Background
Methods
Statistical analysis
Results
Survival analysis
Parameters at FD | N | Category | Total | Death during 12 months from FD | Log-rank test | |||
---|---|---|---|---|---|---|---|---|
ob-served | mis-sing | n = 60 | % | X2(DF) | p(X2) | |||
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 |
Parameter | Category | DF | Estimate b | Standard error(b) | Wald X2
| p | OR(95% CI) |
---|---|---|---|---|---|---|---|
Intercept
| 1 | -2.323 | 0.702 | 10.93 |
0.001
| ||
AGE
| <=65
| 0.000 | Reference | 1 | |||
66-75
| 1 | 0.121 | 0.473 | 0.07 | 0.80 | 1.13 (0.45 - 2.85) | |
76 + | 1 | 1.822 | 0.709 | 6.60 |
0.01
|
6.2 (1.5 - 25) | |
DOD
|
0-6
| 0.000 | Reference | 1 | |||
7-12
| 1 | -0.866 | 0.523 | 2.74 | 0.10 | 0.42 (0.15 - 1.17) | |
13-24
| 1 | -0.808 | 0.594 | 1.85 | 0.17 | 0.45 (0.14 - 1.4) | |
25+ | 1 | -2.999 | 1.151 | 6.78 |
0.01
|
0.050 (0.005 - 0.48) | |
Diff_BMI
| <1
| 0.000 | Reference | 1 | |||
1- < 2
| 1 | 0.227 | 0.591 | 0.15 | 0.70 | 1.26 (0.39 - 4.0) | |
> = 2
| 1 | 1.038 | 0.512 | 4.11 |
0.04
|
2.8 (1.04 - 7.7) | |
FRS
|
Quintile 1: 37-40
| 0.000 | Reference | 1 | |||
Quintile 2: 34-36
| 1 | 0.588 | 0.796 | 0.55 | 0.46 | 1.8 (0.38 - 8.6) | |
Quintile 3: 31-33
| 1 | 0.953 | 0.795 | 1.47 | 0.23 | 2.6 (0.55 - 12) | |
Quintile 4: 27-30
| 1 | 2.556 | 0.786 | 10.57 |
0.001
|
12.9 (2.8- 60) | |
Quintile 5: 00-26
| 1 | 3.520 | 0.823 | 18.28 | <.0001
|
33.8 (6.7 - 170) | |
Testing the global null hypothesis:
BETA=
0
| |||||||
Likelihood ratio test
| 11 | 87.67 | <.0001
| ||||
R
2
| 0.543 |