Table 2.
Clinical data for the ALS patients used in the study
| Greek cohort |
Italian cohort |
Both cohorts |
|||||||
|---|---|---|---|---|---|---|---|---|---|
| Total | Anti-LRP4 antibody-positive | Anti-LRP4 antibody-negative | Total | Anti-LRP4 antibody-positive | Anti-LRP4 antibody-negative | Total | Anti-LRP4 antibody-positive | Anti-LRP4 antibody-negative | |
| Total no. of patients | 51 | 12 | 39 | 53 | 12 | 41 | 104 | 24 | 80 |
| % anti-LRP4 positive | 23.5 | 22.6 | 23.1 | ||||||
| Mean age at onset (years) | 60.8 | 67.4 | 58.6 | 53.8 | 54.6 | 53.6 | 57.2 | 61.0 | 56.0 |
| Mean disease duration (months) | 35.6 | 19.5 | 41.0 | 36.9 | 35.5 | 37.3 | 36.3 | 27.5 | 39.1 |
| ALSFRS-R scale | 36.6 | 42.1 | 34.4 | 35.1 | 34.0 | 35.4 | 35.8 | 38.1 | 34.9 |
| F/M ratio | 0.70 | 0.50 | 0.77 | 1.21 | 1.40 | 1.16 | 0.93 | 0.85 | 0.95 |
| Upper motor (predominant) | 20/3% (64.5%) | 6/10 (60.0%) | 14/21 (66.7%) | 3/39 (7.7%) | 1/9 (11.1%) | 2/30 (6.7%) | 23/70 (32.9%) | 7/19 (36.8%) | 16/51 (31.3%) |
| Lower motor (predominant) | 11/31 (35.5%) | 4/10 (40.0%) | 7/21 (33.3%) | 36/39 (92.3%) | 8/9 (88.9%) | 28/30 (93.3%) | 47/70 (69.3%) | 12/19 (66.7%) | 35/51 (70.4%) |
| Bulbar (onset) | 17/38 (44.7%) | 8/10 (80.0%) | 9/28 (32.1%) | 12/53 (22.6%) | 2/12 (16.7%) | 10/41 (24.4%) | 29/91 (31.9%) | 10/22 (45.5%) | 19/69 (27.5%) |
| Spinal (onset) | 21/38 (55.3%) | 2/10 (20.0%) | 19/28 (67.9%) | 41/53 (77.4%) | 10/12 (83.3%) | 31/41 (75.6%) | 62/91 (68.9%) | 12/22 (54.5%) | 50/72 (72.5%) |
| EMG | 7 in 1 seg | 2 in 1 seg | 5 in 1 seg | 7 in 1 seg | 2 in 1 seg | 5 in 1 seg | |||
| 13 in 2 seg | 8 in 2 seg | 5 in 2 seg | 4 in 2 seg | 11 in 3 seg | 4 in 2 seg | 17 in 2 seg | 8 in 2 seg | 9 in 2 seg | |
| 18 in 3 seg | 2 in 3 seg | 16 in 3 seg | 42 in 3 seg | 31 in 3 seg | 60 in 3 seg | 13 in 3 seg | 47 in 3 seg | ||
| 1 in 4 seg | 1 in 4 seg | 1 in 4 seg | 1 in 4 seg | ||||||
| El Escorial classification1 | 7 defin. | 7 defin. | 7 defin. | 7 defin. | |||||
| 30 prob. | 9 prob. | 21 prob. | 36 prob. | 10 prob. | 26 prob. | 66 prob. | 19 prob. | 47 prob. | |
| 7 pos. | 3 pos. | 4 pos. | 11 pos. | 1 pos. | 10 pos. | 18 pos. | 4 pos. | 14 pos. | |
ALSFRS-R, revised ALS functional rating scale; defin, definite; EMG, electromyography (data refer to the number of segments with EMG findings); pos, possible; prob. probable; seg, segments.
1
By the end of the study all possible ALS patients had become either probable or definite. The diagnosis of probable ALS, supported by laboratory data, based on revised El Escorial criteria, is practically equivalent to definite ALS.