目的：总结并分析视野为中心暗点的视神经病变的病因和临床特点，为临床诊治提供参考。方法：回顾性病例研究。分析2018年8月至2020年3月期间，在中山大学中山眼科中心神经眼科专科门诊就诊，视野表现为中心暗点且随访1年以上的视神经病变患者的资料。患者双眼均行最佳矫正视力、眼压、裂隙灯显微镜及前置镜、频域光学相干断层扫描、视野、颅脑和眼眶核磁共振检查，静脉采血行血常规、血生化、肝肾功能、感染指标(乙肝、丙肝、梅毒、HIV及结核T-spot)检查及Leber遗传性视神经病变的线粒体DNA和OPA1基因检测。结果：共纳入20例患者，病因诊断构成为：Leber遗传性视神经病变9例(45%)，显性视神经萎缩2例(10%)，乙胺丁醇中毒性视神经病变6例(30%)，营养性视神经病变2例(10%)和特发性脱髓鞘性视神经病变1例(5%)。遗传性视神经病变的视力预后差，特别是Leber遗传性视神经病变，78%的随访视力(≥1年)不高于0.1。伴有mtDNA或OPA1基因突变的乙胺丁醇中毒性视神经病变患者，视力预后差。结论：视野为中心暗点表现的视神经病变，主要为遗传、中毒和营养性视神经病变。遗传性视神经病变具有不完全外显率的特点，视野为中心暗点的视神经病变需行基因检测排除遗传性视神经病变。

Objective: To summarize and analyze the etiology and clinical features of optic neuropathy with visual field defect of central scotoma as a reference for clinical diagnosis and treatment. Methods: In the retrospective case study, the data of patients admitted in Neuro-ophthalmic Department of Zhongshan Ophthalmic Center of Sun Yat-sen University from August 2018 to March 2020, who presented with visual field defect of central scotoma and were followed up for more than 1 year, were analyzed. Both eyes of all the patients underwent best corrected visual acuity, intraocular pressure, slit lamp microscope and front mirror, spectral domain optical coherence tomography, humphry visual field tests and MRI of brain and orbit. We examined the blood routine, biochemical test, renal and liver function, infection indicators (hepatitis B, hepatitis C, syphilis, HIV and tuberculosis T-spot), mitochondrial DNA and OPA1 gene detection of Leber hereditary optic neuropathy. The follow-up time of the patients in neuro-ophthalmic department was more than 1 year. Results: A total of 20 patients were recruited. Among them, the etiological diagnosis consisted of 9 patients of Leber hereditary optic neuropathy (45%), 2 of dominant optic atrophy (10%), 6 of ethambutol-induced optic neuropathy (30%), 2 of nutritional optic neuropathy (10%) and 1 of idiopathic demyelinating optic neuropathy (5%). The patients with hereditary optic neuropathy showed a poorer visual prognosis, especially Leber hereditary optic neuropathy, with 78% of follow-up visual acuity (≥1 year) not higher than 0.1. The visual prognosis of ethambutol-induced optic neuropathy patients with mtDNA or OPA1 gene was poor. Conclusions: The optic neuropathy of visual field defects with central scotoma includes mainly hereditary, toxic and nutritional optic neuropathy. Hereditary optic neuropathy is characterized by incomplete penetrance, and genetic testing is required to exclude hereditary optic neuropathy if the visual field is the central scotoma.

青光眼睫状体炎综合征(Posner-Schlossman syndrome，PSS)表现为单眼反复发作性非肉芽肿性前葡萄膜炎，伴有眼压升高，可发展为慢性继发性青光眼，最终导致视神经损伤。尽管PSS总体预后良好，但仍有部分患者因反复眼压骤升造成视神经损伤持续进展，甚至导致失明。目前，PSS的确切病因尚不明确，治疗方式以控制炎症及眼压为主。本文将从病因及治疗两方面阐述PSS的研究现状，以期为PSS相关基础研究及临床诊治提供思路及参考。

Posner-Schlossman syndrome (PSS), also called glaucomatocyclitic crisis, is characterized by recurrent non-granulomatous anterior uveitis, accompanied by elevated intraocular pressure. It is able to develop into chronic secondary glaucoma and eventually lead to optic nerve injury. Although the overall prognosis of PSS is favourable, there are still some patients whose optic nerve injury continues to progress and even lead to blindness due to recurrent attacks of ocular hypertension. At present, the exact cause of PSS is not clear, and the treatment is mainly to control inflammation and intraocular pressure. This article will elaborate the research status of PSS from two aspects of etiology and treatment to provide ideas and reference for the basic research clinical diagnosis and treatment of PSS.