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

H. Mitsumoto, J. R. Aprille, S. H. Wray, R. Nemni, Walter G Bradley

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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 cc 1 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
StatePublished - May 26 1983
Externally publishedYes

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Chronic Progressive External Ophthalmoplegia
Mitochondria
Respiration
Cytochromes
Skeletal Muscle

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Chronic progressive external ophthalmoplegia (CPEO) : Clinical, morphologic, and biochemical studies. / Mitsumoto, H.; Aprille, J. R.; Wray, S. H.; Nemni, R.; Bradley, Walter G.

In: Neurology, Vol. 33, No. 4, 26.05.1983, p. 452-461.

Research output: Contribution to journalArticle

Mitsumoto, H, Aprille, JR, Wray, SH, Nemni, R & Bradley, WG 1983, 'Chronic progressive external ophthalmoplegia (CPEO): Clinical, morphologic, and biochemical studies', Neurology, vol. 33, no. 4, pp. 452-461.
Mitsumoto, H. ; Aprille, J. R. ; Wray, S. H. ; Nemni, R. ; Bradley, Walter G. / Chronic progressive external ophthalmoplegia (CPEO) : Clinical, morphologic, and biochemical studies. In: Neurology. 1983 ; Vol. 33, No. 4. pp. 452-461.
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