Neurorehabilitation, Neurology Unit and Neurophysiology Unit, San Raffaele Scientific Institute, 20132 Milan, Italy.
Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, 20132 Milan, Italy.
Int J Mol Sci. 2023 Jul 28;24(15):12096. doi: 10.3390/ijms241512096.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with variable phenotypic expressions which has been associated with autonomic dysfunction. The cardiovascular system seems to be affected especially in the context of bulbar involvement. We describe four new cases of Tako-Tsubo syndrome (TTS) in ALS patients with an appraisal of the literature. We present a late-stage ALS patient with prominent bulbar involvement that presented TTS during hospitalization. We then retrospectively identify three additional ALS-TTS cases reporting relevant clinical findings. TTS cardiomyopathy has been observed in different acute neurological conditions, and the co-occurrence of ALS and TTS has already been reported. Cardiovascular autonomic dysfunctions have been described in ALS, especially in the context of an advanced diseases and with bulbar involvement. Noradrenergic hyperfunction linked to sympathetic denervation and ventilatory deficits coupled in different instances with a trigger event could play a synergistic role in the development of TTS in ALS. Sympathetic hyperfunctioning and ventilatory deficits in conjunction with cardiac autonomic nerves impairment may play a role in the development of TTS in a context of ALS.
肌萎缩侧索硬化症（ALS）是一种具有可变表型表达的神经退行性疾病，与自主神经功能障碍有关。心血管系统似乎特别受延髓受累的影响。我们描述了 4 例肌萎缩侧索硬化症患者的 Tako-Tsubo 综合征（TTS）新病例，并对文献进行了评估。我们介绍了一名晚期肌萎缩侧索硬化症患者，其延髓受累明显，在住院期间出现 TTS。然后，我们回顾性地确定了另外 3 例肌萎缩侧索硬化症-TTS 病例，报告了相关的临床发现。TTS 心肌病已在不同的急性神经系统疾病中观察到，并且已经报道了 ALS 和 TTS 的同时发生。心血管自主神经功能障碍已在 ALS 中描述，特别是在晚期疾病和延髓受累的情况下。去甲肾上腺素能功能亢进与交感神经去神经支配和通气不足有关，在不同情况下与触发事件有关，可能在 ALS 中 TTS 的发展中发挥协同作用。交感神经功能亢进和通气不足以及心脏自主神经损伤可能在 ALS 背景下 TTS 的发展中起作用。
