Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease

•  Heart disease has been attributed as a major cause of death in patients with Huntington's disease (HD). While little is known about cardiac complication(s) in HD, dysfunction of the autonomic nervous system (ANS) may play a role. •  Using a mouse model of HD, we demonstrated that even from an early HD phase, there was enhanced sympathetic and parasympathetic nervous activities, leading to an unstable heart beat, various arrhythmias and, ultimately, sudden arrhythmic death. •  Greater numbers of active neurons were found in brain regions important for autonomic regulation in HD mice, suggesting a centrally mediated mechanism that was underlying the ANS‐cardiac dysfunction. •  Our findings provide insight into a likely neurocardiac cause of death in HD, and warrant further clinical investigation.