | 7.87 | 19 | 10.98 | 113 | 8.27 | 0.165 |
| Family history of ALS/FTD ° | 180 | 15.07 | 21 | 12.14 | 201 | 14.70 | 0.308 |
| BMI at diagnosis °, Kg/m2 | 24.57 | 4.01 | 23.12 | 3.54 | 24.41 | 3.99 | <0.001 |
| Weight loss at diagnosis °, number | 507 | 55.11 | 80 | 70.17 | 587 | 56.77 | 0.002 |
| Weight loss at diagnosis, kg | 3.92 | 6.02 | 5.14 | 6.49 | 4.06 | 6.09 | 0.043 |
| Progression rate at diagnosis *, points/month | 0.95 | 1.18 | 1.43 | 1.31 | 1.01 | 1.21 | <0.001 |
| Mean ALSFRS-R score at diagnosis °, points | 38.97 | 7.32 | 34.60 | 8.82 | 38.44 | 7.64 | <0.001 |
| Mean FVC value at diagnosis °, % | 86.00 | 25.37 | 72.33 | 27.13 | 84.63 | 25.85 | <0.001 |
| Genetic analysis ° | 0.111 | ||||||
| no mutations | 471 | 86.58 | 52 | 94.55 | 523 | 87.31 | 0.091 |
| C9ORF72 expansion | 39 | 7.17 | 0 | 0.00 | 39 | 6.51 | 0.040 |
| SOD1 mutations | 17 | 3.13 | 1 | 1.82 | 18 | 3.01 | 0.589 |
| FUS mutations | 5 | 0.92 | 2 | 3.64 | 7 | 1.17 | 0.074 |
| TARDBP mutations | 4 | 0.74 | 0 | 0.00 | 4 | 0.67 | 0.523 |
| Other genes involvement | 8 | 1.47 | 0 | 0.00 | 8 | 1.34 | 0.365 |
| Death | 1046 | 76.68 | 202 | 91.40 | 1248 | 78.73 | <0.001 |
| Total | 1391 | 86.24 | 222 | 13.76 | 1613 | 100 | |
SD: standard deviation; spinal UL: spinal upper limb; spinal LL: spinal lower limb; UMNp: upper motor neuron predominant; FVC: forced vital capacity; FTD: frontotemporal dementia. * Progression rate at diagnosis was calculated as the monthly decline in ALSFRS-R scores, assuming a total score of 48 at onset; it was calculated for 1162 total patients. ° Data not available for all patients; FVC% value was listed for 600 subjects; family history and FTD data were available for 1367 patients, weight loss for 1034 cases, and BMI for 1114 cases. Onset type was available for 1310 cases, and the phenotype for 1314. Genetic tests were performed on 599 patients.