The ketogenic (or keto) diet can also be used for seizure control. The classic ketogenic diet is a high fat, low carbohydrate diet in which 90% of calories come from fat. This diet causes ketosis, which is associated with seizure control.
The diet produces improvement in about 20% of people with intractable seizures and is typically used when multiple anticonvulsants fail or when there are problems with anticonvulsant side effects. The diet works best in children under age 4, and the family and child must be motivated to follow the diet and avoid cheating. The diet works best for atonic (sudden loss of tone — head drops, falls) and absence (staring, eye blinking) seizures but can work for all seizure types.
Vagal Nerve Stimulator
The vagal nerve stimulator, or VNS, can be used to control seizures. It is a small device implanted by surgery at the base of the neck on the vagal nerve and is then set to stimulate the nerve at a certain frequency. The frequency of stimulation can then be adjusted to give the best seizure control.
The VNS achieves a substantial improvement in seizure control in about 10% of those with difficult-to-control seizures. It works best when the person has an aura and can activate the device to abort a full seizure.
Risks of VNS placement include hoarseness after surgery and infection around the device.
Epilepsy surgery is an option for patients with very difficult-to-control focal seizures coming from one part of the brain. It can sometimes be used in a disorder affecting the entire brain if the seizures are uncontrolled and there is one area from which most seizures seem to arise.
Breakthrough Seizures, Epilepsy Care and Stopping Anticonvulsants
When on treatment, breakthrough seizures may occur associated with non-compliance (inconsistent use) with medications, too little sleep, illnesses and fever, and possibly stress, or for no reason. When there are ongoing seizures without an obvious reason (e.g,. missed medication doses), the medicine can be increased, changed, or medication can be added. When there is frequent recurrence with prolonged seizures or clusters of seizures, rectal valium (Diastat) or nasal midazolam (Nayzilam) can be used to stop seizures and avoid frequent emergency room visits.
Total epilepsy care should include medication for seizure control with adjustments for side effects and if needed, psychosocial support, educational recommendations and accommodations, behavioral management, and vocational counseling.
If an individual is on a seizure medication, it is helpful to share this information with therapists and school or program teams and to include information on the possible side effects, as these may be a factor for intervention planning and it will be important for all of those working with the person to be aware of all factors related to the seizure treatment.
Seizure medications can be stopped after being two years seizure-free, especially if the EEG is normal. Longer treatment may be recommended if the person has an underlying condition associated with significant seizure risk or if the EEG still shows strong epileptic activity. If a patient is on multiple medications, typically one at a time would be weaned after the person is seizure-free for a year or two. There is always some risk of recurrence no matter how long weaning is delayed past two years seizure-free, but the risk does not really change after two years. Medication can be restarted if seizures recur after weaning.
Data on the Treatment of Seizures in FXS
Based on the large study from the CDC-funded FORWARD project (8) some information in known about how many individuals with Fragile X syndrome and seizures were on medication for seizures during their time in follow up through the FORWARD project. In this study, antiepileptic drugs were more often used in males (60.6%) than females (34.8%) and females more often required more than one medication (see Fig. 4).