ISSN:
1432-0533
Keywords:
Cytochromec oxidase deficiency
;
Ragged-red fibers
;
Mitochondrial myopathy
;
Chronic progressive external ophthalmoplegia (CPEO)
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Histochemical investigations were carried out on skeletal muscle biopsies from ten patients with chronic progressive external ophthalmoplegia with ragged-red fibers (RRF). In addition to the RRF, mild myopathic change consisting of variation in size of both type 1 and 2 fibers was seen in all patients, as well as neuropathic change in eight. Scattered fibers with absent cytochromec oxidase (CCO) activity (focal deficiency) were seen in all patients. In serial sections, CCO deficiency did not always occupy the entire length of a fiber but was localized segmentally to regions measuring several hundred micrometers in lenghth, suggesting the heterogeneity of CCO activity even in the same fiber.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00689593
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