What is Lennox-Gastaut Syndrome (LGS)? Let's talk about it!

Does your little one have frequent seizures? Does he or she have major changes in learning and behavior? If so, what I'm going to say may be very important to you. Today we're going to talk about Lennox-Gastaut Syndrome , or (LGS) for short, a severe and difficult-to-treat epilepsy condition. This can start at a young age. But don't worry, new medications and treatments are now available.

What exactly is Lennox-Gastaut syndrome (LGS)? Who gets it?

Simply put, LGS is a severe form of epilepsy that begins in childhood, usually before the age of 10, and especially between the ages of 3 and 5. It is slightly more common in boys. However, it is not a very common condition. It affects about one to two people in a million. About 1% to 2% of all epilepsy patients have LGS.

How does this affect our bodies?

Epilepsy is a disorder in which the brain's electrical signals, or information, are transmitted between cells. (LGS) is a more severe form of epilepsy. There are several causes:

• In LGS, more than one type of seizure occurs. Some of these types of seizures can cause serious injury to the child.
• These seizures can damage the brain itself. Children with LGS can have severe brain damage, learning difficulties, and developmental delays. This means that the child may lose or forget things they have already learned, such as walking and talking. This is a very sad situation.
• (LGS) is usually very resistant to treatment. This means that it is difficult to control with medication. Medication is the first treatment, but often several types of medication are given at the same time. Even if they are given, medication alone is rarely able to completely stop the seizures.

What are the symptoms of (LGS)?

A child with LGS can exhibit a variety of symptoms. Some of them are noticeable in everyday life and activities, while others are only noticeable during seizures.

Learning and behavior problems

It is common for children with LGS to have learning difficulties and behavioral problems . These can appear soon after the onset of seizures, and they can worsen as the seizures continue. These learning and intellectual problems are often permanent, meaning they can persist into adulthood.

Children with LGS have difficulty learning, making friends, and communicating with others. They may also have difficulty regulating their emotions. Some children may also show symptoms similar to those of children with Autism Spectrum Disorder .

Seizure stage

A seizure can typically occur in three stages:

1. Prodrome or aura: This is something that some people experience before a seizure, although not always. This can be a mildly unpleasant feeling. Changes in vision, changes in other senses, or changes in mood or behavior may occur at this time.

2. Seizure: This is when a seizure occurs. This can vary depending on the type of seizure (we will discuss the different types of seizures below).

3. Post-seizure recovery: This is the period after the seizure has ended. This is when a person regains full consciousness.

Types of seizures seen in LGS

There are several main types of seizures seen in LGS. The most common types of seizures and their effects are as follows:

• Atonic seizures:
• Loss of muscle control/fainting: This is also called a "drop attack" because it involves a sudden loss of control and falling to the ground, like a puppet whose strings have broken. This is very dangerous because the child has no control when they fall and can suffer serious, even life-threatening, injuries to the head, neck, chest, and back.
• Partial loss of consciousness: This seizure may not cause complete loss of consciousness.
• Short duration: These seizures last very short, like a few seconds.

• Tonic convulsions:
• Whole body stiffness/freezing: This is when all the muscles in the body suddenly contract, making the body completely stiffen as if it were turned to stone.
• A special pose: When on the ground, their legs and shoulders are on the ground, their backs can be arched. The arms can be extended above their heads, or the arms can be clenched and the elbows pointed out to the sides, and the fists can be close to the hips.
• Most often occurs during sleep: These most often occur while sleeping. But they can also occur during the day. If this happens while the child is awake, there is a risk of injury because the child may fall.
• Short duration: This also lasts from a few seconds to about a minute.

• Atypical absence seizures:
• Eyes open and staring blankly: In this seizure, consciousness is lost, but the eyes remain open. Therefore, this can often be mistaken for daydreaming, lack of focus, and a sense of being out of place.
• Repetitive movements: During this seizure, you may see movements of the eyes and mouth. There may also be movements of the hands.
• Short duration: This also lasts no more than a few seconds.

Important: Children with LGS may have seizures in "clusters," meaning several seizures in a short period of time. More than two-thirds of children develop a condition called "Status Epilepticus." This is a medical emergency!

What is Status Epilepticus?

This means one of two things:

• A single, prolonged seizure lasting between 5 and 30 minutes.
• More than one seizure occurs within 30 minutes, and the person does not regain full consciousness during those seizures.

Status epilepticus is a medical emergency. It is particularly dangerous because it can cause permanent brain damage and even death.

