Inoue Maki, Himori Noriko, Kunikata Hiroshi, Takeshita Takayuki, Aizawa Naoko, Shiga Yukihiro, Omodaka Kazuko, Nishiguchi Koji M, Takahashi Hidetoshi, Nakazawa Toru
Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Department of Retinal Disease Control, Tohoku University Graduate School of Medicine, Sendai, Japan.
Acta Ophthalmol. 2016 Nov;94(7):e580-e585. doi: 10.1111/aos.12999. Epub 2016 Mar 3.
To evaluate the optic nerve head (ONH) microcirculation in autosomal dominant optic atrophy (ADOA) patients.
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.
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).
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.
评估常染色体显性遗传性视神经萎缩（ADOA）患者的视神经乳头（ONH）微循环。
本研究纳入了12例ADOA患者的22只眼睛，根据包括家族史和OPA1基因突变在内的临床发现进行诊断。选取24例年龄匹配受试者的24只正常眼睛作为对照，随机选择右眼或左眼。分别使用光学相干断层扫描（OCT）和激光散斑血流图（LSFG）测定ONH处的视盘周围视网膜神经纤维层厚度（cpRNFLT）和平均模糊率（MBR）。对于每个ONH象限（上方、颞侧、下方和鼻侧），还分别通过将该象限的组织MBR除以整个ONH的组织MBR以及将该象限的cpRNFLT除以整个ONH的cpRNFLT来计算MBR和cpRNFLT比率。
ADOA患者所有象限的平均模糊率（MBR）均显著低于对照组（各象限p < 0.001）。ADOA患者仅颞侧象限的MBR比率显著降低（p < 0.001）。同样，ADOA患者所有象限的cpRNFLT均较低（各象限p < 0.001），且颞侧象限的cpRNFLT比率较低（p < 0.001）。
ADOA患者颞侧视盘血流减少与颞侧cpRNFLT降低有关，提示两者均由乳头黄斑束受损所致。乳头黄斑束的解剖特征可能使其特别易受ADOA中发生的线粒体功能障碍诱导的损伤。
