Cardiovascular safety of low-dose fenfluramine in Dravet syndrome: A review of its benefit-risk profile in a new patient population

By |2017-07-24T18:48:44-05:00July 24th, 2017|Categories: DSF Research Review|Tags: , , , |

(Schoonjans AS, et al. 2017). Fenfluramine is currently in a Phase 3 clinical trial for Dravet syndrome sponsored by Zogenix, Inc. While primarily used in the 1980s and 1990s for obesity, there were reports of it reducing seizures in the pediatric epilepsy population. It was withdrawn from the market in 1997 due to reports of [...]

Rare variants of small effect size in neuronal excitability genes influence clinical outcome in Japanese cases of SCN1A truncation-positive Dravet syndrome.

By |2017-07-24T17:16:45-05:00July 24th, 2017|Categories: DSF Research Review|Tags: |

(Hammer MF, et al. 2017) There is a broad spectrum of severity in Dravet syndrome, even among patients who have the same SCN1A mutation, which suggests other genes may play a role in modifying the clinical outcome. Mouse lines with a specific mutation can exhibit differing severities depending on the strain of mouse with which [...]

Age-related “Sleep/nocturnal” tonic and tonic clonic seizure clusters are underdiagnosed in patients with Dravet syndrome

By |2017-07-22T16:02:18-05:00July 22nd, 2017|Categories: DSF Research Review|Tags: , |

(Losito E, et al. 2017) Of 33 patients with Dravet syndrome older than eleven years in a French cohort, 26 experienced a switch from mainly daytime seizures to mainly sleep/nocturnal (S/N) seizures between age 4 and age 11. The S/N seizures were usually brief, often occurred in clusters of 2-15 per night, and were often [...]

Efficacy of adjunctive vagus nerve stimulation in patients with Dravet syndrome: A meta-analysis of 68 patients

By |2017-07-22T15:42:07-05:00July 22nd, 2017|Categories: DSF Research Review|Tags: |

(Dibue-Adjei M, et al. 2017) A review of the literature on vagus nerve stimulation (VNS) therapy in patients with Dravet syndrome resulted in 13 studies (68 patients total). Of those patients, approximately 53% experienced a 50% reduction or more in seizures. Twenty-eight patients reported seizure data with enough detail to determine that the average seizure [...]

Cacna1q is a genetic modifier of epilepsy in a mouse model of Dravet syndrome

By |2017-07-09T16:21:51-05:00July 9th, 2017|Categories: DSF Research Review|Tags: , |

(Calhoun, et al. 2017) There are several different mouse models for Dravet syndrome, and researchers have shown that the same disease-causing mutation can present differently depending on which strain of mouse is used in breeding. This suggests there may be genetic modifiers that influence the severity of Dravet syndrome. The authors of this article had [...]

Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome

By |2017-07-09T15:56:26-05:00July 9th, 2017|Categories: DSF Research Review|Tags: , |

(Devinsky, et al. 2017) The long-anticipated results from the first randomized, double blind, placebo controlled trial of cannabidiol (CBD) in Dravet syndrome show that cannabidiol can help alleviate seizures in this syndrome. While there have been anecdotal stories of success with CBD in Dravet syndrome since as early as 2012, this trial represents the first [...]