According to a story from Science & Technology Research News, a recent study has identified that area of the brain that is associated with “silent” seizures that can appear in Dravet syndrome, a rare form of epilepsy that affects children at a very young age. Silent seizures tend to appear as the disorder progresses. They can occur up to 50 times in a day but since they do not cause visible convulsions, they may not be noticed by doctors or parents. This research has also led to two new approaches that could address silent seizures in Dravet syndrome.
About Dravet Syndrome
Dravet syndrome is a form of severe epilepsy that usually begins by the sixth month of life. The disease is most characterized by frequent seizures that are sometimes triggered by fever or hot temperatures. In most cases, the syndrome is caused by mutations of the SCN1A gene. Most of the time, these mutations are not heritable and appear spontaneously. The predominant symptom of Dravet syndrome is seizures; varying types of seizures often occur as the disorder progresses, as well as ataxia, developmental delays, behavioral disorders that may resemble autism, and cognitive impairment. Seizures can be potentially lethal. Management strategies for Dravet syndrome may include a ketogenic diet, anticonvulsant medications, cognitive rehabilitation therapy, and cannabidiol (CBD). This rare disorder is estimated to appear anywhere from 1 in every 20,000 to 1 in every 40,000 births. To learn more about Dravet syndrome, click here.
Treating Silent Seizures
Getting rid of silent seizures in these patients could result in major improvements in quality of life. The area of the brain that the researchers focused on was called the thalamus, a region that plays a role in attention, sleep, cognition, and consciousness itself. In a mouse model, the scientists were surprised to find that inhibitory cells were more active in this region than they expected.
Silent seizures often result in periods of up to 30 seconds where the child appears to disengage from their surroundings or lose consciousness, without convulsing. When a mouse with Dravet syndrome was treated with an drug called EBIO1, the drug was able to reduce symptoms by halting the random activity of inhibitory cells that occurs during silent seizures.
These results suggest that EBIO1, which has never been tested for silent seizures, should be trialled in Dravet syndrome patients. Another method that the scientists used was called optogenetics, which allowed them to alter the activity of neurons when a seizure began and shut it down. This approach will require more testing before it can be used on humans.
Check out the original study here.