Research & Literature

Efficacy of antiepileptic drugs for the treatment of Dravet syndrome with different genotypes.

Shi XY, Tomonoh Y, Wang WZ, et al.

Brain & development 2016; (38(1)):40-6.

[Analysis of parental origin of de novo SCN1A mutations in Dravet syndrome].

Sun H, Zhang Y, Xu X, et al.

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 2015; (32(4)):457-61 doi:10.3760/cma.j.issn.1003-9406.2015.04.001.

A population-based post mortem study of sudden unexpected death in epilepsy.

Clark D, Riney K

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2016; (23()):58-62 doi:10.1016/j.jocn.2015.04.027.

Incidence of Dravet Syndrome in a US Population.

Wu YW, Sullivan J, McDaniel SS, et al.

Pediatrics 2015; (136(5)):e1310-5 doi:10.1542/peds.2015-1807.

Pharmacological considerations in the use of stiripentol for the treatment of epilepsy.

Verrotti A, Prezioso G, Stagi S, et al.

Expert opinion on drug metabolism & toxicology 2016; (12(3)):345-52 doi:10.1517/17425255.2016.1145657.

Pharmacotherapy for Dravet Syndrome.

Wallace A, Wirrell E, Kenney-Jung DL

Paediatric drugs 2016; (18(3)):197-208 doi:10.1007/s40272-016-0171-7.

Prevalence and Characteristics of Vaccination Triggered Seizures in Dravet Syndrome in Hong Kong: A Retrospective Study.

Wong PT, Wong VC

Pediatric neurology 2016; (58()):41-7.

Dravet Syndrome: Diagnosis and Long-Term Course.

Connolly MB

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2016; (43 Suppl 3()):S3-8 doi:10.1017/cjn.2016.243.

A deleterious Nav1.1 mutation selectively impairs telencephalic inhibitory neurons derived from Dravet Syndrome patients.

Sun Y, Paşca SP, Portmann T, et al.

eLife 2016; (5()).

Mortality in Dravet syndrome: A review.

Shmuely S, Sisodiya SM, Gunning WB, et al.

Epilepsy & behavior : E&B 2016; (64(Pt A)):69-74 doi:10.1016/j.yebeh.2016.09.007.

Mortality in Dravet syndrome.

Cooper MS, Mcintosh A, Crompton DE, et al.

Epilepsy research 2016; (128()):43-47 doi:10.1016/j.eplepsyres.2016.10.006.

From genotype to phenotype in Dravet disease.

Gataullina S, Dulac O

Seizure 2017; (44()):58-64 doi:10.1016/j.seizure.2016.10.014.

Use of social media to assess the effectiveness of vagal nerve stimulation in Dravet syndrome: A caregiver's perspective.

Ali R, Elsayed M, Kaur M, et al.

Journal of the neurological sciences 2017; (375()):146-149 doi:10.1016/j.jns.2017.01.057.

Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy.

Brigo F, Igwe SC, Bragazzi NL

The Cochrane database of systematic reviews 2017; (5()):CD010483 doi:10.1002/14651858.CD010483.pub4.

Trial of Cannabidiol for Drug-Resistant Seizures in the Dravet Syndrome.

Devinsky O, Cross JH, Laux L, et al.

The New England journal of medicine 2017; (376(21)):2011-2020 doi:10.1056/NEJMoa1611618.

Anticonvulsant effect of cannabidiol in the pentylenetetrazole model: Pharmacological mechanisms, electroencephalographic profile, and brain cytokine levels.

Vilela LR, Lima IV, Kunsch ÉB, et al.

Epilepsy & behavior : E&B 2017; (75()):29-35 doi:10.1016/j.yebeh.2017.07.014.

Cannabidiol attenuates seizures and social deficits in a mouse model of Dravet syndrome.

Kaplan JS, Stella N, Catterall WA, Westenbroek RE

Proceedings of the National Academy of Sciences of the United States of America 2017; (114(42)):11229-11234 doi:10.1073/pnas.1711351114.

