Table 1 Characteristics of patients

Age at FSHD score evaluation – years

mean ± SD

36.4 ± 9.0

35.6 ± 10.0

35.0 ± 9.5

40.5 ± 8.7

 18–39

n (%)

88 (56.1%)

29 (63.0%)

66 (63.5%)

21 (34.4%)

 40–60

n (%)

69 (43.9%)

17 (37.0%)

38 (36.5%)

40 (65.6%)

Age at symptoms onset – years (*)

mean ± SD

20.5 ± 10.4

28.3 ± 12.0

-

27.3 ± 14.3

 0–17

n (%)

79 (50.3%)

12 (26.1%)

-

20 (32.8%)

 18–39

n (%)

65 (41.4%)

26 (56.5%)

-

22 (36.1%)

 40–60

n (%)

13 (8.3%)

8 (17.4%)

-

19 (31.1%)

Disease duration – years (*)

mean ± SD

15.8 ± 11.1

7.3 ± 9.0

-

13.3 ± 11.9

 0–9

n (%)

56 (35.7%)

30 (65.2%)

-

30 (49.2%)

 10–19

n (%)

47 (30.0%)

10 (21.7%)

-

12 (19.7%)

 20–29

n (%)

33 (21.0%)

5 (10.9%)

-

13 (21.3%)

 30–50

n (%)

21 (13.4%)

1 (2.2%)

-

6 (9.8%)

Gender - male

n (%)

81 (51.6%)

26 (56.5%)

39 (37.5%)

29 (47.5%)

Practiced Pas

n (%)

66 (42.0%)

24 (52.2%)

30 (28.8%)

14 (23.0%)

 non-organized training

n (%)

17 (10.8%)

6 (13.0%)

7 (6.7%)

5 (8.2%)

organized training 

n (%)

49 (31.2%)

18 (39.1%)

23 (22.1%)

9 (14.8%)

 medium-high cardiovascular commitment

n (%)

51 (32.5%)

19 (41.3%)

26 (25.0%)

12 (19.7%)

FSHD score [0–15]

mean ± SD

5.8 ± 3.0

2.2 ± 1.7

0.0 ± 0.2

6.5 ± 3.8

 0–1

n (%)

1 (0.6%)

21 (45.7%)

104 (100.0%)

5 (8.2%)

 2–4

n (%)

62 (39.5%)

20 (43.5%)

0 (0.0%)

17 (27.9%)

 5–10

n (%)

80 (51.0%)

5 (10.9%)

0 (0.0%)

28 (45.9%)

 11–15

n (%)

14 (8.9%)

0 (0.0%)

0 (0.0%)

11 (18.0%)

DRA dimension – number of alleles

mean ± SD

24.7 ± 6.3

29.3 ± 5.3

32.7 ± 5.1

26.6 ± 7.1

 11–19

n (%)

32 (20.4%)

2 (4.3%)

0 (0.0%)

9 (14.8%)

 20–30

n (%)

97 (61.8%)

27 (58.7%)

33 (31.7%)

35 (57.4%)

 31–35

n (%)

23 (14.6%)

10 (21.7%)

44 (42.3%)

13 (21.3%)

 36–41

n (%)

5 (3.2%)

7 (15.2%)

27 (26.0%)

4 (6.6%)