Seizure-like episodes and EEG abnormalities in patients with long QT syndrome


The congenital long QT syndrome (cLQTS) is caused by mutations in genes coding for cardiac ion channels 1. cLQTS is associated with prolongation of the QT interval in the ECG and a propensity to torsade de pointes arrhythmia, syncope and sudden cardiac death2,3 with incomplete penetrance. So far, 19 LQTS susceptibility genes have been identified 4 of which many are expressed both in heart and brain 5. In 75 % the mutations have been found in genes coding for the potassium channels KCNQ1 and KCNH2 or the sodium channel SCN5A 4.


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