Treatment options for patients with Lennox-Gastaut Syndrome consists of anti-epileptic drug therapy, the ketogenic diet, vagus nerve stimulation, or in a few cases, brain surgery to separate the two halves of the brain by cutting the large fiber bundle called the corpus callosum. In addition, the family needs to have a treatment plan available for seizure emergencies, for routine medical illnesses, and medications to avoid that could potentially worsen seizure control. Additionally, inactivity and drowsiness are known to precipitate seizures in LGS. This association is important when a treatment plan is developed, as the neurologist should avoid sedative drugs and the child should be in a stimulating environment. Some children improve with a reduction in total seizure medications, even though they may not have complete seizure control.

When patients with LGS experience medical illness (such as a common cold or viral gastroenteritis) it is critically important they continue to take their seizure medication on their regular schedule. If they are vomiting and are unable to keep their medication down, they may need to be admitted to the hospital for intravenous medication or have developed an emergency treatment plan with their pediatric neurologist, as to temporary treatment when they are unable to take medications by mouth consistently. Aggressive treatment of their medical illness and vomiting should be performed by their primary care physician, with the goal of keeping them on their regular seizure medication to prevent a potential seizure emergency (status epilepticus). Additionally, if they have a cold, older antihistamines (i.e., Benadryl or chlorpheniramine) may lower the seizure threshold resulting in increased frequency of seizures. Alternative medications such as newer antihistamines (such as Claritin or Allegra) or nasal preparations to treat seasonal allergies are preferable.

Every patient with Lennox-Gastaut Syndrome should have a plan developed for what to do if they miss a dose of medication and for treatment of seizure emergencies. For missed dose of medication, the treatment plan may be as simple as making it up as soon as it is realized, depending on which medication the patient is taking, the plan can be further individualized. Each family should plan for how to treat seizure emergencies as these are not uncommon in LGS. There are several out of hospital treatment options available for this including various formulations of benzodiazepines. A specific treatment plan should be tailored to the patient by the pediatric neurologists and discussed with the family with the clear guidelines as to when to administer the medication, when to repeat the dose (if necessary) and when to proceed to the emergency department for further care. Having a plan in place will allow the best possible treatment of the seizure emergency when it does occur and allow the family and the patient to have the best possible quality of life.

Patients with Lennox-Gastaut Syndrome typically have seizures that are resistant to treatment. As such, it is unlikely that the child will be placed on a single medication or treatment and have all their seizures controlled. Knowing this, the family should be aware that there will likely be changes in the child’s treatment, depending on the response to therapy, other treatments that the child is initially on, and the side effects that the child may or may not experience. Unfortunately, there are no studies that have been performed to evaluate what is the best sequence of treatments in a child with Lennox-Gastaut Syndrome or even which combinations of therapy are most effective. Additionally, treatment is further complicated because some anti-epileptic drugs that may be of benefit for a certain seizure type may increase the frequency of another type or bring out a new seizure type (I will discuss these later). Neurologists should aggressively treat the atonic and generalized tonic-clonic seizures (that lead to injuries). These are the more disabling seizures, and while the goal is to reduce the frequency of all seizure types, reducing these should be a priority.

There are three basic groups of therapies available for a child with Lennox-Gastaut Syndrome. These are medications, the ketogenic diet and surgical options. Most children and adults, will require more than one therapy and over time, may require treatment with multiple therapies in each of these categories, given the refractory nature of the seizures associated with LGS. Each of these treatment options will be discussed individually.

Author: James Wheless, M.D.
Topic Editor: Robert Fisher, M.D., Ph.D.
Last Reviewed: 8/7/09