Chronic progressive external ophthalmoplegia (CEPO): Clinical, morphologic, and biochemical studies

Hiroshi Mitsumoto, June R. Aprille, Shirley H. Wray, Raffaello Nemni, Walter G. Bradley

Research output: Contribution to journalArticlepeer-review

Abstract

We studied skeletal muscles from eight chronic progressive external ophthalmoplegia patients with ragged-red fibers (group A), five CPEO patients without ragged-red fibers (group B), and five controls. The EM morphometric fraction of structurally abnormal mitochondria was increased in group A, and there was a similarly increased fraction of normal-appearing mitochondria in group B. State 3 respiration and uncoupled respiration were severely decreased in both groups. The morphometric mitochondrial fraction and respiratory functions were inversely related in all three groups. The cytochrome content in group A was normal; cytochromes b and cc1 were decreased in group B. These studies point to a central role of mitochondrial dysfunction in all types of CPEO, but the basic abnormalities remain to be elucidated.

Original languageEnglish
Pages (from-to)452-461
Number of pages10
JournalNeurology
Volume33
Issue number4
Publication statusPublished - 1983

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

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