文摘
摘要目的:新标准的诊断慢性淋巴细胞炎症与桥的血管周的增强对类固醇(快船)。
背景:快船被定义为1)亚急性脑干体征和症状;2)点状的,曲线增强病变主要涉及脑桥;3)类固醇敏感;4)缺乏替代诊断,和5)血管周的lympho-histiocytic浸润在脑活检。自2010年首次描述,原发性中枢神经系统血管炎(PCNSV),中枢神经系统原发性中枢神经系统淋巴瘤(PCNSL)和lymphomatoid肉芽肿病(CNSLYG)能完成的,至少在最初阶段,所有的五个快船标准。
设计/方法:基于我们报道法国快船队列,我们提出将六分之一标准添加到快船定义:6)复发缓和课程缺乏immunosupprevive疗法,与复发(s)满足上述标准或一分之四标准缺乏脑活检;和评估的能力,这个新的标准来区分从CLIPPERS-mimickers快船。快船怀疑患者分为5类:PPERS一分之四标准)(一个攻击,柏与一分之五标准)(一个攻击,CPPERS(复发(s)一分之四标准),快船队(复发(s)一分之五标准),和PPERS-plus非典型结果有关的一分之四标准之一出席第一次攻击或复发。
结果:在42名患者中加入6在第一个攻击,被分配到PPERS-plus 6 PPERS, 4成理柏,14到CPPERS, 12到快船。两个6 PPERS患者和3 4柏患者提出第二个攻击非典型结果和加入PPERS-plus类别。所有PPERS-plus患者比快船有另一个诊断:3 PCNSV 4中枢神经系统来讲,2 PCNSL, 2例伴有全身性淋巴瘤。相反,没有一个26患者分为CPPERS和快船PPERS-plus袭击,显示和快船保持最后的诊断。
结论:这个新标准似乎区分从CLIPPERS-mimickers快船。
研究支持:没有
披露:Taieb博士没有披露。Allou博士没有披露。Kaphan博士没有披露。Audoin博士没有披露。博士Pelletier没有披露。Ouallet博士没有披露。Desbordes博士没有披露。Brassat博士已经收到个人活动与生原体补偿,赛诺菲安万特Genzyme, Teva,诺华,默克公司差旅费用和个人费用教育计划和董事会的参与。Castelnovo博士没有披露。Thouvenot博士已经收到个人补偿活动Teva,生原体,和诺华科学顾问委员会成员或扬声器。 Dr. Rigau has nothing to disclose. Dr. Arquizan has nothing to disclose. Dr. ayrignac has nothing to disclose. Dr. Limousin has nothing to disclose. Dr. Loussouarn has nothing to disclose. Dr. Magy has nothing to disclose. Dr. Rousselot has nothing to disclose. Dr. Lefaucheur has nothing to disclose. Dr. De Seze has received personal compensation for activities with Merck, Biogen, Novartis, Roche, Teva, LFB, Alexion, and Sanofi Genzyme. Dr. De Seze has received research support from LFB and Novartis. Dr. Tranchant has nothing to disclose. Dr. Lebrun-Freney has nothing to disclose. Dr. Bejot has nothing to disclose. Dr. Guiraud has nothing to disclose. Dr. Camdessanche has nothing to disclose. Dr. Pico has nothing to disclose. Dr. van Oosten has nothing to disclose. Dr. Kerschen has nothing to disclose. Dr. Fominykh has nothing to disclose. Dr. Mulero has nothing to disclose. Dr. Tintore-Subirana has received personal compensation for activities with Bayer-Schering, Merk-Serono, Biogen, Teva, Sanofi-Aventis, and Novartis as a consultant and speaker. Dr. Seebach has nothing to disclose. Dr. Psimaras has nothing to disclose. Dr. Hoang-Xuan has received personal compensation in an editorial capacity for ""Revue neurologique"" (offical journal of the French Society of Neurology)- Elsevier editor. Dr. Labauge has nothing to disclose.
