J Korean Child Neurol Soc.  1999 May;6(2):388-393.

A Case of Idiopathic Long QT Syndrome Presenting as Epilepsy

Affiliations
  • 1Department of Pediatrics, Ulsan University College of Medicine, Seoul, Korea.

Abstract

"Idiopathic long QT syndrome" is characterized by prolongation of the QT interval due to unusual electrocardiographic repolarization abnormality and associated with variable clinical manifestations from no specific symptoms in lifetime to syncope or even sudden death. The prognosis of this syndrome is very grave and motality is approximately 50% within 10 years among untreated symptomatic patients after the initial syncope. But this sudden onset syncope may be misdiagnosed as epilepsy, being treated with antiepileptic drug for many years. However, this high mortality has been significantly reduced to less than 5% by the effective therapy. Therefore, it is crucial to make an early and accurate dianosis. We exprienced a case of 34 months old male who presented with recurrent syncopal attacks. He had no specific neurological abnomal finding except congenital deafness. He had normal EEG and brain MRI findings but ECG showed prolonged QT interval (QTc= 0.5), findings of which were compatible with long QT syndrome. He is currently being followed at OPD, but the pateint is still experiencing syncopal attack despite of treatment with beta-blocker, atenolol. Therefore, we are considering an insertion of pacemaker or performing thoracic sympathectomy.

Keyword

Long QT syndrome; Epilepsy; Syncopal attack

MeSH Terms

Atenolol
Brain
Child, Preschool
Deafness
Death, Sudden
Electrocardiography
Electroencephalography
Epilepsy*
Humans
Long QT Syndrome*
Magnetic Resonance Imaging
Male
Mortality
Prognosis
Sympathectomy
Syncope
Atenolol
Full Text Links
  • JKCNS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr