Lennox-Gastaut syndrome (LGS) in children

Lennox-Gastaut syndrome (LGS) is a rare and severe form of epilepsy that begins in childhood. Children with LGS often have seizures and have several types of seizures.

This condition is difficult to treat, but researchers are looking for new treatments. Finding practical and emotional support is essential to help you provide your child with the best quality of life and cope with the challenges and stress that this disease brings.

Seizures usually begin between the ages of 2 and 6 years. Children with LGS have learning difficulties and developmental delays (such as sitting, crawling, walking) that can be moderate to severe. They may also have behavior problems.

Each child develops differently, and it is impossible to predict how a child with LGS will behave. While most children have continuous seizures and some form of learning disability, some may respond well to treatment and have fewer seizures.

Others may continue to have frequent seizures, as well as problems with thinking, development, and behavior, and will need help with daily living. Some parents find that a special diet called a ketogenic diet helps.

Causes

Doctors don't always know what caused LGS in a child. In some cases, this may be caused by:

Lack of oxygen during birth
Severe brain injuries linked with pregnancy or birth, such as low birth weight or premature birth
Brain infections (such as encephalitis, meningitis, or rubella)
Seizures that start in infancy, called infantile spasms or West’s syndrome
A brain problem called cortical dysplasia, where some nerve fibers in the brain don’t line up right during development in the womb
Tuberous sclerosis, where non-cancerous tumors form in many places throughout the body, including the brain
Genetics

Symptoms

Children with LGS have frequent and severe seizures. And they often have different types of seizures, including:

Atonic convulsions. Also called "falling fits" because the person loses muscle tone and may fall to the ground. Their muscles may tremble. These seizures are brief and usually last a few seconds.
Tonic convulsions. These seizures cause a person's body to tense up and can last anywhere from a few seconds to a minute. They usually occur when a person is sleeping. If they occur while a person is awake, they can lead to a fall. Like atonic seizures, they are also called drop attacks.
Absence seizures. During these seizures, the person may stare blankly, nod their head, or blink their eyes rapidly.

In some children, the first sign of LGS is a continuous seizure lasting 30 minutes, or continuous seizures without complete recovery in between. It's called status epilepticus, and it's a medical emergency.

People with LGS may also have slower reaction times. Some have problems with learning and processing information. They may also have behavior problems.

Getting a diagnosis

Your doctor will want to know:

When did you first notice a problem?
Has your child had seizures? How many? How frequent?
How long did it last, and how would you describe what happened?
Does your child have any medical conditions or take any medications?
Were there any complications during birth?
Do you know if your child has any brain injuries?
Does your child have problems with learning or behavior?

Your doctor will look for three signs to diagnose LGS:

Multiple kinds of seizures that are hard to control
Developmental delays or intellectual disability

Questions for the doctor

Does my child need any more tests?
Have you treated other children with this condition?
What treatment do you recommend?
How will treatment make my child feel?
What can I do to keep my child safe during seizures?
Are there clinical trials my child could take part in?
How do I connect with other families who have children with LGS?

Treatment

Medicines

Doctors may prescribe various medications to treat attacks of LGS. The goal is to reduce the number of seizures with drugs that cause the fewest side effects. Finding the right treatment for your child will likely take time and close coordination with your doctor. Medicines used to treat seizures include:

Cannabidiol
Clobazam
Felbamate
Lamotrigine
Rufinamide
Topiramate
Valproate, valproic acid

Usually, no medication completely controls seizures. The doctor will monitor your child's medications closely, especially if your child is taking more than one medication at a time.

Diets

A special high-fat, low-carb diet called the ketogenic diet helps some people with epilepsy, including some children with LGS. It is a diet high in fat, low in protein and low in carbohydrates. It must be started in a certain way and very strictly observed, therefore the supervision of a doctor is necessary.

Your doctor will keep a close eye on whether and when you can lower your drug levels. Since the diet is very specific, your child may need to take vitamin or mineral supplements.

Doctors don't know why the ketogenic diet works, but some research suggests that children with epilepsy who follow the diet are more likely to have less seizures or medication.

Some children may also benefit from a modified Atkins diet. It is slightly different from the ketogenic diet. You don't have to restrict calories, protein, or fluids. Also, you don't weigh or measure the food. Instead, you watch your carbs.

People with intractable seizures have also tried a low glycemic index diet. This diet focuses on the type of carbs as well as how much a person is eating.

Medical marijuana

Much attention is being paid to the use of medical marijuana to treat children with epilepsy, and many families are interested in learning more. Physicians have not yet studied the use of medical marijuana in children with LGS, and most studies on its use for epilepsy have focused on short-term benefits. More research is needed to find out if this is a safe and effective treatment for children with LGS, according to the Lennox-Gastaut Foundation.

Surgery

If medications and other treatments do not reduce the number of seizures, a doctor may suggest surgery.

The vagus nerve stimulator is a small device placed on the arm or near the chest. It sends electrical impulses to the vagus nerve, which runs from the abdomen to the brain. The nerve then sends these impulses to the brain to help control seizures. The operation is performed under general anesthesia and lasts about an hour.

The Neuropace Responsive Stimulator is a device that is placed inside the skull and connected to the brain. It detects any abnormal electrical activity and then sends electrical impulses to the brain to try and prevent seizures from occurring.

Body callosotomy separates the left and right hemispheres of the brain. This prevents seizures that start in one part of the brain from spreading to the opposite side. Typically, this is only offered to people with severe, uncontrollable seizures that cause them to fall and injure themselves. A person who has had a body callosotomy will need to stay in the hospital for 2-4 days and continue taking antiepileptic drugs after they go home.

What to expect

Raising a child with LGS is difficult. If your child has frequent seizures, they may need to wear a helmet to protect them in the event of a fall. You may have to deal with behavioral problems, such as the side effects of antiepileptic drugs.

There is no cure for LGS, although there are many studies looking for more effective treatments.

Each child with LGS has different needs. Most of them continue to have seizures and mental retardation after growing up. Some can live on their own, but most will need help with daily activities. They may have to live in a group home or nursing home.

It is important that parents and siblings receive the support they need as caregivers and family members dealing with this serious illness. Connecting with other families facing the same issues can help you feel less isolated, and getting advice and information from others can make everyday life easier.