Idebenone Treatment into Patients with Friedreich Ataxia and Quinone Deficience

The benzoquinone ring of idebenone can undergo reversible redox reactions, which influence the electron balance in mitochondria. Early clinical studies of idebenone at a dose of 5 mg·kg-1·d-1 mainly demonstrated the efficacy of idebenone on Friedreich ataxia(FRDA)-associated cardiomyopathy. More recent trials have shown that higher doses of idebenone and longer time of idebenone treatment not only could improve cardiomyopathy, but improve neurological function in the patients with FRDA. Especially the stabilizing function of idebenone was obvious for a long time in paediatric patients. Meanwhile, idebenone could improve cerebral mitochondrial metabolism in the patients with mitochondrial encephalopathy.