Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Dec;60 Suppl 3:S39-S48.
doi: 10.1111/epi.16334.

Dravet Syndrome: Treatment Options and Management of Prolonged Seizures

Affiliations
Review

Dravet Syndrome: Treatment Options and Management of Prolonged Seizures

J Helen Cross et al. Epilepsia. .

Abstract

Over time, with careful delineation of Dravet syndrome, we have gained experience in treatments most likely to lead to improvement in seizures, as well as those that should be avoided. Sodium valproate, clobazam, stiripentol, and topiramate are all medications that may lead to benefit, as well as the ketogenic diet. Bromides may be utilized in resistant cases. However, equally important are outlining prompt rescue treatment for prolonged seizures and avoidance of precipitants. Newer agents including cannabidiol and fenfluramine have been demonstrated to be of benefit in clinical trials. We propose an algorithm for management, but appreciate that the positioning of newer agents is yet to be established.

Keywords: Dravet syndrome; antiepileptic drugs; cannabidiol; fenfluramine; stiripentol; treatment.

Similar articles

See all similar articles

Cited by 1 article

References

REFERENCES

    1. Catarino CB, Liu JYW, Liagkouras I, et al. Dravet syndrome as epileptic encephalopathy: evidence from very long-term course and neuropathology. Brain. 2011;134:2982-3010.
    1. Takayama R, Fujiwara T, Shigematsu H, et al. Long-term course of Dravet syndrome: a study from an epilepsy center in Japan. Epilepsia. 2014;55:528-38.
    1. Lagae L, Brambilla I, Mingorance A, Gibson E, Battersby A. Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey. Dev Med Child Neurol. 2018;60:63-72.
    1. Nabbout R, Chemaly N, Chipaux M, et al. Encephalopathy in children with Dravet syndrome is not a pure consequence of epilepsy. Orphanet J Rare Dis. 2012;8:176.
    1. Chiron C, Marchand MC, Tran A, et al. Stiripentol in severe myoclonic epilepsy in infancy: a randomised placebo-controlled syndrome-dedicated trial. STICLO study group. Lancet. 2000;356:1638-42.

MeSH terms

Feedback