Brugada in ALS: Heart beyond neurons

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder characterized by upper and lower-motor neurons involvement. However, growing evidence suggests a non-neuronal involvement with a particular attention to autonomic disfunction and consequent cardiovascular complication and sudden-cardiac death.(1) In this contest, many authors focused on arrhythmic complications in ALS patients and in other motor neuron diseases such as Kennedy's Disease (KD).(2) While, Brugada Syndrome(BS) is the most frequent arrhythmia associated with KD, only one case of type2 BS has been described in ALS(3).