Leading experts from our Professional Advisory Board – Dr. Angel Hernandez of Cook Children’s Medical Center in Texas and Dr. David Ficker from the University of Cincinnati Academic Health Center – talk about the treatment of children and adults with LGS in our Ask the Expert Series: Spotlight on Lennox-Gastaut Syndrome.

What treatment options are available for people with LGS?

The first line of treatment for any person with epilepsy, including children and adults with Lennox Gastaut syndrome are seizure medications. Seizures associated with LGS usually are not controlled with one seizure medication. Two or more medications are often needed. Even then, medicines may not control seizures enough and the person continues to be at risk for injuries, seizure emergencies, and even worsening of cognition and behavior from ongoing seizures.

When seizures persist or don't respond to medicines, it’s time to look at other treatments. For example, dietary therapies, devices, surgery, or research trials should be considered.

Seizure medications

  • Seizure medications specific for people with LGS have been approved by the Food and Drug Administration (FDA).
  • Other seizure medications may also work with this condition. The choice of treatment will also consider the person's age and other associated conditions. 
  • Sedating drugs should be avoided, since inactivity and drowsiness can precipitate seizures in LGS.
  • Some children improve with a reduction in total seizure medications, even though they may not have complete seizure control.

Dietary therapy

  • Dietary therapies specific for treating epilepsy have been tested in children and in adults to a lesser extent. These diets include the Ketogenic diet, Modified Atkins diet, and Low Glycemic Index diet.
  • These diets restrict some food choices so they can be difficult to stay on for long periods. The ketogenic diet is most restrictive and is used more often in children or people who get their food and nutrition through a tube in their stomach. Parents or other caregivers can control the types and amount of foods given. The Modified Atkins diet and Low Glycemic diet are more liberal and easier to use. These are being tried more in adolescent and adults.
  • Learn more about dietary therapies in epilepsy and follow our KetoNews.

Surgery and Devices

A type of brain surgery, called corpus callosotomy, can be done to separate some of the connections between the two sides of the brain.

  • This surgery has been very helpful to decrease the frequency of atonic or drop attacks, tonic clonic, and tonic seizures. Since these are the most frequent seizures in LGS, and cause the most injury, corpus callostomy used to be used more often for people with LGS.
  • This type of surgery has been done less frequently in recent years and usually if dietary therapy or vagus nerve stimulation has not helped.

The most common device used in people with Lennox Gastaut syndrome is vagus nerve stimulation with VNS Therapy®.  

  • Surgery is needed to place the device in the left chest area and the left side of the neck. It does not require surgery in the brain.
  • Repeat surgeries may be needed after a number of years to replace the generator when the battery in it wears down, or to replace an electrode wire. This type of surgery is an outpatient procedure that usually takes 1 to 2 hours at most.

A new type of device called responsive neurostimulation (RNS®) was approved in the U.S in 2013. This therapy is indicated for people who have seizures beginning in more than one area or in an area that can not be surgically removed. Since people with LGS have seizures from broad areas of the brain, this treatment does not appear to be helpful for LGS at this time.

Managing medical illnesses and seizure emergencies

  • When patients with LGS experience medical illness (such as a common cold or viral gastroenteritis(commonly called a ‘stomach bug’), it is critically important to continue taking seizure medication on a 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 and fluids.  
  • Everyone needs an emergency plan to temporarily treat seizures when someone can't take their usual medicines by mouth.
  • Get aggressive treatment of their medical illness and vomiting to prevent a potential seizure emergency such as status epilepticus.
  • If they have a cold, some antihistamines (i.e., Benadryl or chlorpheniramine) may lower the seizure threshold resulting in more seizures. Alternative medications such as Claritin or Allegra or nasal preparations to treat seasonal allergies may be better to use.
  • Some antibiotics can worsen seizures too. If an antibiotic is needed, ask the medical doctor to contact your neurologist before starting an antibiotic. 

Tips to Remember:

  • Families should be aware that changes in their loved ones’s treatment will likely be needed over time, depending on the response to therapy, other treatments that the child or adult is initially on, and the side effects that they may have.
  • Unfortunately, there are no studies that can tell the best treatment for each person, or which one should be tried first, second or third. There’s also no clear agreement on which combination of therapy may be best for people with LGS.
  • Treatment can be complicated because some seizure medications that help one seizure type may worsen other seizure types. At times, a new seizure type may be seen on certain medicines. 
  • Don't settle for having frequent seizures! Work together with the epilepsy/neurology team to aggressively treat the atonic and generalized tonic-clonic seizures. These are the more disabling seizures and can cause many injuries.  While the goal is to reduce the frequency of all seizure types, reducing the atonic and tonic clonic seizures should be a priority.

 

Authored By: 
Patricia O. Shafer RN, MN
Elaine Kiriakopoulos MD, MSc
Joseph I. Sirven, MD
Authored Date: 
Thursday, November 27, 2014