Choi Seok-Jin, Hong Yoon-Ho, Shin Je-Young, Yoon Byung-Nam, Sohn Sung-Yeon, Park Chan Soon, Sung Jung-Joon
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.
Department of Neurology, Seoul National University-Seoul Metropolitan Government Boramae Medical Center, Seoul, Republic of Korea.
J Neurol Sci. 2017 Apr 15;375:289-293. doi: 10.1016/j.jns.2017.02.012. Epub 2017 Feb 8.
To investigate the frequency, features, and prognosis of takotsubo cardiomyopathy (TTC) in patients with amyotrophic lateral sclerosis (ALS).
We reviewed detailed clinical, laboratory, and cardiovascular data from 64 ALS patients (38 men and 26 women) who underwent echocardiographic evaluation for various reasons at a single referral center between January 2011 and December 2015.
TTC was diagnosed in 9 ALS patients (4 men and 5 women). Mean age was 61.3years (range 55-71years), and median disease duration was 51.5months (range 18-134months). All patients were bulbar or cervical onset, and were at advanced stages of ALS when TTC was diagnosed. Acute exacerbation of dyspnea was an invariable presentation, and chest discomfort mimicking acute coronary syndrome was present in 2 patients. Six patients had significant hypotension requiring intravenous fluid challenge and inotropic support. Three patients showed altered mentality, and 2 of them suffered cardiopulmonary arrest.
TTC should be suspected in ALS patients presenting with acute exacerbation of dyspnea and chest discomfort, particularly at advanced stages of the disease. This study highlights the need for proper evaluation and management of cardiac dysfunction in ALS.
探讨肌萎缩侧索硬化症（ALS）患者中应激性心肌病（TTC）的发生率、特征及预后。
我们回顾了2011年1月至2015年12月期间在单一转诊中心因各种原因接受超声心动图评估的64例ALS患者（38例男性和26例女性）的详细临床、实验室和心血管数据。
9例ALS患者（4例男性和5例女性）被诊断为TTC。平均年龄为61.3岁（范围55 - 71岁），疾病中位病程为51.5个月（范围18 - 134个月）。所有患者均为延髓或颈髓起病，诊断TTC时均处于ALS晚期。呼吸困难急性加重是常见表现，2例患者出现类似急性冠状动脉综合征的胸部不适。6例患者有明显低血压，需要静脉补液和使用血管活性药物支持。3例患者出现意识改变，其中2例发生心肺骤停。
对于出现呼吸困难急性加重和胸部不适的ALS患者，尤其是疾病晚期患者，应怀疑TTC。本研究强调了对ALS患者心脏功能障碍进行恰当评估和管理的必要性。
