Finsterer Josef, Stöllberger Claudia, Ho Chen-Yu
a Neurological Department, Krankenanstalt Rudolfstiftung , Vienna , Austria.
b 2nd Medical Department with Cardiology and Intensive Care Medicine , Krankenanstalt Rudolfstiftung , Vienna , Austria.
Int J Neurosci. 2018 Dec;128(12):1207-1210. doi: 10.1080/00207454.2018.1486830. Epub 2018 Aug 20.
Takotsubo syndrome (TTS) is a non-ischaemic cardiomyopathy with sudden but transient systolic dysfunction. TTS mimics myocardial infarction clinically, chemically, and electrocardiographically but echocardiography typically shows apical ballooning and coronary angiography is normal. TTS has not been reported in a patient with myasthenia gravis (MG) and polymyositis due to a malignant thymoma.
Two weeks prior to admission, a 76-year-old female developed dysarthria, chronic coughing and disabling myalgias of the entire musculature. Since there was hyper-CKemia and elevated troponin, myocardial infarction was suspected. During swallowing of the antithrombotic medication on admission, she experienced apnoea, requiring cardio-pulmonary resuscitation with intubation and mechanical ventilation. Further diagnostic work-up precluded coronary heart disease but revealed TTS. Upon neurologic work-up, MG and polymyositis were diagnosed but the response to cholinergic drugs and plasmapheresis was poor. TTS was attributed to stress and anxiety from MG-associated respiratory insufficiency. The further course was complicated by recurrent supraventricular bradyarrhythmias and respiratory insufficiency. Upon thoracic CT a thymoma was suspected. Two months after admission, the mediastinal tumour was resected and malignant thymoma WHO BII infiltrating the mediastinum (modified Masaoka-Koga II/2) was diagnosed.
This case shows that TTS may be triggered by stress from respiratory insufficiency during a myasthenic crisis, MG may be associated with polymyositis, cholinergic medication may trigger bradyarrhythmias, and cholinergic drugs and plasmapheresis may exhibit a poor effect if malignant thymoma and polymyositis are present.
应激性心肌病（TTS）是一种非缺血性心肌病，具有突发但短暂的收缩功能障碍。TTS在临床、化学和心电图方面类似心肌梗死，但超声心动图通常显示心尖气球样变，冠状动脉造影正常。尚未有重症肌无力（MG）合并恶性胸腺瘤所致多肌炎患者发生TTS的报道。
入院前两周，一名76岁女性出现构音障碍、慢性咳嗽和全身肌肉严重肌痛。由于存在高肌酸激酶血症和肌钙蛋白升高，怀疑为心肌梗死。入院时服用抗血栓药物吞咽时，她出现呼吸暂停，需要进行插管和机械通气的心肺复苏。进一步的诊断检查排除了冠心病，但发现了TTS。经神经学检查，诊断为MG和多肌炎，但对胆碱能药物和血浆置换的反应不佳。TTS归因于MG相关呼吸功能不全引起的应激和焦虑。病程进一步因反复出现的室上性缓慢性心律失常和呼吸功能不全而复杂化。胸部CT怀疑有胸腺瘤。入院两个月后，切除纵隔肿瘤，诊断为WHO BII型恶性胸腺瘤浸润纵隔（改良Masaoka-Koga II/2）。
本病例表明，TTS可能由重症肌无力危象期间呼吸功能不全引起的应激触发，MG可能与多肌炎相关，胆碱能药物可能引发缓慢性心律失常，如果存在恶性胸腺瘤和多肌炎，胆碱能药物和血浆置换可能效果不佳。
