Table 1.
Clinical characteristics of ALS patients and unadjusted associations with ALS survival
| Characteristics | All N=135 |
HR (95%CI) |
|---|---|---|
| Age at onset (in years), mean (sd) | 56.1 (12.8) | 1.04 (1.01, 1.07)b |
| Sex, n (%) | ||
| Male | 86 (64%) | Reference |
| Female | 49 (36%) | 1.40 (0.72, 2.71) |
| Diagnosis, n (%) | ||
| ALS | 129 (96%) | Reference |
| ALS-FTSDa | 6 (4%) | 1.54 (0.47, 5.04) |
| Site of onset, n (%) | ||
| Spinal | 99 (73%) | Reference |
| Other | 36 (27%) | 2.52 (1.31, 4.82) |
| C9orf72 mutation, n (%) | ||
| No | 128 (95%) | Reference |
| Yes | 7 (5%) | 1.52 (0.36, 6.34) |
| Smoking, n (%) | ||
| Never | 79 (59%) | Reference |
| Ever | 56 (41%) | 0.69 [0.36, 1.34] |
| BMI (kg/m2) | ||
| <18, n (%) | 4 (3%) | 1.52 (0.45, 5.13) |
| 18 to <25, n (%) | 60 (44%) | Reference |
| 25 to <30, n (%) | 44 (33%) | 0.48 (0.21, 1.10) |
| >=30, n (%) | 27 (20%) | 0.46 (0.17, 1.23) |
| Diagnostic delay (years), median (25th %, 75th %) | 1.0 (0.6, 2.0) | 0.71 (0.56, 0.89)b |
| Baseline ALSFRS-R, median (25th %, 75th %) | 37.0 (32.0, 41.0) | 0.99 (0.94, 1.03)c |
*
Abbreviations: ALS, amyotrophic lateral sclerosis; ALS-FTSD, ALS-frontotemporal spectrum disorder; C9orf72 mutation, chromosome 9 open reading frame 72 repeat expansion; ALSFRS-R, Revised Amyotrophic Lateral Sclerosis Functional Rating Scale; HR, hazard ratio; CI, confidence interval.
a
Includes ALS with frontotemporal dementia, and ALS with behavioral or cognitive impairment.
b
Per year increase
c
Per +1 point difference in ALSFRS-R score