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Case Report

Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy

1
Department of Pediatric Cardiology, University Hospital Liège, 4000 Liège, Belgium
2
Department of Cardiology, University Hospital Liège, 4000 Liège, Belgium
3
Department of Genetics, University Hospital Liège, 4000 Liège, Belgium
*
Author to whom correspondence should be addressed.
Academic Editor: Fernando Cordido
Clin. Pract. 2022, 12(5), 677-685; https://doi.org/10.3390/clinpract12050070
Received: 22 July 2022 / Revised: 18 August 2022 / Accepted: 23 August 2022 / Published: 26 August 2022
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. View Full-Text
Keywords: congenital long QT syndrome; Jervell and Lange-Nielsen syndrome; genetics; ventricular arrhythmia; seizures; beta-blocker congenital long QT syndrome; Jervell and Lange-Nielsen syndrome; genetics; ventricular arrhythmia; seizures; beta-blocker
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MDPI and ACS Style

Levaux, J.; Farhat, N.; Van Casteren, L.; Bulk, S.; Seghaye, M.-C. Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy. Clin. Pract. 2022, 12, 677-685. https://doi.org/10.3390/clinpract12050070

AMA Style

Levaux J, Farhat N, Van Casteren L, Bulk S, Seghaye M-C. Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy. Clinics and Practice. 2022; 12(5):677-685. https://doi.org/10.3390/clinpract12050070

Chicago/Turabian Style

Levaux, Joachim, Nesrine Farhat, Lieve Van Casteren, Saskia Bulk, and Marie-Christine Seghaye. 2022. "Cerebral Seizures in an Adolescent with Jervell and Lange-Nielsen Syndrome: It May Not Be Epilepsy" Clinics and Practice 12, no. 5: 677-685. https://doi.org/10.3390/clinpract12050070

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