Patient characteristics of the enrolled population
| Characteristic | All patients (N=433) | Stratified by MND-subtype | ||
| ALS (N=271) | PMA (N=74) | PLS (N=88) | ||
| Age at study enrolment, years | 65 (10) | 64 (10) | 67 (9) | 66 (10) |
| Sex, male | 295 (68%) | 186 (69%) | 57 (77%) | 52 (60%) |
| Symptom duration,* months | 51 (78) | 36 (40) | 79 (78) | 143 (125) |
| Diagnostic delay,* months | 17 (25) | 12 (16) | 26 (47) | 34 (49) |
| Site of symptom onset, bulbar | 82 (19%) | 65 (24%) | 2 (3%) | 15 (17%) |
| ALSFRS-R total score | 32 (10) | 31 (10) | 33 (10) | 35 (8) |
| ∆FRS, points per month | −0.37 (.38) | −0.48 (0.41) | −0.26 (0.28) | −0.13 (0.19) |
| Riluzole use, yes | 271 (63%) | 214 (79%) | 46 (62%) | 11 (13%) |
| Gastrostomy, yes | 67 (16%) | 58 (21%) | 5 (7%) | 4 (5%) |
| Respiratory support, yes | 87 (20%) | 62 (23%) | 18 (24%) | 7 (8%) |
| Presence of frontotemporal dementia† | 9 (2%) | 7 (3%) | 0 (0%) | 2 (2%) |
Data are expressed as mean (SD) or n (%).
*Data are expressed as median (IQR).
†Determined at diagnosis, cognitive status unknown at time of questionnaire.
ALS, amyotrophic lateral sclerosis; ALSFRS-R, ALS functional rating scale; ∆FRS, ALSFRS-R – 48/symptom duration; MND, motor neuron disease; PLS, primary lateral sclerosis; PMA, progressive muscular atrophy.