原发性视网膜色素变性临床分级方法初探

A study of clinical classification in primary retinitis pigmentosa

目的探讨原发性视网膜色素变性的临床分级方法,以据此判断该病病情的临床严重性。方法将患者按自觉症状分成I、Ⅱ、Ⅲ、Ⅳ4个等级,选择各个等级资料(眼底照相、视网膜电流图、视野)齐全的患者159人,分析他们在年龄、视力、色觉异常、视网膜色素沉着、视网膜血管变细和视盘颜色变淡6个因素上的差异,找出与患者自觉症状最相关的指标,以此作为衡量病变严重程度的指标。结果视网膜色素沉着、视网膜血管变细和视盘颜色变淡三者的出现与患者白天活动障碍的出现比较同步,而三者中又以视网膜色素沉着最具客观性和直观性。结论在原发性视网膜色素变性患者,一旦出现眼底色素沉着,从病理上和临床上都说明视网膜中视杆细胞和视锥细胞的结构和功能已发生严重破坏,病情不可逆转。建议将眼底色素沉着作为衡量原发性视网膜色素变性病情严重性的主要指标。

Objective To investigate a clinical classification method in primary retinitis pigmentos in case to estimate the clinical severity of the disease. Methods 159 continuous primary retinitis pigmentos patients with full clinical materials including fundus photograph, electroretinogram and visual field were divided into four groups as group Ⅰ （ slight moving obstacle in dark）, group Ⅱ （severe moving obstacle in dark）, group Ⅲ（slight moving obstacle in bright） and group Ⅳ （ severe moving obstacle in bright ） according to their symptoms. Six factors including ages, visual acuities, prevalence rates in color vision abnormality, retinal perivascular black bone-spicules, arterial narrowing and waxy pallor of the optic nerve head were analysed to find factor（s） most correlative to the severity of the disease. Results The appearance of retinal perivascular black bone-spicules, arterial narrowing and waxy pallor of the optic nerve head is most correlative to the patients ＇ moving obstacle in bright. Among the three, bone-spicules is most in objectivity. Conclusion In primary retini- tis pigmentos, the appearance of retinal perivascular black bone-spicules means the structure and function of photoreceptor are damaged beyond retrieve pathologically and clinically. It is suggested to take the appearance of retinal perivascular black bone-spicules as the main factor in the estimation of the clinical severity in primary retinitis pigmentosa.