Table 2.

Functional outcome measures at baseline, visit 4 (day 63), visit 5 (day 180) and visit 6 (day 300)

Outcome measures Mean±SD (median; range; N)	baseline	V4	V5	V6	p-value baseline-V4	p-value baseline-V5	p-value baseline-V6
RULM [score]	32.32±7.39	32.58±7.31	32.76±7.31	33.06±7.33	0.096	0.163	0.048
	(37.00; 11–37; 19)	(37.00; 12–37; 19)	(37.00; 12–37; 17)	(37.00; 12–37; 16)			d = 0.1; r = 0.05
HFMSE [score]	35.16±21.14	36.84±20.65	38.59±20.13	39.50±20.58	0.112	0.153	0.201
	(42.00; 0–64; 19)	(44.00; 0–64; 19)	(46.00; 0–64; 17)	(46.50; 100–613; 12)
6MWT[m]	369.50±126.62	384.73±131.80	378.83±147.17	377.75±156.60	0.058	0.002	0.010
	(389.00; 181–550; 10)	(405.00; 175–608; 11)	(410.00; 135–610; 12)	(429.50; 400–613; 12)		d = 0.1; r = 0.03	d = 0.1; r = 0.03
ALSFRS [score]	32.17±4.94	32.65±4.68	32.57±5.58	33.07±5.56	0.875	0.255	0.729
	(34.00; 23–38; 18)	(34.00; 23–83; 17)	(34.00; 20–39; 14)	(34.00; 20–39; 15)
FVC [% ]	94.54±15.45	96.31±16.50	98.52±14.48	99.54±12.42
	(91.8; 59–122; 17)	(92.80; 58–126; 19)	(94.70; 79–128; 17)	(96.25; 83–129; 16)	0.317	0.329	0.335
Peak cough flow [l/min]	376.11±183.12	430.00±160.48	461.54±147.24	435.63±125.23	0.311	0.044	0.285
	(345.00; 86–800; 14)	(355.00; 230–800; 14)	(450.00; 300–800; 13)	(450.00; 260–575; 8)		d = 0.5; r = 0.25
MRC [sum score]	113.95±22.91(115.00;	115.37±22.67(115.00;	117.65±24.85(119.00;	118.50±24.63(119.50;
	46–154; 19)	46–154; 19)	46–154; 17)	58–158; 16)	0.490	0.209	0.214

m = meter; l = liter, n.d. = not done; RULM = Revised Upper Limb Module; HFMSE = Hammersmith Functional Motor Scale Expanded; 6MWT = 6-Minute Walk Test; ALSFRS = Amyotrophic Lateral Sclerosis Functional Rating Scale; FVC = Forced Vital Capacity.