摘要：

目的: 总结伴脑膜炎和（或）脑炎的Vogt-小柳-原田综合征的临床特征。方法与结果: 回顾分析首都医科大学附属北京同仁医院2009年1月至2022年12月诊断与治疗的23例Vogt-小柳-原田综合征患者的临床特点，20例（86.96%）并发神经系统症状，以头痛为主（19例，82.61%）；11例（47.83%）脑脊液压力增高，14例（60.87%）白细胞计数增加，11例（47.83%）蛋白定量升高；头部MRI以白质脱髓鞘改变为主（8/14例），视神经MRI表现为视神经和视乳头炎症性病变（12/14例）；视觉诱发电位主要呈现潜伏期延长（10/16例），光学相干断层扫描主要表现为视网膜渗出（8/15例）和视网膜神经上皮层脱离（7/15例）；眼底照相以视盘水肿为主（17/18例），荧光素眼底血管造影主要表现为葡萄膜炎（9/15例）。经激素冲击和静脉注射免疫球蛋白治疗后，14例接受随访，神经系统症状完全缓解，11例视力预后良好，3例视力改善不明显。结论: Vogt-小柳-原田综合征可伴发脑膜炎和（或）脑炎症状，其脑脊液特点与病毒性脑膜炎相似，对于早期疑似Vogt-小柳-原田综合征的患者，可完善腰椎穿刺脑脊液检查和影像学检查等辅助早期诊断与治疗。

关键词: 葡萄膜脑膜脑炎综合征, 脑膜炎, 脑炎, 脑脊髓液, 磁共振成像

Abstract:

Objective: To summarize the clinical characteristics of Vogt-Koyanagi-Harada syndrome (VKHS) combing with meningitis/encephalitis patients. Methods and Results: The clinical data of the inpatients of Beijing Tongren Hospital, Capital Medical University from January 2009 to December 2022 with VKHS combing with meningitis/encephalitis were retrospectively analyzed. A total of 23 patients were included in the study, of which 20 patients (86.96%) had neurological symptoms, with headache being the most common (19 cases, 82.61%), 11 patients (47.83%) had increased cerebrospinal fluid (CBF) pressure, 14 patients (60.87%) had increased CBF white blood cells, and 11 patients (47.83%) had increased CBF protein. The brain MRI mainly showed white matter demyelination (8/14 cases). Optic nerve MRI showed inflammatory lesions of optic nerve and optic papilla (12/14 cases). The visual evoked potential (VEP) mainly showed prolonged latency (10/16 cases). The optical coherence tomography (OCT) showed retinal exudation (8/15 cases) and retinal neurocortical detachment (7/15 cases). The main results of fundus photography were disc edema (17/18 cases). The main manifestation of fundus fluorescein angiography (FFA) was uveitis (9/15 cases). After intravenous hormone and intravenous immunoglobulin (IVIg) treatment, 14 patients were followed up, and neurological symptoms were completely relieved, 11 patients had good visual prognosis, and 3 patients had no obvious visual improvement. Conclusions: VKHS might present with meningitis/encephalitis symptoms, and the CSF characteristics of these patients were similar to those of viral meningitis. For those with early suspicions of VKHS, CBF examination and imaging examination might assist in early diagnosis and treatment.

Key words: Uveomeningoencephalitic syndrome, Meningitis, Encephalitis, Cerebrospinal fluid, Magnetic resonance imaging