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首页> 外文期刊>Journal of the Neurological Sciences: Official Bulletin of the World Federation of Neurology >Neurophysiological characteristics of MuSK antibody positive Myasthenia Gravis mice: Focal denervation and hypersensitivity to acetylcholinesterase inhibitors
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Neurophysiological characteristics of MuSK antibody positive Myasthenia Gravis mice: Focal denervation and hypersensitivity to acetylcholinesterase inhibitors

机译：MuSK抗体阳性重症肌无力小鼠的神经生理特征：局部神经支配和对乙酰胆碱酯酶抑制剂的超敏性

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摘要

Myasthenia Gravis (MG) patients with antibodies against the muscle specific tyrosine kinase (MuSK +) typically present with focal fatigue and atrophy of the facial and bulbar muscles, along with unbeneficial reactions upon administration of acetylcholinesterase inhibitors (AChEIs). This study addresses the neurophysiological characteristics in facial versus limb muscles, before and after intraperitoneal injection of AChEIs, in mice immunized with MuSK. We performed in-vivo neurophysiological examinations in the masseter and gastrocnemius muscles of mice with MuSK + experimental autoimmune MG (EAMG) and in healthy control mice before and after administration of AChEIs. Abnormal spontaneous activity (fibrillations) was observed in the masseter muscle of MuSK + mice. Furthermore, 94% of MuSK-immunized mice displayed so called extra discharges (EDs) upon administration of a therapeutic AChEI dose, in contrast to 22% of the control mice, indicating neuromuscular hyperactivity. These findings support functional denervation in the masseter muscle and neuromuscular hypersensitivity already at a standard dose of AChEIs in MuSK + EAMG.
机译：重症肌无力（MG）患者的抗肌肉特异性酪氨酸激酶（MuSK +）抗体通常表现为局灶性疲劳和面部和延髓肌萎缩，以及服用乙酰胆碱酯酶抑制剂（AChEIs）时出现无益反应。这项研究解决了在用MuSK免疫的小鼠腹膜内注射AChEIs之前和之后面部和四肢肌肉的神经生理学特征。在使用AChEIs之前和之后，我们在使用MuSK +实验性自身免疫性MG（EAMG）的小鼠的咬肌和腓肠肌以及健康对照小鼠中进行了体内神经生理学检查。在MuSK +小鼠的咬肌中观察到异常的自发活动（纤颤）。此外，与22％的对照小鼠相比，94％的MuSK免疫小鼠在给予治疗性AChEI剂量后表现出所谓的额外放电（EDs），表明神经肌肉过度活跃。这些发现支持在标准剂量的MuSK + EAMG中的AChEIs下，咬肌的神经功能失灵和神经肌肉超敏反应。

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