Other symptoms and related seizure types

• Tonic-clonic seizures: Tonic-clonic seizures (formerly called "grand mal") begin in the same way as tonic seizures, but they have an additional phase. The clonic phase is when the body jerks, the most common feature of the seizure, occur. Tonic and tonic-clonic seizures usually last about two minutes.
• Quick, short, jerky movements: Myoclonic seizures are sudden, jerky movements. They last only a few seconds, but sometimes they can last longer. People who don't have seizures can also have these kinds of "myoclonic jerks." For example, they can cause hiccups or suddenly wake up from sleep.
• Sensory, mental, or other symptoms: Partial or focal seizures affect only a limited part of the body.

What are the causes of LGS?

There are several possible causes of Lennox-Gastaut syndrome. Some of the causes are present before a child is born. Others are caused by events that occur during childhood. In about a quarter of cases of ``(LGS)``, no clear cause can be identified.

Here are some of the identified reasons:

• Problems with brain development before birth.
• Severe head injuries (traumatic brain injuries or concussions).
• Infections that damage the brain, such as meningitis or encephalitis.
• Inherited metabolic diseases, for example `(Mitochondrial diseases)`.
• Brain damage caused by lack of oxygen before or during birth (cerebral hypoxia).
• Tuberous sclerosis.
• Brain tumors.
• West syndrome (this is another type of epilepsy, which can later develop into LGS).

Researchers have identified "de novo" mutations that are common among people with LGS. De novo means "from the beginning." These are errors in a person's DNA that occur suddenly, randomly. That is, the mutation is caused by an error in the DNA of the sperm or egg that produced the person, but not by an error in the DNA of either parent.

However, there may be a genetic influence on the condition. About 30% of people with LGS have a family history of epilepsy. However, more research is needed to determine exactly how much of a genetic factor this is.

Is this contagious?

No, Lennox-Gastaut syndrome is not contagious. It is not spread from one person to another. However, some infectious diseases can cause brain damage and cause ``LGS''. However, not everyone who has those infections will develop ``LGS''.

How is Lennox-Gastaut syndrome (LGS) diagnosed?

A number of tests are usually done to diagnose LGS, and a doctor will perform a neurological exam. A pediatrician may suspect the condition based on what the parents say about their child's symptoms, especially if the child has learning disabilities or behavioral problems.

What tests are done to diagnose this?

The most commonly used tests are:

• Electroencephalogram (EEG): This involves placing several electrodes on the scalp with a conductive gel to measure the electrical activity of the brain. An EEG is important in diagnosing LGS because it shows a distinctive electrical pattern in the brain waves.
• Magnetic Resonance Imaging (MRI): This uses a powerful magnet and a computer to take high-resolution pictures of the inside of the body, especially the brain. This is very helpful in detecting problems with brain development.
• Lab testing: These tests check blood and urine to see if there are other possible causes of the seizures. They look at things like blood chemistry, liver enzymes, and kidney function. While these tests cannot definitively diagnose LGS, they can help rule out other conditions that may mimic LGS.
• Genetic testing: This can help determine whether there are inherited conditions that can cause LGS, or whether there are spontaneous mutations.

In addition, other tests may be done to rule out similar conditions. Your doctor will tell you more about this.

How can this be treated? Can it be cured?

There are several treatment options for LGS. However, the condition is notoriously difficult to treat. In most cases, one or a combination of these treatments may be used:

• Medications
• Dietary therapies
• Brain surgery or implanted devices

Medications

Lennox-Gastaut syndrome is often highly resistant to medication. That's why it's so difficult to manage. In many cases, multiple medications are needed. Another complicating factor is that some antiseizure medications, which control some types of seizures, can increase the frequency of other types of seizures.

When the condition is resistant to treatment, doctors may try to prevent certain types of seizures or reduce their frequency. For example, atonic seizures are especially important to prevent, as they are associated with a high risk of injury from falls.

Dietary therapies

For many people with severe epilepsy, dietary changes can help reduce the frequency of seizures or stop them altogether. The ketogenic diet for epilepsy – a diet high in protein and fat and low in carbohydrates – is often recommended by doctors. It can be a good option when medication has not worked.

Although the ketogenic diet is a popular treatment for severe epilepsy, it has a few drawbacks. This diet only works if you follow it exactly. This is because eating too many carbohydrates, such as sugar and starchy foods, can trigger seizures. However, other modified diets can also help. If you're interested, your doctor can help you try one of these diets.

