Supraventricular Tachycardia During Status Epilepticus in Dravet Syndrome: A Link Between Brain and Heart?

详细信息    查看全文

• 作者：Marco Daverio ; MD^a ; Ornella Ciccone ; MD^a ; Clementina Boniver ; MD^a ; Luca De Palma ; MD^a ; Domenico Corrado ; MD ; PhD^b ; Marilena Vecchi ; MD^a ; ^{vecchi@pediatria.unipd.it" class="auth_mail" title="E-mail the corresponding author}
• 关键词：arrhythmia ; channelopathy ; Dravet syndrome ; SCN1A ; paroxysmal supraventricular tachycardia
• 刊名：Pediatric Neurology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：56
• 期：Complete
• 页码：69-71
• 全文大小：526 K

文摘

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.