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Review
. 2025 Jul 1;34(3):125-132.
doi: 10.4103/ant.ANT-D-25-00005. Epub 2025 Sep 29.

Dravet Syndrome: Past, Present, and Future

Yi-Hsuan Liu  1 , Cheng-Yen Kuo  1 , I-Ching Chou  2 , Ting-Rong Hsu  3 , Kuang-Lin Lin  1   4 ; Society for Neurological Rare Disorders-Taiwan
Affiliations
Review

Dravet Syndrome: Past, Present, and Future

Yi-Hsuan Liu et al. Acta Neurol Taiwan. .

Abstract

Dravet syndrome (DS) is a severe and rare developmental epileptic encephalopathy and genetic epilepsy characterized by the onset of seizures before 1 year of age, typically prolonged generalized tonic-clonic or hemiclonic seizures, specific triggers such as vaccination, high body temperature, or photic stimulation, and normal development before seizure onset. It was proposed in 1989 and confirmed as a genetic epilepsy in 2001 due to SCN1A gene mutations. The seizures are pharmacoresistant, and the prognosis is poor. Comorbidities include movement disorders, intellectual disabilities, behavioral and psychiatric issues, and a high risk of sudden unexpected death in epilepsy (SUDEP). Despite significant advances in understanding the natural course of DS and its pathophysiological mechanisms, management remains difficult. Current treatments, including various antiseizure medications and neuromodulation therapies, rarely achieve complete seizure freedom and fail to prevent cognitive decline. The review aims to summarize the advancements in diagnosis, optimal management, and the prognosis of DS into adulthood while discussing the ongoing challenges, unresolved needs, and potential areas for future research. In addition, it highlights the importance of early intervention, prognostic factors, and the critical need for effective management strategies beyond seizure control, encompassing cognitive preservation, SUDEP prevention, and addressing sleep problems.

Keywords: Development epileptic encephalopathy; Dravet syndrome; epilepsy; pharmacoresistant epilepsy; seizure management.

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References

REFERENCES

    1. Poke G, Stanley J, Scheffer IE, Sadleir LG. Epidemiology of developmental and epileptic encephalopathy and of intellectual disability and epilepsy in children. Neurology 2023;100:e1363–75.
    1. Claes L, Del-Favero J, Ceulemans B, Lagae L, Van Broeckhoven C, De Jonghe P. De novo mutations in the sodium-channel gene SCN1A cause severe myoclonic epilepsy of infancy. Am J Hum Genet 2001;68:1327–32.
    1. Depienne C, Trouillard O, Saint-Martin C, Gourfinkel-An I, Bouteiller D, Carpentier W, et al. Spectrum of SCN1A gene mutations associated with Dravet syndrome: Analysis of 333 patients. J Med Genet 2009;46:183–91.
    1. Dravet C. Severe epilepsies of infancy. Vie Méd 1987;8:543–8.
    1. Ogino T, Ohtsuka Y, Amano R, Yamatogi Y, Ohtahara S. An investigation on the borderland of severe myoclonic epilepsy in infancy. Jpn J Psychiatry Neurol 1988;42:554–5.

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