Treatment Strategies for Dravet Syndrome.

Knupp KG, Wirrell EC

CNS drugs 2018; (32(4)):335-350 doi:10.1007/s40263-018-0511-y.

Influence of contraindicated medication use on cognitive outcome in Dravet syndrome and age at first afebrile seizure as a clinical predictor in SCN1A-related seizure phenotypes.

de Lange IM, Gunning B, Sonsma ACM, et al.

Epilepsia 2018; (59(6)):1154-1165 doi:10.1111/epi.14191.

Nocturnal supervision and SUDEP risk at different epilepsy care settings.

van der Lende M, Hesdorffer DC, Sander JW, Thijs RD

Neurology 2018; (91(16)):e1508-e1518 doi:10.1212/WNL.0000000000006356.

Current Treatment Strategies and Future Treatment Options for Dravet Syndrome.

Ziobro J, Eschbach K, Sullivan JE, Knupp KG

Current treatment options in neurology 2018; (20(12)):52 doi:10.1007/s11940-018-0537-y.

A two-hit story: Seizures and genetic mutation interaction sets phenotype severity in SCN1A epilepsies.

Salgueiro-Pereira AR, Duprat F, Pousinha PA, et al.

Neurobiology of disease 2019; (125()):31-44 doi:10.1016/j.nbd.2019.01.006.

A Phase 1, Open-Label, Pharmacokinetic Trial to Investigate Possible Drug-Drug Interactions Between Clobazam, Stiripentol, or Valproate and Cannabidiol in Healthy Subjects.

Morrison G, Crockett J, Blakey G, Sommerville K

Clinical pharmacology in drug development 2019; (8(8)):1009-1031 doi:10.1002/cpdd.665.

The Ketogenic and Modified Atkins Diet Therapy for Children With Refractory Epilepsy of Genetic Etiology.

Jagadish S, Payne ET, Wong-Kisiel L, et al.

Pediatric neurology 2019; (94()):32-37 doi:10.1016/j.pediatrneurol.2018.12.012.

Autism spectrum disorder and cognitive profile in children with Dravet syndrome: Delineation of a specific phenotype.

Ouss L, Leunen D, Laschet J, et al.

Epilepsia open 2019; (4(1)):40-53 doi:10.1002/epi4.12281.

Stiripentol: A Novel Antiseizure Medication for the Management of Dravet Syndrome.

Buck ML, Goodkin HP

The Annals of pharmacotherapy 2019; (53(11)):1136-1144 doi:10.1177/1060028019856008.

Association of quality of paediatric epilepsy care with mortality and unplanned hospital admissions among children and young people with epilepsy in England: a national longitudinal data linkage study.

Hargreaves DS, Arora S, Viveiro C, et al.

The Lancet. Child & adolescent health 2019; (3(9)):627-635 doi:10.1016/S2352-4642(19)30201-9.

Dravet Syndrome: An Overview.

Anwar A, Saleem S, Patel UK, et al.

Cureus 2019; (11(6)):e5006 doi:10.7759/cureus.5006.

Recent Advances in the Drug Treatment of Dravet Syndrome.

Wirrell EC, Nabbout R

CNS drugs 2019; (33(9)):867-881 doi:10.1007/s40263-019-00666-8.

Sudden unexpected death in epilepsy. A nationwide population-based study.

Einarsdottir AB, Sveinsson O, Olafsson E

Epilepsia 2019; (60(11)):2174-2181 doi:10.1111/epi.16349.

Fenfluramine for Treatment-Resistant Seizures in Patients With Dravet Syndrome Receiving Stiripentol-Inclusive Regimens: A Randomized Clinical Trial.

Nabbout R, Mistry A, Zuberi S, et al.

JAMA neurology 2020; (77(3)):300-308 doi:10.1001/jamaneurol.2019.4113.

Fenfluramine hydrochloride for the treatment of seizures in Dravet syndrome: a randomised, double-blind, placebo-controlled trial.

Lagae L, Sullivan J, Knupp K, et al.

Lancet (London, England) 2019; (394(10216)):2243-2254 doi:10.1016/S0140-6736(19)32500-0.

Dravet syndrome: Early electroclinical findings and long-term outcome in adolescents and adults.

Darra F, Battaglia D, Dravet C, et al.

Epilepsia 2019; (60 Suppl 3()):S49-S58 doi:10.1111/epi.16297.

Predicting the impact of sodium channel mutations in human brain disease.

Noebels JL

Epilepsia 2019; (60 Suppl 3()):S8-S16 doi:10.1111/epi.14724.

Cardiac arrhythmias in Dravet syndrome: an observational multicenter study.

Shmuely S, Surges R, Helling RM, et al.

Annals of clinical and translational neurology 2020; (7(4)):462-473 doi:10.1002/acn3.51017.

Electrophysiological Alterations of Pyramidal Cells and Interneurons of the CA1 Region of the Hippocampus in a Novel Mouse Model of Dravet Syndrome.

Dyment DA, Schock SC, Deloughery K, et al.

Genetics 2020; (215(4)):1055-1066 doi:10.1534/genetics.120.303399.

Cannabidiol anticonvulsant effect is mediated by the PI3Kγ pathway.

Lima IVA, Bellozi PMQ, Batista EM, et al.

Neuropharmacology 2020; (176()):108156 doi:10.1016/j.neuropharm.2020.108156.

Efficacy of the ketogenic diet in patients with Dravet syndrome: A meta-analysis.

Wang YQ, Fang ZX, Zhang YW, et al.

Seizure 2020; (81()):36-42 doi:10.1016/j.seizure.2020.07.011.

Therapeutic advances in Dravet syndrome: a targeted literature review.

Strzelczyk A, Schubert-Bast S

Expert review of neurotherapeutics 2020; (20(10)):1065-1079 doi:10.1080/14737175.2020.1801423.

Cardiovascular safety of fenfluramine in the treatment of Dravet syndrome: Analysis of an ongoing long-term open-label safety extension study.

Lai WW, Galer BS, Wong PC, et al.

Epilepsia 2020; (61(11)):2386-2395 doi:10.1111/epi.16638.

Cognitive, behavioral, and social functioning in children and adults with Dravet syndrome.

Brown A, Arpone M, Schneider AL, et al.

Epilepsy & behavior : E&B 2020; (112()):107319 doi:10.1016/j.yebeh.2020.107319.

Convulsive seizures and some behavioral comorbidities are uncoupled in the Scn1aA1783V Dravet syndrome mouse model.

Fadila S, Quinn S, Turchetti Maia A, et al.

Epilepsia 2020; (61(10)):2289-2300 doi:10.1111/epi.16662.

Efficacy and safety of Fenfluramine hydrochloride for the treatment of seizures in Dravet syndrome: A real-world study.

Specchio N, Pietrafusa N, Doccini V, et al.

Epilepsia 2020; (61(11)):2405-2414 doi:10.1111/epi.16690.

Foot-floor contact pattern in children and adults with Dravet Syndrome.

Wyers L, Di Marco R, Zambelli S, et al.

Gait & posture 2021; (84()):315-320 doi:10.1016/j.gaitpost.2020.12.030.

The probability of sudden unexpected death in epilepsy given postictal prone position.

Esmaeili B, Dworetzky BA, Glynn RJ, Lee JW

Epilepsy & behavior : E&B 2021; (116()):107775 doi:10.1016/j.yebeh.2021.107775.

Deconstructing Dravet syndrome neurocognitive development: A scoping review.

Bertuccelli M, Verheyen K, Hallemans A, et al.

Epilepsia 2021; (62(4)):874-887 doi:10.1111/epi.16844.

Risk of SUDEP during infancy.

Trivisano M, Muccioli L, Ferretti A, et al.

Epilepsy & behavior : E&B 2022; (131(Pt B)):107896 doi:10.1016/j.yebeh.2021.107896.

Oxygen for seizures, more questions than answers: A scoping review.

