ALS is currently considered a multisystem disorder on the basis of its involvement of other organ systems leading to significant mortality and morbidity [
1‐
5]. Most patients with this disease experience respiratory failure secondary to progressive chest wall weakness and pulmonary embolism resulting from their immobility. In our patient’s case, we emphasize that cardiovascular involvement may also contribute to the disease mortality. In a French ALS study by Gil
et al., who researched the leading causes of mortality, 3.4% of patients died as a result of myocardial infarction or dysrhythmias, and a few patients had sudden cardiac death [
3]. The cardiovascular aberrations, such as QT prolongation, heart rate-blood pressure dyssynchrony, and nocturnal hypotension during the course of the disease have frequently been reported in this patient population. This could be due to decreasing sympathetic tone secondary to degeneration of the intermediolateral column in the upper levels of the spinal cord, as previously shown in pathological studies by Hirohide
et al. [
6]. Brugada syndrome is an inherited arrhythmia syndrome characterized by coved (type 1) or saddleback (types 2 and 3) ST-segment elevations (≥2 mm), followed by deep T-wave inversions in leads V1–V3. It is associated with increased risk of sudden cardiac death, especially in young male adults of Southeast Asian origin. Although genetic mutations in several sodium and calcium channel genes have been identified, an imbalance in sympathetic and parasympathetic nervous systems in the pathogenesis of Brugada syndrome has been proposed [
7,
8]. Brugada syndrome, which can predispose patients to develop ventricular tachyarrhythmia and circulatory collapse, has not been reported in patients with ALS, which can predispose them to develop ventricular tachyarrhythmia and circulatory collapse, as happened in our patient. Kennedy’s disease, another type of motor neuron disease, is known to be associated with all types of Brugada syndrome [
4]. We hypothesize that patients with ALS are also at high risk for this pattern, given the changes in the efferent sympathetic dysfunction shared with Kennedy’s disease. Our patient did not have any preexisting mutations to develop Brugada syndrome, ruling out the possibility of a casual association, but rather had an important pathophysiological mechanism of motor neuron diseases that needs to be explored further. Because patients with ALS tend to live longer with improved respiratory management, identifying and managing potential causes of acute cardiorespiratory failure, such as ventricular tachyarrhythmias, need further emphasis. Periodic surveillance with electrocardiography to identify patterns of Brugada syndrome and QTc prolongation may be considered to identify patients at high risk for potential cardiac arrest.