DSF Research Review
Welcome to DSF’s Research Review! We use this space to keep you up to date on the most exciting studies that are published each month on Dravet syndrome and SCN1A. Each review includes a link to the original abstract or article (where possible), as well as a lay-person’s summary. Have questions? Email our team.
Looking for more information on research? Click on the button below to go to a list of pubmed published scientific articles on Dravet syndrome.
November 2019
Stiripentol: A Review in Dravet Syndrome
This article is a summary of the efficacy and safety of stiripentol (Diacomit), a drug indicated for use in patients with Dravet syndrome as an adjunctive therapy in conjunction with clobazam (Onfi) and [...]
Early hippocampal hyperexcitability followed by disinhibition in a mouse model of Dravet syndrome
Dravet syndrome can be conceptualized as a three-stage disease - the first stage (febrile; up to age 1) is typically characterized by prolonged complex febrile seizures and status epilepticus; followed by a second [...]
October 2019
Fenfluramine HCl (Fintepla®) Provides Long-Term Clinically Meaningful Reduction in Seizure Frequency: Results of an Open-Label Extension Study
This is a report on results from an open-label extension study of fenfluramine in children and young adults (2–18 years old) with Dravet syndrome. "Open-label extension" means that the participants have participated in [...]
dCas9-based Scn1a gene activation restores inhibitory interneuron excitability and attenuates seizures in Dravet syndrome mice
Our chromosomes are paired, and therefore we have two copies of almost every gene. The mutations that cause Dravet Syndrome affect only one copy of the Scn1a gene, which leads to production of [...]
Reduced efficacy and risk of seizure aggravation when cannabidiol is used without clobazam
Cannabidiol oral solution (Epidiolex) was recently approved in the United States for the treatment of seizures associated with Dravet syndrome. It is typically used as an adjunct with other medication(s). This paper highlights [...]
Gait abnormalities in people with Dravet syndrome: A cross-sectional multi-center study
In reviews of Dravet syndrome, statements about gait are usually very generalized, along the lines of: " by the age of 13 years, up to 80% of people with Dravet syndrome walk with [...]
September 2019
Dravet syndrome and parkinsonism: A case report investigating the dopaminergic system
This paper was interesting because one doesn't see too many reports focused on adult Dravet patients. It has been found that Parkinsonism is a motor condition that often develops in adults with Dravet [...]
August 2019
Donepezil increases resistance to induced seizures in a mouse model of Dravet syndrome
As seen in many of these research summaries, models that reliably mimic human Dravet syndrome, such as engineered mice and zebrafish, allow labs to try out various potential drugs in a quick and [...]