Table 2 Clinical features of patients
Patients | n = 30 | ||
|---|---|---|---|
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% |