title: Antioxidant use in Friedreich ataxia.
abstract: Many antioxidants have been suggested as potential treatments for Friedreich ataxia, but have not been tested in clinical trials. We found that a majority of patients in our cohort already use such antioxidants, including idebenone, which is not available at a pharmaceutical grade in the United States. Younger age, cardiomyopathy and shorter GAA repeat length were independent predictors of idebenone use, but no factors predicted use of other antioxidants. This confirms that non-prescription antioxidant use represents a major confounder to formal trials of existing and novel agents for Friedreich ataxia.
authors.affiliation: Department of Neurology, University of Pennsylvania School of Medicine, USA.
authors.forename: Lauren
authors.initials: L
authors.lastname: Myers
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Farmer
authors.forename: Robert B
authors.initials: RB
authors.lastname: Wilson
authors.forename: Lisa
authors.initials: L
authors.lastname: Friedman
authors.forename: Amy
authors.initials: A
authors.lastname: Tsou
authors.forename: Susan L
authors.initials: SL
authors.lastname: Perlman
authors.forename: Sub H
authors.initials: SH
authors.lastname: Subramony
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Gomez
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Ashizawa
authors.forename: George R
authors.initials: GR
authors.lastname: Wilmot
authors.forename: Katherine D
authors.initials: KD
authors.lastname: Mathews
authors.forename: Laura J
authors.initials: LJ
authors.lastname: Balcer
authors.forename: David R
authors.initials: DR
authors.lastname: Lynch
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000975:Antioxidants / Q000008:administration & dosage*; D009202:Cardiomyopathies / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D002986:Clinical Trials as Topic / Q000592:standards; D015331:Cohort Studies; D015986:Confounding Factors, Epidemiologic; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005621:Friedreich Ataxia / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000187:drug effects / Q000235:genetics; D004366:Nonprescription Drugs / Q000627:therapeutic use*; D018384:Oxidative Stress / Q000187:drug effects / Q000235:genetics; D018579:Patient Selection; D015990:Placebo Effect; D012651:Self Medication / Q000706:statistics & numerical data* / Q000639:trends; D019680:Trinucleotide Repeat Expansion / Q000187:drug effects / Q000235:genetics; D014451:Ubiquinone / Q000008:administration & dosage / Q000031:analogs & derivatives*
chemical_list: D000975:Antioxidants; D004366:Nonprescription Drugs; D014451:Ubiquinone; C036619:idebenone