Baseline characteristics
| Ireland | The Netherlands | Apuglia | Lombardy | Piedmont and Valle d’Aosta | ||||||
| Patient | Control | Patient | Control | Patient | Control | Patient | Control | Patient | Control | |
| n | 177 | 349 | 791 | 1880 | 141 | 213 | 186 | 190 | 262 | 290 |
| Male gender, % | 59.9 | 60.5 | 60.4 | 59.9 | 57.4 | 53.1 | 53.8 | 53.2 | 52.7 | 52.8 |
| Bulbar site of onset, % | 23.7 | 35.3 | 26.5 | 29.6 | 37.0 | |||||
| Age at onset, years | 63.4 (11.5) | 62.8 (10.4) | 61.8 (10.8) | 63.6 (10.6) | 64.2 (10.8) | |||||
| Age at diagnosis, years | 64.6 (11.5) | 63.9 (10.4) | 63.3 (10.8) | 64.6 (10.6) | 65.3 (10.8) | |||||
| Age at survey, years | 65.1 (11.5) | 65.4 (11.0) | 64.2 (10.3) | 64.0 (9.6) | 63.9 (10.8) | 63.8 (11.7) | 65.3 (10.6) | 65.5 (11.1) | 65.8 (10.8) | 64.2 (11.7) |
| EEC, % | ||||||||||
| Definite | 52.4 | 18.8 | 33.3 | 21.1 | 26.7 | |||||
| Probable | 28.6 | 41.1 | 36.2 | 41.6 | 28.6 | |||||
| Laboratory supported probable | 0 | 25.0 | 2.2 | 17.3 | 17.2 | |||||
| Possible | 19.0 | 15.1 | 28.3 | 20.0 | 27.5 | |||||
| Education, % | ||||||||||
| ISCED 5–8 | 16.4 | 21.8 | 26.0 | 28.3 | 7.6 | 9.9 | 9.3 | 36.4 | 6.3 | 15.9 |
| ISCED 0–4 | 83.6 | 78.2 | 74.0 | 71.7 | 92.4 | 90.1 | 90.7 | 63.6 | 93.7 | 84.1 |
| C9orf72 repeat expansion*, % | 9.2 | 9.7 | NA | 8.8 | 8.8 | |||||
| Smoking, % | ||||||||||
| Current | 19.4 | 11.2 | 21.5 | 13.8 | 22.1 | 21.0 | 19.8 | 18.0 | 18.9 | 15.6 |
| Former | 36.6 | 40.2 | 45.9 | 52.6 | 33.6 | 26.7 | 36.3 | 34.4 | 28.7 | 34.6 |
| Never | 44.0 | 48.6 | 32.6 | 33.6 | 44.3 | 52.4 | 44.0 | 47.6 | 52.4 | 49.8 |
| Pack-years | 15.6 (25.8) | 13.5 (22.0) | 13.0 (16.8) | 11.1 (15.1) | 15.3 (25.4) | 14.5 (22.9) | 13.9 (19.0) | 10.2 (18.6) | 12.0 (18.9) | 11.5 (18.4) |
| Alcohol, % | ||||||||||
| Current | 72.7 | 75.4 | 78.1 | 87.7 | 54.3 | 40.5 | 59.6 | 62.7 | 56.4 | 61.3 |
| Former | 10.5 | 5.9 | 8.0 | 4.0 | 7.9 | 2.4 | 7.6 | 3.0 | 6.8 | 6.3 |
| Never | 16.9 | 18.6 | 13.9 | 8.3 | 37.8 | 57.1 | 32.7 | 34.3 | 36.8 | 32.4 |
| Lifetime MET scores | ||||||||||
| Leisure time activities† | 1.82 (2.29) | 1.35 (1.50) | 1.39 (1.99) | 1.34 (1.47) | 0.46 (0.98) | 0.37 (0.83) | 0.81 (1.41) | 0.75 (1.62) | 1.01 (1.20) | 0.98 (1.32) |
| Occupational activities | 4.67 (3.90) | 3.93 (3.02) | 3.22 (2.52) | 3.21 (2.53) | 4.28 (4.33) | 2.88 (2.96) | 3.52 (2.88) | 2.32 (1.85) | 4.03 (2.58) | 3.37 (2.33) |
| All activities‡ | 6.23 (4.51) | 5.28 (3.68) | 4.64 (3.34) | 4.57 (3.01) | 4.87 (4.30) | 3.32 (3.18) | 4.43 (3.39) | 3.16 (2.57) | 5.05 (2.85) | 4.41 (2.81) |
Values are expressed as mean±SD when appropriate.
*Missings in C9orf72: 4 (2.3%) in Ireland, 57 (7.2%) in The Netherlands, 139 (98.6%) in Apuglia, 106 (57.0%) in Lombardy, 0 (0%) in Piedmont and Valle d’Aosta.
†Leisure time activities: combination of sports and hobbies.
‡All activities: combination of sports, hobbies and occupations.
EEC, El Escorial Criteria; ISCED, International Standard Classification of Education; MET, metabolic equivalent of task; NA, not applicable.