| [94] |
668 |
R |
56 |
36§ |
39 |
Age, bulbar (worse) |
Highly selected cohort (ALS Foundation) |
NS |
| [32] |
768 |
R |
NS |
NS |
12.8 (ALS) 28.8 (PMA) 4.3 (PBP) |
Type on onset; age; protein content of CSF (worse if >61 mg/dl); presence of hyperactive reflexes or Babinski response (worse). Geographical region not significant |
Questionnaire to 24 neurological institutes in Japan. Only deceased cases included |
|
| [95] |
515 |
R |
50.8 |
NA |
NA |
ALS worse than PMA. Older age, bulbar, left limbs (worse) |
Only deceased cases |
0 |
| [96] |
155 |
R |
48 |
>72§ |
56 § |
Age, diffusion of symptoms at time of diagnosis |
PMA only |
3 |
| [97] |
318 |
R |
56.6 |
36 |
29 |
Bulbar (worse) |
Israel national neurological register |
NS |
| [98] |
27 |
R |
50.3 |
24 |
8 |
None |
FALS only |
0 |
| [9] |
336 |
R |
57 |
60 (48 for ALS) |
35 |
Age Type of onset non-significant |
Highly selected population |
0 |
| [65] |
194 |
P |
59 |
36 |
32 |
Age, ALS score, ALS duration |
|
3 |
| [8] |
708 |
R |
57 (m), 60 (f) (ALS) 49 (m) 54 (f) (benign ALS) |
38 |
28 |
Plateau in survival of younger patients |
162 more cases excluded due to various reasons |
1 |
| [99] |
138 |
P |
57.8 (m) 60.7 (w) |
43.2 (m) 38.4 (w) |
NA |
Age |
Only deceased patients from a series of 246 |
0 |
| [60] |
167 |
P |
56 |
NA |
48 |
Age, presence of bulbar symptoms at entry, presence of fasciculations. Prevalent cases better than incident cases (4.7 vs. 2.1 years). |
5 ALS centers in U.S. |
1 |
| [53] |
144 |
R |
55 |
NA |
NA |
Effect of distressing psychological factors (COX: OR 2.24 [1.08–4.64]) |
Patients from 3 ALS centers across U.S. |
0 |
| [10] |
831 |
R |
55.7 |
35 |
37.5 |
Age, diagnostic delay, slope of Appel score |
|
0 |
| [11] |
158 |
R |
62.7 |
25.0–26.9 |
14.7 |
COX: upper limbs worse than lower Bulbar worse than spinal Age at onset |
|
NS |
| [34] |
71 |
R |
|
31.2 |
25 |
Gender (women worse), age Bulbar not significant |
|
NS |
| [5] |
90 |
P |
56.8 |
23 (17 from onset of study + 6 pre-study) |
NA |
None |
Placebo arm of IGF-I trial |
NS |
| [12] |
307 |
R |
60 |
16.8§ |
NA |
Age, bulbar, time from onset to diagnosis |
11 additional patients were excluded because they were lost to follow-up FALS not significant |
0 (3%) |
| [28] |
62 |
P |
57 |
38.4 |
NA |
COX: linear estimate of decline in foot dorsiflexion strength, FVC%, grip strength |
Patients included in two trials |
NS |
| [16] |
245 |
P |
53.1 (surviving) 59.6 (deceased) |
NA |
NA |
Age; bulbar onset; body weight; weight change from prestudy; FVC; pCO2; ALSFRS score; muscle strength megascore |
Placebo arm of CNTF trial. Comparison between surviving and deceased patients |
0 |
| [40] |
105 |
R |
57.1 |
32 |
|
FVC at study entry (<80% worse than FVC ≥80%); bulbar (worse) |
The analysis included only the 74 patients for whom FVC was available COX: age, bulbar onset, diagnostic delay (per month), FVC% |
30 |
| [39] |
155 |
P |
54.2 |
47 |
NA |
Bulbar onset and >55 years worse COX: age, bulbar, faster progression in the first year of observation |
92 other patients were excluded because of missing data |
0 |
| [13] |
841 |
R |
56 |
43 |
NA |
Age, referral delay, El Escorial classification |
Several variables with missing data |
NA |
| [67] |
985 |
R |
NA |
39 |
NA |
Mean age of onset lower in long survivors (43 vs. 57) |
Analysis of long survivors (>120 months) from a tertiary center database |
22 |
| [14] |
793 |
R |
56 |
34.8 |
24 |
Diagnostic delay, age, bulbar onset, diagnosis ≥1990 better than before 1990 |
|
0 |
| [41] |
267 |
R |
61.7 |
33.6 |
NA |
COX: symptom duration, ALSFRS-R, FVC at baseline |
ALS referral center in New York City |
3 |
| [15] |
1398 671 |
R |
62.5 61.7 |
26 26 |
NA NA |
COX: Age (≥65, >65), disease duration at diagnosis (<2, >2 years), plasma creatinine, atrophy diffusion, pyramidal signs, spasticity, fasciculation, muscle strength (MMT), cough, swallowing, sVC |
Several variables with missing data, replaced with statistical artifices Patients separated in 2 cohorts |
0 |
| [56] |
81 |
P |
NA |
NA |
NA |
FTLD significantly worse in univariate analysis but not in multivariate. COX: age and bulbar onset significant |
FLTD was significantly associated with bulbar onset and older age at onset |
0 |
| [100] |
123 |
R |
63.3 |
27 |
23 |
Age, lower limb onset (better), area of residence (mountainous areas worse), and work (agricultural workers worse) |
|
0 |
| [29] |
1034 |
R |
54.1 |
41.4 |
28 |
COX: FVC < 75% at baseline; age at onset, bulbar onset, duration at diagnosis PEG and NIPPV not significant |
A series of three papers concerning the same large series of cases (see also ref. 55 and 91) |
0 |
| [61] |
1034 |
R |
54.1 |
41.4 |
28 |
Prolonged survival related to younger age, limb site of onset and longer diagnostic delay, lower baseline AALSS, lower AALSS- preslope and higher baseline FVC |
|
0 |
| [101] |
1034 |
R |
54.1 |
41.4 |
28 |
Prolonged survival in more recent patients (1984–1998 vs. 1999–2004) |
|
0 |
| [50] |
479 |
R |
58 |
42.8 |
20 |
Prolonged survival related to young age at onset (<40), spinal onset, FVC >80 at diagnosis, longer diagnostic delay, ALSFRS-R score-ratio < 1.185 |
ALSFRS-R score ratio was calculated as ALSFRS-R score at first visit – ALSFRS-R score at last visit/time between first and last visit in months |
18.90% |
