Electrical and autonomic cardiac function in patients with Dravet syndrome

Angelica Bibiana Delogu, Domenica Immacolata Battaglia, Gaetano Antonio Lanza, Filippo Crea, Antonella Spinelli, Charlotte Dravet, Alessia De Nisco, Costantino Romagnoli, A Saracino

Research output: Contribution to journalArticle

Abstract

Dravet syndrome (DS) is an epileptic encephalopathy related mainly to mutations in the SCN1A gene, encoding for neuronal sodium channels. Patients with DS have a high risk of sudden unexpected death in epilepsy (SUDEP). In this study we investigated whether patients with DS present abnormalities in electrical and autonomic cardiac function. To this aim we assessed ventricular repolarization and heart rate variability (HRV) on standard electrocardiography (ECG) and on 24-h ECG Holter monitoring, respectively, in 20 patients affected by DS (6.8 ± 4 years, 11 female). As age- and sex-matched control groups, we also studied 20 patients with other epileptic syndromes receiving antiepileptic drugs (ES/AED, 6.0 ± 5 years, 12 female), 20 patients with other epileptic syndromes without treatment (ES/no-AED, 6.7 ± 4 years, 10 female), and 20 healthy children (HC, 7.2 ± 5 years, 11 females). Data analysis showed that patients with DS had depressed HRV variables compared to both ES patients (ES/AED and ES/no-AED) and HC control group, whereas no significant differences in HRV variables were found between ES patients (with and without treatment) and HC. There was no significant difference between patients with DS and all the other control groups in RR intervals, QT, and QTc interval analysis. In conclusion, DS patients display an imbalance of cardiac autonomic function toward a relative predominance of adrenergic tone compared to both healthy children and patients with other forms of epilepsy, independent of antiepileptic therapy. Follow-up studies should clarify the clinical significance of this autonomic impairment and whether HRV analysis can be helpful in predicting the risk of sudden death in patients with DS.
Original languageEnglish
Pages (from-to)55-58
Number of pages4
JournalEpilepsia
Volume52 Suppl 2
DOIs
Publication statusPublished - 2011

Keywords

  • Autonomic Nervous System
  • Cardiovascular Physiological Phenomena
  • Child
  • Child, Preschool
  • Electrocardiography
  • Electrocardiography, Ambulatory
  • Epilepsies, Myoclonic
  • Female
  • Heart Rate
  • Humans
  • Male
  • Nerve Tissue Proteins
  • Sodium Channels
  • Syndrome

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