Skeletal muscle pathology in chronic progressive external ophthalmoplegia with ragged-red fibers.

Abstract:

: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 cytochrome c 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 length, suggesting the heterogeneity of CCO activity even in the same fiber.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Yamamoto M,Nonaka I

doi

10.1007/BF00689593

subject

Has Abstract

pub_date

1988-01-01 00:00:00

pages

558-63

issue

6

eissn

0001-6322

issn

1432-0533

journal_volume

76

pub_type

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