Clinical experience with high-dose idebenone in Friedreich ataxia

• Published in: Journal of neurology Vol. 256; no. Suppl 1; pp. 42 - 45
• Main Authors: Schulz, Jörg B., Di Prospero, Nicholas A., Fischbeck, Kenneth
• Format: Journal Article
• Language: English
• Published: Darmstadt Steinkopff-Verlag 01.03.2009; Springer Nature B.V
• Subjects: Antioxidants - therapeutic use; Ataxia; Cardiomyopathies - drug therapy; Cardiomyopathies - etiology; Cardiomyopathy; Clinical Protocols; Clinical trials; Clinical Trials as Topic - methods; Dose-Response Relationship, Drug; Friedreich Ataxia - complications; Friedreich Ataxia - drug therapy; Heart; Humans; Medicine; Medicine & Public Health; Nervous System Diseases - drug therapy; Nervous System Diseases - etiology; Neurology; Neuroradiology; Neurosciences; Oxidative stress; Ubiquinone - analogs & derivatives; Ubiquinone - therapeutic use; Aachen Germany; United States > US; Germany; rare disorders; Friedreich ataxia; clinical trials; cardiomyopathy; ICARS; FARS; neurologic; oxidative stress; idebenone

Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably determined in these studies; when neurologica...