Ubidecarenone in the treatment of mitochondrial myopathies: A multi-center double-blind trial

N. Bresolin, C. Doriguzzi, C. Ponzetto, C. Angelini, I. Moroni, E. Castelli, E. Cossutta, A. Binda, A. Gallanti, S. Gabellini, G. Piccolo, A. Martinuzzi, E. Ciafaloni, E. Arnaudo, L. Liciardello, A. Carenzi, G. Scarlato

Research output: Contribution to journalArticlepeer-review


Forty-four patients with mitochondrial myopathies were treated with Ubidecarenone (CoQ10) for 6 months in an open multi-center trial. No side effects of the drug were observed. Sixteen patients showing at least 25% decrease of post-exercise lactate levels were selected as responders. Responsiveness was apparently not related to CoQ10 level in serum and platelets or to the presence or absence of mtDNA deletions. The responders were treated for a further 3 months with CoQ10 or placebo in the second blind part of the trial; no significant differences were observed between the 2 groups. It is not clear why CoQ10 had therapeutic effects in some patients and not in others with the same clinical presentation and biochemical defect, and we failed to identify candidate responders before treatment. At the dose of CoQ10 used in this study (2 mg/kg/day) the therapy requires a long administration time before a response is seen.

Original languageEnglish
Pages (from-to)70-78
Number of pages9
JournalJournal of the Neurological Sciences
Issue number1-2
Publication statusPublished - 1990


  • Mitochondrial myopathy
  • mtDNA deletion
  • Ubiquinone

ASJC Scopus subject areas

  • Ageing
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology


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