Leber遗传性视神经病变患者和携带者视网膜电图改变  

Electroretinogram changes in patients and carriers with Leber hereditary optic neuropathy

作　　者：缪青梅 程宇芳 元佳佳 郑红梅[1] 陈长征[1] Miao Qingmei;Cheng Yufang;Yuan Jiajia;Zheng Hongmei;Chen Changzheng(Department of Ophthalmology,Renmin Hospital of Wuhan University,Wuhan 430060,China)

机构地区：[1]武汉大学人民医院眼科中心,武汉430060

出　　处：《中华眼底病杂志》2022年第12期988-993,共6页Chinese Journal of Ocular Fundus Diseases

基　　金：国家自然科学基金青年科学基金(82101115);武汉大学自主创新基金青年项目(2042021kf0094)。

摘　　要：目的观察Leber遗传性视神经病变(LHON)患者和携带者图形视网膜电图(PERG)和闪光视网膜电图(FERG)的明视负波反应(PhNR)特征。方法横断面观察性研究。2021年2~11月于武汉大学人民医院眼科中心检查确诊的LHON患者32例64只眼(LHON组)和患者家系中携带相同基因突变而视力正常的成员15例30只眼(携带者组)纳入研究。LHON组患者均为男性(100.0%,32/32);年龄(23.34±7.41)岁。携带者组15例30只眼中,男性2例(13.3%,2/15),女性13例(86.7%,12/15);年龄(43.44±7.65)岁。选取同期健康志愿者24名48只眼作为对照组。其中,男性8名(33.3%,8/24),女性16名(66.7%,16/24);年龄(23.42±2.54)岁。所有受检者均采用视觉电生理仪行PERG、FERG检查。记录PERG的P50波、N95波振幅和FERG的PhNR、a波、b波振幅。采用光相干断层扫描仪测量受检眼视盘周围视网膜神经纤维层(pRNFL)厚度,包括平均以及鼻侧、上方、下方、颞侧象限。三组受检眼之间a波、b波、PhNR、P50波、N95波振幅以及pRNFL厚度比较行单因素方差分析;不同参数之间的相关性行Pearson相关性分析。结果与对照组比较,LHON组、携带者组患眼PhNR振幅显著降低,差异有统计学意义(F=11.973,P<0.001)。相关性分析结果显示,LHON患眼PhNR振幅与鼻侧、颞侧象限pRNFL厚度显著相关(r=0.249、0.272,P=0.048、0.030)。LHON组、携带者组、对照组受检眼PERG的P50波振幅比较,差异无统计学意义(F=1.342,P=0.265)。LHON组、对照组受检眼N95波振幅比较,差异无统计学意义(P=0.960)。结论与对照组比较,LHON患者和携带者FERG的PhNR振幅显著降低。Objective To observe the characteristics of pattern electroretinogram(PERG)and the photopic negative response(PhNR)of flash electroretinogram(FERG)in patients and carriers with Leber hereditary optic neuropathy(LHON).Methods A cross sectional,observational study.Thirty-two patients(64 eyes)diagnosed with LHON(LHON group)and 15 normal members with the same mutation in patient's family(carrier group)were included in this study from February 2021 to November 2021 in the Department of Ophthalmology of Renmin Hospital of Wuhan University.All patients in LHON group were males(100.0%,32/32)and the average age was 23.34±7.41 years.In the carrier group of 15 cases(30 eyes),there were 2 males(13.3%,2/15)and 13 females(86.7%,12/15).The average age was 43.44±7.65 years.Twenty-four healthy subjects(48 eyes)in the same period were selected as the control group.Among them,there were 8 males(33.3%,8/24)and 16 females(66.7%,16/24).The average age was 23.42±2.54 years.All subjects were examined with the GT-2008V-VI visual electrophysiology instrument of Chongqing Gotec Medical Equipment Limited Company for PERG and FERG.P50 and N95 amplitudes of PERG and PhNR,a wave and b wave amplitudes of FERG were recorded.The peripapillary retinal nerve fiber layer(pRNFL)thicknesses of the nasal,superior,temporal,inferior and average quadrants were measured by spectral domain optical coherence tomography(SD-OCT).The amplitudes of a wave,b wave,PhNR,P50 wave,N95 wave and pRNFL thickness between the three groups were compared by one-way ANOVA.Pearson correlation analysis was used to analyze the correlation between different parameters.Results Compared with the control group,the amplitudes of PhNR in LHON group and carrier group decreased significantly(F=11.973,P<0.001).The results of correlation analysis showed that the amplitude of PhNR in LHON group was significantly correlated with the thickness of nasal and temporal pRNFL(r=0.249,0.272;P=0.048,0.030).There was no significant difference in P50 wave amplitude between patients,carriers and c

关 键 词：LEBER遗传性视神经病变 视网膜电图 明视负波反应 视网膜神经纤维层厚度 

分 类 号：R774.6[医药卫生—眼科]