11例迟发性视锥细胞营养不良患者的临床特征观察  

Clinical features of 11 patients with late-onset cone dystrophy

作　　者：王明扬[1] 王光璐[1] 

机构地区：[1]首都医科大学附属北京同仁医院北京同仁眼科中心北京市眼科学与视觉科学重点实验室,100730

出　　处：《中华眼底病杂志》2014年第3期274-277,共4页Chinese Journal of Ocular Fundus Diseases

摘　　要：目的 观察迟发性视锥细胞营养不良（LOCD）患者的临床特征.方法 临床检查确诊的LOCD患者11例15只眼纳入研究.其中,男性7例,女性4例.年龄50～79岁,平均年龄60.2岁.患者畏光和昼盲症状不明显.视力≤0.05者4只眼;0.06～0.2者5只眼;0.3～1.0者6只眼.所有患者行视力、裂隙灯显微镜、间接检眼镜、闪光视网膜电图（F-ERG）、多焦视网膜电图（mfERG）检查.行荧光素眼底血管造影（FFA）检查11只眼、光相干断层扫描（OCT）检查13只眼,色觉检查6只眼.结果 行色觉检查的6只眼中,红绿色盲4只眼;色弱2只眼.15只眼中,眼底检查正常11只眼;黄斑色素紊乱4只眼.行FFA检查的11只眼中,正常5只眼;黄斑色素紊乱表现为荧光遮蔽及透见荧光点相间4只眼;黄斑卵圆形萎缩灶2只眼.F-ERG检查结果显示,明视b波振幅下降6只眼.其中,轻度4只眼;中重度各1只眼.明视、暗视b波振幅下降9只眼.mfERG检查结果显示,距中心凹7°范围内的视锥细胞中心异常5只眼;中心、距中心凹7°范围以外的周边部分均异常10只眼.OCT检查结果显示,黄斑色素上皮改变3只眼.黄斑中心凹视网膜厚度正常8只眼;黄斑薄变5只眼.黄斑薄变的5只眼,黄斑厚度83～111μm.结论 LOCD患者早期眼底黄斑区正常,晚期呈卵圆形萎缩.可有色觉异常.F-ERG检查表现为早期明视b波振幅下降或晚期明视、暗视b波振幅均下降.mfERG检查表现为视锥细胞中心和（或）周边部异常.Objective To observe the clinical features of late-onset cone dystrophy （LOCD）.Methods Eleven patients （15 eyes） of LOCD were enrolled in this study.The patients included 7 males and 4 females.The age was ranged from 50 to 79 years,with a mean age of 60.2 years.There was no obvious photophobia and hemeralopia.The visual acuity was less than or equal to 0.05 in 4 eyes,0.06-0.2 in 5 eyes,0.3-1.0 in 6 eyes.Visual acuity,slit lamp microscope,indirect ophthalmoscopy,flash electroretinogram （FERG） and multifocal electroretinograms （mfERG） were examined for all patients,fundus fluorescein angiography （FFA） for 11 eyes,optical coherence tomography （OCT） and chromoptometry for 6 eyes.Results There were 6 eyes with red/green color blindness,2 eyes with color weakness.Normal fundus was found in 11 eyes,while derangement of macular pigment epithelial in 4 eyes.FFA results showed that there were 5 eyes with normal fundus,4 eyes with blocked fluorescent spots,2 eyes with oval macular atrophy.FERG results showed that in cone response,the amplitude was lower in 6 eyes （including mild decrease in 4 eyes,moderate decrease in 1 eye and severe decrease in 1 eye） ; both in cone and rod response,the amplitude were lower in 9 eyes.mfERG results showed that central part of the cone （less than 7 degree from the center） was damaged in 5 eyes,both central and peripheral part （outside of 7 degree） of the cone were damaged in 10 eyes.OCT results showed that pigment derangement in 3 eyes,fovea was normal in 8 eyes,thinned in 5 eyes （foveal thickness was 83-111 μm）.Conclusions The fundus manifestations of LOCD patients are variable,from normal fundus to oval macular atrophy.FERG is abnormal,which mainly in cone response at early stage and both in cone and rod response at late stage.Central part and （or） peripheral part of the cone are abnormal by mfERG.

关 键 词：视网膜营养不良 视网膜电描记术 色觉缺陷 

分 类 号：R774[医药卫生—眼科]