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原发性中枢神经系统血管炎
引用本文:刘云,李子孝,张星虎.原发性中枢神经系统血管炎[J].大连大学学报,2007,28(3):72-74,80.
作者姓名:刘云  李子孝  张星虎
作者单位:首都医科大学,附属北京天坛医院,北京,100050
摘    要:目的]探讨原发性中枢神经系统血管炎(PACNS)的临床与影像学表现,提出诊断及鉴别诊断要点.方法]对2例疑诊PACNS的病例进行常规MR I、MRA扫描,分析常规MR I病变部位、信号特点、强化特征及MRA情况.结果]2例均有头痛症状,无语言障碍,表现为肢体轻度无力,体检左肢肌力5级减,轻度中枢性面瘫.MR I显示病灶位于单侧,主要位于白质内,可累及灰质,分布以额叶、顶叶为主,小脑、胼胝体、丘脑亦受累,T1呈略低信号,T2呈略高信号,增强后两例均有多发斑片状或脑回样强化.MRA 2例均正常.1例腰穿CSF-OB(+)、CSF-24 h IgG鞘内合成率增高,1例脑脊液髓鞘碱性蛋白增高,未见寡克隆区带,24 h IgG合成率正常.1例免疫全套:抗组蛋白抗体及抗核抗体筛检试验阳性,以上提示病变与自身免疫性相关,但不具有特异性.2例ANCA均正常.结论]PACNS临床和影像学表现多样,MR I、MRA、DSA对诊断有一定价值.

关 键 词:原发性中枢神经系统血管炎  诊断  影像学检查
文章编号:1008-2395(2007)03-0072-04
修稿时间:2007-03-27

Primary Angitis of Central Nervous System
LIU Yun,LI ZI-xiao,ZHANG Xing-hu.Primary Angitis of Central Nervous System[J].Journal of Dalian University,2007,28(3):72-74,80.
Authors:LIU Yun  LI ZI-xiao  ZHANG Xing-hu
Institution:Department of Neurology,BJ Tiantan Hospital ,the Capital Medical University,Beijing 100050, China
Abstract:Objective]To explore the clinical and imaging feature,diagnosis and differential diagnosis of primary angitis of central nervous system(PACNS).Methods]Two patients suspected as PACNS were performed magnetic resonance imaging(MRI) and angiography(MRA).We analyzed the characteristics of MRI and MRA.Results]Two patients both had headache and felt slight weakness of limbs.Physical examination showed the muscle power of left limbs was less than 5 degree and they had slight facial paralysis.MRI showed the disease focus was appeared as long T1 and long T2 signal mainly in white matter involved frontal and parietal lobes on one side and the cerebellum,corpus callosum and thalamus were also involved.After gadolinium enhanced the focus was reinforced as patching and gyrus-like.The MRA of two patients were both normal.The oligoclonal bands(OB) of cerebrospinal fluid(CSF) in one patient was positive and 24 h-IgG was higher than normal.While for the other patient,the myelin basic protein of CSF was in high level and the OB and 24 h-IgG were normal.The anti-histone antibody and antinuclear antibody(ANA) in one patient were positive and the anti-neutrophil cytoplasmic antibody(ANCA) of two patients was negative.Conclusion]There may be no specificity in clinical and imaging manifestation of PANCS,but MRI,MRA and digital subtraction angiography(DSA) will be helpful to diagnosis.
Keywords:primary angitis of central nervous system  diagnosis  image inspection
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