Vogt-Koyanagi-Harada (VKH) syndrome is an immune-mediated granulomatous disease which affects melanin-rich organs like eyes, skin, nervous system, and ears. Neurological and auditory manifestations usually precede the involvement of other sites. Patients may manifest with “complete” or “incomplete” syndrome. We report two patients who presented with acute headache and impaired vision. Fundus examination revealed optic disc hyperemia and exudative retinal detachment which provided a clue for the diagnosis at the bedside. Fundus fluorescein angiogram (FFA) revealed abnormal dye leakage, whereas B scan showed choroid thickening. Cerebrospinal fluid (CSF) pleocytosis contrasted with unremarkable brain magnetic resonance imaging and lack of meningeal signs. Melanophagocytosis was evidenced by melanin-laden macrophages in CSF and skin biopsy. This finding is specific for VKH syndrome and helps to clinch the diagnosis even when the complete syndrome is not present cross-sectionally. VKH syndrome should be suspected in patients with aseptic meningitis if tests for common infectious and immune-mediated diseases are negative.


中文翻译：

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Vogt-koyanagi-harada 综合征 - 神经科医生的观点


Vogt-Koyanagi-Harada (VKH) 综合征是一种免疫介导的肉芽肿性疾病，会影响眼睛、皮肤、神经系统和耳朵等富含黑色素的器官。神经和听觉表现通常先于其他部位的受累。患者可能表现为“完全”或“不完全”综合征。我们报告了两名出现急性头痛和视力受损的患者。眼底检查发现视盘充血和渗出性视网膜脱离，为床边诊断提供了线索。眼底荧光素血管造影 (FFA) 显示异常染料渗漏，而 B 扫描显示脉络膜增厚。脑脊液 (CSF) 细胞增多与不显着的脑磁共振成像和缺乏脑膜体征形成对比。脑脊液和皮肤活检中载有黑色素的巨噬细胞证实了黑色素吞噬作用。这一发现是 VKH 综合征特有的，即使在横断面不存在完整综合征的情况下，也有助于确定诊断。如果对常见传染性和免疫介导疾病的检测结果为阴性，则对于无菌性脑膜炎患者，应怀疑 VKH 综合征。