Article (Scientific journals)
Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy
Levaux, Joachim; Farhat, Nesrine; VAN CASTEREN, Lieve et al.
2022In Clinics and Practice, 12 (5), p. 677-685
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Keywords :
General Medicine
Abstract :
[en] A 13-year-old girl with Jervell and Lange-Nielsen syndrome associated congenital long QT syndrome (LQTS) and central deafness was admitted for generalized seizures. LQTS had been diagnosed after birth and confirmed at genetic testing. β-blocker treatment was immediately started. Despite this, since the age of 12 months, recurrent cerebral seizures occurred leading to the diagnosis of epilepsy. Anti-convulsive therapy was initiated but without success. At the last admission, nadolol dosage seemed infratherapeutic. Considering malignant ventricular arrhythmias as the cause of seizures, the β-blocker dosage was adjusted to weight and levels of magnesium and potassium optimized. Furthermore, the patient received an implantable Medtronic Reveal LINQ Recorder®. Since then, the adolescent has been asymptomatic with no arrhythmia documented. LQTS is due to one or more mutations of genes coding for ion channels. It may induce malignant ventricular arrhythmias and is a major cause of sudden cardiac death in children. Generalized cerebral seizures are extra-cardiac manifestations caused by decreased cerebral perfusion during ventricular arrhythmia. They are commonly misinterpreted as manifestations of epilepsy. For any patient with known or unknown LQTS who presents seizures with resistance to anti-convulsive therapy, a cardiac electrophysiological investigation should be performed promptly to ensure etiological diagnosis and optimize treatment.
Disciplines :
Cardiovascular & respiratory systems
Pediatrics
Author, co-author :
Levaux, Joachim  ;  Centre Hospitalier Universitaire de Liège - CHU
Farhat, Nesrine  ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
VAN CASTEREN, Lieve ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de cardiologie
BULK, Saskia ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de génétique
Seghaye, Marie-Christine ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Language :
English
Title :
Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy
Publication date :
26 August 2022
Journal title :
Clinics and Practice
ISSN :
2039-7275
eISSN :
2039-7283
Publisher :
MDPI AG
Volume :
12
Issue :
5
Pages :
677-685
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 21 October 2022

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