Lennox Gastaut Syndrome LGS

Medications

The first treatment for any person with epilepsy, including children and adults with Lennox-Gastaut syndrome, is antiseizure medications. Seizures with LGS usually are not controlled with one seizure medicine. 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 worsening of cognition (thinking) and behavior.

Most seizures in LGS don’t respond well to usual seizure medicines, and side effects can be hard to tolerate. Partial relief from seizures may be found with medications.

• One of the most common seizure medicines used in LGS is valproic acid.
• Medicines that have proven to reduce seizures in many persons with LGS are lamotrigine, topiramate, felbamate, rufinamide, clobazam, alternative therapies and most recently fenfluramine. However, other medications may also be helpful.

When seizures don't respond to medicines, it’s time to look at other treatments.

Other Treatments

Dietary Therapies

Dietary treatments can often reduce the number of seizures in people with LGS. The number or doses of some seizure medications may be lowered if diet therapy works well.

The dietary therapies used for LGS and other forms of epilepsy 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 often used when several medications have failed. It can also be easily given 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 ketogenic diet is prescribed by a physician and carefully monitored by a dietitian.
• The modified Atkins diet and low glycemic diet are more liberal and easier to use. These are being tried more in adolescent and adults.

Follow our Keto News to stay up to date about dietary therapies.

Surgery and Devices

Rarely, people with Lennox-Gastaut syndrome will be found to have a focal area of brain that can be removed to help control seizures.

Corpus Callosotomy

• This type of brain surgery 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 drop attacks. Since these are the most frequent seizures in LGS and cause the most injury, corpus callostomy is often considered for people with LGS.

Vagus Nerve Stimulation (VNS)

• 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.
• Discuss this option with your health care team.

Rescue Medications

About 50 to 75% of people with LGS have one or more episodes of non-convulsive status epilepticus.

• This type of seizure emergency consists of ongoing atypical absence seizures.
• The person’s awareness is altered and they may appear confused for long periods.
• They may have frequent to near continuous twitching of the arms or face (myoclonic seizures).
• Atonic symptoms, such as a drop of the head or part of the body, may happen with clusters of brief tonic (stiffening) seizures too.

Convulsive status epilepticus can also occur in people with LGS. This type of seizure emergency happens when a generalized seizure with loss of consciousness lasts 5 minutes or longer or repeated seizures happen without the person coming back to awareness between seizures.