1. Progression of myopathology in Kearns-Sayre syndrome: a morphological follow-up study.
- Author
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Reichmann H, Gold R, Meurers B, Naumann M, Seibel P, Walter U, and Klopstock T
- Subjects
- Blotting, Southern, Cytochrome-c Oxidase Deficiency, Follow-Up Studies, Humans, Immunohistochemistry, Male, Middle Aged, Mitochondria, Muscle ultrastructure, Kearns-Sayre Syndrome pathology, Muscles pathology
- Abstract
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.
- Published
- 1993
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