Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis
Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis
复制标题
DOI：
10.1007/s10384-020-00780-x
复制
发表时间：
2020-11-12
影响因子：
2.4
通讯作者：
Miyake, Yozo
中科院分区：
文献类型：
--
作者：
Hirakata, Toshiaki;Fujinami, Kaoru;Miyake, Yozo
目的用负性视网膜电图（ERG）对8例单侧严重视神经恐惧症患者进行临床鉴别，这些患者眼底基本正常，视力良好，但视锥、视杆细胞功能严重受损。研究设计多中心回顾性观察病例系列。方法对患者进行全面的眼科检查，包括最佳矫正视力（BCVA）、全视野视网膜电图（ffERG）、多焦视网膜电图（mfERG）、眼底照相、OCT等，并进行系统和遗传学检查。结果6例患者的平均发病年龄（± SD）为60.0 ± 8.4岁，尽管眼底外观基本正常，最佳矫正视力良好，但患眼存在严重的视神经恐惧症。患眼的暗适应亮闪光ERG具有相对良好的a波和抑制的b波，即，负ERG。视锥ERG和明视长时程ERG的B波和d波几乎检测不到。杆ERG严重降低;然而，只有两名患者主诉夜盲症。在5名患者中，mfERG在周边消失，但在中央视网膜保留，导致良好的BCVA。电生理结果表明，严重的弥漫性功能障碍的内层视网膜影响双极细胞的ON-和OFF-途径，并在5例患者有一个减少内核层的厚度。7例患者视网膜动脉变细。两名患者的血清中检测到抗视网膜抗体。结论8例单侧视网膜电图阴性患者的共同特征提示单侧急性视网膜内层功能障碍是一种新的疾病。在大多数患者中，唯一的主观抱怨是恐惧症。
Purpose To establish with negative electroretinogram (ERG) the clinical entity of eight patients with unilateral severe photophobia, essentially normal fundus, good visual acuity, and severe cone and rod dysfunction. Study design Multicenter retrospective observation case series. Methods Comprehensive ophthalmologic examinations were performed, including best-corrected visual acuity (BCVA), full-field ERGs and multifocal ERGs (mfERGs), fundus photographs, and OCT. Systemic and genetic examinations were performed. Results The mean (+/- SD) age at the onset was 60.0 +/- 8.4 years, and the six patients noticed severe photophobia in the affected eye in spite of almost normal fundus appearance and good BCVA. The dark-adapted bright flash ERGs in the affected eye had relatively well-preserved a-waves and depressed b-waves, i.e., a negative ERG. Cone ERGs and both b- and d-waves of the photopic long-duration ERGs were almost undetectable. Rod ERGs were severely reduced; however, only two patients complained of night blindness. In five patients, the mfERGs were extinguished in the periphery but preserved in the central retina, resulting in good BCVA. Electrophysiological findings indicated a severe diffuse dysfunction of the inner retina affecting bipolar cells of both ON- and OFF-pathways, and in five patients there was a reduction in the thickness of the inner nuclear layer. In seven patients the retinal arteries were attenuated. Anti-retinal antibodies were detected in the serum of two patients. No genetic causes were found. Conclusions The common features in the eight patients with unilateral negative ERGs suggest a new disease entity of unilateral acute inner retinal layer dysfunction. In most patients, the only subjective complain was photophobia.