Vogt-小柳-原田综合征五例临床及神经影像学特点

目的 探讨Vogt-小柳-原田综合征的临床及神经影像学特点。方法 回顾我院收治的5例Vogt-小柳-原田综合征患者，总结其临床、脑脊液细胞学、神经影像学特点及药物治疗的效果。结果 5例患者均在发病早期确诊葡萄膜炎，并出现头痛症状，3例出现脑膜刺激征，所有患者颅脑MRI强化扫描均显示软脑膜较均匀强化。脑脊液白细胞计数均增高[(4 - 196)×106/L]。5例患者经大剂量激素联合环磷酰胺治疗均得到有效控制。结论 假性脑膜炎是Vogt -小柳-原田综合征最常见的中枢神经系统表现，脑脊液细胞学检查主要表现为白细胞计数增高，颅脑MRI平扫及强化对该病的诊断具有一定价值。大剂量激素联合环磷酰胺治疗疗效确切。

Vogt-Koyanagi-Harada syndrome: 5 cases report with clinical and neuroimaging features

Objective To investigate the clinical and neuroimaging features of Vogt-KoyanagiHarada syndrome ( VKH ). Methods Cerebrospinal fluid ( CSF ), neuroimaging examination, clinical manifestation and pharmacotherapy features were investigated in 5 patients diagnosed as VKH. Results All 5 patients were diagnosed as uveitis in the early stage of disease. All patients suffered “ headache”.Meningeal irritation sign was appeared in 3 cases. The MRI enhanced scan of all 5 cases showed abnormal enhancement of meninges. CSF examination showed increased leukocyte number ((4--196) × 106/L). All patients were alleviated with combination therapy of high dose of steroid with cyclophosphamide.Conclusions VKH is a systemic disease that usually involving the uvea, central nervous system, internal ear and the skin. MRI and CSF examination are valuable for diagnosis. High dose of steroid combined with cyclophosphamide is an effective therapeutic strategy.