視神經(jīng)脊髓炎與多發(fā)硬化的關(guān)系.doc

每月一主題-視神經(jīng)脊髓炎與多發(fā)性硬化的關(guān)系每月一主題-視神經(jīng)脊髓炎與多發(fā)性硬化的關(guān)系視神經(jīng)脊髓炎(Neuromyelitis optica, NMO)又稱Devics disease，是我國常見的脫髓鞘病，有些學(xué)者認(rèn)為屬多發(fā)性硬化的亞型，但目前越來越多學(xué)者認(rèn)為屬于獨(dú)立的疾病。NMO 目前被認(rèn)為是某些病因引起針對(duì)水通道蛋白AQP4產(chǎn)生特異性自身抗體NMO-IgG的不同于MS的離子通道性自身免疫病。其主要特點(diǎn)是合并有視神經(jīng)與脊髓的脫髓鞘性變。二者同時(shí)或先后發(fā)病，急性或亞急性病程。實(shí)驗(yàn)研究結(jié)果提示，NMO-IgG可以調(diào)制AQP4的功能，對(duì)NMO存在潛在的致病性。分成幾個(gè)課題來導(dǎo)論視神經(jīng)脊髓炎與多發(fā)性硬化的臨床表現(xiàn)異同視神經(jīng)脊髓炎與多發(fā)性硬化的致病因異同視神經(jīng)脊髓炎與多發(fā)性硬化的檢查與診斷視神經(jīng)脊髓炎與多發(fā)性硬化的治療與結(jié)局異同請(qǐng)選擇高質(zhì)量、近期或大型的研究來討論，也可以選擇綜述的文獻(xiàn)先分享最近得2篇綜述，讓不清楚個(gè)中差異的讀者了解Neuromyelitis optica: an overview點(diǎn)擊下載重新認(rèn)識(shí)視神經(jīng)脊髓炎點(diǎn)擊下載 關(guān)于視神經(jīng)脊髓炎和多發(fā)性硬化兩者的鑒別，Wingerchuk這篇綜述被引用次數(shù)較多，兩者區(qū)別如下：此外，在這篇文獻(xiàn)中Wingerchuk還提出了視神經(jīng)脊髓炎疾病譜的概念NMO的病變部位多出現(xiàn)于于第三、四腦室、側(cè)腦室周圍、下丘腦等區(qū)域The spectrum of neuromyelitis optica點(diǎn)擊下載NMO和MS均可以出現(xiàn)腦部脫髓鞘改變，兩者M(jìn)RI有不同的特征。shoichi等研究發(fā)現(xiàn)89%的NMO患者顱腦MRI檢查可以出現(xiàn)異常，最主要的特征病變部位有“云霧狀強(qiáng)化”（Cloud-like Enhancement），而MS腦內(nèi)病灶強(qiáng)化多為卵圓狀，病灶邊緣模糊?！癈loud-like Enhancement”Is a Magnetic Resonance Imaging Abnormality Specific to Neuromyelitis Optica點(diǎn)擊下載看看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. 結(jié)論：MS特征性的多反應(yīng)性抗病毒體液免疫反應(yīng)幾乎不見于NMO，不考慮患者NMO-IgG。我們的發(fā)現(xiàn)進(jìn)一步證實(shí)MS和NMO病理機(jī)制不同這一事實(shí)。原文點(diǎn)擊下載 再來看Arch Neurol. 2007;64(6):903-905. 的Is Neuromyelitis Optica Distinct From Multiple Sclerosis?Something for Lumpers and Splitters 作者解釋了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，并從以下幾個(gè)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?結(jié)論是：Notwithstanding all of this substantial progress