4
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(E)T-1). Comparison of demographic and clinical characteristics of patients obtained from the collaborating centers or from secondary sources.
Variable Patients found through Patients found through p collaborating centers secondary sources
Number of cases 193 28 -
Mean age at onset (years) 62.4 (11.4) 65.9 (10.5) n.s
(SD)
Mean age at diagnosis 63.3 (11.2) 66.8 (10.5) n.s (years) (SD)
Time delay 11.1 (9.7) 11.3 (6.3) n.s.
onset-diagnosis (months)
Gender (% women) 43.0% 45.7% n.s.
Site of onset (% bulbar) 31.6% 34.5% n.s.
Bulbar signs at 60.1% 57.1% n.s.
diagnosis (%)
Respiratory involvement 23.3% 17.9% n.s.
at diagnosis (%)
PEG (%) 24.4% 17.9% n.s.
NIV (%) 10.4% 10.7% n.s.
Tracheostomy (%) 6.7% 3.6% n.s.
Median survival time 905 860 n.s.
(days)
3-year survival rate 40.5% 37.1% n.s.*
*Log-rank test
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(E)T-2). Median survival time and 3-year survival rates of the variables included in the analysis
Variable Level Number of median 3-year surv. p^
patients survival rate (SD)
time (days)
Gender Men 110 880 41.1 (4.8) 0.89
Women 83 975 39.8 (5.2)
Age-groups £50 20 2000 80.0 (8.9) 0.0002
(years) 51-60 34 1275 58.8 (8.4)
61-70 79 885 33.8 (5.3)
³71 60 730 27.9 (5.1)
Time delay <16 150 805 34.2 (3.9) 0.0003
onset- diagnosis ³16 42 1400 62.8 (7.4)
(months)
Site of onset* Bulbar 61 700 24.6 (5.7) 0.03
Upper limbs 55 1340 52.8 (6.9)
Lower limbs 77 1050 46.0 (5.8)
Bulbar signs at Yes 116 805 30.4 (4.8) 0.0002
diagnosis No 77 1400 59.0 (6.3)
Respiratory Yes 45 885 34.5 (5.1) 0.0001
involvement at No 148 1445 75.0 (8.9)
diagnosis
El Escorial status Definite 98 700 26.9 (4.6) 0.0001**
at diagnosis Probable 73 1035 48.3 (6.5)
Possible 16 1065 47.8 (10.4)
Suspected 6 1765 66.6 (12.7)
FALS Yes 10 1430 67.1 (15.3) 0.18
No 183 915 40.0 (3.6)
MRC, upper limbs <1.0 111 1095 47.8 (4.7) 0.01
progression rate# ³1.0 82 865 30.5 (5.1)
MRC, lower limbs <1.5 138 1080 47.9 (4.2) 0.0002
progression rate# ³1.5 55 700 21.8 (5.8)
MRC, neck <0.015 148 975 43.3 (4.1) 0.09
progression rate# ³0.015 45 790 31.1 (6.9)
Bulbar score <0.1 112 1200 50.9 (4.7) 0.0003
progression rate# ³0.1 81 730 25.8 (4.9)
FVC progression <3% 129 1110 50.0 (7.2) 0.0001
rate# ³3% 64 605 13.2 (5.5)
Norris Scale score <2.5 103 1155 52.5 (5.6) 0.0018
progression rate# ³2.5 90 790 34.7 (5.5)
^Log-rank test. *In one case onset was at respiratory level. This patient was treated with NIV but died 5 months after onset. **Definite ALS versus the other three categories. The result is also significant (p=0.004) when all categories are matched without grouping. #Mean number of points lost per month in the six months after diagnosis (for details, see text).