视神经脊髓炎与多发硬化的关系.doc

每月一主题-视神经脊髓炎与多发性硬化的关系每月一主题-视神经脊髓炎与多发性硬化的关系视神经脊髓炎(Neuromyelitis optica, NMO)又称Devics disease，是我国常见的脱髓鞘病，有些学者认为属多发性硬化的亚型，但目前越来越多学者认为属于独立的疾病。NMO 目前被认为是某些病因引起针对水通道蛋白AQP4产生特异性自身抗体NMO-IgG的不同于MS的离子通道性自身免疫病。其主要特点是合并有视神经与脊髓的脱髓鞘性变。二者同时或先后发病，急性或亚急性病程。实验研究结果提示，NMO-IgG可以调制AQP4的功能，对NMO存在潜在的致病性。分成几个课题来导论视神经脊髓炎与多发性硬化的临床表现异同视神经脊髓炎与多发性硬化的致病因异同视神经脊髓炎与多发性硬化的检查与诊断视神经脊髓炎与多发性硬化的治疗与结局异同请选择高质量、近期或大型的研究来讨论，也可以选择综述的文献先分享最近得2篇综述，让不清楚个中差异的读者了解Neuromyelitis optica: an overview点击下载重新认识视神经脊髓炎点击下载 关于视神经脊髓炎和多发性硬化两者的鉴别，Wingerchuk这篇综述被引用次数较多，两者区别如下：此外，在这篇文献中Wingerchuk还提出了视神经脊髓炎疾病谱的概念NMO的病变部位多出现于于第三、四脑室、侧脑室周围、下丘脑等区域The spectrum of neuromyelitis optica点击下载NMO和MS均可以出现脑部脱髓鞘改变，两者MRI有不同的特征。shoichi等研究发现89%的NMO患者颅脑MRI检查可以出现异常，最主要的特征病变部位有“云雾状强化”（Cloud-like Enhancement），而MS脑内病灶强化多为卵圆状，病灶边缘模糊。“Cloud-like Enhancement”Is a Magnetic Resonance Imaging Abnormality Specific to Neuromyelitis Optica点击下载看看J Neurol Neurosurg Psychiatry 2008;79:1134-1136 doi:10.1136/jnnp.2007.133330 Background: A polyspecific, intrathecal humoral immune response against neurotropic viruses such as measles, rubella and varicella zoster virus (MRZ reaction, MRZR) is present in 80100% of patients with multiple sclerosis (MS), but has not to date been evaluated in patients with neuromyelitis optica (NMO). Aims: To evaluate whether MRZR distinguishes NMO and MS. Methods: 20 patients with NMO and 42 with MS were included. The intrathecal synthesis of antibodies against measles, rubella and varicella zoster virus was detected by calculation of the respective antibody indices (AI). Results: A positive MRZ reaction, as defined by a combination of at least two positive AIs, was found in 37/42 MS, but in only 1/20 NMO patients (p0.0001). Median AI values differed significantly between the groups (p0.0005). Conclusions: The polyspecific antiviral humoral immune response characteristic for MS is widely missing in NMO, irrespective of the NMO-IgG status of the patients. Our findings further strengthen the case for NMO being pathologically distinct from MS. 结论：MS特征性的多反应性抗病毒体液免疫反应几乎不见于NMO，不考虑患者NMO-IgG。我们的发现进一步证实MS和NMO病理机制不同这一事实。原文点击下载 再来看Arch Neurol. 2007;64(6):903-905. 的Is Neuromyelitis Optica Distinct From Multiple Sclerosis?Something for Lumpers and Splitters 作者解释了lumpers 是指similar disorders should be characterized under a common rubric，而 splitters 是指similarly appearing disorders should be differentiated on a pathophysiologic, genetic, or phenotypic basis，并从以下几个方面做了分析：LUMPING AND SPLITTINGMS VS NMO: DISTINCTIONS AND SIMILARITIESUTILITY OF THE NMO-IgG ANTIBODYTHE NMO-IgG ANTIBODY AND PATHOGENICITYDO MS AND NMO DISPLAY DIFFERENTIAL TREATMENT RESPONSIVENESS?NMO AND MS: LUMP IT OR SPLIT IT?结论是：Notwithstanding all of this substantial progress