|
Gender, M/F
|
80 M/60 F
|
M
|
F
|
|
Age at recruitment, mean ± SD
|
57 ± 16
|
69
|
42
|
|
Months from symptoms onset, median (min, max)
|
6 (0–373)
|
12
|
16
|
|
Body mass index, kg/m2
|
28.5 ± 9.5
|
19.7
|
23.3
|
|
Respiratory symptoms
|
100%
|
yes
|
yes
|
|
• Dyspnea during exercise
|
86.4%
|
yes
|
yes
|
|
• Dyspnea at rest
|
35.9%
|
no
|
yes
|
|
• Ineffective cough
|
41.1%
|
yes
|
yes
|
|
• Ortopnea
|
43.5%
|
no
|
no
|
|
• Fatigue
|
84.3%
|
yes
|
yes
|
|
• Airways infections
|
44.1%
|
no
|
yes
|
|
Sleep disorders
|
36.4%
|
yes
|
yes
|
|
• Nocturnal restlessness
|
44.2%
|
yes
|
yes
|
|
• Frequent reawaken
|
40.0%
|
no
|
yes
|
|
• Nocturnal apnoea
|
41.4%
|
no
|
no
|
|
• Snoring
|
30.0%
|
no
|
no
|
|
• Morning sleepiness
|
25.0%
|
no
|
yes
|
|
• Morning headache
|
20.0%
|
yes
|
no
|
|
• Day sleepiness
|
39.2%
|
yes
|
yes
|
|
Acute respiratory failure at recruitment
|
28.5%
|
yes
|
yes
|
|
Myalgia
|
52.1%
|
no
|
no
|
|
CPKaemia, IU/L
|
345 ± 700
|
206
|
471
|
|
AST, U/L
|
27 ± 13
|
44
|
56
|
|
PaCO2, mmHg
|
43 ± 12
|
54.2
|
46
|
|
Upright FVC % predicted
|
67 ± 25
|
62
|
66
|
| △Upright-Supine FVC% |
−18 ± 20
|
−28
|
−31
|
|
Lower-girdle muscle weakness, %
|
48.6%
|
yes
|
yes
|
|
Upper-girdle muscle weakness, %
|
35.7%
|
yes
|
yes
|
|
Walton&Gardner-Medwin Scale
|
2.9 ± 3.1
|
7
|
4
|
|
GAA activity, microMol/L/h
|
10.1 ± 6.6
|
0.36
|
0.71
|
