scholarly journals Sudden unexpected death in Dravet syndrome: Respiratory and other physiological dysfunctions

2013 ◽  
Vol 189 (2) ◽  
pp. 324-328 ◽  
Author(s):  
Franck Kalume
Keyword(s):  
Sudden Unexpected Death ◽  
Dravet Syndrome ◽  
Unexpected Death

scholarly journals Sudden unexpected death in a mouse model of Dravet syndrome

2013 ◽  
Vol 123 (4) ◽  
pp. 1798-1808 ◽  
Author(s):  
Franck Kalume ◽  
Ruth E. Westenbroek ◽  
Christine S. Cheah ◽  
Frank H. Yu ◽  
John C. Oakley ◽  
...  
Keyword(s):  
Mouse Model ◽  
Sudden Unexpected Death ◽  
Dravet Syndrome ◽  
Unexpected Death

scholarly journals Sudden unexpected death in genetic epileptic encephalopathies: a role of neurocardiac genes

2018 ◽  
Vol 10 (3) ◽  
pp. 63-70
Author(s):  
E. D. Belousova ◽  
M. A. Shkolnikova
Keyword(s):  
High Risk ◽  
Sudden Unexpected Death ◽  
Dravet Syndrome ◽  
Unexpected Death ◽  
Sodium Potassium ◽  
Epileptic Encephalopathies ◽  
Increased Risk ◽  
Clinical Conditions ◽  
Cultured Cardiomyocytes

It is well known that sudden unexpected death in epilepsy (SUDEP) is one of the most significant factors of mortality in epileptic patients. There is an increased risk of SUDEP in genetic epileptic encephalopathies (EE), partly because those syndromes are associated with mutations in the “neurocardiac” genes, which have been implicated in both epilepsy and cardiac arrhythmias. In these clinical conditions, functions of ion selective channels (sodium, potassium and etc.) are affected; for example, in children with Dravet syndrome, the risk of SUDEP is 40 times higher than that in children with common epilepsy syndromes. In a murine model of SCN1A epilepsy, a prolongation of QT interval coincided with a seizure; in addition, an excessive excitability of cultured cardiomyocytes was demonstrated. A high risk of SUDEP is characteristic for EE caused by mutation in the SCN8A gene. Other prognostic biomarkers of SUDEP may include mutations in sodium channel genes, such as SCN4A, SCN10A, and SCN11A. Our knowledge about SUDEP associated with potassium channel dysfunctions is still very limited. There are likely some mutations in other genes, that can modify (increase or decrease) the risk of SUDEP in EE. If patients with genetic EE are indeed at a high risk for SUDEP, they must be followed up by cardiologists alongside with neurologists. Provided this hypothesis is proved, any newly diagnosed arrhythmia should be carefully monitored and treated (with medications and/or interventions), in order to improve the survival rate in genetic EE.

Two autopsy cases of sudden unexpected death from Dravet syndrome with novel de novo SCN1A variants

2020 ◽  
Vol 42 (2) ◽  
pp. 171-178 ◽  
Author(s):  
Yukiko Hata ◽  
Yuko Oku ◽  
Hiromichi Taneichi ◽  
Tomomi Tanaka ◽  
Noboru Igarashi ◽  
...  
Keyword(s):  
De Novo ◽  
Sudden Unexpected Death ◽  
Dravet Syndrome ◽  
Unexpected Death

scholarly journals Effect of Vagus Nerve Stimulation in an Adult Patient with Dravet Syndrome: Contribution to Sudden Unexpected Death in Epilepsy Risk Reduction?

2013 ◽  
Vol 69 (2) ◽  
pp. 119-121 ◽  
Author(s):  
Marianna Spatola ◽  
Pierre-Yves Jeannet ◽  
Claudio Pollo ◽  
Christian Wider ◽  
Robyn Labrum ◽  
...  
Keyword(s):  
Vagus Nerve ◽  
Adult Patient ◽  
Risk Reduction ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Sudden Unexpected Death ◽  
Dravet Syndrome ◽  
Unexpected Death

ECG-Patterns of Focal Epileptic Seizures, the Role in Sudden Unexpected Death in Epilepsy: A Prospective Long-Term Study

2019 ◽  
Author(s):  
Svetlana Serdyuk ◽  
Karapet V. Davtyan ◽  
Sergey G. Burd ◽  
Oksana M. Drapkina ◽  
Sergey A. Boytsov ◽  
...  
Keyword(s):  
Epileptic Seizures ◽  
Sudden Unexpected Death ◽  
Unexpected Death ◽  
Term Study ◽  
Long Term Study
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