Leber遗传性视神经病
视网膜
眼科
医学
明视
亚临床感染
视神经病变
神经纤维层
视网膜电图
内科学
昼夜节律
内分泌学
视神经
作者
Qingmei Miao,Yufang Cheng,Hongmei Zheng,Jiajia Yuan,Changzheng Chen
出处
期刊:Mitochondrion
[Elsevier]
日期:2023-05-01
卷期号:70: 111-117
被引量:1
标识
DOI:10.1016/j.mito.2023.04.002
摘要
To analyze the functional and structural changes in retinal ganglion cells (RGCs) and their axons that occur during Leber's hereditary optic neuropathy (LHON) using photopic negative response (PhNR) and spectral domain optical coherence tomography (SD-OCT).Individuals diagnosed with LHON and their family members were invited to participate in this cross-sectional study. PhNR and OCT were used. The PhNR amplitude and peripapillary retinal nerve fiber layer (pRNFL) thicknesses were compared among the three groups. In addition, affected individuals were divided into subacute, dynamic and chronic phases based on disease duration in order to evaluate the decay in RGCs function and structure.73 affected and 30 carriers with a m.11778G > A mutation were included. PhNR amplitude and the thickness of pRNFL significantly decreased in affected individuals and carriers compared to that of the controls (P<0.001). However, there was no difference between the carriers and the controls (P>0.05). There was no difference in the PhNR amplitude of different phases (P = 0.464). In the subacute phase, only temporal pRNFL thickness decreased significantly (P<0.001). PRNFL thickness decreased significantly in dynamic phase (P<0.001). Temporal pRNFL thickness continued to decrease in the chronic phase (P = 0.042).In the subacute phase, the function of RGCs was severely impaired. Thickness of pRNFL decreased significantly in four quadrants during disease progression. In the chronic phase, pRNFL thickness decreased slightly. Carriers have shown RGCs dysfunction before pathological changes occur, suggesting subclinical abnormalities.
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