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Reichmann, Heinz; Gold, R.; Meurers, B.; Naumann, M.; Seibel, P.; Walter, U. und Klopstock, Thomas (1993): Progression of myopathology in Kearns-Sayre syndrome. A morphological follow-up study. In: Acta Neuropathologica, Vol. 85, Nr. 6: S. 679-681


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.