title: Effects of idebenone on color vision in patients with leber hereditary optic neuropathy.
abstract: BACKGROUND
  The authors investigated the correlation of protan and tritan color vision with disease characteristics in Leber hereditary optic neuropathy (LHON). The authors also characterized the therapeutic potential of idebenone in protecting patients from developing dyschromatopsia in LHON.
  
  
  METHODS
  Color contrast data of 39 LHON patients participating in a randomized, double-blind placebo-controlled intervention study were evaluated. Patients reported disease onset <5 years before enrolment and were genetically confirmed. Protan and tritan color contrast sensitivity was measured using a computer graphics method in patients receiving idebenone (Catena; 900 mg/d; N = 28) or placebo (N = 11) for 6 months.
  
  
  RESULTS
  Mean age of patients was 28.1 years, 87.2% were men, 76.9% carried the m11778G>A mutation, and mean duration since onset was 2 years. Assessing protan and tritan color vision at baseline revealed a high degree of color confusion even in young patients (<25 years) and with a short history of disease (<1 year). Treatment with idebenone improved tritan color vision compared with placebo (P = 0.008 at week 24); a similar trend was seen for protan. The effect of idebenone was most prominent in patients with discordant visual acuity (interocular difference of logMAR >0.2). In this subgroup, the treatment effect at week 24 was 20.4% (P = 0.005) in favor of idebenone for the tritan color domain and 13.5% (P = 0.067) for the protan domain.
  
  
  CONCLUSIONS
  This study confirms that protan and tritan color confusion is an early symptom in LHON. Treatment with idebenone can protect from loss of color vision, particularly in patients who are at imminent risk of further vision loss.
authors.affiliation: Department of Ophthalmology (GR, FS), Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Guenther
authors.initials: G
authors.lastname: Rudolph
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimitriadis
authors.forename: Boriana
authors.initials: B
authors.lastname: Büchner
authors.forename: Suzette
authors.initials: S
authors.lastname: Heck
authors.forename: Jasmina
authors.initials: J
authors.lastname: Al-Tamami
authors.forename: Florian
authors.initials: F
authors.lastname: Seidensticker
authors.forename: Christian
authors.initials: C
authors.lastname: Rummey
authors.forename: Mika
authors.initials: M
authors.lastname: Leinonen
authors.forename: Thomas
authors.initials: T
authors.lastname: Meier
authors.forename: Thomas
authors.initials: T
authors.lastname: Klopstock
mesh_terms: D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000627:therapeutic use*; D003118:Color Perception / Q000502:physiology*; D004311:Double-Blind Method; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D029242:Optic Atrophy, Hereditary, Leber / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D010859:Pigmentation Disorders / Q000151:congenital* / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D012873:Skin Diseases, Genetic / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D016896:Treatment Outcome; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D000975:Antioxidants; D014451:Ubiquinone; C036619:idebenone