• DOI:10.1212/01.WNL.0000068549.52812.0F
• Corpus ID: 36556782

Idebenone treatment in Friedreich’s ataxia

@article{Buyse2003IdebenoneTI,
  title={Idebenone treatment in Friedreich’s ataxia},
  author={Gunnar M. Buyse and Luc Mertens and Giovanni Di Salvo and I Matthijs and Frank Weidemann and B{\'e}n{\'e}dicte Eyskens and Willy Goossens and Nathalie Goemans and George R. Sutherland and Johan L. K. Van Hove},
  journal={Neurology},
  year={2003},
  volume={60},
  pages={1679 - 1681}
}

• G. Buyse, L. Mertens, +7 authors J. V. Van Hove
• Published 27 May 2003
• Medicine
• Neurology

The authors report 1-year prospective data on eight patients with Friedreich ataxia. Idebenone did not halt the progression of ataxia. At the end of therapy, cardiac ultrasound demonstrated significant reduction of cardiac hypertrophy in six of eight patients. Cardiac strain and strain rate imaging showed that the reduction of hypertrophy is preceded by an early and linear improvement in cardiac function. Idebenone reduced erythrocyte protoporphyrin IX levels in five of six patients with elevated baseline levels; however, changes did not consistently relate to cardiac improvement.