Gill Dalvir, Liu Kan
Department of Internal Medicine, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA.
Department of Cardiology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA.
Am J Emerg Med. 2017 Jul;35(7):1012. doi: 10.1016/j.ajem.2016.12.050. Epub 2016 Dec 22.
51-year-old female who presented with progressive paresthesia, numbness of the lower extremities, double vision, and trouble walking. Physical exam was remarkable for areflexia, and ptosis. Her initial EKG showed nonspecific ST segment changes and her Troponin T was elevated to 0.41ng/mL which peaked at 0.66ng/mL. Echocardiogram showed a depressed left ventricular ejection fraction to 35% with severely hypokinetic anterior wall and left ventricular apex was severely hypokinetic. EMG nerve conduction study showed severely decreased conduction velocity and prolonged distal latency in all nerves consistent with demyelinating disease. She was treated with 5days of intravenous immunoglobulin therapy to which she showed significant improvement in strength in her lower extremities. Echocardiogram repeated 4days later showing an improved left ventricular ejection fraction of 55% and no left ventricular wall motion abnormalities. Takotsubo cardiomyopathy is a rare complication of Miller-Fisher syndrome and literature review did not reveal any cases. Miller-Fisher syndrome is an autoimmune process that affects the peripheral nervous system causing autonomic dysfunction which may involve the heart. Due to significant autonomic dysfunction in Miller-Fisher syndrome, it could lead to arrhythmias, blood pressure changes, acute coronary syndrome and myocarditis, Takotsubo cardiomyopathy can be difficult to distinguish. The treatment of Takotsubo cardiomyopathy is supportive with beta-blockers and angiotensin-converting enzyme inhibitors are recommended until left ventricle ejection fraction improvement. Takotsubo cardiomyopathy is a rare complication during the acute phase of Miller-Fisher syndrome and must be distinguished from autonomic dysfunction as both diagnoses have different approaches to treatment.
一名51岁女性,出现进行性感觉异常、下肢麻木、复视及行走困难。体格检查发现无反射和上睑下垂。其初始心电图显示非特异性ST段改变,肌钙蛋白T升高至0.41ng/mL,峰值为0.66ng/mL。超声心动图显示左心室射血分数降至35%,前壁严重运动减弱,左心室心尖严重运动减弱。肌电图神经传导研究显示所有神经的传导速度严重降低,远端潜伏期延长,符合脱髓鞘疾病。她接受了5天的静脉注射免疫球蛋白治疗,下肢力量有显著改善。4天后复查超声心动图显示左心室射血分数提高至55%,且无左心室壁运动异常。应激性心肌病是米勒-费希尔综合征的一种罕见并发症,文献回顾未发现相关病例。米勒-费希尔综合征是一种自身免疫过程,影响周围神经系统,导致自主神经功能障碍,可能累及心脏。由于米勒-费希尔综合征存在显著的自主神经功能障碍,可导致心律失常、血压变化、急性冠状动脉综合征和心肌炎,应激性心肌病可能难以鉴别。应激性心肌病的治疗以支持治疗为主,推荐使用β受体阻滞剂和血管紧张素转换酶抑制剂,直至左心室射血分数改善。应激性心肌病是米勒-费希尔综合征急性期的一种罕见并发症,必须与自主神经功能障碍相鉴别,因为这两种诊断的治疗方法不同。