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Washington University Experience | MUSCLE | Myopathies with Mitochondrial Pathology | 28A6 (Case 28) muscle_038 - CopyA

Large vacuoles with engulfed debris. (electron micrographs) ---- Comment: Ultrastructural examination of this skeletal muscle biopsy shows numerous abnormalities. The most prominent feature involves mitochondria, consisting of increased numbers collected in the subsarcolemma and within the deep sarcoplasm of occasional fibers. These mitochondria are associated with a variety of lipid droplets ranging from small to large, disorganization of mitochondrial cristae, and small numbers of matrix densities. Some fibers have increased numbers of autophagosomes and lipofuscin. COX staining shows 2 fibers with negative staining. SDH shows abnormal internal architecture, but no fibers have mitochondrial proliferation.

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