Table 1.
N | 278 |
---|---|
Age (years) | 62.8 ± 11.4 (28–88) |
Sex (M/F) | 64%/36% |
Education (years) | 11.6 ± 4.4 (5–24) |
Handedness (right/left) | 94.6%/5.4% |
Disease duration (months) | 16.7 ± 14.3 (2–120) |
ALSFRS-R | |
Total | 39.4 ± 5.6 (22–48) |
Bulbar | 10.5 ± 1.9 (4–12) |
Spinal – lower limbs | 11.2 ± 3.9 (0–16) |
Spinal – upper limbs | 6.3 ± 1.7 (0–8) |
Respiratory | 11.3 ± 1.4 (5–12) |
ΔFS | 0.8 ± 0.7 (0–5.2) |
KSS | |
Stage 0 | 2% |
Stage 1 | 36.5% |
Stage 2 | 33.7% |
Stage 3 | 23.3% |
Stage 4 | 4.4% |
MiToS | |
Stage 0 | 77.5% |
Stage 1 | 20.1% |
Stage 2 | 2.4% |
PEG | 0.4% |
NIV | 4% |
Genetics | |
C9orf72 | 6.8% |
SOD1 | 2.9% |
TARDBP | 3.2% |
FUS | 0.4% |
MoCA | |
Raw scores | 23.6 ± 3.6 (11–30) |
Below-cut-off scoresa | 6.1% |
ECAS | |
Total | 100.4 ± 18.1 (39–29) |
ALS-specific | 74.3 ± 14.8 (22–97) |
ALS-nonspecific | 26.2 ± 4.9 (9–34) |
Language | 23.5 ± 3.9 (10–28) |
Fluency | 16.5 ± 5.5 (0–24) |
Executive | 34.3 ± 7.6 (7–47) |
Memory | 14.8 ± 4.5 (1–22) |
Visuo-spatial | 11.4 ± 1 (6–12) |
ECAS-CI | 0.7 ± 0.9 (0–5) |
ΔFS, progression rate; ALS, amyotrophic laterals sclerosis; ALSFRS-R, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised; ECAS, Edinburgh Cognitive and Behavioural ALS Screen; F, female; KSS=King’s staging system; M, male; MiToS, Milano-Torino staging system; MoCA, Montreal Cognitive Assessment; NIV, non-invasive ventilation; PEG, percutaneous endoscopic gastrostomy.