Table 2 M50 and D50 median separation.

	Same separation	Differing separation	Differing separation		Sig.
	Same separation	Differing separation	Low M50 and high D50	High M50 and low D50
	n = 164	n = 58	n = 29	n = 29
M50 in months	14.9 (8.19–29.7)	14.6 (10.7–22.0)	10.8 (8.10–13.0)	21.7 (16.8–27.7)	***
MUSIX200 in months	19.0 (10.1–34.0)	13.5 (8.83–19.7)	10.8 (8.19–13.7)	18.6 (13.2–23.3)	***
CMAP50 in months^$	17.6 (9.69–38.6)	17.5 (11.2–23.7)	12.8 (8.85–15.4)	22.9 (18.3–30.8)	***
D50 disease progression model parameters
rD50 at MUNIX	0.30 (0.20–0.41)	0.26 (0.13–0.39)	0.16 (0.10–0.26)	0.39 (0.23–0.51)	***
D50 in months	28.1 (17.3–48.2)	28.6 (20.3–38.7)	38.5 (31.0–47.5)	20.7 (15.6–25.6)	***
Demographic and clinical parameters
Age at MUNIX	66.3 (58.7–72.4)	64.1 (57.5–72.4)	59.3 (54.9–66.4)	67.1 (58.7–75.4)	**
Gender (female/male)	74/90	23/35	7/22	16/13
Disease duration at MUNIX in months	15.7 (8.73–30.7)	13.4 (8.88–19.3)	13.6 (9.52–16.9)	13.1 (7.89–23.9)	*
ALS phenotype
Classic	92	36	23	13	**
Bulbar	57	22	6	16	**
Flail arm	4	0	0	0
Flail leg	2	0	0	0
Pyramidal	4	0	0	0
PLMN	5	0	0	0
ALSFRS-R Subscores
Bulbar	11 (8–12)	10 (7–12)	11 (11–12)	7 (6–10)	***
Fine motor	9 (6–11)	10 (7–12)	10 (9–12)	8 (5–11)	**
Gross motor	9 (5–11)	9 (6–11)	9 (7–12)	8 (5–11)
Respiratory	12 (10–12)	12 (10–12)	12 (11–12)	10 (8–12)	***

Values are given as median and interquartile range or numbers.
ALS amyotrophic lateral sclerosis, ALSFRS-R revised amyotrophic lateral sclerosis functional rating scale, CMAP compound muscle action potential, MUNIX motor unit number index, MUSIX motor unit size index, LSPR laboratory-supported probable, PLMN pure lower motor neuron, Sig significant difference between the “differing separation” subgroups (*p ≤ 0.05, **p ≤ 0.01, ***p ≤ 0.001) ^$related to n = 160 at “same separation” and n = 56 at “differing separation”/n = 27 at “high M50 and low D50” because 6 patients had no loss of function in comparison to the mean of CMAP of healthy controls. Phenotype in accordance to Chio et al.²⁵.

Search