Total for the last 12 months
number of access : ?
number of downloads : ?
ID 113228
Title Alternative
TTS in ALS and Synucleinopathies
Yamamoto, Yuki Tokushima University
Matsubara, Tomoyasu Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
Unai, Yuki Tokushima University
Tsukamoto, Ai Tokushima Hospital
Takamatsu, Naoko Tokushima University
Hayashi, Shinya Kaminaka Hospital
Oda, Masaya Mifukai Vihara Hananosato Hospital
Mori, Atsuko Itsuki Hospital
Nishida, Yoshihiko Itsuki Hospital
Watanabe, Shunsuke Tokushima University
Murayama, Shigeo Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
takotsubo syndrome
amyotrophic lateral sclerosis
Parkinson’s disease
dementia with Lewy bodies
multiple system atrophy
sympathetic nerve
Content Type
Journal Article
Takotsubo syndrome (TTS) is an acute cardiac syndrome characterized by regional left ventricular dysfunction with a peculiar circumferential pattern, which typically results in apical ballooning. Evidence indicates a pivotal role of catecholamines in TTS, and researchers have discussed multiple hypotheses on the etiology, including multivessel coronary spasm, myocardial stunning, excessive transient ventricular afterload, and cardiac sympathetic overactivity with local noradrenaline spillover. Although central nervous system disorders, such as stroke and epilepsy, are known to trigger TTS, the incidence and clinical features of TTS in neurodegenerative disorders are poorly understood. Here, we retrospectively examined TTS cases in a single-center cohort composed of 250 patients with amyotrophic lateral sclerosis (ALS) and 870 patients with synucleinopathies [582 patients with Parkinson’s disease (PD), 125 patients with dementia with Lewy bodies (DLB), and 163 patients with multiple system atrophy (MSA)] and identified 4 (1.6%, including 2 women) cases with ALS and no cases with synucleinopathies. Two ALS patients underwent autopsy and the pathological findings were compatible with the chronological changes identified in catecholamine-induced cardiomyopathy. A literature review identified 16 TTS cases with ALS, 1 case each with PD and DLB, and no cases with MSA. When current and previous TTS cases with ALS were concatenated: 55% (11/20) were female; 35% (7/20) had a bulbar-onset and 45% (9/20) had a limb-onset; the mean age of TTS onset was 63.3 ± 9.0 years and the mean interval time from ALS onset to TTS development was 4.9 ± 3.0 years; no (0/16) patients developed TTS within 12 months after ALS onset; 50% (10/20) underwent artificial ventilations; the mortality was 17% (3/18); and most cases had precipitating factors, and TTS development was associated with gastrostomy, tracheostomy, or infections in 45% (9/20) of the patients. This study demonstrated that ALS is a considerable predisposing factor of TTS and that synucleinopathies rarely cause TTS. The distinct TTS incidence between ALS and synucleinopathies may be due to cardiac sympathetic overactivity in ALS and may also be affected by cardiac sympathetic denervation in synucleinopathies. Moreover, the etiology of TTS in ALS may be reasonably explained by the two-hit theory.
Journal Title
Frontiers in Neurology
Start Page
Published Date
Copyright © 2018 Izumi, Miyamoto, Fujita, Yamamoto, Yamada, Matsubara, Unai, Tsukamoto, Takamatsu, Nodera, Hayashi, Oda, Mori, Nishida, Watanabe, Ogawa, Uehara, Murayama, Sata and Kaji.This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY)( The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
DOI (Published Version)
URL ( Publisher's Version )
FullText File
University Hospital
Medical Sciences