Table 1.
Demographic, clinical, and neuropsychological measures of patients, divided into pseudobulbar (PSB) and non-pseudobulbar (nPSB), and healthy controls (HC); data are shown as mean (standard deviation) or count (percentage)
| Variables | HC (n = 26) |
ALS (n = 27) |
PSB (n = 13) |
nPSB (n = 14) |
HC vs ALS p-value |
PSB vs nPSB p-value |
|---|---|---|---|---|---|---|
| Age, years | 58 (12) | 58 (9) | 56 (9) | 60 (10) | 0.947 | 0.271 |
| Education, years | 11 (4) | 11 (4) | 9 (3) | 12 (5) | 0.824 | 0.134 |
| Sex, male | 20 (76.9%) | 23 (85.2%) | 11 (84.6%) | 12 (85.7%) | 0.453 | 0.328 |
| Disease Duration, months | n.a | 21 (21) | 16 (17) | 25 (25) | n.a | 0.24 |
|
Disease onset (bulbar/upper limbs/lower limbs) |
n.a | 5/10/12 | 4/5/4 | 1/5/8 | n.a | 0.157 |
|
Disease phenotype (classic/bulbar/FL/FA/pyramidal) |
n.a | 3/3/5/5/11 | 2/2/1/3/5 | 1/1/4/2/6 | n.a | 0.332 |
| ALSFRS-R total score | n.a | 43 (3) | 42 (4) | 43 (3) | n.a | 0.773 |
| Bulbar subscore | n.a | 11 (1) | 11 (1) | 12 (1) | n.a | 0.031 |
| Fine motor subscore | n.a | 10 (2) | 10 (2) | 10 (2) | n.a | 0.599 |
| Gross motor subscore | n.a | 10 (2) | 10 (2) | 9 (2) | n.a | 0.147 |
| Respiratory subscore | n.a | 12 (0.4) | 12 (0.6) | 12 (0) | n.a | 0.221 |
| UMN score | n.a | 8 (4) | 8 (4) | 8 (5) | n.a | 0.778 |
| King’s clinical staging (1/2/3)* | n.a | 11/13/3 | 5/5/3 | 6/8/0 | n.a | 0.305 |
| MMSE | 29(1) | n.a | ||||
| ECAS total score | n.a | 88 (25) | 83 (22) | 92 (27) | n.a | 0.326 |
| Language | n.a | 21 (5) | 20 (4) | 22 (5) | n.a | 0.441 |
| Verbal fluency | n.a | 16 (7) | 16 (7) | 16 (8) | n.a | 0.993 |
| Executive functions | n.a | 27 (11) | 24 (11) | 29 (11) | n.a | 0.269 |
| Memory | n.a | 13 (5) | 12 (5) | 14 (5) | n.a | 0.205 |
| Visuospatial abilities | n.a | 11 (1) | 10 (1) | 11 (1) | n.a | 0.214 |
| CNS-LS | n.a | 12 (4) | 16 (2) | 9 (2) | n.a | < 0.001 |
| Laughing | n.a | 7 (3) | 9 (3) | 5 (1) | n.a | < 0.001 |
| Crying | n.a | 5 (2) | 6 (2) | 4 (1) | n.a | 0.003 |
| BDI-II | n.a | 7 (6) | 6 (6) | 8 (7) | n.a | 0.449 |
ALS Amyotrophic Lateral Sclerosis; ALSFRS-R ALS Functional Rating Scale Revised; BDI-II Beck Depression Inventory -II; ECAS Edinburgh Cognitive and Behavioural ALS Screen; FA flail arm; FL flail leg; MMSE Mini Mental State Examination; HC Healthy Controls; χ2 Chi-square test; UMN upper motor neuron
* According to the King’s clinical staging system, the number of regions involved gives the stage