Table 1.
Parameters at FD | N | Category | Total | Death during 12 months from FD | Log- rank test | |||
---|---|---|---|---|---|---|---|---|
ob-served | mis-sing | n = 60 | % | X 2 (DF) | p (X 2 ) | |||
Age | 176 | 0 | ≤ 65 | 69 | 18 | 26% | 24.13 (2) | < 0.0001 |
66-75 | 84 | 25 | 30% | |||||
> 75 | 23 | 17 | 74% | |||||
Gender | 176 | 0 | male | 96 | 31 | 32% | 0.29 (1) | 0.59 |
female | 80 | 29 | 36% | |||||
Site of onset | 176 | 0 | bulbar | 62 | 25 | 40% | 1.71 (1) | 0.19 |
spinal | 114 | 35 | 31% | |||||
Site of spinal onset | 114 | 0 | upper limbs | 39 | 10 | 26% | 2.58 (4) | 0.64 |
lower limbs | 60 | 20 | 33% | |||||
upper + lower limbs | 8 | 3 | 38% | |||||
trunk | 7 | 2 | 29% | |||||
DOD [months] | 176 | 0 | 0 - 6 | 60 | 27 | 45% | 9.41 (3) | 0.024 |
07-12 | 61 | 17 | 28% | |||||
13-24 | 37 | 15 | 41% | |||||
≥ 25 | 18 | 1 | 6% | |||||
BMI [kg/ m 2 ] | 173 | 3 | < 25 | 92 | 34 | 37% | 0.97 (1) | 0.33 |
≥ 25 | 81 | 24 | 30% | |||||
Diff-BMI [kg/ m 2 ] | 173 | 3 | < 1 | 87 | 14 | 16% | 33.14 (2) | < 0.0001 |
1 - < 2 | 31 | 11 | 35% | |||||
≥ 2 | 55 | 33 | 60% | |||||
EEC | 176 | 0 | possible | 31 | 6 | 19% | 16.21 (3) | 0.001 |
probable (lab. conf.) | 54 | 12 | 22% | |||||
probable | 63 | 26 | 41% | |||||
definite | 28 | 16 | 57% | |||||
FRS | 173 | 3 | < 32 | 72 | 44 | 61% | 46.92 (1) | < 0.0001 |
≥ 32 | 101 | 14 | 14% | |||||
FVC [%] | 153 | 23 | < 80 | 31 | 19 | 61% | 28.61 (1) | < 0.0001 |
≥ 80 | 122 | 27 | 22% | |||||
PCO 2 [mm Hg] | 134 | 42 | < 42 | 112 | 33 | 29% | 15.97 (1) | < 0.0001 |
≥ 42 | 22 | 14 | 64% | |||||
CK [U/ l] | 153 | 23 | < 150 | 73 | 27 | 37% | 1.29 (1) | 0.26 |
≥ 150 | 80 | 24 | 30% | |||||
Pre-alcohol cons. status | 149 | 27 | no | 62 | 26 | 42% | 2.99 (1) | 0.084 |
yes | 87 | 19 | 22% | |||||
Pre-smoking status | 151 | 25 | no | 85 | 23 | 27% | 0.69 (1) | 0.41 |
yes | 66 | 23 | 35% | |||||
social life | 153 | 23 | living in a partnership | 109 | 28 | 26% | 1.59 (1) | 0.21 |
single person | 44 | 19 | 43% | |||||
Abbreviations | DOD | delay between onset and diagnosis | FD | first diagnosis | ||||
BMI | body mass index | FRS | functional rating scale | |||||
Diff-BMI | difference between BMI at diagnosis and six months before | FVC | forced vital capacity | |||||
PCO2 | carbondioxide pressure in blood | |||||||
EEC | revised El Escorial criteria | CK | creatinine kinase |