Table 1.

Cross‐sectional study. Demographic and clinical features of the ABC and PGB cohorts.

	ALS‐slower		ALS‐faster		C9+ve ALS		Healthy controls
	ABC	PGB	ABC	PGB	ABC	PGB	HC‐1	HC‐2
	N = 36	N = 54	N = 45	N = 55	N = 26	N = 20	N = 76	N = 64
Male (%)	23 (64%)	29 (54%)	22 (40%)	28 (51%)	13 (50%)	9 (45%)	27 (36%)	26 (41%)
Age at baseline, years
Mean (±SD)	63.4 ± 9.4	57.1 ± 13.1	63.3 ± 11.3	61.0 ± 10.7	57.2 ± 10.4	56.35 ± 8.8	58.7 ± 8.5	61.5 ± 9.3
Site of disease onset
Bulbar	7	9	18	19	9	7	(n/a)
Limb	29	46	22	36	15	13	(n/a)
Bulbar and Limb	0	0	0	0	0	0	(n/a)
Other**	1	5	3	7	2	2	(n/a)
Time from onset to diagnosis, months
Median (interquartile range)	38.5 (5.6–92.8)	11.0 (1.05–30.86)	12.3 (2.34–27)	6.2 (0.33–25.31)	13.4 (12.6–26.28)	9.9 (1.12–24.9)	(n/a)
Baseline ALSFRS‐R
Mean (±SD)	38.7 ± 7.6	–	32.9 ± 7.6	–	35.4 ± 8.9	–	(n/a)
Survival, months
Median (interquartile range)	63.8 (28.4–114.3)	41.6 (15.0–85.0)	23.3 (3.4–53.5)	34.0 (19.2–38.0)	28.8 (7.8–73.3)	29.4 (8.5–21.8)	(n/a)
Baseline ΔFRS (points/month)
Mean (±SD)	−0.25 ± 0.14	−0.43 ± 0.21	−1.11 ± 0.79	−1.17 ± 0.97	−0.81 ± 0.72	−0.90 ± 0.68	(n/a)
ALSFRS‐R slope (points/months)
Mean (±SD)	−0.2 ± 0.2	−0.2 ± 0.2	−1.5 ± 1.2	−1.7 ± 0.8	−1.2 ± 0.9	−1.1 ± 0.9	(n/a)

ALSFRS‐R, Amyotrophic lateral sclerosis functional rating scale‐revised; ABC, ALS Biomarkers Study Cohort; PGB, phenotype–genotype biomarker (PGB) study; Other**, onset in other regions (e.g., respiratory); C9+ve ALS, ALS patients with a mutation in the C9orf72 gene; Baseline ΔFRS (points/month), estimated ALSFRS‐R rate of progression from onset (assumed score 48) to baseline visit; ALSFRS‐R change (in points/month), ALSFRS‐R rate of progression calculated using ALSFRS‐R scores collected at longitudinal study visits (post‐baseline slope); Survival, survival duration from symptom onset to permanent assisted ventilation/tracheostomy, death, or censoring. HC‐1, HC‐2, healthy control groups