Cohort demographics and phenotypes
Total no of patients | 100 |
Age (years), mean (range) | 60.4 (22–87) |
Male | 54% |
Female | 46% |
Site of first muscle weakness | |
Upper limb | 40% |
Lower limb | 39% |
Bulbar | 19% |
Respiratory | 1% |
Trunk | 1% |
Disease subtype | |
ALS | 83% |
LMN predominant | 12% |
UMN predominant | 5% |
Family history of ALS | 7% |
Time from onset of first muscle weakness (months), mean (SD, range) | 20.8 (20.4, 1–134) |
Time from onset of first muscle weakness until death (months), mean (SD, range)* | 28.5 (21.0, 7–85) |
ALSFRS-R, mean (range) at first assessment | 36.9 (15–47) |
Revised El-Escorial criteria at first assessment | |
Clinically probable laboratory supported | 36% |
Clinically probable | 30% |
Clinically suspected | 15% |
Clinically definite | 13% |
Clinically possible | 6% |
ALS, amyotrophic lateral sclerosis; ALSFRS-R, revised ALS-Functional Rating Score; LMN, lower motor neuron; UMN, upper motor neuron.