中文摘要：

目的分析神经白塞病（NBD）的临床症状、影像学特征及脑脊液成分改变情况,探讨该病早期诊断及预后评估的方法。方法回顾性分析本院2006—2012年收治的15例NBD患者的临床症状、神经系统损害表现、影像学特点、脑脊液检查结果、其他实验室检查结果、治疗及预后情况。结果 15例NBD患者均有白塞病的基本临床表现,出现神经系统症状的时间在确定白塞病后8个月-12.5年,平均4.4年。其中单纯中枢神经系统损害12例,周围神经损害2例,两者同时合并者1例。颅脑MRI显示,病灶部位分别为半球9例（60%）,脑干6例（40%）,小脑4例（27%）,丘脑2例（13%）,脊髓2例（13%）。7例脑脊液检查显示：抗髓鞘因子（AMSF）、白介素6（IL-6）水平均升高;5例寡克隆区带（OB）阳性;5例髓鞘碱性蛋白水平（0.58-16.45 nmol/L）升高。其他实验室检查如红细胞沉降率（20-78 mm/h）和C反应蛋白（CRP）水平（5.2~36.2 mg/L）明显增高。15例患者给予泼尼松加环磷酰胺治疗后均好转出院;随访8例,其中5例治疗过程中反复缓解-复发,2例无复发,1例脑干损害死亡。结论 NBD临床表现无特异性,可发生在中枢神经系统的任何部位,影像学改变有助于临床诊断,脑脊液AMSF、IL-6及血清CRP水平检测有助于早期诊断及鉴别诊断。

英文摘要：

Objective To explore the methods of early diagnosis and prognosis of Neuro- Behcet＇s disease（NBD） through analyzing clinical manifestation,imaging characteristics and change of cerebrospinal fluid composition.Methods A retrospective survey on clinical manifestation,the lesions in nervous system,imaging characteristics,results of cerebrospinal fluid and other laboratory examination,therapy and prognosis was performed in 15 NBD patients who were treated in our hospital between 2006 and 2012.Results All the 15 patients had basic clinical manifestation of NBD.The nervous system symptoms appeared in the eighth month to 12.5 years（mean time = 4.4 years） after the diagnosis of NBD.Among the 15 cases,12 cases had pure central nervous system damage,2 cases had peripheral nervous system lesion,1 case had both of them.The magnetic resonance image showed the lesion existed in cerebral hemisphere（9 cases,60%）,brainstem（6 cases,40%）,cerebellum（4 cases,27%）,thalamus（2 cases,13%） and spinal cord（2 cases,13%）.The results of cerebrospinal fluid of 7 cases showed that level of anti myelin sheath factor（AMSF） and interleukin- 6（IL- 6） increased,5 cases showed OB positive,5 cases showed elevated MBP level（0.58- 16.45 nmol / L）.Other laboratory examination results：erythrocyte sedimentation rate（20-78 mm/h） and serum C reactive protein（CRP） level（5.2- 36.2 mg/L） increased significantly.All 15 patients improved and discharged after prednisone and cyclophosphamide treatment.8 cases received follow- up,5 cases of them showed recurrent trends during treatment,no recurrence happened in 2 cases,and 1 case died from brain stem lesion.Conclusion The clinical manifestation of NBD showed no specificness,any site of central nervous system can be damaged by NBD.The alteration in imageology contribute to clinical diagnosis.Test for SMSF and IL- 6 in CSF and CRP level in serum contribute to the early diagnosis and differential diagnosis of NBD.