Susceptibility to seizure-induced sudden death in DBA/2 mice is altered by adenosine

Faingold, Carl L.; Randall, Marc; Kommajosyula, Srinivasa P.

doi:10.1016/j.eplepsyres.2016.05.007

Cited by 33 publications

(51 citation statements)

References 60 publications

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Section: Introductionmentioning

confidence: 99%

“…This is expected to maximize the severity of the AGS response. Priming can create susceptibility to AGS even in mouse strains that do not acutely respond to AGS …”

Section: Introductionmentioning

confidence: 99%

“…Animal models of sudden unexpected death in epileptic patients (SUDEP) have been valuable tools used to explore the underlying mechanisms of this phenomenon in hopes of treating and preventing death due to epilepsies. [1][2][3][4][5][6] Typically, mouse models of SUDEP use audiogenic seizures (AGS), induced with a sound stimulation, to induce SUDEP. 7 Immediately after the sound presentation, the mouse manifests a stereotyped behavior for which one can identify successively wild running, a clonic seizure, a tonic-clonic seizure when the mouse falls on its flanks, and a tonic seizure with an extension of the limbs toward the tail, followed, or not, by death.…”

Section: Introductionmentioning

confidence: 99%

“…13 AGS in DBA/1 mice are evident from postnatal days 21 to 100. 14 The vast majority of the AGS studies involve the DBA/2 inbred strain simply because (1) it is an AGS responder, and (2) it is a mouse strain that is well described genetically and phenotypically. Recently, DBA/1 has been highlighted as an alternate model of AGS for its larger time frame of susceptibility, despite the lack of data about this strain compared to the DBA/2 strain.…”

Section: Introductionmentioning

confidence: 99%

“…Priming can create susceptibility to AGS even in mouse strains that do not acutely respond to AGS. [2][3][4]16 To study pharmacological interventions for SUDEP in mouse models, one often gives the pharmacological intervention after the mouse has been resuscitated after cardiorespiratory arrest induced by an AGS and then tests the mouse for AGS response with the assumption that without the pharmacological intervention the mouse would have cardiorespiratory arrest after an AGS (ie, determinism). This concept of determinism, that the same innate response will occur in response to the same stimulus under the same conditions, is often applied to lethal (experimentally a seizure-induced respiratory arrest [SIRA]) and nonlethal AGS (non-SIRA), with the assumption that a SIRA is followed by a SIRA during the next test or a non-SIRA is followed by a non-SIRA during the next test.…”

Section: Introductionmentioning

confidence: 99%

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Audiogenic seizure as a model of sudden death in epilepsy: A comparative study between four inbred mouse strains from early life to adulthood

et al. 2020

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Objective Mouse models of sudden unexpected death in epileptic patients (SUDEP) using audiogenic seizures (AGS) are valuable because death can occur following a sound‐induced seizure in the absence of any pharmacologic or electric component. However, only a few strains of mice are AGS prone, and the vast majority of studies involve DBA/2 or DBA/1 inbred strains. With the goal of characterizing the variation of AGS susceptibility with age, and of offering a larger panel of mice available for AGS studies, we performed a comparative study of the variability in AGS responses. Methods The variation of AGS with age was determined in two classically used inbred strains of mice, DBA/2 and DBA/1, and two additional strains, BALB/c and 129/SvTer. As AGS‐stimulated tonic seizures can be lethal or nonlethal, even in the same inbred strain, in a second experiment, we addressed whether there is an innate capacity to reproduce the same response after a tonic AGS, referred to as “determinism,” in the DBA/2J, DBA/1J, and 129/SvTer mouse strains. Results Results show that the 129/SvTer mouse is a more versatile model of SUDEP due to its wider age range of susceptibility compared to the DBA/2J and DBA/1J mouse strains. In addition, we show that determinism is not consistently evident in DBA/2J and 129/SvTer strains after AGS. Hence, one cannot be certain that a lethal AGS will always be lethal in successive testing after resuscitation and vice versa in these two mouse strains. Significance These studies highlight the phenotypic variability of AGS in different mouse strains, show the value of an additional mouse strain, 129/SvTer, for studies using AGS, and thus provide valuable information for future studies of AGS and SUDEP.

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Section: Introductionmentioning

confidence: 99%

“…This is expected to maximize the severity of the AGS response. Priming can create susceptibility to AGS even in mouse strains that do not acutely respond to AGS …”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Audiogenic seizure as a model of sudden death in epilepsy: A comparative study between four inbred mouse strains from early life to adulthood

et al. 2020

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Postictal Death Is Associated with Tonic Phase Apnea in a Mouse Model of Sudden Unexpected Death in Epilepsy

Wenker

Teran

Wengert

et al. 2021

Annals of Neurology

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Objective: Sudden unexpected death in epilepsy (SUDEP) is an unpredictable and devastating comorbidity of epilepsy that is believed to be due to cardiorespiratory failure immediately after generalized convulsive seizures. Methods: We performed cardiorespiratory monitoring of seizure-induced death in mice carrying either a p.Arg1872Trp or p.Asn1768Asp mutation in a single Scn8a allele-mutations identified from patients who died from SUDEP-and of seizure-induced death in pentylenetetrazole-treated wild-type mice. Results: The primary cause of seizure-induced death for all mice was apnea, as (1) apnea began during a seizure and continued for tens of minutes until terminal asystole, and (2) death was prevented by mechanical ventilation. Fatal seizures always included a tonic phase that was coincident with apnea. This tonic phase apnea was not sufficient to produce death, as it also occurred during many nonfatal seizures; however, all seizures that were fatal had tonic phase apnea. We also made the novel observation that continuous tonic diaphragm contraction occurred during tonic phase apnea, which likely contributes to apnea by preventing exhalation, and this was only fatal when breathing did not resume after the tonic phase ended. Finally, recorded seizures from a patient with developmental epileptic encephalopathy with a previously undocumented SCN8A likely pathogenic variant (p.Leu257Val) revealed similarities to those of the mice, namely, an extended tonic phase that was accompanied by apnea. Interpretation: We conclude that apnea coincident with the tonic phase of a seizure, and subsequent failure to resume breathing, are the determining events that cause seizure-induced death in Scn8a mutant mice.

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Autonomic Involvement in Childhood Epilepsy

Parisi,

Sciruicchio,

D’Agnano

et al. 2023

Autonomic Disorders in Clinical Practice

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Susceptibility to seizure-induced sudden death in DBA/2 mice is altered by adenosine

Cited by 33 publications

References 60 publications

Audiogenic seizure as a model of sudden death in epilepsy: A comparative study between four inbred mouse strains from early life to adulthood

Audiogenic seizure as a model of sudden death in epilepsy: A comparative study between four inbred mouse strains from early life to adulthood

Postictal Death Is Associated with Tonic Phase Apnea in a Mouse Model of Sudden Unexpected Death in Epilepsy

Autonomic Involvement in Childhood Epilepsy

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