Referenced in: none

Automatically associated channels: Cav1.1 , Nav1.4

Title: Atypical arrhythmic complications in familial hypokalemic periodic paralysis.

Authors: Stefano Maffè, Fabiana Signorotti, Antonello Perucca, Massimo Bielli, Uros Hladnik, Elena Ragazzoni, Elisabetta Maduli, Paola Paffoni, Pierfranco Dellavesa, Anna Maria Paino, Franco Zenone, Umberto Parravicini, Nicolò Franchetti Pardo, Lorenzo Cucchi, Marco Zanetta

Journal, date & volume: J Cardiovasc Med (Hagerstown), 2009 Jan , 10, 68-71

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/19708131

Abstract
Familial hypokalemic periodic paralysis is an autosomal dominant muscle disorder characterized by episodic attacks of muscle weakness, accompanied by a decrease in blood potassium levels. It is based on genetic mutations in the genes CACNA1S (most frequent, encoding the skeletal muscle calcium channel) and SCN4A (10% of cases, encoding the sodium channel). Few cases have been reported with cardiac dysrhythmia. We report a rare case of a patient with a novel SCN4A mutation who presented, on ECG, extreme bradycardia and syncopal sinus arrest that required a temporary pacemaker implant