期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 24, 期 15, 页码 -出版社
MDPI
DOI: 10.3390/ijms241512096
关键词
motor neuron disease; autonomic system; mortality; non-invasive ventilation; PEG; NIMV; vascular; cardio-vascular
This study summarizes four cases of amyotrophic lateral sclerosis (ALS) patients with Tako-Tsubo syndrome (TTS) and reviews the relevant literature, revealing the association between ALS and TTS. Among these cases, one ALS patient developed TTS during hospitalization, and three additional cases with ALS-TTS were identified retrospectively. The study found that cardiovascular autonomic dysfunction in ALS is especially pronounced in cases with bulbar involvement.
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.
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