Fig. 1

Muscle histology of all 3 SRP cases: a-f haematoxylin and eosin (H&E) stain showing scattered necrotic fibres (arrows) and atrophic fibres (open arrows) across the biopsies. c and f Numerous regenerating fibres (open arrow heads) could also be seen in case 3. g-i there was very little staining for CD3 in case 1 (arrow) and none in case 2 h. Moderate levels of CD3 were seen in case 3 in the endomysial compartment (i, arrows). j-l there was diffuse MHC class 1 up regulation in all 3 cases, but case 3 had higher levels at the sarcolemma and within fibres. m-o MAC C5b9 stained necrotic fibres in all cases (arrows) and also some endomysial capillaries (not shown). p-r there was scattered expression of neonatal myosin in the muscle fibres of case 1 and 2 (p & q, arrows) and expression in numerous fibres in case 3 r. Scale bars: a, b, f, m-o, q and r – 100 μm. c, g-l, p - 250 μm. d and e - 50 μm