Published online April 9, 2007
PEDIATRICS Vol. 119 No. 5 May 2007, pp. e1206-e1211 (doi:10.1542/peds.2006-2628)
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EXPERIENCE & REASON

Brugada Syndrome Masquerading as Febrile Seizures

Jonathan Robert Skinner, MB, ChB, FRACP, FRCPCH, MDa,b, Seo-Kyung Chung, BScb,c, Carey-Anne Nel, BSc, MSc(Med)b,d, Andrew Neil Shelling, BPhEd, BSc, PhDb,d, Jackie Robyn Crawford, NZCSa,b, Neil McKenzie, MB, ChB, FRACPe, Ralph Pinnock, MB, ChB, DCH(SA), MHSc, FRACPf, John Kerswell French, BMedSc, MB, ChB, MSc, PhDb,g and Mark Ian Rees, BSc, PhDb,h

a Greenlane Paediatric and Congenital Cardiac Services
f Department of Paediatrics, Starship Children's Hospital, Grafton, Auckland, New Zealand
b Cardiac Inherited Disease Group, Auckland City Hospital, Grafton, Auckland, New Zealand
c Departments of Molecular Medicine
d Obstetrics and Gynaecology, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand
e Department of Paediatrics, Christchurch Public Hospital, Christchurch, New Zealand
g Department of Cardiology, Liverpool Hospital, Liverpool, New South Wales, Australia
h Molecular Neuroscience Group, School of Medicine, University of Wales Swansea, Swansea, United Kingdom

ABSTRACT

Fever can precipitate ventricular tachycardia in adults with Brugada syndrome, but such a link has not been reported in children. A 21-month-old white girl presented repeatedly with decreased conscious level and seizures during fever. During a typical episode, rapid ventricular tachycardia was documented. The resting 12-lead electrocardiogram revealed a Brugada electrocardiogram signature. Resting electrocardiograms of the asymptomatic brother and mother were normal, but fever in the mother and pharmacologic stress with ajmaline in the brother revealed Brugada electrocardiogram features. Genetic testing revealed an SCN5A mutation in the affected family members.

Key Words: fever • seizures • ventricular tachycardia • cardiac channelopathies • Brugada syndrome

Abbreviations: ECG, electrocardiogram • SCN5A, gene encoding for sodium channel, voltage-gated, type V, alpha • SCN5A/Nav1.5, gene product/protein sodium channel, voltage-gated, type V, alpha • dHPLC, denaturing high-pressure liquid chromatography

Accepted Nov 1, 2006.