Efficacy of stiripentol in Dravet syndrome with or without SCN1A mutations

By |2018-01-23T11:23:03+00:00October 31st, 2017|Categories: DSF Research Review|Tags: |

(Cho et al. 2017)  This small study of 32 patients with clinically-diagnosed Dravet syndrome sought to determine whether stiripentol's efficacy differed among those with and without SCN1A mutations. 15 patients had definite SCN1A mutations, and 17 had variants of unknown significance or benign variants. The severity of DS did not differ significantly between the two groups. Stiripentol [...]

Cannabidiol attenuates seizures and social deficits in a mouse model of Dravet syndrome

By |2018-01-22T15:17:21+00:00October 15th, 2017|Categories: DSF Research Review|Tags: , |

(Kaplan, et al. 2017) Parents often report "improved behavior" or "improved cognition" when they initiate cannabidiol (CBD) treatment in their children with Dravet syndrome, with and without improved seizure control. This project sought to measure the effect of CBD on seizure control, autistic-like behaviors, and specific neuron action. They found that when mice were given [...]

Dravet syndrome and its mimics: Beyond SCN1A

By |2017-10-06T16:39:38+00:00October 5th, 2017|Categories: DSF Research Review|

(Steel D, et al. 2017). In this critical review, the authors examine the literature surrounding each of the genes that have been associated with severe epilepsy syndromes and describe their characteristics and associated symptoms. Although each of these mutations has been found in a small number of patients with DS, with the exception of SCN1A they [...]

Population Pharmacokinetics of Stiripentol in Paediatric Patients with Dravet Syndrome Treated with Stiripentol, Valproate and Clobazam Combination Therapy

By |2017-10-05T16:26:15+00:00October 5th, 2017|Categories: DSF Research Review|Tags: , |

(Peigne S, et al. 2017) Although stiripentol has been used to treat patients with Dravet syndrome for several years, its pharmacokinetics (how the body processes the medication after a dose) are not always well understood by prescribing physicians. In this study, 35 patients with Dravet syndrome who were already taking clobazam, sodium valproate, and stiripentol [...]