Smith AN, Abraham J, Shankar R

Acta neurologica Scandinavica 2021; (144(6)):719-729 doi:10.1111/ane.13508.

Sudden Unexpected Death in Epilepsy (SUDEP): A Review of Risk Factors and Possible Interventions in Children.

Wicker E, Cole JW

The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2021; (26(6)):556-564 doi:10.5863/1551-6776-26.6.556.

International consensus on the use of [18F]-FDG PET/CT in pediatric patients affected by epilepsy.

Tian M, Watanabe Y, Kang KW, et al.

European journal of nuclear medicine and molecular imaging 2021; (48(12)):3827-3834 doi:10.1007/s00259-021-05524-8.

Parental experiences and perspectives on the value of seizure detection while caring for a child with epilepsy: A qualitative study.

van Westrhenen A, de Lange WFM, Hagebeuk EEO, et al.

Epilepsy & behavior : E&B 2021; (124()):108323 doi:10.1016/j.yebeh.2021.108323.

Serotonin 5-HT4 receptors play a critical role in the action of fenfluramine to block seizure-induced sudden death in a mouse model of SUDEP.

Tupal S, Faingold CL

Epilepsy research 2021; (177()):106777 doi:10.1016/j.eplepsyres.2021.106777.

Impact of fenfluramine on the expected SUDEP mortality rates in patients with Dravet syndrome.

Cross JH, Galer BS, Gil-Nagel A, et al.

Seizure 2021; (93()):154-159 doi:10.1016/j.seizure.2021.10.024.

SCN1A Mutation-Beyond Dravet Syndrome: A Systematic Review and Narrative Synthesis.

Ding J, Li X, Tian H, et al.

Frontiers in neurology 2021; (12()):743726 doi:10.3389/fneur.2021.743726.

Development and Validation of a Prediction Model for Early Diagnosis of SCN1A-Related Epilepsies.

Brunklaus A, Pérez-Palma E, Ghanty I, et al.

Neurology 2022; (98(11)):e1163-e1174 doi:10.1212/WNL.0000000000200028.

Serotonin receptors in epilepsy: Novel treatment targets?

Sourbron J, Lagae L

Epilepsia open 2022; (7(2)):231-246 doi:10.1002/epi4.12580.

Pre-hospital and emergency department treatment of convulsive status epilepticus in adults: an evidence synthesis.

Cruickshank M, Imamura M, Booth C, et al.

Health technology assessment (Winchester, England) 2022; (26(20)):1-76 doi:10.3310/RSVK2062.

Tauopathy and Epilepsy Comorbidities and Underlying Mechanisms.

Hwang K, Vaknalli RN, Addo-Osafo K, et al.

Frontiers in aging neuroscience 2022; (14()):903973 doi:10.3389/fnagi.2022.903973.

Genetic Landscape of SCN1A Variants in a Turkish Cohort with GEFS+ Spectrum and Dravet Syndrome.

Türkyılmaz A, Tekin E, Yaralı O, Çebi AH

Molecular syndromology 2022; (13(4)):270-281 doi:10.1159/000521330.

Two Perspectives on Dravet Syndrome: Viewpoints from the Clinician and the Caregiver.

Bluvstein J, Wenniger S

Neurology and therapy 2023; (12(2)):343-350 doi:10.1007/s40120-023-00450-3.

Investigational new drugs for the treatment of Dravet syndrome: an update.

Janković SM, Janković SV, Vojinović R, Lukić S

Expert opinion on investigational drugs 2023; (32(4)):325-331 doi:10.1080/13543784.2023.2193680.

Multimodal nocturnal seizure detection in children with epilepsy: A prospective, multicenter, long-term, in-home trial.

van Westrhenen A, Lazeron RHC, van Dijk JP, et al.

Epilepsia 2023; (64(8)):2137-2152 doi:10.1111/epi.17654.

Electroencephalography characteristics related to risk of sudden unexpected death in epilepsy in patients with Dravet syndrome.

