Cazzolli et al reported prognostic factors affected survival of a patient with amyotrophic lateral sclerosis (ALS). Noninvasive ventilation can be tolerated unless oral secretions become severe because of bulbar involvement. The authors are from the ALS Care Project of Canton, Ohio, Atrium Health Neurosciences Institute, University of North Carolina, Tokyo Metropolitan Institute of Medical Science, the University of Ottawa and other institutions.

Patient selection: amyotrophic lateral sclerosis (ALS) on noninvasive ventilation (NIV)



(1) oral secretion scale (OSS), from 4 (normal) to 0 (constant pooling)

(2) duration of noninvasive ventilation in hours per day


OSS Score

Median Survival


11 months


5 months


5 months


1 months




Am OSS of 0 or 1 is associated with:

(1) a high risk of being unable to tolerate NIV

(2) a need for hospice or planned tracheostomy


Duration of NIV

Median Survival

24 hours per day

21 months

17 to 23 hours per day

8 months

4 to 16 hours per day

6 months

< 4 hours per day

2 months


Additional factors associated with survival from the multivariate analysis:

(1) site of onset limb (vs bulbar; HR 1.5)

(2) disrupted sleep (HR 2.3)

(3) upright dyspnea before NIV (HR 1.9)

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