Table 1.
Participants' background.
| Measure | ALS without cognitive impairment (n = 25) | ALS with cognitive impairment (n = 13) |
|---|---|---|
| Demographic background | ||
| Recruitment location (HRO/MD) | 20/5 | 4/9 |
| Sex (f/m) | 9/16 | 4/9 |
| Age (years) | 54.52 (10.20) | 60.77 (8.59) |
| Cognitive reserve measure | 9.16 (1.99) | 8.62 (0.60) |
| Education (years) | 13.24 (2.44) | 11,77 (1.74) |
| ISCED level | 4.80 (1.08) | 4.23 (0.60) |
| Verbal IQ | 92.56 (14.81) | 90.85 (7.96) |
| Clinical presentation | ||
| Disease duration until final MRI (months) | 49.75 (39.82) | 41.64 (30.44) |
| ALSFRS‐R at baseline | 39.00 (4.92) | 38.62 (4.81) |
| Progression speed δ between baseline & final MRI | 0.61 (0.51) | 0.54 (0.60) |
| SOD1 mutation | 3 (12%) | 0 |
| VAPB mutation | 1 (4%) | 0 |
| Tested, no mutation found | 21 (84%) | 13 (100%) |
| Disease onset (%) | ||
| Bulbar | 4 (16%) | 2 (15%) |
| Spinal | 15 (60%) | 10 (77%) |
| Unknown | 6 (24%) | 1 (8%) |
| El Escorial criteria (%) | ||
| Not applicable | 5 (20%) | 3 (23%) |
| Possible ALS | 10 (40%) | 6 (46%) |
| Probable ALS | 2 (24%) | 3 (23%) |
| Definite ALS | 4 (6%) | 1 (8%) |
| Phenotype (%) | ||
| Classical ALS | 14 (56%) | 6 (46%) |
| Predominant upper motor neuron | 2 (8%) | 3 (23%) |
| Primary lateral sclerosis | 3 (12%) | 0 |
| Progressive muscular atrophy | 3 (12%) | 2 (15%) |
| Flail arm syndrome | 1 (4%) | 0 |
| Flail leg syndrome | 1 (4%) | 2 (15%) |
| Uncertain | 1 (4%) | 0 |
ALS, amyotrophic lateral sclerosis; HRO, Rostock; MD, Magdeburg; ISCED, international standard classification of education; IQ, intelligence quotient.