TABLE 5.

Cox proportional hazard regression at univariate and multivariate analysis.

Variable	Observations (n)	Univariate analysis		Multivariate analysis (n = 71)
		HR (95% CI)	P‐value	HR (95% CI)	P‐value
Sex (male as reference)	109	0.8 (0.5–1.27)	0.35
Age at onset	109	1.01 (1–1.03)	0.18
Diagnostic delay	109	0.94 (0.91–0.97)	<0.001
BMI at sampling	109	0.94 (0.89–0.99)	0.03
Mutation (WT as reference)	84	1	0.50
c9orf72		1.7 (0.72–4)	0.23
Other		1.26 (0.4–4.1)	0.70
FTD	109	1.62 (0.83–3.15)	0.16
Cognitive changes	110	1.94 (1.07–3.53)	0.03
Behavioral changes	110	1.65 (0.92–2.95)	0.09
Site of onset (spinal as reference)	109	1.1 (0.67–1.82)	0.71
FVC at sampling	106	0.99 (0.98–1)	0.01	0.97 (0.96–0.97)	0.001
ALSFRS‐R at sampling	109	0.98 (0.96–1.01)	0.16
Time to generalization	108	0.97 (0.95–0.99)	<0.001
DPRSA	109	1.31 (1.16–1.48)	<0.001	2.02 (1.49–2.74)	< 0.001
NfLserum	76	1.004 (1.002–1.01)	<0.001
NfLCSF	72	1 (1–1.00004)	0.002
NfHserum	107	1 (1–1.0002)	0.03
NfHCSF	93	1 (1–1.0002)	<0.001	1.000 (1.000–1.000)	< 0.001
SerpinA1serum	107	1 (0.99–1.02)	0.45
SerpinACSF	94	1.05 (1.02–1.09)	0.003
SerpinA1ratio	92	3.68 (1.73–7.83)	0.001	5.16 (2.34–11.36)	< 0.001
CHI3L1serum	107	1 (0.996–1.004)	0.97
CHI3L1CSF	94	1.01 (1–1.008)	0.003
BBB damage index	49	1.22 (0.33–4.53)	0.76

Note: Hazard ratios are presented with 95% confidence interval and relative p‐value. Figures in bold type denote statistical significance. See Methods section for calculation of clinical variables.

Abbreviations: ALSFRS‐R, Amyotrophic Lateral Sclerosis Functional Rating Scale‐Revised; BBB damage index, blood–brain barrier damage index, calculated by the ratio of Albumin_CSF/Albumin_serum, multiplied per 100; BMI, body mass index; CHI3L1, chitinase‐3‐like protein‐1; CI, confidence interval; CSF, cerebrospinal fluid; DPR_SA, disease progression rate at sampling; FTD, frontotemporal dementia; FVC, forced vital capacity; HR, hazard ratio; NfL, neurofilament light chain; NfH, neurofilament heavy chain; WT, wild‐type.