Kim JY, Shin J, Kim L, Kim SH

Frontiers in neurology 2023; (14()):1222721 doi:10.3389/fneur.2023.1222721.

Diagnosis, epilepsy treatment and supports for neurodevelopment in children with Dravet Syndrome: Caregiver reported experiences and needs.

Soto Jansson J, Bjurulf B, Dellenmark Blom M, et al.

Epilepsy & behavior : E&B 2024; (151()):109603 doi:10.1016/j.yebeh.2023.109603.

A systematic literature review on the global epidemiology of Dravet syndrome and Lennox-Gastaut syndrome: Prevalence, incidence, diagnosis, and mortality.

Sullivan J, Benítez A, Roth J, et al.

Epilepsia 2024; (65(5)):1240-1263 doi:10.1111/epi.17866.

An Update on Stiripentol Mechanisms of Action: A Narrative Review.

Bacq A, Depaulis A, Castagné V, et al.

Advances in therapy 2024; (41(4)):1351-1371 doi:10.1007/s12325-024-02813-0.

Enhancing the action of serotonin by three different mechanisms prevents spontaneous seizure-induced mortality in Dravet mice.

Guo J, Min D, Farrell EK, et al.

Epilepsia 2024; (65(6)):1791-1800 doi:10.1111/epi.17966.

The influence of risk factors, biomarkers and care settings on SUDEP counseling.

Valdrighi A, Laze J, Farooque P, et al.

Epilepsy & behavior : E&B 2024; (156()):109845 doi:10.1016/j.yebeh.2024.109845.

Determinants of successful ictal SPECT injection in phase 1 epilepsy presurgical evaluation: Findings from the pediatric epilepsy research consortium surgery database project.

Joshi C, Singh R, Liu G, et al.

Epilepsia open 2024; (9(4)):1467-1479 doi:10.1002/epi4.12986.

Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review.

Specchio N, Auvin S, Strzelczyk A, et al.

Epilepsia open 2024; (9(6)):2017-2036 doi:10.1002/epi4.13036.

Eating habits and behaviors in children with Dravet syndrome: A case-control study.

Laliberté A, Siafa L, Soufi A, et al.

Epilepsia 2025; (66(1)):e1-e6 doi:10.1111/epi.18179.

Pediatric Epilepsy Management: Special Considerations.

Nickels K, Joshi C

Seminars in neurology 2025; (45(2)):189-197 doi:10.1055/a-2531-3610.

Emerging Genetic Therapies in Epilepsy.

Sullivan J, Hood V

Seminars in neurology 2025; (45(2)):298-302 doi:10.1055/a-2544-1530.

Spotlight on mechanism of sudden unexpected death in epilepsy in Dravet syndrome.

Shao W, Liu L, Gu J, et al.

Translational psychiatry 2025; (15(1)):84 doi:10.1038/s41398-025-03304-8.

Cenobamate in pediatric epilepsy and developmental and epileptic encephalopathies: Efficacy, safety, and syndrome-specific considerations.

Samanta D

Epilepsy & behavior : E&B 2025; (173()):110787 doi:10.1016/j.yebeh.2025.110787.

Dravet syndrome diagnosed in adults.

Dudley AM, Peña-Ceballos J, El-Naggar H, et al.

Practical neurology 2026; (26(2)):124-132 doi:10.1136/pn-2025-004743.

Adult-Onset Neurological Deterioration in Dravet Syndrome Associated With a Novel SCN1A Missense Variant (p.Gly1371Asp): A Case Report.

Yokoyama K, Miyazaki S, Murayama K, et al.

Cureus 2025; (17(9)):e93228 doi:10.7759/cureus.93228.

Status Epilepticus.

Siegel CR, Khoujah D

Emergency medicine clinics of North America 2026; (44(1)):187-202 doi:10.1016/j.emc.2025.08.011.

Long-term seizure reduction with vagus nerve stimulation in Dravet syndrome.

Bajaj S, Ivaniuk A, Bruenger T, et al.

Developmental medicine and child neurology 2026; doi:10.1111/dmcn.70127.