Sheremet N L, Ronzina I A, Andreeva N A, Zhorzholadze N V, Murakhovskaya Yu K, Nevinitsyna T A, Shmelkova M S, Krylova T D, Tsygankova P G, Gerasimidi E S, Lyamzaev K G, Skulachev M V, Karger E M
Research Institute of Eye Diseases, Moscow, Russia.
Research Centre for Medical Genetics, Moscow, Russia.
Vestn Oftalmol. 2022;138(2):5-14. doi: 10.17116/oftalma20221380215.
To study the capabilities of electrophysiological and psychophysical examination methods for assessment of the functional state of ganglion cells, retina and optic nerve in patients with hereditary optic neuropathy (HON).
The study included 60 patients (118 eyes) with a genetically confirmed diagnosis of HON. All study patients underwent visual field test (VFT), spectral optical coherence tomography (OCT), flash and pattern visual evoked potentials (VEP) (Flash-VEP, FVEP; Pattern-VEP, PVEP), photopic electroretinography with photonegative response (PhNR) registration and the color vision test. In 24 patients (46 eyes), these parameters were assessed before the start of treatment and one year later. The treatment involved the mitochondria-targeted antioxidant SkQ1 - plastoquinonyl-decyl-triphenylphosphonium bromide (PDTP) in the form of eye drops.
The main PVEP components for 1.0° and 0.3° were registered in 20% and in 14% of patient eyes with HON and high visual functions, respectively. After one year of PDTP use, a significant decrease in P100 peak latency was found only in the group with disease duration of ≤1.5 years as of the time of treatment start (<0.05). Significant differences were observed in the PhNR amplitude (<0.004) between patients of the main and the control groups, as well as in the PhNR amplitude between patients with visual acuity of ≤0.1 and ≥0.13 (<0.01). Patients with high visual functions were found to have a correlation between the PhNR amplitude, GCC thickness and the global loss index (GLV).
Along with VFT, OCT and color vision tests, electrophysiological studies are one of the main methods of examining patients with HON. After one year of PDTP use, there was a significant decrease in the FVEP P2 peak latency in the group with a disease duration of ≤1.5 years as of the time of treatment start. The PhNR amplitude in patients with high visual functions was found to correlate with structural changes in the ganglion cell layer and the retinal nerve fiber layer.
研究电生理和心理物理学检查方法评估遗传性视神经病变（HON）患者神经节细胞、视网膜及视神经功能状态的能力。
该研究纳入60例经基因确诊的HON患者（118只眼）。所有研究患者均接受了视野测试（VFT）、光谱光学相干断层扫描（OCT）、闪光及图形视觉诱发电位（VEP）（闪光VEP，FVEP；图形VEP，PVEP）、记录负向光反应的明视视网膜电图（PhNR）及色觉测试。24例患者（46只眼）在治疗开始前及1年后评估了这些参数。治疗采用线粒体靶向抗氧化剂SkQ1——以滴眼液形式存在的质体醌基癸基三苯基溴化鏻（PDTP）。
主要PVEP成分中，1.0°和0.3°的成分分别在20%和14%的视力良好的HON患者眼中被记录到。使用PDTP 1年后，仅在治疗开始时疾病持续时间≤1.5年的组中发现P100峰潜伏期显著缩短（<0.05）。主要组与对照组患者之间的PhNR振幅存在显著差异（<0.004），视力≤0.1和≥0.13的患者之间的PhNR振幅也存在显著差异（<0.01）。发现视力良好的患者中，PhNR振幅、神经节细胞复合体（GCC）厚度与整体损失指数（GLV）之间存在相关性。
除VFT、OCT及色觉测试外，电生理研究是检查HON患者的主要方法之一。使用PDTP 1年后，治疗开始时疾病持续时间≤1.5年的组中FVEP的P2峰潜伏期显著缩短。发现视力良好的患者的PhNR振幅与神经节细胞层和视网膜神经纤维层的结构变化相关。
