Table 2

Univariable survival analysis disclosed that a higher rate of disease progression (ΔFS), along with lower Amyotrophic Laterals Sclerosis Rating Scale-Revised (ALSFRS-R) score, older age of onset (≥70 years), bulbar onset disease and shorter disease duration were predictors of survival

Predictor variableNumberMedian survival time from initial visit (years)HR95% CIp Value
Gender
 Male931.41
 Female711.40.920.64 to 1.300.626
Age
 <701041.61
 ≥706011.771.23 to 2.550.002
Site of onset
 Limb951.61
 Bulbar690.91.761.23 to 2.510.002
Symptom duration (years)
 0.7411.32.521.45 to 4.39<0.001
 0.7–1421.42.741.61 to 4.68<0.001
 1.1–2401.22.811.62 to 4.89<0.001
 >24151<0.001
Riluzole
 Yes1271.50.70.46 to 1.050.087
 No370.71
PEG tube
 Yes811.41.360.96 to 1.940.09
 No831.51
NIV
 Yes341.31.240.83 to 1.850.304
 No1301.41
ΔFS tertiles
 <0.47562.41
 0.47–1.11541.61.771.12 to 2.820.015
 >1.11540.73.742.40 to 5.82<0.001
ALSFRS-R quartiles
 ≤33480.62.371.40 to 4.040.002
 34–39421.51.650.94 to 2.920.084
 40–43421.51.720.99 to 3.010.056
 ≥44322.61
  • The presence of a percutaneous endoscopic gastrostomy (PEG) tube and non-invasive ventilation did not significantly impact on survival in the present cohort.