Dravet Syndrome

Dravet syndrome is a rare and severe form of epilepsy. Severe myoclonic epilepsy in infancy was described for the first time by Charlotte Dravet in 1978 in Marseille. Common characteristics were observed, such as it appearing during the first year of life, sensitivity to fever, various types of seizures particularly myoclonic, and mental retardation. The following descriptions then led to the recognition of a genuine epileptic syndrome. Renzo Guerrini contributed to the understanding of its genetic factors and its response to antiepileptic medication. New drugs have emerged since and studies on cognitive disorders have enabled to determine the delayed development. The authors offer a summary of Dravet syndrome, and in particular, a description of the electroclinical characteristics that will help towards a better diagnosis. The different treatment options are illustrated in the work, which also provides an update on the genetics. While a large number of questions remain on the understanding of this syndrome, this monograph is an essential tool for practitioners to improve the management of children with epilepsy.