Twitch response of striated muscle in patients with progressive external ophthalmoplegia, mitochondrial myopathy and focal cytochrome c-oxidase deficiency

A. Moglia, E. Alfonsi, G. Piccolo, A. Lozza, A. Arrigo, E. Bollani, S. Malaguti

Research output: Contribution to journalArticle

Abstract

Some aspects of the contractile properties of skeletal muscle in patients with progressive external ophthalmoplegia (PEO), mitochondrial myopathy and focal cytochrome c-oxidase deficiency were investigated by studying the twitch response (TR) of the tibialis anterior muscle both at rest and after maximum isometric contraction. The results of needle electromyography were normal in four of the six examined patients, and myopathic in the remaining two. A slowing in muscle relaxation was the most frequently observed abnormality; significantly prolonged muscle contraction times and reduced twitch torque potentiation values after isometric contraction were also detected. TR abnormalities in PEO patients may be due either to a dysfunction of the contractile machinery depending upon impaired muscle energy supply or to altered muscle fiber characterized by the predominance of type I slow fiber. In addition to conventional electromyographic investigations, TR study may be a useful diagnostic tool in PEO patients.

Original languageEnglish
Pages (from-to)159-166
Number of pages8
JournalItalian Journal of Neurological Sciences
Volume16
Issue number3
DOIs
Publication statusPublished - Apr 1995

Keywords

  • contraction time
  • cytochrome c-oxidase deficiency
  • mitochondrial myopathy
  • progressive external ophthalmoplegia
  • relaxation time
  • twitch response
  • twitch torque

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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