CAJ | 학술논문

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。
목적：분석신경백새병（NBD）적림상증상、영상학특정급뇌척액성분개변정황，탐토해병조기진단급예후평고적방법。방법회고성분석본원2006-2012년수치적15례 NBD 환자적림상증상、신경계통손해표현、영상학특점、뇌척액검사결과、기타실험실검사결과、치료급예후정황。결과15례 NBD 환자균유백새병적기본림상표현，출현신경계통증상적시간재학정백새병후8개월~12.5년，평균4.4년。기중단순중추신경계통손해12례，주위신경손해2례，량자동시합병자1례。로뇌 MRI 현시，병조부위분별위반구9례（60%），뇌간6례（40%），소뇌4례（27%），구뇌2례（13%），척수2례（13%）。7례뇌척액검사현시：항수초인자（AMSF）、백개소6（IL -6）수평균승고；5례과극륭구대（OB）양성；5례수초감성단백수평（0.58~16.45 nmol/ L）승고。기타실험실검사여홍세포침강솔（20~78 mm/ h）화 C 반응단백（CRP）수평（5.2~36.2 mg/ L）명현증고。15례환자급여발니송가배린선알치료후균호전출원；수방8례，기중5례치료과정중반복완해-복발，2례무복발，1례뇌간손해사망。결론NBD 림상표현무특이성，가발생재중추신경계통적임하부위，영상학개변유조우림상진단，뇌척액 AMSF、IL -6급혈청 CRP 수평검측유조우조기진단급감별진단。
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 ret-rospective 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 be-tween 2006 and 2012. Results All the 15 patients had basic clinical manifestation of NBD. The nervous system symptoms ap-peared 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 reso-nance 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 im-proved and discharged after prednisone and cyclophosphamide treatment. 8 cases received follow - up,5 cases of them showed re-current 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 di-agnosis and differential diagnosis of NBD.