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情绪应激作为重症肌无力危象和伴发的应激性心肌病的触发因素:一例报告

Emotional stress as a trigger of myasthenic crisis and concomitant takotsubo cardiomyopathy: a case report.

作者信息

Beydoun Said R, Wang Jingtian, Levine Reed Loring, Farvid Ali

机构信息

Department of Neurology, University of Southern California, Los Angeles, California, USA.

出版信息

J Med Case Rep. 2010 Dec 3;4:393. doi: 10.1186/1752-1947-4-393.

Abstract

INTRODUCTION

Myasthenia gravis is a neuromuscular junction post-synaptic autoimmune disorder. Myasthenic crisis is characterized by respiratory failure requiring mechanical ventilation. Takotsubo cardiomyopathy is a rare clinical syndrome defined as a profound but reversible left ventricular dysfunction in the absence of coronary artery disease.

CASE PRESENTATION

We report a unique case of a 60-year-old Hispanic woman with myasthenia gravis who developed takotsubo cardiomyopathy and concomitant myasthenic crisis that appear to have been triggered by a stressful life event. On admission, she presented with severe mid-sternal chest pain and shortness of breath shortly after a personally significant stressful life event. A pertinent neurological examination showed bilateral facial weakness and right ptosis. The left ventriculogram showed apical ballooning with hyperdynamic proximal segments with sparing of the apex. Her troponin I level was elevated, while cardiac catheterization revealed no significant coronary artery disease. The findings were consistent with takotsubo cardiomyopathy. Shortly after cardiac catheterization, she developed bilateral ophthalmoparesis and significant bulbar and respiratory muscle weakness. Forced vital capacity values were persistently less than 1 L. The patient developed respiratory failure and required endotracheal intubation. After plasmapheresis and corticosteroid treatment, her clinical course improved with successful extubation. A normal left ventricle chamber size and a normal ejection fraction were noted by an echocardiogram repeated 10 months later.

CONCLUSION

This is the first reported case of the simultaneous triggering of both takotsubo cardiomyopathy and myasthenic crisis by the physiologic consequences of a state of severe emotional stress. We hypothesize that the mechanism underlying the rare association of takotsubo cardiomyopathy with myasthenic crisis involves excessive endogenous glucocorticoid release, a high-catecholamine state, or a combination of both. We advocate careful cardiac monitoring of myasthenia gravis patients during acute emotional or physical stress, as there is potential risk of developing takotsubo cardiomyopathy.

摘要

引言

重症肌无力是一种神经肌肉接头突触后自身免疫性疾病。重症肌无力危象的特征是呼吸衰竭需要机械通气。应激性心肌病是一种罕见的临床综合征,定义为在无冠状动脉疾病的情况下出现严重但可逆的左心室功能障碍。

病例介绍

我们报告了一例独特的病例,一名60岁的西班牙裔重症肌无力女性患者,并发应激性心肌病和重症肌无力危象,似乎是由一次重大生活应激事件引发的。入院时,在经历一次对个人意义重大的生活应激事件后不久,她出现了严重的胸骨后胸痛和呼吸急促。相关神经系统检查显示双侧面部无力和右侧上睑下垂。左心室造影显示心尖部膨出,近端节段动力增强,心尖部未受累。她的肌钙蛋白I水平升高,而心脏导管检查显示无明显冠状动脉疾病。这些发现符合应激性心肌病。心脏导管检查后不久,她出现双侧眼球运动障碍以及明显的延髓和呼吸肌无力。用力肺活量值持续低于1升。患者出现呼吸衰竭,需要气管插管。经过血浆置换和皮质类固醇治疗,她的临床病程有所改善,成功脱机。10个月后复查超声心动图显示左心室腔大小正常,射血分数正常。

结论

这是首例报告的因严重情绪应激状态的生理后果同时引发应激性心肌病和重症肌无力危象的病例。我们推测应激性心肌病与重症肌无力危象罕见关联的潜在机制涉及内源性糖皮质激素过度释放、高儿茶酚胺状态或两者兼而有之。我们主张在重症肌无力患者急性情绪或身体应激期间进行仔细的心脏监测,因为存在发生应激性心肌病的潜在风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d89/3003678/63deb5ad252f/1752-1947-4-393-1.jpg

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