Progression of myopathology in Kearns-Sayre syndrome

Reichmann, Heinz and Gold, R. and Meurers, B. and Naumann, M. and Seibel, P. and Walter, U. and Klopstock, Thomas (1993): Progression of myopathology in Kearns-Sayre syndrome. A morphological follow-up study. In: Acta Neuropathologica, Vol. 85, No. 6: pp. 679-681

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.

• Item Type: Article
• Keywords: Myopathy, Kearns-Sayre-syndrome, Cytochrome c oxidase deficiency
• Collections: Medicine
• Subjects: 600 Technology > 610 Medicine and health