Clinical, electrophysiological and pathologic correlations in a severe murine experimental autoimmune neuritis model of Guillain-Barre syndrome
Clinical, electrophysiological and pathologic correlations in a severe murine experimental autoimmune neuritis model of Guillain-Barre syndrome
复制标题
DOI：
10.1016/j.jneuroim.2009.11.019
复制
发表时间：
2010-02-26
影响因子：
3.3
通讯作者：
Ubogu, Eroboghene E.
中科院分区：
文献类型：
--
作者：
Xia, Robin H.;Yosef, Nejla;Ubogu, Eroboghene E.
SJL/J小鼠的严重小鼠实验性自身免疫性神经炎（sm-EAN）是最近描述的，但不完全表征的格林巴利综合征（GBS）小鼠模型。在疾病过程中的电生理和病理表征是设计可能与GBS发病机制相关的机制研究的必要前提。Sm-EAN是一种具有弥漫性脱髓鞘性多发性神经病的电生理学证据的单相疾病，严重程度最高时伴有轴突丢失。回归分析表明，在疾病过程中神经肌肉严重程度评分和电生理参数之间存在很强的相关性。在坐骨神经中病理学观察到与单核细胞浸润（F4/80+巨噬细胞> CD 3 + T淋巴细胞> CD 19 + B淋巴细胞）相关的进行性多灶性或弥漫性脱髓鞘伴轴突丢失，在最严重程度时达到峰值。回归分析显示严重程度评分与炎性细胞计数之间有很强的相关性，相关数据表明单核细胞浸润。以及脱髓鞘和轴突缺失与sm-EAN中观察到的神经肌肉无力直接相关。高诱导率以及与AIDP的病理相似性使sm-EAN成为使用客观结果测量（C）研究人类周围神经炎症发病机制的稳健模型。2009 Elsevier B V.保留所有权利。
Severe murine experimental autoimmune neuritis (sm-EAN) in SJL/J mice is a recently described, but incompletely characterized mouse model of Guillain-Bari-e syndrome (GBS) Electrophysiological and pathologic characterization during the disease course is a necessary prerequisite to designing mechanistic studies that may be relevant to GBS pathogenesis. Sm-EAN is a monophasic disorder with electrophysiological evidence for a diffuse demyelinating polyneuropathy with axonal loss at peak severity. Regression analyses demonstrated strong correlations between neuromuscular severity scores and electrophysiological parameters during the disease course Progressive multi-focal or diffuse demyelination with axonal loss was observed pathologically in sciatic nerves in association with mononuclear cell infiltrates (F4/80+ macrophages>CD3+ T-lymphocytes>CD19 + B-lymphocytes), peaking at maximal severity. Regression analyses demonstrated strong correlations between severity scores and inflammatory cell counts The correlative data imply that mononuclear infiltration. as well as demyelination and axonal loss are directly related to the observed neuromuscular weakness in sm-EAN The high induction rates, as well as pathologic similarities with AIDP make sm-EAN a robust model to study the pathogenesis of human peripheral nerve inflammation using objective outcome measures (C) 2009 Elsevier B V. All rights reserved.