News

The U.S. Food and Drug Administration (FDA) has approved Biocodex’s Diacomit (stiripentol) as an add-on therapy for seizures associated with Dravet syndrome in patients 2 years and older who are undergoing treatment with Onfi (clobazam), an anticonvulsant therapy marketed by Lundbeck. Diacomit is used as an adjunctive (add-on) therapy for treating …

Long-term use of sodium channel blockers in the first five years of disease has been linked to poorer cognitive function in Dravet syndrome patients. The study, “Influence of contraindicated medication use on cognitive outcome in Dravet syndrome and age at first afebrile seizure as a clinical predictor in…

A compound isolated from spider venom called Hm1a helps reduce seizure susceptibility and mortality in mice with Dravet syndrome, according to researchers. Their study, “Selective NaV1.1 activation rescues Dravet syndrome mice from seizures and premature death,” was published in PNAS. Dravet syndrome is resistant to several pharmaceutical therapies…

Specific nerve cells are critically involved in the development of epileptic seizures, hyperactivity, autistic-like behaviors, and cognitive declines in Dravet syndrome, according to a mouse study from Japan. The research, “Impairments in social novelty recognition and spatial memory in mice with conditional deletion of Scn1a in parvalbumin-expressing…

Perampanel, as an add-on therapy, efficiently reduced seizure rates in children with Dravet syndrome, a real-world study shows. The study, “Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics,” was published in the journal Epilepsy &…

The ketogenic diet, which is high in fats and low in carbs, helped reduce the frequency of seizures and improve cognitive function in children with Dravet syndrome who are resistant to standard therapies, a small study in China found. The study, “Prospective study of the efficacy of a ketogenic…

Zogenix reported positive top-line results from a second confirmatory Phase 3 study investigating the use of ZX008 (low-dose fenfluramine hydrochloride) in treating children and young adults with Dravet syndrome, and said that it plans to request regulatory approval. The Phase 3 trial (NCT02926898) — also known as study…

Add-on therapy with Fycompa (perampanel) is safe and effective in preventing seizures in adolescents with treatment-resistant refractory epilepsy, according to a real-world retrospective study from Taiwan. The study, “Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian…

A new mutation associated with Dravet syndrome was identified in an Iranian family with suspected disease, according to case report. The study, “Pathogenic significance of SCN1A splicing variants causing Dravet syndrome: improving diagnosis with targeted sequencing for variants by in silico analysis,” was published in Clinical Neurology and Neurosurgery.

A phenomenon known as “genetic mosaicism” may contribute to milder symptoms in people with Dravet syndrome and related disorders, a new study shows. The study, “Mosaicism of de novo pathogenic SCN1A variants in epilepsy is a frequent phenomenon that correlates with variable phenotypes,” was published in the journal…