Dravet syndrome is a rare and severe form of epilepsy characterized by seizures that usually begin in infancy.
A number of approved treatments exist that can help control seizures in Dravet patients. These must be catered to the child’s needs, and primarily work to reduce the number and severity of seizures.
Anticonvulsant therapies
Seizures associated with Dravet syndrome are often resistant to treatment and, for most patients, two or more seizure medications or anticonvulsants are needed to treat the multiple types of seizures experienced. However, not all medications can be prescribed for children, making the management of this condition even more difficult.
The primary anticonvulsants used to treat Dravet syndrome are valproate and clobazam, but stiripentol and topiramate may also be prescribed. Other options include levetiracetam and potassium bromide.
It is important to avoid a type of anticonvulsant therapy known as sodium channel blockers, as these can worsen seizures in Dravet syndrome. These include Dilantin (phenytoin), Cerebyx (fosphenytoin), Tegretol (carbamazepine), Trileptal (oxcarbazepine), Lamictal (lamotrigine), and Banzel (rufinamide).
Vagus nerve stimulation (VNS)
Vagus nerve stimulation (VNS) may be useful for some children. One vagus nerve can be found on each side of the body, running from the brainstem to the chest and abdomen. In VNS, a device is surgically implanted under the skin of the chest, and a wire is threaded under the skin to connect the device to the left vagus nerve.
When activated, the device sends electrical signals along the vagus nerve to the brainstem, which then sends signals to certain areas of the brain.  A type of neuromodulation, vagus nerve stimulation is designed to change how brain cells work by giving electrical stimulation to those areas involved in seizures.
An alternative to the VNS device, which does not require surgery, has been approved for use in Europe to treat epilepsy but has not yet been approved in the U.S. It is called the Monarch external Trigeminal Nerve Stimulation (eTNS) system.
Ketogenic diet
A ketogenic diet may be prescribed by a physician for those patients who do not respond well to anticonvulsant therapy. A ketogenic diet is low in carbohydrates and high in fat and protein. It increases the levels of ketones, a byproduct of fat metabolism, in the blood, which may help control seizures.
Patients placed on a ketogenic diet should be monitored by the prescribing physician, usually in coordination with a pediatric dietitian.
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