Missing data | Population (n=735) Median (IQR) or counts | Not PBA CNS-LS<13 (n=526) | PBA CNS-LS≥13 (n=209) (28.4%) | p-Value | PBA crying-predominant (n=109) | PBA laughter-predominant (n=100) | p-Value | |
Age (years) | 60.4 (52–68.9) | 60.2 | 60.8 | ns | 62.0 | 57.1 | <0.05 | |
Female | 315 | 196 | 119 (37.8%) | <0.0001 | 77 (64.7%) | 42 (35.3%) | <0.0001 | |
Male | 420 | 330 | 90 (21.4%) | 32 (35.6%) | 58 (64.4%) | |||
Bulbar onset | 2 | 202 | 116 | 86 (42.6%) | <0.0001 | 39 | 47 | ns |
Non-bulbar onset | 531 | 408 | 123 (23.2%) | 70 | 53 | |||
Days from onset | 399 (243–727) | 399 | 411 | ns | 383 | 423 | ns | |
ALSFRS-R | 165 | 38 (32–42) | 39 | 35 | <0.0001 | 35 | 34 | ns |
Bulbar subscore | 167 | 10 (8–12) | 11 | 8 | <0.0001 | 9 | 8 | <0.05 |
Fine motor subscore | 9 (7–11) | 9 | 9 | <0.01 | 8 | 9 | ns | |
Gross motor subscore | 8 (6–10) | 9 | 7 | <0.001 | 6.5 | 8 | ns | |
Respiratory subscore | 11 (9–12) | 12 | 10 | <0.0001 | 11 | 10 | .099 | |
Pre-slope ALSFRS-R† | 165 | −0.64 (−1.13 to –0.35) | −0.56 | −0.96 | <0.0001 | −1.00 | −0.80 | ns |
ALS (UMN+LMN) UMN-predominant LMN-predominant | 6 | 575 83 71 | 409 51 64 | 166 (28.9%) 32 (38.6%) 7 (9.9%) | <0.001 | 88 16 4 | 78 16 3 | ns |
Cognition normal Cognitive impairment | 10 | 593 132 | 438 82 | 155 (26.1%) 50 (37.9%) | <0.01 | 83 25 | 78 25 | ns |
Dysarthria/dysphagia (−) Dysarthria/dysphagia (+) | 167 | 334 234 | 286 129 | 48 (14.4%) 105 (44.9%) | <0.0001 | 32 50 | 16 55 | <0.05 |
EQ-5D | 10 | 0.71 (0.60–0.82) | 0.75 | 0.68 | <0.001 | 0.60 | 0.69 | <0.05 |
EQ-5D VAS | 332 | 60 (42.5–88) | 66.5 | 50 | <0.0001 | 49 | 56 | .085 |
Dextromethorphan-quinidine | 102/735 | 41 | 61 | <0.0001 | 31 | 30 | ns | |
Any antidepressant | 413/735 | 265 | 148 | <0.0001 | 77 | 71 | ns | |
Riluzole | 505/735 | 366 | 139 | ns | 71 | 68 | ns | |
Baclofen | 194/735 | 114 | 80 | <0.0001 | 32 | 48 | <0.01 |
*Defined by which z-score subscore was higher.
†Preslope ALSFRS-R = (Initial ALSFRS-R at first assessment − 48)/months from onset of weakness.
Counts and median values are reported for categorical and continuous variables, respectively. Wilcoxon rank-sum test and Pearson’s χ2 test are used to compare continuous and categorical variables, respectively.
ALSFRS-R, Revised ALS Functional Rating Scale; CNS-LS, Center for Neurologic Study-Lability Scale; EQ-5D, EuroQol five dimensions questionnaire; LMN, lower motor neuron; ns, not significant; PBA, pseudobulbar affect; UMN, upper motor neuron; VAS, visual analogue scale.