It acts as a GABAergic agent and as a positive allosteric modulator of GABAA receptor. Stiripentol, can improve focal refractory epilepsy, as well as Dravets syndrome, supplemented with clobazam and valproate was approved in Europe in 2007 as a therapy for Dravet syndrome and has been found to reduce overall seizure rate by 70%. In cases with more drug-resistant seizures, topiramate and the ketogenic diet are used as alternative treatments.Cannabidiol (CBD) was approved in United States for treatment of Dravet syndrome in 2018. A 2017 study showed that the frequency of seizures per month decreased from 12 to 6 with the use of cannabidiol, compared with a decrease from 15 to 14 with placebo.In 2020, fenfluramine was approved for the medical treatment in the European Union and the USA. Epidemiology
Dravet syndrome is a severe form of epilepsy, responsible for roughly 10% of cases in children. It is a rare genetic disorder that affects an estimated 1 in every 20,000–40,000 births. COVID-19
Although it is not clear whether people with Dravet syndrome are specially vulnerable to COVID-19 infection, recent publications have shown that affected individuals and their families have suffered some indirect consequences during the COVID-19 pandemic, such as healthcare barriers, loss of therapies or economic issues. History
Charlotte Dravet first described severe myoclonic epilepsy of infancy in Centre Saint Paul, Marseille, France in 1978 and the name was later changed to Dravet syndrome in 1989.
Similar descriptions were given by Bernardo Dalla Bernardina in Verona.Charlotte Figi, who was diagnosed as having Dravet syndrome, was the focus of a cause célèbre to provide a means for use of cannabidiol for persons with intractable seizures. She died from pneumonia, possibly caused by COVID-19, in April, 2020. References


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