Table 2 Participant characteristics

Age

121

100

44.8 ± 13.0

21

43

85

Sex

Male

55

44

Female

66

55

Age at onset

121

100

27.7 ± 9.6

15

26

61

Body mass index

121

100

21.4 ± 4.4

12.1

21

37.8

Normal

65

54

Underweight

36

30

Obesity

20

16

Walking capability

Ambulant without assistance

24

20

Ambulant with assistance

45

37

Non-ambulant

52

43

Wheelchair use

Never

43

36

Part-time

28

23

Full time

50

41

Respiratory function (%FVC)

Performed

79

65

88.4 ± 23.7

16.4

92.8

130

Not performed

42

35

Normal (FVC ≥ 80%)

26

33*

Decreased

53

67*

Nocturnal NPPV

2

2

Cardiac function

Performed

35

29

(EF, ES)

Not performed

86

71

Normal

35

100*

Decreased

0

0*

Grip power (kg)

Performed

96

79

5.5 ± 7.2

0

2.9

30

Not performed

25

21

Grip power = 0

36

38*

CK (Iu/L)

Performed

120

99

357.8 ± 209.0

11

209

459

Not performed

1

1