摘要：

目的 总结慢性进展型神经白塞综合征的临床特征。方法 回顾分析 2018 年 5 月 24 日收治的 1 例慢性进展型神经白塞综合征患者的诊断与治疗经过；并以 neuro Behçet's syndrome、neuro Behçet's disease、chronic progressive，以及白塞、神经白塞、慢性进展为中英文检索词， “AND”为检索策略，计算机检索 1980 年 1 月 1 日至 2018 年 12 月 1 日美国国立医学图书馆生物医学信息检索系统（PubMed）和中国知网中国知识基础设施工程（CNKI）、万方数据库中慢性进展型神经白塞综合征相关临床研究和病例报告，总结其社会人口学、临床表现、实验室和影像学特点，以及治疗原则及预后。结果 共检索 14 篇国外文献计 122 例患者，结合本文病例，共纳入 123 例患者。分析结果显示，慢性进展型神经白塞综合征好发于中年男性，高峰发病年龄 45 ~ 47 岁，呈慢性病程；表现为共济失调、假性延髓麻痹和认知功能障碍；脑脊液白细胞介素?6 水平升高为其特异性实验室指标，脑干萎缩是特征性影像学改变；治疗原则以糖皮质激素联合免疫抑制剂治疗为主，多预后不良。结论 慢性进展型神经白塞综合征是白塞综合征的少见亚型，易误诊或漏诊，临床表现不典型，脑脊液和 MRI 检查对明确诊断具有重要提示意义，病残率和病死率较高。

关键词: 贝赫切特综合征, 中枢神经系统, 弥散磁共振成像, 白细胞介素6

Abstract:

Objective To summarize the clinical manifestations of chronic progressive parenchymal neuro-Behçet's syndrome (CPPNBS). Methods The diagnosis and treatment of one patient with CPPNBS was reviewed and analyzed. Taking neuro Behçet's syndrome, neuro Behçet's disease AND chronic progressive (in English and Chinese) as search terms, retrieve in databases such as PubMed, China National Knowledge Infrastructure (CNKI) and Wanfang Data, in order to collect clinical studies and case reports of CPPNBS from January 1, 1980 to December 1, 2018, summarizing the sociodemographic, clinical, laboratory and imaging features, treatment and prognosis of CPPNBS. Results A total of 14 English articles with 122 cases were enrolled after searching. Combined with our case, there were 123 cases. CPPNBS was common in male, and the peak onset age ranged 45-47 years old, with a chronic disease course. The common clinical manifestations were ataxia, pseudobulbar palsy and cognitive impairment. Elevated level of interleukin-6 (IL-6) in cerebrospinal fluid (CSF) was the specific laboratory index. Brain stem atrophy in MRI was the specific imaging feature. Glucocorticoids and immunosuppressive agents were the main treatment option. The prognosis was poor. Conclusions CPPNBS is a rare subtype of Behçet's syndrome (BS), and could be easily missed or misdiagnosed. The clinical manifestations are not specific. CSF and MRI examinations are important for the diagnosis. The prognosis of this disease is poor. The rates of disability and mortality are high.

Key words: Behcet syndrome, Central nervous system, Diffusion magnetic resonance imaging, Interleukin-6