副肿瘤性视网膜视神经病变临床特征初步分析

Clinical characteristics of paraneoplastic retinopathy and optic neuropathy

目的探讨副肿瘤性视网膜视神经病变患者的临床特征及其临床意义。方法系列病例研究。2006年10月至2012年3月解放军总医院眼科连续诊治副肿瘤性视网膜视神经病变患者8例（16只眼），行眼部照相、视觉电生理、荧光素眼底血管造影、相干光断层扫描、眼底自发荧光、视野、超声、MRI检查及腰穿脑脊液化验、副肿瘤综合征抗体检测等，并经门诊和（或）电话随访患者的病情变化和结局，分析其病变类型、临床表现及治疗结果等。结果8例患者中，癌症相关性视网膜病变3例、双眼弥漫性葡萄膜黑色素细胞增生2例，副肿瘤性视神经病变3例。5例患者进行了副肿瘤综合征的抗体检测，3例结果阳性，2例结果阴性。8例患者中，肿瘤源自肺癌4例，源自侵袭性胸腺瘤、肾癌、急性淋巴细胞白血病、宫颈癌各1例。患者均主诉视物模糊或视力进行性下降，并有2例主诉视物遮挡，其他主诉包括眼前闪光感、炫目、复视、眼痛各1例。其中1例患者是由于进行性视力下降首诊于眼科后发现患有恶性肿瘤。在8例16只眼中，随访结束时无光感的6只眼，光感至0．05的5只眼，视力≥0．4的5只眼。5例患者接受糖皮质激素治疗，总体疗效不明显。结论各型副肿瘤性视网膜视神经病变有其相应的临床表现，副肿瘤综合征尤其是双眼弥漫性葡萄膜黑色素细胞增生可能是一种转移前病变。

Objective To analyze the clinical characteristics of paraneoplastic retinopathy and optic neuropathy（ PRON ）. Methods Case series study. Eight patients were enrolled from October 2006 to March 2012 visited in ophthalmology department, the People Liberation Army General Hospital. The patients were underwent a series of examinations, including fundus photography, visual electrophysiology, fundus fluorescein angiography, optic coherent tomography, fundus autoflnorescent imaging, perimetry, ultrasonography, magnetic resonance imaging, spinal tap and cerebrospinal fluid test, paraneoplastic syndrome （PNS） antibody test. The patients were followed up in outpatient department and （or） by phone. The clinical manifestation,entity types, and treatment were analyzed. Results Of the eight patients, there were cancer associated retinopathy （CAR） 3 cases, bilateral diffuse uveal melanocytic proliferation （BDUMP） 2 cases and paraneoplastie optic neuropathy（PON） 3 cases. Five patients were detected the PNS antibodies and revealed three patients with positive results. As for the primary malignancy, four of the eight patients were lung carcinoma, others included invasive thymoma, kidney cancer, acute lymphocytic leukemia and cervical cancer, each for one case. All the patients complained vision blurring or progressive visual decrease. Other complaints included dark shadow in two patients, shimmering, dazzling, double vision and eye pain, each in one patient. One patient complained progressive decreased vision in both eyes prior to the diagnosis of lung cancer. Of the 16 eyes of the eight patients, there were six patients with no light perception vision, five from light perception to 0.05, and other five with no less than 0.4 vision, in the end of the follow up.Five patients were treated with steroid with unsatisfactory efficacy. Conclusions Each entity of PRON has its own clinical characteristics. PRON especially BDUMP may be a pre-metastatic disease.