Demographic and clinical characteristics
| ALS (n=277) | PLS (n=75) | PMA (n=143) | P values | |||
| ALS/PLS | ALS/PMA | PLS/PMA | ||||
| Male, n (%) | 159 (57) | 47 (63) | 108 (76) | NS | <0.01 | <0.05 |
| Age at screening, mean years (range) | 67.5 (33–86) | 66.9 (52–82) | 65.6 (24–85) | NS | NS | NS |
| Disease duration at testing, mean years (range) | 1.3 (0–8) | 11.4 (4–29) | 5.0 (0–41) | <0.01 | <0.01 | <0.01 |
| Bulbar onset, n (%) | 81 (29.3) | 10 (13.3) | 1 (0.7) | NS | <0.01 | <0.01 |
| ALSFRS-R at testing, mean±SD | 39.4±5.0 | 34.9±6.6 | 39.4±5.6 | <0.01 | NS | <0.01 |
| C9ORF72 mutation | 18 (9.2) | 1 (1.6) | 2 (1.6) | NS | <0.01 | NS |
| Level of education | ||||||
| ISCED 0–4, n (%) | 224 (81) | 50 (67) | 115 (80) | <0.01 | NS | <0.05 |
| ISCED 5–6, n (%) | 53 (19) | 25 (33) | 28 (20) | |||
| Left-handed, n (%) | 22 (8) | 6 (8) | 11 (8) | NS | NS | NS |
| Ambidextrous, n (%) | 7 (3) | 2 (3%) | 2 (1) | |||
| HADS | ||||||
| HADS-A score, mean±SD | 5.0±2.8 | 3.9±2.5 | 4.8±2.6 | <0.05 | NS | NS |
| HADS-D score, mean±SD | 4.0±2.5 | 4.1±2.4 | 3.9±2.6 | NS | NS | NS |
Availability of data: ALSFRS-R: ALS 91%, PLS 75%, PMA 77%; HADS: ALS 100%, PLS 48%, PMA 29%.
ALS, amyotrophic lateral sclerosis; ALSFRS-R, Revised ALS Functional Rating Scale; HADS, Hospital Anxiety and Depression Scale; ISCED, International Standard Classification of Education 1997; PLS, primary lateral sclerosis; PMA, progressive spinal muscular atrophy.