Fig. 2.

Muscle biopsy findings. (a) H&E, scale bar = 100 μm and (b) Gomori trichrome, scale bar = 50 μm reveal evidence of subsarcolemmal mitochondrial accumulation consistent with “ragged-red” changes in the patient’s biopsy. (c) Several fibres are depleted for COX activity (asterisked; scale bar = 100 μm), which is accentuated following the histochemical demonstration of sequential COX and SDH activities (d; scale bar = 250 μm). (e) Electron microscopy revealed focal subsarcolemmal accumulation of abnormal mitochondria in muscle with typical parking lot-like inclusions, scale bar = 500 nm.