文献标题：Absence of cortical demyelination in neuromyelitis optica
文献出处：Neurology.2010.75(23): 2103-2109
期刊影响因子：8.172
一项最新的研究结果显示：皮质脱髓鞘病变的缺乏可以进一步用来区别视神经脊髓炎和多发性硬化。
此项研究目的为评估视神经脊髓炎患者大脑中的皮质脱髓鞘病变的发生情况。视神经脊髓炎是一个自身免疫炎症反应性脱髓鞘疾病，主要病变靶位点是中枢神经系统的富含水通道蛋白-4的区域。因为水通道蛋白-4通常 在正常皮质中存在高表达，所以我们预测在视神经脊髓炎中可出现皮质脱髓鞘病变。
这是一项神经病理学的横断面分析，研究了对患有临床和（或）病理学确诊的视神经脊髓炎系列疾病的19例患者在尸检时获得的前脑和小脑组织切片。
结果对19例存档的视神经脊髓炎病例进行细致的免疫组化分析后证实了水通道蛋白-4在大脑和小脑皮质中的正常分布，且没有证据表明其存在皮质脱髓鞘现象。
医脉通推荐英文原文摘要
Neurology.2010.75(23):2103-2109.doi: 10.1212/WNL.0b013e318200d80c
Absence of cortical demyelination in neuromyelitis optica
B.F.Gh. Popescu,PhD, J.E. Parisi,et al.
Objective
To asses the presence of cortical demyelination in brains of patients with neuromyelitis optica (NMO). NMO is an autoimmune inflammatory demyelinating disease that specifically targets aquaporin-4–rich regions of the CNS. Since aquaporin-4 is highly expressed in normal cortex, we anticipated that cortical demyelination may occur in NMO.
Methods
This is a cross-sectional neuropathologic study performed on archival forebrain and cerebellar tissue sections from 19 autopsied patients with a clinically and/or pathologically confirmed NMO spectrum disorder.
Results
Detailed immunohistochemical analyses of 19 archival NMO cases revealed preservation of aquaporin-4 in a normal distribution within cerebral and cerebellar cortices, and no evidence of cortical demyelination.
Conclusions
This study provides a plausible explanation for the absence of a secondary progressive clinical course in NMO and shows that cognitive and cortical neuroimaging abnormalities previously reported in NMO cannot be attributed to cortical demyelination. Lack of cortical demyelination is another characteristic that further distinguishes NMO from MS.
文献来源
B.F.Gh. Popescu,PhD, J.E. Parisi,et al.Absence of cortical demyelination in neuromyelitis optica.Neurology.2010.75 (23): 2103-2109.[PubMed链接 | 期刊网站链接]
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