Table 4.
Myopathological changes of the present MFM patient cohort.
| Patient no. | Necrosis (%) | Regeneration (%) | Central nuclei (%) | Eosinophilic bodies (%) | Cytoplasmic bodies (%) | Amorphous deposits (%) | Non-rimmed vacuoles (%) | Rimmed vacuoles (%) | Rubbed out fibers (%) | Desmin (%) | BAG3 (%) | αB crystallin (%) | Interstitial proliferation |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 0.9 | 0.5 | 15.0 | 6.6 | 0.5 | 16.0 | 0.2 | 0.1 | 5.6 | 12.8 | 9.2 | 14.1 | + |
| 2 | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA |
| 3 | 2.9 | 2.1 | 22.2 | 3.1 | 2.5 | 3.8 | 11.5 | 3.5 | 6.5 | 19.7 | 3.8 | 3.3 | ++ |
| 4 | 1.1 | 0.9 | 9.5 | 1.5 | 0.4 | 4.9 | 2.5 | 1.5 | 6.8 | 5.5 | 1.8 | 1.5 | – |
| 5 | 0.1 | 0.4 | 38.4 | 6.0 | 0.6 | 5.1 | 0.4 | 0.0 | 5.3 | 9.0 | 3.6 | 3.4 | + |
| 6 | 0.2 | 0.3 | 11.1 | 2.2 | 1.1 | 4.1 | 0.2 | 0.0 | 2.3 | 3.7 | 3.1 | 4.6 | + |
| 7 | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA |
| 8 | 0.6 | 0.0 | 3.4 | 0.1 | 0.0 | 0.2 | 0.0 | 0.0 | 0.1 | 1.0 | 0.1 | 0.2 | ++ |
| 9 | 0.3 | 0.1 | 1.0 | 0.5 | 6.7 | 6.8 | 0.0 | 0.0 | 0.8 | 6.4 | 5.7 | 6.5 | – |
| 10 | 0.4 | 0.0 | 1.8 | 2.4 | 1.4 | 2.2 | 0.4 | 0.0 | 2.0 | 4.3 | 3.6 | 2.3 | ++ |
| 11 | 0.6 | 0.4 | 12.4 | 0.0 | 0.0 | 0.0 | 0.4 | 1.8 | 0.0 | 0.0 | 0.0 | 0.0 | ++ |
| 12 | 0.3 | 0.2 | 18.5 | 0.0 | 0.0 | 0.0 | 0.9 | 0.6 | 0.0 | 0.0 | 0.0 | 0.0 | – |
| 13 | 0.1 | 0.0 | 10.0 | 2.4 | 4.4 | 1.0 | 0.0 | 0.0 | 0.0 | 0.2 | 0.0 | 0.5 | + |
| 14 | 1.3 | 0.9 | 31.4 | 1.1 | 4.5 | 0.0 | 0.2 | 0.0 | 1.4 | 5.0 | 1.8 | 3.9 | + |
| 15 | 0.0 | 0.2 | 10.5 | 0.2 | 0.0 | 0.0 | 0.0 | 0.0 | 0.4 | 0.8 | 0.3 | 0.0 | + |
| 16 | 0.5 | 1.1 | 86.8 | 0.1 | 0.3 | 0.3 | 2.6 | 4.0 | 0.9 | 4.3 | 1.8 | 0.0 | + |
| 17 | 1.3 | 0.6 | 79.3 | 0.4 | 0.6 | 11.9 | 2.9 | 0.3 | 0.0 | 8.8 | 1.5 | 0.8 | + |
| 18 | 2.7 | 0.7 | 36.7 | 1.8 | 0.0 | 2.0 | 2.0 | 0.0 | 0.7 | 1.5 | 1.1 | 1.3 | ++ |
−, no interstitial proliferation; +, ++ corresponds to mild, moderate interstitial proliferation.