Table 1 Multivariate analyses, with all factors analysed in the same Cox models.

Category		Survival, years (HR (95% CI))		p		Disease progression, years (HR (95% CI))		p
Baseline FVC <75%		2.07 (1.73 to 2.48)		<0.001		1.56 (1.28 to 1.90)		<0.001
Age at onset, years		1.04 (1.03 to 1.05)		<0.001		1.01 (0.99 to 1.01)		0.01
Limb vs. bulbar onset		1.09 (0.87 to 1.37)		0.4		1.34 (1.07 to 1.68)		0.009
FS to FE time, months		0.95 (0.94 to 0.96)		<0.001		0.98 (0.97 to 0.98)		<0.001
Women versus men		1.10 (0.92 to 1.33)		0.3		1.20 (0.99 to 1.43)		0.05
Riluzole (ever versus never)		1.14 (0.94 to 1.38)		0.2		1.12 (0.95 to 1.32)		0.2
NIV (ever versus never)		0.77 (0.62 to 0.95)		0.02		ND		ND
PEG (ever versus never)		0.74 (0.62 to 0.88)		0.001		ND		ND

In most patients who received riluzole, therapy was initiated at the time of the first examination and diagnosis, prior to a documented 20 point progression. In contrast, NIV initiation and PEG placement were generally performed in patients who had reached an advanced degree of disability. Thus, the Cox model for disease progression did not include NIV and PEG use.