|
Proportion of female (%)
| |
n = 11
|
33%
|
|
Median current age (years)
| |
n = 28
|
52
|
|
Median age at biopsy (years)
| |
n = 27
|
38
|
|
Onset age
|
Before 1 years old
|
7
|
23,3%
|
|
First decade
|
8
|
26,7%
|
|
Second decade
|
3
|
10,0%
|
|
Third decade
|
2
|
6,7%
|
|
Fourth decade
|
1
|
3,3%
|
|
Fifth decade
|
3
|
10,0%
|
|
After 50 years old
|
3
|
10,0%
|
|
Unknown
|
3
|
10,0%
|
|
Clinical features
|
Asymptomatic
|
1
|
3,3%
|
|
Muscles disease (fatigability, exercise intolerance, muscle weakness)
|
21
|
70,0%
|
|
Cardiac involvement (cardiomegaly, rhythmic abnormalities)
|
10
|
33,3%
|
|
Hepatic involvement (hypoglycemia, hepatopathy)
|
18
|
60,0%
|
|
Respiratory involvement
|
3
|
10,0%
|
|
Biopsied muscle
|
L. biceps brachii
|
10
|
33,3%
|
|
L. vastus (medialis or lateralis)
|
7
|
23,3%
|
|
L. Deltoid
|
5
|
16,7%
|
|
L. Palmaris
|
1
|
3,3%
|
|
Tibial anterior
|
1
|
3,3%
|
|
R. Rectus femoris
|
1
|
3,3%
|
|
Unknown
|
5
|
16,7%
|
