Takotsubo cardiomyopathy in amyotrophic lateral sclerosis.
OBJECTIVE
To investigate the frequency, features, and prognosis of takotsubo cardiomyopathy (TTC) in patients with amyotrophic lateral sclerosis (ALS).


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.
Seok-Jin
SJ
Choi
Department of Neurology, Seoul National University-Seoul Metropolitan Government Boramae Medical Center, Seoul, Republic of Korea.
Yoon-Ho
YH
Hong
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.
Je-Young
JY
Shin
Department of Neurology, Inha University Hospital, Incheon, Republic of Korea.
Byung-Nam
BN
Yoon
Department of Neurology, Eulji University Hospital, Daejeon, Republic of Korea.
Sung-Yeon
SY
Sohn
Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea.
Chan Soon
CS
Park
Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea. Electronic address: jjsaint@snu.ac.kr.
Jung-Joon
JJ
Sung
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D015331:Cohort Studies; D003402:Creatine Kinase / Q000097:blood; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D054549:Takotsubo Cardiomyopathy / Q000097:blood / Q000175:diagnosis* / Q000209:etiology*; D019210:Troponin I / Q000097:blood
D019210:Troponin I; D003402:Creatine Kinase
Amyotrophic lateral sclerosis; Autonomic dysfunction; Takotsubo cardiomyopathy