severe myoclonic epilepsy in infancy (SMEI); Dravet syndrome

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Epidemiology

  • rare

Genetics

Clinical manifestations

Management

More general terms

References

  1. OMIM https://mirror.omim.org/entry/607208
  2. 2.0 2.1 Devinsky O, Cross JH, Laux L et al Trial of Cannabidiol for Drug-Resistant Seizures in the Dravet Syndrome. N Engl J Med 2017; 376:2011-2020. May 25, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28538134 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMoa1611618
    Berkovic SF Cannabinoids for Epilepsy - Real Data, at Last N Engl J Med 2017; 376:2075-2076. May 25, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28538129 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMe1702205
  3. 3.0 3.1 Fiore K Fenfluramine Works in Dravet Syndrome Seizure reductions seen for former weight-loss agent in phase III trial. MedPage Today. Dec 04, 2017 https://www.medpagetoday.com/meetingcoverage/aes/69638
    Lagae L, et al ZX008 (fenfluramine HCl oral solution) in Dravet syndrome: Results of a phase III randomized double-blind placebo- controlled trial American Epilepsy Society (AES) 2017; Abstract 2.434.
  4. George J Antisense Oligonucleotide Shows Potential in Dravet Syndrome. Investigational STK-001 appears well tolerated in early studies. MedPage Today December 9, 2022 https://www.medpagetoday.com/meetingcoverage/aes/102175

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