Table 1.
Demographic and clinical characteristics of the study cohort
| ALS cohort | ||||
|---|---|---|---|---|
| n | % | Mean | Median | |
| Sex | ||||
| M | 438 | 63.6 | ||
| F | 251 | 36.4 | ||
| Site of onset | ||||
| Bulbar | 155 | 22.6 | ||
| Spinal | 531 | 77.5 | ||
| Phenotype | ||||
| Classic | 367 | 53.5 | ||
| Bulbar | 137 | 20.0 | ||
| Respiratory | 14 | 2.0 | ||
| Flail arm | 30 | 4.4 | ||
| Flail leg | 17 | 2.5 | ||
| PMA | 27 | 3.9 | ||
| UMN predominant | 68 | 9.9 | ||
| PLS | 26 | 3.8 | ||
| Age at onseta | 60.4 | 61.9 | ||
| Time to NIVb | 27.1 | 20.1 | ||
| Time to PEGb | 26.9 | 23.2 | ||
| ALSFRS-R | 38.2 | 40.0 | ||
| Progression rate | 0.86 | 0.60 | ||
| Survivalb | 37.6 | 26.8 | ||
NIV non-invasive ventilation; PEG percutaneous endoscopic gastrostomy; PMA progressive muscular atrophy; PLS primary lateral sclerosis; UMN upper motor neuron
aExpressed in years
bExpressed in months