Lennox-Gastaut Syndrome
What Is Lennox-Gastaut Syndrome?
Lennox-Gastaut syndrome is a rare type of intractable epilepsy. Children with the condition will have:
- several different kinds of seizures
- some degree of intellectual disability and developmental delay
- abnormal findings on an EEG (a test to see brain waves/electrical activity)
Lennox-Gastaut (LEN-eks gas-TOE) syndrome begins when kids are 3 to 5 years old. It's a lifelong condition that requires a high level of care.
What Kinds of Seizures Happen in Lennox-Gastaut Syndrome?
There are different seizure types in Lennox-Gastaut syndrome. They can cause a variety of symptoms.
Tonic seizures:
- muscles suddenly become stiff
- last about 20 seconds
- usually involve both sides of the body, but can involve only one side
- often happen during sleep
Atonic seizures:
- muscles briefly and unexpectedly go weak or limp (called "drop attacks")
- last less than 20 seconds
- can involve the whole body or just certain parts
- after the seizure, the child recovers quickly
Other types:
- atypical absence seizures: staring spells with blinking, smacking or chewing, rubbing hands together (called automatisms)
- myoclonic seizures: brief muscle twitches or jerks in the neck, shoulders, upper arms, and face
- tonic-clonic seizures: convulsions with rhythmic jerks, rigid muscles, loss of consciousness, and eyes rolling back
What Causes Lennox-Gastaut Syndrome?
Lennox-Gastaut syndrome can be caused by multiple conditions. For example, early brain injury from infection or trauma, gene mutations, or problems with the way the brain was formed before birth all can lead to the condition. Sometimes, it has no clear cause.
How Is Lennox-Gastaut Syndrome Diagnosed?
A pediatric neurologist (a doctor who treats brain, spine, and nervous system problems) can diagnose the condition by doing tests such as:
- an EEG to see if there are abnormal brainwaves
- VEEG, or video electroencephalography (EEG with video recording) to better understand the seizure types a child has
- MRI to get very detailed images of the brain
How Is Lennox-Gastaut Syndrome Treated?
Seizures in Lennox-Gastaut syndrome are typically intractable and don't usually respond well to medicines. (Intractable means "not easily managed or relieved.") Medicines doctors try include:
- Depakote (sodium valproate), Lamictal (lamotrigine), Topamax (topiramate), Zonegran (zonisamide), Banzel (rufinamide), Epidiolex (cannabadiol/CBD), Onfi (clobazam), Felbatol (felbamate)
Other treatments include:
- vagus nerve stimulation
- ketogenic diet
- surgery, such as corpus callosotomy where the "bridge" that connects the two brain hemispheres is cut. This works best for tonic and atonic seizures, and might not help the other types of seizures.
How Can Parents Help?
Caring for a child with Lennox-Gastaut syndrome can be challenging. Work with your child's care team to set up needed medical visits and therapies. Work them to create a treatment plan that provides your child with a good quality of life.
Make sure that you and other adults and caregivers (family members, babysitters, teachers, coaches, etc.) know what to do during a seizure. Your child may need to wear a helmet to prevent head injury during seizures.
What Else Should I Know?
If your child has Lennox-Gastaut syndrome, your doctor and the care team can answer questions and offer support. They also might be able to recommend a local support group. Online organizations can help too, such as: