Journal Article10.1016/J.HRTHM.2013.10.010
Prevalence and spectrum of electroencephalogram-identified epileptiform activity among patients with long QT syndrome.
TL;DR: While the overall prevalence of epilepsy among patients with LQTS is low, 10 of 68 of the patients who presented with seizures/seizure-like episodes had EEG-identified epileptiform activity, further supporting the shared pathogenetic link hypothesis of this KCNH2-encoded potassium channel that is expressed in both the heart and the brain.
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About: This article is published in Heart Rhythm. The article was published on 01 Jan 2014. The article focuses on the topics: Epilepsy & Epileptologist.
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Citations
Sudden unexpected death in epilepsy: epidemiology, mechanisms, and prevention.
TL;DR: Because generalised tonic-clonic seizures precede most cases of SUDEP, patients must be better educated about prevention and the value of nocturnal monitoring to detect seizures and postictal stimulation is unproven but warrants further study.
585
Mechanisms of sudden unexpected death in epilepsy: the pathway to prevention.
TL;DR: Evidence for the mechanisms that cause cardiac, respiratory and arousal abnormalities during the ictal and postictal period is summarized and potential cellular mechanisms underlying these abnormalities are highlighted, such as a defect in the serotonergic system, ICTal adenosine release, and changes in autonomic output.
The heart of epilepsy: Current views and future concepts
Sharon Shmuely,M. van der Lende,Robert J. Lamberts,Josemir W. Sander,Roland D. Thijs,Roland D. Thijs +5 more
TL;DR: The fascinating borderland of epilepsy and cardiovascular conditions is discussed, focusing on epidemiology, clinical presentations and possible mechanisms for shared pathophysiology, and a call for validated screening instruments and guidelines aiding the early identification and treatment of CV comorbidity in epilepsy.
114
Sudden unexpected death in epilepsy: The neuro-cardio-respiratory connection
TL;DR: Neuroimaging and molecular genetic studies may provide insights into the causes of SUDEP and identify potential biomarkers for risk stratification of patients susceptible to SUDEP.
88
Genetic Basis of Sudden Unexpected Death in Epilepsy
Richard D. Bagnall,Richard D. Bagnall,Douglas E. Crompton,Douglas E. Crompton,Christopher Semsarian,Christopher Semsarian,Christopher Semsarian +6 more
TL;DR: A mounting body of evidence is highlighted for the involvement of genetic risk factors in SUDEP, including respiratory dysfunction, cardiac arrhythmia and postictal generalized electroencephlogram suppression.
References
A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome
Mark E. Curran,Igor Splawski,Katherine W. Timothy,Vincent Gm,Eric D. Green,Keating Mt,Keating Mt +6 more
TL;DR: In this article, the authors investigated patients with long QT syndrome (LQT), an inherited disorder causing sudden death from a ventricular tachyarrythmia, torsade de pointes.
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Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias.
Qing Wang,Mark E. Curran,Igor Splawski,Timothy C. Burn,J. M. Millholland,T. J. VanRaay,Jiaxiang Shen,Katherine W. Timothy,Vincent Gm,Vincent Gm,T. De Jager,Peter J. Schwartz,J.A. Towbin,Arthur J. Moss,Donald L. Atkinson,Gregory M. Landes,Timothy D. Connors,M T Keating +17 more
TL;DR: In this article, positional cloning was used to establish KVLQT1 as the chromosome 11-linked LQT 1 gene responsible for the most common inherited cardiac arrhythmia.
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SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome
Qing Kenneth Wang,Jiaxiang Shen,Jiaxiang Shen,Igor Splawski,Donald L. Atkinson,Donald L. Atkinson,Zhizhong Li,Jennifer L. Robinson,Arthur J. Moss,Jeffrey A. Towbin,Mark T. Keating +10 more
TL;DR: Genetic linkage between LQT3 and polymorphisms within SCN5A, the cardiac sodium channel gene, and single strand conformation polymorphism and DNA sequence analyses suggest that mutations in SCN 5A cause chromosome 3-linked LQt and indicate a likely cellular mechanism for this disorder.
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Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death
Anton Jervell,Fred Lange-Nielsen +1 more
TL;DR: Three deaf-mute children all suffered attacks of fainting, probably Adams-Stokes seizures caused by standstill of the heart, and 3 of the children died in such attacks at the ages of 4, 5, and 9 years, respectively.
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hERG K+ Channels: Structure, Function, and Clinical Significance
TL;DR: The human ether-a-go-go related gene (hERG) encodes the pore-forming subunit of the rapid component of the delayed rectifier K(+) channel, Kv11.1, which is the gene product involved in chromosome 7-associated long QT syndrome (LQTS), an inherited disorder associated with a markedly increased risk of ventricular arrhythmias and sudden cardiac death.
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