근위축성측삭경화증 환자에서 발생한 takotsubo 심근병증

학위논문 (석사)-- 서울대학교 대학원 : 의학과, 2017. 2. 홍윤호.Objective: To investigate the frequency, features, and prognosis of takotsubo cardiomyopathy (TTC) in patients with amyotrophic lateral sclerosis (ALS). Methods: Among 624 ALS patients seen at a single referral center between January 2011 and December 2015, we retrospectively reviewed detailed clinical, laboratory, and cardiovascular data from 64 ALS patients (38 men and 26 women) who underwent echocardiographic evaluation for various reasons. Results: TTC was diagnosed in 9 ALS patients (4 men and 5 women). Mean age was 61.3 years (range 55–71 years), and median disease duration was 51.5 months (range 18–134 months). 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. Conclusion: 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 and autonomic dysfunction in ALS.Introduction 1 Materials and Methods 3 Results 5 Discussion 8 Conclusion 10 References 11 Tables and Figures 15 Abstract (in Korean) 19Maste