Monitoring Cardiac Function During Idebenone Therapy in Friedreich's Ataxia

Title:Monitoring Cardiac Function During Idebenone Therapy in Friedreich's Ataxia

Volume: 21 Issue: 4

Author(s): Tomas Palecek, Michal Fikrle, Eduard Nemecek, Lenka Bauerova, Petr Kuchynka, William E. Louch, Ivan Spicka and Romana Rysava

Affiliation:

Keywords: Idebenone, Friedreich’s ataxia, Echocardiography.

Abstract: Friedreich’s ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of death (59%) in FA patients. Cardiac related death occurs at a significantly younger age than non-cardiac related death. Idebenone is a short-chain quinone analogue with a potent free-radical scavenger action. This drug has the potential to preserve and even improve mitochondrial function.Studies on Idebenone treatment showed rather conflicting results on FA cardiomyopathy. The present article reviews the clinical features of FA cardiomyopathy, imaging techniques used to diagnose, follow and monitor therapy which aimed to revert FA cardiomyopathy.

Cite this article as:

Palecek Tomas, Fikrle Michal, Nemecek Eduard, Bauerova Lenka, Kuchynka Petr, Louch E. William, Spicka Ivan and Rysava Romana, Monitoring Cardiac Function During Idebenone Therapy in Friedreich's Ataxia, Current Pharmaceutical Design 2015; 21 (4) . https://dx.doi.org/10.2174/138161282104141204142917