Clinical characteristics for patients with amyotrophic lateral sclerosis (ALS) as a group and divided into three tertiles as defined by the rate of disease progression (ΔFS)
| Characteristic | All n=164 | ΔFS group 1 (<0.47) n=56 | ΔFS group 2 (0.47–1.11) n=54 | ΔFS group 3 (>1.11) n=54 |
|---|---|---|---|---|
| Gender (%) | ||||
| Male | 57 | 55 | 65 | 50 |
| Female | 43 | 45 | 35 | 50 |
| Site of onset (%) | ||||
| Limb | 55 | 66 | 54 | 44 |
| Bulbar | 44 | 33 | 46 | 54 |
| Respiratory | 1 | 2 | 0 | 2 |
| Phenotype (%) | ||||
| ALS | 84 | 74 | 83 | 96 |
| ALS-FTD | 4 | 0 | 12 | 0 |
| Flail-arm variant | 4 | 10 | 0 | 2 |
| PBP | 4 | 4 | 6 | 2 |
| PLS | 3 | 8 | 0 | 0 |
| PMA | 1 | 4 | 0 | 0 |
| Median age at visit 1 | 64.7 (58, 73) | 63.3 (58, 71) | 64.7 (59, 75) | 69.4 (58, 75) |
| Symptom duration (years) | 1.0 (0.6, 2) | 2.2 (1.2, 4.9) | 1.0 (0.6, 1.5) | 0.6 (0.4, 0.9) |
| Median ALSFRS score visit 1 | 39 (33, 43) | 42 (36, 44) | 40 (36, 43) | 34 (26, 38) |
| Median ΔFS score at visit 1 | 0.67 (0.33, 1.47) | 0.24 (0.13, 0.34) | 0.67 (0.60, 0.94) | 2.69 (1.47, 4) |
The median Amyotrophic Laterals Sclerosis Rating Scale-Revised (ALSFRS-R) was significantly lower in the highest tertile ΔFS group. Atypical ALS phenotypes such as primary bulbar palsy (PBP), primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) were infrequent, but more common in the lowest ΔFS group. All data is expressed as median (IQR).
FTD, frontotemporal dementia.