Table 2 ECAS outcome scores in treatment-naive patients with SMA

Language (max. 28)

28.0 (27–28)

27.0 (25–28)

U = 22.5; Z = − 2.728; p = 0.009¹; r = 0.57

below cut-off, n (%)

0 (0.0)

2 (14.3)

p = 0.502²; OR = 1.17

Verbal Fluency (max. 24)

14.0 (8–20)

12.0 (10–22)

U = 56.5; Z = − 0.421; p = 0.688¹; r = 0.09

below cut-off, n (%)

3 (33.3)

4 (28.6)

p = 0.999²; OR = 0.80

Executive Functions (max. 48)

40.0 (28–43)

38.5 (28–44)

U = 55.0; Z = − 0.506; p = 0.643¹; r = 0.11

below cut-off, n (%)

2 (22.2)

2 (14.3)

p > 0.999²; OR = 0.58

Memory (max. 24)

15.0 (12–19)

17.0 (11–21)

U = 42.5; Z = − 1.301; p = 0.201¹; r = 0.27

below cut-off, n (%)

0 (0.0)

0 (0.0)

p > 0.999²

Visuospatial (max. 12)

12.0 (12)

12.0 (11–12)

U = 54.0; Z = − 1.161; p = 0.600¹; r = 0.24

below cut-off, n (%)

0 (0.0)

1 (7.1)

p > 0.999²; OR = 1.01

ALS-specific (max. 100)

84.0 (66–90)

78.0 (64–89)

U = 51.0; Z = − 0.757; p = 0.477¹; r = 0.16

below cut-off, n (%)

2 (22.2)

7 (50.0)

p > 0.999²; OR = 1.25

Non-ALS-specific (max. 36)

27.0 (24–31)

29.0 (23–32)

U = 43.0; Z = − 1.270; p = 0.224¹; r = 0.26

below cut-off, n (%)

0 (0.0)

0 (0.0)

p > 0.999²

ECAS total (max. 136)

111.0 (91–116)

105.5 (92–119)

U = 56.0; Z = − 0.442; p = 0.688¹; r = 0.09

below cut-off, n (%)

2 (22.2)

1 (7.1)

p = 0.538²; OR = 0.27