Summary
We report on the progression of myopathology by comparing two biopsies from a patient with a Kearns-Sayre-Syndrome. The first biopsy was taken in 1979 and showed 10% ragged-red fibers. Myopathic changes were slight including internal nuclei and fiber splitting in 10% of the fibers. Electron microscopy revealed typical mitochondrial abnormalities with regard to number and shape. In 1989 a second biopsy was performed for an extended analysis of mitochondrial DNA. This time less than 5% of all fibers were ragged-red. Severe myopathic changes could be detected which so far has rarely been reported in mitochondrial cytopathy.
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Supported by the Deutsche Forschungsgemeinschaft (Re 265/6-1 and Re 265/7-1)
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Reichmann, H., Gold, R., Meurers, B. et al. Progression of myopathology in Kearns-Sayre syndrome: a morphological follow-up study. Acta Neuropathol 85, 679–681 (1993). https://doi.org/10.1007/BF00334681
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DOI: https://doi.org/10.1007/BF00334681