Brain surgery and implanted devices

In some cases, especially when other treatments have not worked, surgery is the best option. Here are some types of surgery:

• Vagus Nerve Stimulation (VNS): This involves implanting a device into the body that delivers a mild electrical current to the 10th cranial nerve, the vagus nerve – which is directly connected to the brain. This current can reduce the frequency and severity of seizures. Over time, its effects increase. This means fewer seizures and less severe seizures. Implanting this device is a reversible procedure, with low risks and a short recovery time, so it is often considered before major surgery.
• Corpus callosotomy: The corpus callosum is a part of the brain that acts as a bridge between the left and right hemispheres. When the corpus callosum is severed, the abnormal signals that cause seizures stop going back and forth. This can reduce the frequency of seizures, or even stop severe, disabling seizures altogether.
• Hemispherectomy: This involves surgically removing one side of the brain. While this may seem extreme, specialists can use it to stop uncontrollable seizures. Later, physical therapy can often help the brain adjust to the change. The brain remaps itself, allowing the remaining half to take over the functions of the removed side.
• Resection: Structural anomalies in the brain can sometimes cause LGS. If doctors can find such an abnormality, they may be able to remove the damaged or diseased brain tissue and stop the seizures. However, because in LGS, seizure activity is in more than one area of ​​the brain, this is usually not a very successful option for LGS.

Complications/side effects of treatment

Complications of treatment are specific to each treatment. Therefore, it is best to talk to your doctor about the complications and side effects of the treatment you or your child are receiving. The doctor can then give you information that is appropriate for your condition, your circumstances, and other medical conditions.

How soon will I feel better after treatment? How long will it take to recover?

The recovery time after treatment varies greatly depending on the treatment methods used and your overall condition. Your doctor is the best person to tell you exactly what to expect and how long it will take to recover.

Can Lennox-Gastaut syndrome (LGS) be prevented?

In most cases, LGS is not a preventable condition. Nor can the risk of developing it be reduced. The only exception is to prevent head and brain injuries that can result from LGS. The only way to prevent this condition from occurring is to have your children wear approved, certified helmets.

What should I expect if my child or I have this condition?

Lennox-Gastaut syndrome is a condition that usually has severe consequences. Many children with this condition develop learning disabilities and permanent brain damage that limits their mental abilities. Also, because atonic seizures (drop attacks) are common, and they often lead to falls, children with this condition are at increased risk of injury.

This condition is also difficult to treat. Many children with it require lifelong specialist care because they are unable to care for themselves. However, some people with this condition only have minor brain damage, meaning they can live independently.

How long does (LGS) last?

Unless treatment is given to completely stop the seizures (which is very rare), LGS is a lifelong condition.

What is the outlook for this situation?

The biggest risks of Lennox-Gastaut syndrome are brain damage from uncontrolled seizures, or from falls caused by seizures. Because of those risks, the mortality rate for people with ``LGS'' within 10 years of diagnosis is between 3% and 7%.

In some cases, medical procedures can stop seizures caused by LGS. This stops the condition, but it cannot repair damage to the brain from previous seizures.

How do I take care of myself? / How do I take care of my child?

If you or a loved one has LGS, it is very important to follow your doctor's instructions exactly. This includes:

• Taking medication as prescribed.
• Going to see the doctor as recommended.
• Monitoring seizures and avoiding triggers or situations that may cause seizures.

When should I see the doctor?

Your or your child's doctor will tell you to come in for follow-up visits as needed. You should also see your doctor if your seizure symptoms change significantly or if they affect your normal daily routine and activities.

When should I go to the Emergency Treatment Unit (ETU) ?

If you or your child has a seizure or multiple seizures that meet the criteria for a medical emergency called status epilepticus , you should go to the emergency room at your nearest hospital. This means in the following situations:

• If a single seizure lasts between 5 and 30 minutes.
• If two or more seizures occur within 30 minutes and the person does not regain full consciousness during the seizures.

Take-Home Message

Lennox-Gastaut syndrome (LGS) is a severe, early-onset form of epilepsy. It can often have a profound, sometimes lifelong impact on a child's life. Although it is difficult to treat, new medications, surgeries, and treatments can help reduce the severity of the condition and limit the spread of the disease. Rarely, treatment can completely cure the condition. However, more research is needed to determine whether or not this condition can be treated or cured. If your child has any of these symptoms, it is best to see a doctor as soon as possible.

` Lennox-Gastaut syndrome, LGS, epilepsy, seizures, pediatrics, brain disorders, neurological diseases