title: The reduction of temporal optic nerve head microcirculation in autosomal dominant optic atrophy.
abstract: OBJECTIVE
  To evaluate the optic nerve head (ONH) microcirculation in autosomal dominant optic atrophy (ADOA) patients.
  
  
  METHODS
  This study comprised 22 eyes of 12 ADOA patients, diagnosed according to clinical findings including family history and the presence of mutations in the OPA1 gene. Twenty-four normal eyes of 24 age-matched subjects, with either the right or left eye randomly selected for use, served as controls. Circumpapillary retinal nerve fibre layer thickness (cpRNFLT) and mean blur rate (MBR) in the ONH were determined with optical coherence tomography (OCT) and laser speckle flowgraphy (LSFG), respectively. For each ONH quadrant (superior, temporal, inferior and nasal), the MBR and cpRNFLT ratio was also calculated by dividing tissue MBR in that quadrant by tissue MBR in the entire ONH and by dividing cpRNFLT in that quadrant by cpRNFLT in the entire ONH respectively.
  
  
  RESULTS
  Mean blur rate (MBR) in all quadrants was significantly lower in the ADOA patients than in the controls (p < 0.001 in each). The MBR ratio was significantly lower in the ADOA patients only in the temporal quadrant (p < 0.001). Similarly, cpRNFLT was lower in the ADOA patients in all quadrants (p < 0.001 in each), and the cpRNFLT ratio was lower in the temporal quadrant (p < 0.001).
  
  
  CONCLUSIONS
  Reduced blood flow in the temporal optic disc in ADOA patients is associated with reduced temporal cpRNFLT, suggesting that both are caused by damage to the papillomacular bundle. The anatomical characteristics of the papillomacular bundle may make it especially susceptible to mitochondrial dysfunction-induced damage, which occurs in ADOA.
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Maki
authors.initials: M
authors.lastname: Inoue
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Himori
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.|Department of Retinal Disease Control, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kunikata
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takeshita
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Aizawa
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Shiga
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Kazuko
authors.initials: K
authors.lastname: Omodaka
authors.affiliation: Department of Advanced Ophthalmic Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Koji M
authors.initials: KM
authors.lastname: Nishiguchi
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takahashi
authors.affiliation: Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan. ntoru@oph.med.tohoku.ac.jp.|Department of Retinal Disease Control, Tohoku University Graduate School of Medicine, Sendai, Japan. ntoru@oph.med.tohoku.ac.jp.|Department of Advanced Ophthalmic Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan. ntoru@oph.med.tohoku.ac.jp.
authors.forename: Toru
authors.initials: T
authors.lastname: Nakazawa
mesh_terms: D000328:Adult; D001783:Blood Flow Velocity; D002648:Child; D004252:DNA Mutational Analysis; D005260:Female; D020558:GTP Phosphohydrolases / Q000235:genetics; D006801:Humans; D017078:Laser-Doppler Flowmetry; D008297:Male; D008833:Microcirculation; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology; D029241:Optic Atrophy, Autosomal Dominant / Q000235:genetics / Q000503:physiopathology*; D009898:Optic Disk / Q000098:blood supply*; D011506:Proteins / Q000235:genetics; D012165:Retinal Ganglion Cells / Q000473:pathology; D041623:Tomography, Optical Coherence; D055815:Young Adult
chemical_list: C443108:OPA3 protein, human; D011506:Proteins; D020558:GTP Phosphohydrolases; C415198:OPA1 protein, human
keywords: OPA1; autosomal dominant optic atrophy; laser speckle flowgraphy; papillomacular bundle