Seizures and arrhythmias

  title={Seizures and arrhythmias},
  author={Jill V. Hunter and Arthur J. Moss},
  pages={208 - 209}
The human ether-a-go-go–related gene ( HERG , now known as KCNH2 ) encodes an ion channel subunit IKr, which subserves a potassium current that repolarizes human heart ventricular cells. Mutations in KCNH2 cause type 2 long QT syndrome (LQT2) by disrupting IKr, increasing cardiac excitability, and even triggering the catastrophic arrhythmia torsades de pointes (TdP),1,2 ventricular tachyarrhythmias, and sudden death. In syncope related to ventricular tachyarrhythmias, loss of consciousness is… Expand
Ryanodine Calcium Channel: A Novel Channelopathy for Seizures
This episode of unresponsiveness in an 18-year-old white male with a history of mild developmental delay and CPVT may have been caused by the same mutations known to precipitate CPVT. Expand
Supraventricular Tachycardia During Status Epilepticus in Dravet Syndrome: A Link Between Brain and Heart?
A 5-month-old girl with Dravet syndrome who presented with paroxysmal supraventricular tachycardia during status epilepticus supports the hypothesis that SCN1A mutation might have a role as a common substrate to both epilepsy and cardiac arrhythmia. Expand
Prevalence and spectrum of electroencephalogram-identified epileptiform activity among patients with long QT syndrome.
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. Expand
Concealed long QT syndrome and intractable partial epilepsy: a case report.
The importance of avoiding "tunnel vision" as patients with long QT syndrome could also have a primary seizure disorder, and it is theorized that abnormal KCHN2-encoded potassium channel repolarization in the brain could result in epilepsy and arrhythmias in longQT syndrome. Expand
Diagnostic value of electrocardiogram during routine electroencephalogram
The frequency of EKG abnormalities captured during routine EEGs was high, with Afib being the most significant, and neurologists are suggested to become more vigilant to EKGs recorded during routineEEG as such findings might have diagnostic and therapeutic implications. Expand
Since the discovery of ion channels in 1970s, the aetiology of many diseases has been traced back to channelopathies. Many toxins produced by snakes, fish, spiders and other insects paralyse the ionExpand
[KCNH2 gene new mutation in a patient with prior diagnosis of epilepsy].
Les HM épileptiques sont exceptionelles et peuvent résulter de lésions temporales ou temporonsulaires, aussi bien droites que gauches. Expand
Ion channels are key components in a wide variety of biological processes that involve rapid changes in cells, such as cardiac, skeletal, and smooth muscle contraction, epithelial transport of nutrients and ions, T-cell activation and pancreatic beta-cell insulin release. Expand
Les canalopathies potassiques, autour du syndrome de Morvan
La maladie decrite en 1890 par Morvan a subi une surprenante expansion en raison notamment d’une etroite parente avec diverses canalopathies potassiques a l’origine de convulsions neonatales familiales benignes. Expand
Detecting pore-lining regions in transmembrane protein sequences
This work presents a method capable of automatically identifying pore-lining regions in transmembrane proteins from sequence information alone, which can be used to determine the pore stoichiometry, and presents the results of applying it to a data set of sequences with available crystal structures. Expand


Long QT syndrome: from channels to cardiac arrhythmias.
Understanding of the mechanisms that control the critical plateau and repolarization phases of the human ventricular action potential has been raised to new levels through these studies, which have clarified the manner in which both potassium and sodium channels regulate this critical period of electrical activity. Expand
Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy
A diagnostic consideration of epilepsy and treatment with antiepileptic drug medications was more common in patients with LQT2, raising the possibility that L QT2-causing perturbations in the KCNH2-encoded potassium channel may confer susceptibility for recurrent seizure activity. Expand
Phenytoin and phenobarbital inhibit human HERG potassium channels
Collective data show that drugs with the same margins (ratio HERG IC50/unbound therapeutic concentration), as PHT and PB, may have arrhythmogenic potential, especially when used in predisposed patients and in the case of drug-drug interactions. Expand
Drug-induced torsades de pointes: the evolving role of pharmacogenetics.
The present strategy of identifying " at-risk compounds" and the potential future strategy involving pharmacogenetics to pinpoint "at-risk hosts" in an effort to curb this rare, unintended, but potentially life-threatening side effect are focused on. Expand
The promiscuous nature of the cardiac sodium current.
An overview of the evidence for the presence of TTX-sensitive Na(V) isoforms in the heart is given and the hypothesis brought forward so far for their direct role in cardiac function is presented, demonstrating the promiscuous nature of the cardiac sodium current at the molecular level. Expand
Structure and function of Kv4-family transient potassium channels.
A surprisingly large number of ancillary subunits and scaffolding proteins that can interact with the primary subunits of Shal-type K(+) channels, resulting in alterations in channel trafficking and kinetic properties are described. Expand
K(v)7 channels: function, pharmacology and channel modulators.
An account on the focused efforts to develop selective modulators, openers as well as blockers, of the K(v)7 channel subtypes, which have been undertaken during recent years are given, along with a discussion of theK(v),7 ion channel physiology and therapeutic indications for modulators of the neuronal K( v)7 channels. Expand
Epilepsy: Getting to the heart of the matter
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  • 2009
Epilepsy : Getting to the heart of the matter . European Paediatrics Review
  • 2007
Structure and function of Kv 4family transient potassium channels
  • Physiol Rev
  • 2004