Table 1.
Background and clinical features of patients that underwent the FAB
| N | 297 |
|---|---|
| Age (years) | 63.1 ± 11.2 (28–88) |
| Sex (M/F) | 62.3%/37.7% |
| Education (years) | 11.6 ± 4.4 (5–24) |
| Handedness (right/left) | 94.6%/5.4% |
| Disease duration (months) | 17.4 ± 15.9 (2–120) |
| ALSFRS-R | |
| Total | 39.2 ± 5.5 (23–48) |
| Bulbar | 10.5 ± 2 (4–12) |
| Spinal–lower limbs | 11.2 ± 3.8 (0–16) |
| Spinal–upper limbs | 6.2 ± 1.8 (1–8) |
| Respiratory | 11.3 ± 1.5 (3–12) |
| ΔFS | 0.8 ± 0.8 (0–5.3) |
| KSS | |
| Stage 0 | 1.9% |
| Stage 1 | 36.2% |
| Stage 2 | 34% |
| Stage 3 | 23.4% |
| Stage 4 | 4.5% |
| MiToS | |
| Stage 0 | 76.6% |
| Stage 1 | 20.8% |
| Stage 2 | 2.6% |
| PEG | 0.3% |
| NIV | 4.1% |
| Genetics | |
| C9orf72 | 7.1% |
| SOD1 | 2.7% |
| TARDBP | 3.4% |
| FUS | 0.3% |
| FAB | |
| Raw scores | 15.7 ± 2.1 (8–18) |
| Below-cut-off scoresa | 12.1% |
| ECAS | |
| Total | 99.8 ± 18.6 (31–129) |
| ALS-specific | 73.8 ± 15.1 (21–97) |
| ALS-nonspecific | 26 ± 5 (9–34) |
| Language | 23.4 ± 3.9 (10–28) |
| Fluency | 16.3 ± 5.6 (0–24) |
| Executive | 34 ± 7.8 (7–47) |
| Memory | 14.6 ± 4.6 (1–22) |
| Visuo-spatial | 11.4 ± 1 (6–12) |
| ECAS-CI | 0.7 ± 0.9 (0–5) |
Notes. ΔFS progression rate, ALS amyotrophic lateral sclerosis, ALSFRS-R Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, ECAS Edinburgh Cognitive and Behavioural ALS Screen, F female, FAB Frontal Assesment Battery, KSS King’s staging system, M male, MiToS Milano-Torino staging system, NIV non-invasive ventilation, PEG percutaneous endoscopic gastrostomy aAppollonio et al. [21]