Background: The possibility that epileptic seizures and arrhythmias are different clinical manifestations of a common channelopathy is an interesting but unproved hypothesis. Patients with Dravet syndrome show heart rate variability and affected individuals with arrhythmias have also been documented. The possibility that a genetic mutation affecting sodium channel functions may predispose to both Dravet syndrome and arrhythmogenic disorders is an interesting hypothesis.
Patient presentation: We describe a 5-month-old girl with Dravet syndrome who presented with paroxysmal supraventricular tachycardia during status epilepticus. She presented to the hospital the first time with afebrile tonic-clonic seizures and then several subsequent times with status epilepticus confirmed with electroencephalography. During two of these episodes she also exhibited paroxysmal supraventricular tachycardia. She received propofol for status epilepticus and adenosine for the arrhythmia. A clinical and genetic (denovo mutation of a sodium channel, SCN1A) diagnosis of Dravet syndrome was made.
Conclusions: Our patient supports the hypothesis that SCN1A mutation might have a role as a common substrate to both epilepsy and cardiac arrhythmia. More studies are needed to better assess genetic, cardiac, respiratory, and autonomic dysfunction in patients with Dravet syndrome.
Keywords: Dravet syndrome; SCN1A; arrhythmia; channelopathy; paroxysmal supraventricular tachycardia.
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