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Idebenone in patients with Friedreich ataxia

Authors
Journal
Neuroscience Letters
0304-3940
Publisher
Elsevier
Publication Date
Volume
306
Issue
3
Identifiers
DOI: 10.1016/s0304-3940(01)01892-4
Keywords
  • Friedreich Ataxia
  • Magnetic Resonance Spectroscopy
  • Idebenone
  • Mitochondrial Impairment
  • Respiratory Function
  • Cardiomyopathy
  • Controlled Trial

Abstract

Abstract Friedreich ataxia (FA), the most common form of degenerative ataxia, is thought to be caused by respiratory deficiency due to mitochondrial iron accumulation and oxidative stress. Idebenone, a free-radical scavenger, protects mitochondrial function in in vitro models of FA. In a placebo-controlled crossover trial we studied the effect of idebenone on respiratory function in nine ambulant FA patients. 31P magnetic resonance spectroscopy demonstrated mitochondrial impairment in vivo in skeletal muscle of all FA patients, but no recovery with idebenone. No effects were seen in clinical scores. Echocardiography did not confirm a preliminary study reporting improvement of FA-associated cardiomyopathy with idebenone.

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