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Muscle biopsy in both patients demonstrated ragged-red fibers, lipid storage, and decreased staining for succinate dehydrogenase (SDH, FAD-dependent mitochondrial respiratory chain Complex II) and COX (mitochondrial respiratory chain Complex IV)

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After the onset of visual acuity decline, the patient underwent ophthalmological evaluation

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We propose a mechanism by which a vicious cycle of oxidative stress and neuroinflammation enhances neurodegenerative processes