Review articleDravet syndrome or genetic (generalized) epilepsy with febrile seizures plus?
Section snippets
Historical perspective
Charlotte Dravet first described Dravet syndrome in 1978 when she identified a group of children with seizures beginning in infancy, early normal development and a devastating course with ongoing seizures and cognitive decline [1]. She recognized that these children presented with episodes of status epilepticus triggered by fever and could be readily discerned from children presenting with other forms of symptomatic generalized epilepsy such as Lennox–Gastaut syndrome. Dravet observed that
Classical Dravet syndrome (or severe myoclonic epilepsy of infancy)
Dravet syndrome comprises a complex phenotype incorporating factors such as age of onset of specific seizure types, a distinctive developmental course and characteristic EEG features. The diagnosis is like a puzzle where the clinician needs to combine all the pieces together to determine if the child has this specific electro clinical syndrome.
In Dravet syndrome, a previously well and normally developing infant of around 6 months of age presents with febrile status epilepticus [12]. This may be
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Genetic therapeutic advancements for Dravet Syndrome
2022, Epilepsy and BehaviorThe evolving genetic landscape of febrile seizures and GEFS +
2022, Febrile Seizures: New Concepts and ConsequencesNew avenues in molecular genetics for the diagnosis and application of therapeutics to the epilepsies
2021, Epilepsy and Behavior