Neuromuscular Immunopathology Research Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas, United States.
J Neuroimmunol. 2010 Feb 26;219(1-2):54-63. doi: 10.1016/j.jneuroim.2009.11.019. Epub 2010 Jan 19.
Severe murine experimental autoimmune neuritis (sm-EAN) in SJL/J mice is a recently described, but incompletely characterized mouse model of Guillain-Barré 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.
严重实验性自身免疫性神经炎（sm-EAN）在 SJL/J 小鼠中是最近描述的，但不完全特征的格林-巴利综合征（GBS）小鼠模型。在疾病过程中的电生理学和病理学特征是设计可能与 GBS 发病机制相关的机制研究的必要前提。sm-EAN 是一种单相疾病，电生理学证据表明弥漫性脱髓鞘多神经病伴有轴突丢失的峰值严重程度。回归分析表明，在疾病过程中，神经肌肉严重程度评分与电生理学参数之间存在很强的相关性。在与单核细胞浸润（F4/80+巨噬细胞>CD3+T 淋巴细胞>CD19+B 淋巴细胞）相关的坐骨神经中观察到进行性多灶性或弥漫性脱髓鞘伴轴突丢失，在最大严重程度时达到峰值。回归分析表明，严重程度评分与炎症细胞计数之间存在很强的相关性。相关数据表明，单核细胞浸润以及脱髓鞘和轴突丢失与 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 的病理相似性使得 sm-EAN 成为使用客观结果措施研究人类周围神经炎症发病机制的强大模型。
