Key result
Novel SCN4A mutation in familial hypokalemic periodic paralysis manifests as extreme bradycardia requiring temporary pacing.
Why the study?
Few cases of cardiac dysrhythmia have been reported in familial hypokalemic periodic paralysis, highlighting a gap in understanding atypical arrhythmic complications.
Case Report (n=1)
Reports a rare case of extreme bradycardia and syncopal sinus arrest requiring pacing in a patient with familial hypokalemic periodic paralysis and a novel SCN4A mutation.
May prompt arrhythmia vigilance in hypokalemic periodic paralysis; extends SCN4A phenotype but leaves causality open.
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
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Maffè et al. (2009) conducted a case report in Familial hypokalemic periodic paralysis (n=1). Novel SCN4A mutation was evaluated. A novel SCN4A mutation in a patient with familial hypokalemic periodic paralysis manifested as extreme bradycardia and syncopal sinus arrest requiring a temporary pacemaker.
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