Degree of histopathology and results from immune staining and muscle fibre size measurements
| Patient/muscle | Age (years)/ sex | Degree of histopathological changes* | Dystrophin† | Desmin† | Vimentin† | CD56† | MCSA type I fibre μm2 (SD) | MCSA type IIa fibre μm2 (SD) | MCSA type IIb fibre μm2 (SD) | MCSA type IIc fibre μm2 (SD) |
|---|---|---|---|---|---|---|---|---|---|---|
| *0, normal muscle; I, mild changes; II, moderate changes; III, moderate to severe changes; IV, severe changes. | ||||||||||
| †Expression of dystrophin decreased and that of desmin increased in some degenerating or regenerating fibres. A, all fibres stained; +, <10% stained; ++, 10–50% stained; +++, >50% stained. | ||||||||||
| CP, cricopharyngeus muscle; F, female; M, male; TA, tibialis anterior muscle; VL, vastus lateralis muscle. | ||||||||||
| 1/TA | 80/F | II | A | A | + | + | 5243 (3117) | 2306 (649) | 2651 (197) | 2129 (1876) |
| 2/VL | 76/M | III | A | A | +++ | + | 5872 (2153) | 5097 (1639) | 1857 (1145) | 1920 (1523) |
| 3/CP | 76/M | III | A | A | + | ++ | 3993 (2751) | 756 (365) | 1421 (819) | 1756 (2563) |
| 3/VL | 75/M | III | A | A | ++ | +++ | 2140 (1581) | 602 (277) | ND | 1153 (683) |
| 3/TA | 75/M | II | A | A | + | + | 7890 (4647) | 0 | 0 | 2459 (490) |
| 4/VL | 75/M | IV | A | A | + | + | 6663 (4455) | 0 | 0 | 1024 (567) |
| 5/TA | 74/F | II | A | A | + | + | 4591 (2665) | 1621 (352) | 1193 (297) | 2275 (1940) |
| 6/VL | 69/M | III | A | A | + | + | 10716 (3450) | 5029 (3117) | 3119 (1712) | 3691 (3232) |
| 6/VL | 69/M | III | A | A | + | ++ | 5560 (2950) | 1506 (936) | 1263 (991) | 1245 (1168) |
| 7/VL | 67/M | III | A | A | + | + | 10233 (7174) | 0 | 0 | 1513 (910) |
| 8/TA | 60/M | III | A | A | + | + | 5616 (4491) | 1142 (831) | 1025 (274) | 1086 (528) |
| 9/VL | 57/F | III | A | A | + | + | 2683 (1685) | 2604 (2018) | 3724 (1582) | 2102 (1270) |
| 10/VL | 57/F | II | A | A | + | + | 4490 (2678) | 2434 (1416) | 2636 (858) | 7043 (1269) |
| 11/TA | 45M | I | A | A | + | + | 8861 (5018) | 14912 (4546) | 8169 (2144) | 10363 (2542) |