Benzodiazepines are a class of anticonvulsant therapies, some of which are also used to treat anxiety. They are sometimes prescribed to treat seizures associated with Dravet syndrome, a serious type of epilepsy disorder characterized by prolonged seizures that begin in the first year of life.
Treatments currently available to treat the condition are primarily palliative, aimed at improving quality of life and reducing the number and severity of seizures.
How benzodiazepines work
Nerve cells communicate with each other via electrical signals, which are sent by changing the flow of charged particles in and out of the nerve cells.
In Dravet syndrome, mutations in the gene providing instructions to make a protein involved in the transport of charged particles in and out of nerve cells causes overactive signaling and leads to seizures or convulsions.
Benzodiazepines enhance the effect of an inhibitory signaling molecule called gamma-aminobutyric acid (GABA). GABA decreases the excitability of nerve cells, which reduces the signal sent by nerve cells during normal signaling. Benzodiazepines bind to a specific site on the receptor protein to which GABA also binds. This increases the effect of GABA in the brain, reducing the severity and incidence of seizures.
Some benzodiazepines can also be used as a rescue medication to stop a seizure once it has begun.
Benzodiazepines and Dravet syndrome
Ativan (lorazepam), Klonopin (clonazapam), and Onfi (clobazam) are benzodiazepines commonly prescribed for the treatment of Dravet syndrome.
Some benzodiazepines used in emergencies to stop seizure activity include Valium (diazepam),  Ativan (lorazepam), and Versed (midazolam). Because patients are unable to swallow during a seizure, these medicines are injected into the bloodstream or administered under the tongue.
Diastat is a diazepam gel preparation that can be administered rectally while Nayzilam (USL261) is a midazolam formulation that can be administered nasally and is currently being investigated as a rescue medicine to stop acute repetitive seizures
Other Information
Benzodiazepines have several side effects including drowsiness, unsteadiness, and problems with memory or concentration.
***
Dravet Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.