Table 1.
Demographic, clinical, and neuropsychological measures of patients and healthy controls; data are shown as mean (standard deviation) or count (percentage)
| Variable | ALS patients (n = 32) |
HCs (n = 21) |
t-test/χ2 | p-value | Below\above cut-off scorea |
|---|---|---|---|---|---|
| Demographics: | |||||
| Age, years | 58.3 (10.3) | 56.0 (10.1) | − 0.8 | 0.40 | - |
| Education, years | 10.5 (4.3) | 12.5 (3.04) | 1.8 | 0.10 | - |
| Sex, male | 25 (78%) | 15 (71%) | 0.3 | 0.60 | - |
| Clinical features: | |||||
| Symptom duration, months | 19.5 (24.6) | - | - | - | - |
| ALSFRS-R total score | 39.0 (6.8) | - | - | - | - |
| Bulbar subscore | 10.7 (2.2) | - | - | - | - |
| Fine motor subscore | 8.5 (3.0) | - | - | - | - |
| Gross motor subscore | 8.6 (2.9) | - | - | - | - |
| Respiratory subscore | 11.1 (1.9) | - | - | - | - |
| Disease Progression Rate* | 0.8 (0.7) | - | - | - | - |
| Upper Motor Neuron score | 7.1 (5.0) | - | - | - | - |
| Neuropsychological measures: | |||||
| Global cognition: | |||||
| Mini Mental State Examination | 28.3 (1.6) | 28.5 (1.1) | 0.5 | 0.60 | 0 |
| ECAS total score | 90.9 (21.3) | 100.6 (9.0) | 1.9 | 0.10 | 4 |
| ECAS subscores: | |||||
| Executive functions | 29.18 (9.75) | 34.29 (5.35) | 2.1 | 0.03 | 17 |
| Fluency | 16.22 (7.26) | 17.67 (4.64) | 0.7 | 0.43 | 11 |
| Language | 21.68 (4.04) | 22.05 (2.69) | 0.3 | 0.71 | 12 |
| Memory | 13.04 (5.35) | 15.86 (3.21) | 2.1 | 0.03 | 7 |
| Visuospatial abilities | 10.7 (1.6) | 10.7 (1.3) | 0.1 | 0.91 | 5 |
| Executive functions: | |||||
| Letter fluency task | 26.9 (11.8) | - | - | - | 5 |
| Semantic fluency task | 20.1 (6.0) | - | - | - | 1 |
| SF Index** | 29.2 (13.4) | - | - | - | - |
| PF Index** | 9.0 (11.3) | - | - | - | - |
| Stroop test – seconds | 24.6 (10.0) | - | - | - | 3 |
| Stroop test – number of errors | 1.0 (2.5) | - | - | - | 1 |
| Memory: | |||||
| RAVLT-immediate recall | 38.5 (9.0) | - | - | - | 3 |
| RAVLT-delayed recall | 8.2 (2.4) | - | - | - | 3 |
| Digit Span forward | 5.1 (0.9) | - | - | - | 1 |
| Digit Span backward | 3.2 (0.8) | - | - | - | 9 |
| Corsi Block-Tapping test | 4.8 (0.7) | - | - | - | 0 |
| Non verbal Intelligence: | |||||
| Coloured Progressive Matrices | 26.3 (4.9) | - | - | - | 0 |
| Behavioural assessment: | |||||
| ALS-FTD Q (cut-off ≥ 22) | 13.1 (9.7) | - | - | - | 4 |
| Hamilton HDRS | 10.0 (4.2) | - | - | - | 2 |
Note. ALS, Amyotrophic Lateral Sclerosis; ALSFRS-R, ALS Functional Rating Scale Revised; ALS-FTD-Q, ALS-Frontotemporal Dementia questionnaire; ECAS, Edinburgh Cognitive and Behavioural ALS Screen; HCs, Healthy Controls; PF, Phonemic fluency; RAVLT, Rey’s Auditory Verbal Learning Test; SF, Semantic fluency; χ2, Chi-square test. *Disease Progression Rate was computed as: 48 – ALSFRS-R/Disease Duration. **Verbal fluency indices were obtained as following: time for generation condition - time for control condition (reading or writing generated words)/total number of items generated; a number of patients with cognitive performance\behavioural symptoms below\above cut-off score