Table 3 ECAS outcome scores of patients with SMA type 2 and 3 before and after treatment initiation

Language (max. 28)

27.0 (25–28)

28.0 (25–28)

Z = − 2.352; p = 0.019 ¹; r = 0.49

below cut-off, n (%)

2 (8.7)

1 (4.3)

p > 0.999²; OR = 0.48

Verbal Fluency (max. 24)

12.0 (8–22)

16.0 (10–24)

Z= − 2.199;p = 0.028¹; r = 0.46

below cut-off, n (%)

7 (30.4)

3 (13.0)

p = 0.284²; OR = 0.34

Executive Functions (max. 48)

39.0 (28–44)

40.0 (32–46)

Z= − 2.469;p = 0.014¹; r = 0.51

below cut-off, n (%)

4 (17.4)

1 (4.3)

p = 0.346²; OR = 0.22

Memory (max. 24)

16.0 (11–21)

18.0 (14–23)

Z= − 2.979;p = 0.003¹; r = 0.62

below cut-off, n (%)

0 (0.0)

0 (0.0)

p > 0.999²

Visuospatial (max. 12)

12.0 (11–12)

12.0 (8–12)

Z = − 1.511; p = 0.131¹; r = 0.81

below cut-off, n (%)

1 (4.3)

4 (17.4)

p = 0.346²; OR = 4.63

ALS-specific (max. 100)

78.0 (64–90)

82.0 (72–96)

Z= − 3.241;p = 0.001¹; r = 0.68

below cut-off, n (%)

11 (47.8)

8 (34.8)

p = 0.550²; OR = 0.58

Non-ALS-specific (max. 36)

28.0 (23–32)

30.0 (24–35)

Z= − 2.754;p = 0.006¹; r = 0.57

below cut-off, n (%)

0 (0.0)

0 (0.0)

p > 0.999²

ECAS total (max. 136)

106.0 (91–119)

113.0 (96–130)

Z= − 3.318;p < 0.001¹; r = 0.69

below cut-off, n (%)

3 (13.0)

0 (0.0)

p = 0.233²; OR = 0.00