Table 2 Association of the baseline ALSFRSr and other clinical characteristics with death or tracheostomy, from Cox proportional hazards models adjusting for age at baseline, sex, and symptom duration (n = 267)

ModelVariableHazard ratio (95% CI)p Value
* n = 250.
ALSFRSr = Amyotrophic Lateral Sclerosis Functional Rating Scale–revised.
1Total ALSFRSr score (dichotomized at the median)
    ≥381.00 (Reference)
    <384.38 (2.79–6.86)<0.001
2Total ALSFRSr score quartiles
    ≥431.00 (Reference)
    ≥38 and <437.08 (2.12–23.66)0.001
    ≥33 and <3812.51 (3.81–41.08)<0.001
    <3327.78 (8.57–90.07)<0.001
3Total ALSFRSr score0.91 (0.89–0.93)<0.001
4*Total ALSFRSr score0.94 (0.91–0.98)<0.001
Forced vital capacity (% predicted)0.99 (0.98–1.00)0.02
5Total ALSFRSr score0.91 (0.89–0.93)<0.001
Riluzole use (ever vs never)0.92 (0.60–1.41)0.7
6Total ALSFRSr score0.91 (0.89–0.93)<0.001
Site of symptom onset
    Upper extremity1.00 (Reference)
    Lower extremity1.28 (0.73–2.23)0.4
    Bulbar1.91 (1.06–3.46)0.03
    Respiratory8.44 (3.68–19.35)<0.001
7Fine motor ALSFRSr subscore0.95 (0.88–1.03)0.2
Gross motor ALSFRSr subscore0.91 (0.84–0.99)0.02
Bulbar ALSFRSr subscore0.94 (0.88–1.01)0.08
Respiratory ALSFRSr subscore0.79 (0.72–0.87)<0.001
8Most affected ALSFRSr subscore (dichotomized at the median)
    ≥71.00 (Reference)
    <74.44 (2.73–7.22)<0.001
9Most affected ALSFRSr subscore quartiles
    ≥91.00 (Reference)
    ≥7 and <94.48 (1.32–15.22)0.02
    ≥4 and <710.33 (3.18–33.57)<0.001
    <420.60 (6.31–67.30)<0.001
10Most affected ALSFRSr subscore0.76 (0.70–0.81)<0.001