Table 3: Numbers of patients exhibiting clinical features according to age of disease onset.
| Clinical feature | Age of disease onset | ||
|---|---|---|---|
| congenital (<2 years) | Childhood (2-16 years) | Adult (>16years) | |
| Creatine Kinase level (IU/L) | |||
| Absent | 8 | 8 | 6 |
| Normal (<250) | 30 | 13 | 5 |
| 250-999 | 8 | 8 | 12 |
| 1,000-2,999 | 0 | 4 | 13 |
| 3,000-9,999 | 1 | 1 | 1 |
| >10,000 | 0 | 1 | 0 |
| Presence of any contractures (including rigid spine) | |||
| yes | 33 | 18 | 16 |
| no | 14 | 17 | 21 |
| Presence of any respiratory dysfunction | |||
| yes | 26 | 7 | 11 |
| no | 21 | 28 | 26 |
| Inheritance pattern | |||
| Recessive | 4 | 7 | 6 |
| Dominant | 4 | 4 | 10 |
| Sporadic | 39 | 24 | 21 |
| Pattern of muscle involvement | |||
| Proximal | 7 | 7 | 8 |
| Proximal>distal | 12 | 10 | 16 |
| Proximal and distal | 18 | 9 | 7 |
| Distal>proximal | 4 | 4 | 3 |
| Distal | 0 | 2 | 1 |
| No weakness | 6 | 3 | 2 |