假性视网膜色素变性临床特征研究

Clinical characteristics of pseudoretinitis pigmentosa: analysis of 26 cases

目的探讨假性视网膜色素变性(pseudo retinitis pigmentosa,PRP)患者的临床特征。方法回顾性分析2014年12月至2018年10月就诊于陆军军医大学第一附属医院眼科的PRP患者26例,并选择年龄匹配的视网膜色素变性(retinitis pigmentosa,RP)患者30例作为对照,分析PRP病因、病程、最佳矫正视力(best corrected visual acuity,BCVA)、眼底特征和全视野闪光视网膜电图(full field flash electroretinogram,FERG)改变。结果 26例PRP患者年龄26~56岁,其中男性12例,女性14例,单眼发病22例22眼(84.6%),双眼发病4例8眼(15.4%),病程6个月至5年,最佳矫正视力无光感~1.2,无夜盲主诉。RP患者30例,男性13例,女性17例,平均病程27年。PRP原发病因:葡萄膜炎(40.0%),视网膜脱离(36.7%),眼外伤(16.7%),其他(6.6%)。病程、BCVA RP和PRP患者相比,差异有统计学意义(P<0.05)。26例PRP患者眼底主要表现为双眼不对称性,视网膜色泽和色素分布以及视盘颜色改变与RP有别。PRP患者FERG显示双眼各波形幅值同步降低、双眼不对称,患眼的眼底改变与FERG幅值降低程度不一致。结论 PRP病因多样,病程短、不伴夜盲,其眼底改变、FERG是PRP与RP鉴别诊断的主要指标。

Objective To analyze the clinical features of patients with pseudoretinitis pigmentosa(PRP). Methods We performed a retrospective case analysis among 26 patients with PRP admitted in our department between December, 2014 and October, 2018, with 30 age-matched patients with RP as the controls. The etiology, disease course, best corrected visual acuity(BCVA), fundus and full-field flash electroretinogram(FERG) changes were compared between the patients with RP and those with PRP. Results Of the 26 patients with PRP(including 12 male and 14 female patients aged from 26 to 56 years), 22(84.6%) had unilateral PRP and 4(15.4%) had bilateral PRP, and the disease course ranged from 6 months to 5 years. The BCVA of these patients ranged from no light perception to 1.2, and none of the patients complained of night blindness. The 30 control patients with RP included 13 male and 17 female patients with a mean disease duration of 27 years. The primary causes of PRP included uveitis(40.0%), retinal detachment(36.7%), ocular trauma(16.7%), and other factors(6.6%). The patients with RP and PRP had significantly different disease durations and BCVA(P<0.05). The patients with PRP typically manifested a bilateral asymmetry of the fundus, with obvious differences in retinal color, pigment distribution and visual disc color change from patients with RP. FERG of the patients with PRP revealed synchronously decreased amplitudes of the waveforms in the bilateral eyes with obvious eye asymmetry, and the reduction of FERG amplitude was not consistent with the changes in the fundus of the affected eyes. Conclusion The causes of PRP are diverse, and the disease course is often short without night blindness. The changes in the fundus and FERG are the main indicators for differential diagnosis between PRP and RP.