Dravet syndrome and parkinsonism: A case report investigating the dopaminergic system

By |2019-09-09T17:20:38-05:00September 9th, 2019|Categories: DSF Research Review|Tags: , , |

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 syndrome. However, it is not well understood how Dravet syndrome, in particular dysfunction of the NaV1.1 sodium channel, is connected to [...]

Donepezil increases resistance to induced seizures in a mouse model of Dravet syndrome

By |2019-08-14T10:34:53-05:00August 14th, 2019|Categories: DSF Research Review|Tags: , , |

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 safe manner. In this study, the researchers used a Dravet mouse model to test the efficacy of donepezil (a.k.a. "Aricept"), an [...]

Engineering brain activity patterns by neuromodulator polytherapy for treatment of disorders

By |2019-07-15T23:15:28-05:00July 14th, 2019|Categories: DSF Research Review|Tags: , , |

This article is interesting for a couple of reasons. First, it describes the use of zebrafish as a model for Dravet syndrome. Zebrafish have become very useful in medical research. They are small, and easy and inexpensive to maintain and employ in drug response studies, especially in comparison to mice. Of course, although they [...]

NaV1.1 and NaV1.6 selective compounds reduce the behavior phenotype in a novel zebrafish model for Dravet Syndrome

By |2019-07-15T23:23:04-05:00July 14th, 2019|Categories: DSF Research Review|Tags: , , |

Dravet syndrome is caused by loss-of-function mutations in the SCN1A gene, which codes for the alpha subunit of the NaV1.1 sodium channel. However, there are other sodium channels, and it is known that gain-of-function mutations in SCN8A, the gene that codes for the alpha subunit of the NaV1.6 sodium channel, can lead to severe [...]

The Efficacy of Ketogenic Diet in 60 Chinese Patients With Dravet Syndrome

By |2019-07-15T23:17:09-05:00July 13th, 2019|Categories: DSF Research Review|Tags: , , |

This was a retrospective study, gathering information on Dravet patients who, over the last nine years, had followed a strict ketogenic diet for at least 12 weeks. In total, there were 60 children identified. The duration of their ketogenic diets ranged from 12 weeks to 54 months, with a median duration of 44 weeks. [...]

Potentiating α2 subunit containing perisomatic GABAA receptors protects against seizures in a mouse model of Dravet syndrome

By |2019-06-10T12:53:23-05:00June 7th, 2019|Categories: DSF Research Review|Tags: , , |

Many of the drugs used to treat Dravet Syndrome work by interacting with GABAA receptors.  Each GABAA receptor is composed of subunits, and there are different versions of receptors depending on which subunits they have. Drugs have unwanted side-effects, and it is worth asking: if we hit just one specific kind of GABAA receptor, [...]