目的：分析单中心神经眼科疾病谱及流行病学特点，为指导神经眼科疾病诊断和治疗提供基础。方法：纳入2010年1月1日—2021年12月31日中国人民解放军总医院神经眼科病区收治的神经眼科疾病患者，从电子病例系统检索和记录所有纳入病例的年龄、性别、地区分布及病种亚型分析。结果：共计7245例神经眼科患者纳入统计，其中男性3331例(46.0%)、女性3914例(54.0%)，男女比例1∶1.2；年龄(38.2±17.5)岁。83.25%(6031/7245)为传入神经系统疾病，9.92%(719/7245)为传出神经系统疾病和眼眶疾病，6.83%(495/7245)未归类。病种分析显示，占比最高的是脱髓鞘性视神经炎(demyelinating optic neuritis,DON)，为40.17%(2910/7245)；占比第二的是非动脉炎性前部缺血性视神经病变(nonarteritic anterior ischemic optic neuropathy,NAION)，为11.37%(824/7245)；占比第三的是外伤性视神经病变5.15%(373/7245)，其中7.85%(569/7245)表现为不明原因视神经萎缩。从年龄分布来看，DON和外伤性视神经病变患者中18~40岁者占比最高(分别为48.63%和44.24%)，NAION患者中41~60岁者占比最高(66.14%)，小于18岁的未成年患者在遗传性视神经病变中占比最高，比例为48.58%。在2226例DON患者中，视神经脊髓炎(neuromyelitis optica,NMO)/视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)比例最高，为60.02%；髓鞘少突胶质细胞糖蛋白抗体(myelinoligodendrocyte glycoprotein antibody,MOG-IgG)阳性视神经炎比例为11.68%；多发性硬化(multiple sclerosis,MS)和MS相关性视神经炎和慢性复发性炎性视神经病变(chronic recurrent inflammatory optic neuropathy,CRION)占比较低，分别是1.8%和2.25%。DON整体患者中，男女比例为1∶3.08；在NMO/NMOSD患者中男女比例为1∶8；MOG阳性视神经炎患者中，男女比例为4∶5；在非典型视神经炎患者中，男性比例高于女性，为1.28∶1；DON患者中，81.79%患者为中青年，MOG阳性视神经炎未成年患者可达41.15%。结论：DON和NAION是神经眼科传入系统疾病最常见两大病种。
Objective: To analyze the spectrum and epidemiological characteristics of neuro-ophthalmic diseases from single center, and to provide basis for guiding the diagnosis and treatment of neuro-ophthalmic diseases. Methods: Patients with neuro-ophthalmic diseases admitted to the neuro-ophthalmology ward of Chinese PLA General Hospital from January 1, 2010 to December 31, 2021 were enrolled. The age, gender, regional distribution and disease subtypes of all included patients were retrieved and recorded from the electronic case system. Results: A total of 7245 patients with neuro-ophthalmic diseases were enrolled, including 3331 males(46.0%)and 3914 females(54.0%), with a male to female ratio of 1:1.2. The average age was 38.2±17.5 years. 83.25%(6031/7245)were afferent nervous system diseases, 9.92% (719/7245)were efferent nervous system diseases and orbital diseases, and 6.83%(495/7245)were not classified. The ratio of demyelinating optic neuritis(DON)was the highest(40.17%,2910/7245), followed by nonarteritic anterior ischemic optic neuropathy(NAION)(11.37%,824/7245)and traumatic optic neuropathy(TON) (5.15%,373/7245). The ratio of optic nerve atrophy with unknown causes was 7.85%(569/7245). Characteristics of age distribution, the DON and TON were more common in 18-40 age group(the proportion were 48.63% and 44.24%,respectively), the NAION was common in 41-60 age group(66.14%), and the hereditary optic neuropathy was common in younger 18 age group (48.58%). In 2226 DON patients, the proportion of neuromyelitis optica(NMO)/neuromyelitis optica spectrum disorder(NMOSD)-optic neuritis(ON)was the highest(60.02%)and myelinoligodendrocyte glycoprotein antibody(MOG-IgG)ON was 11.68%, while multiple sclerosis(MS)-ON and chronic recurrent inflammatory optic neuropathy(CRION)were relatively low(1.8% and 2.25%,respectively). In DON patients, the male to female ratio was 1:3.08. In NMO/NMOSD-ON patients, the ratio of male to female was 1:8, and that of MOG-ON was 4:5. In atypical ON, the ratio of male to female was higher than that of female(1.28:1). In DON patients, 81.79% of patients were young and middle-aged, and the proportion of children with MOG-ON(less than 18 years old)was 41.15%.Conclusions: DON and NAION are the two most common diseases of neuro-ophthalmic afferent system.
后视路病变是视交叉以后的视觉通路其本身或毗邻结构发生病变，引起视觉功能改变的一类疾病。神经眼科医生比较熟悉枕叶病变引起的对称性同侧偏盲，但枕极(纹状皮质的最后部分)的病变产生中心性对称性同向盲点，此类视野改变容易被忽略或误诊。该文报道一例老年男性患者，因双眼视觉清晰度下降、视物变形就诊。眼科检查：最佳矫正视力：右眼0.8，左眼1.0，FM-100检查提示重度色觉异常，颅脑磁共振成像(magnetic resonance imaging,MRI)提示双侧枕叶脑梗死(右侧枕极部，左侧纹状皮质前部)，24-2 Humphrey视野检查可见双眼同向暗点趋势(不典型)，10-2 Humphrey视野检查可见双眼中心视野同向偏盲(暗点)，故而确诊。后视路病变可引起多种特征性的视野改变，可伴有高级视功能异常及其他神经系统症状和体征，是神经眼科的重要组成部分。该例枕极脑梗死病变产生对称性同向性盲点伴色觉改变患者的诊治过程，提示需关注后视路病变视野改变的多样性及其他视觉功能异常，提高早期诊断率，改善患者预后。
The disease of the posterior visual pathway is a kind of lesion in which the visual pathway itselfor its adjacent structure changes after optic chiasma causes pathological changes, resulting in changes in visual function. Neuro-ophthalmologists are familiar with symmetrical ipsilateral hemianopia caused by occipital lobe lesions, but occipital tip (the last part of the striatal cortex) lesions produce central symmetrical homonymous scotomas, which can easily be overlooked or misdiagnosed. This article reported a case of an olderly male patient treated with decreased binocular visual clarity and distortion. Ophthalmology examination: best corrected visual acuity: 0.8 in the right eye, 1.0 in the left eye; FM-100 examination indicated severe dyschromatopsia; cranial magnetic resonance imaging: infarction of bilateral occipital lobe (right portion of the occipital tip and left anterior portion of striate cortex); 24-2 Humphrey field examination showed a tendency of homonymous scotoma in bilateral eyes (atypical); 10-2 Humphrey field examination showed homonymous hemianopia (scotoma) in the central visual field. These results confirm a diagnosis of the disease of the posterior visual pathway. As an important part of neuro-ophthalmology, the posterior visual pathway can cause various characteristic visual field defects, which can be accompanied by advanced visual dysfunction and other neurological symptoms and signs. The diagnosis and treatment process of this case of occipital tip cerebral infarction with symmetrical homonymous blind spot accompanied by color vision changes suggests that attention should be paid to the diversity of visual field changes and other visual functional abnormalities in the posterior visual pathway lesions, so as to improve the early diagnosis rate and prognosis of the patient s.
