Talking about life expectancy with Lennox-Gastaut syndrome (LGS) can be difficult. Each person’s experience is unique, and outcomes vary based on many factors — including overall health and health risks, types of seizures, and access to care. While there’s no one-size-fits-all answer, learning what research shows about longevity in people with LGS can help you better understand what to expect and guide important conversations with your doctor.

In this article, we’ll take a closer look at how and why LGS may affect life expectancy. We’ll also cover ways to support someone living with this condition and help minimize risk as much as possible.

Life Expectancy in Lennox-Gastaut Syndrome

LGS is a rare and severe type of epilepsy. Often beginning in early childhood, it causes multiple types of seizures. Some children with a diagnosis of LGS live well into adulthood — even into their 50s and 60s. For others, complications can develop early in life, especially if seizures happen often and don’t respond well to medication. Every person’s path is different.

Seizure Control as a Factor

One of the biggest reasons for variation in life expectancy is seizure control. Around 80 percent to 90 percent of children continue to have seizures into adulthood. These seizures are often refractory, which means they don’t respond well to medicine. If you have seizures that don’t respond to at least two different epilepsy drugs, it’s known as intractable epilepsy.

One of the biggest reasons for variation in life expectancy with LGS is seizure control.

Seizure control in LGS can be especially tricky because there are multiple types of seizures involved in the disease. These include:

• Atypical absence seizures (beginning gradually without complete loss of consciousness)
• Atonic seizures (drop attacks)
• Myoclonic seizures (muscle jerks and spasms)
• Tonic seizures (stiffening)
• Tonic-clonic seizures (muscle stiffness followed by jerking movements)

Sometimes, medications that treat one type of seizure can make other types worse.

Other Factors in Life Expectancy

However, life expectancy is affected by more than just seizures. Other factors — such as how the brain is affected, injury risk, and other health factors — also come into play.

The risk of early death is also higher because of complications like head injuries, status epilepticus (a long or repeated seizure emergency), breathing issues, or sudden unexpected death in epilepsy (SUDEP). Due to these risks, people with LGS are 24 times more likely to die early compared to the general population. The overall mortality (death) rate for LGS is around 5 percent, but some studies suggest it may be higher in people with very frequent or severe seizures.

Treatment Options

Neurology research is providing more treatment options for people with LGS than ever before. For example, dietary changes such as the ketogenic diet can help reduce the frequency of seizures.

A type of brain surgery called corpus callosotomy can also be performed to reduce the risk of the seizures spreading to other parts of the brain.

While complete recovery is rare, new treatments are providing people with LGS with more options than in the past.

Causes of Early Death in Lennox-Gastaut Syndrome

Children and adults with LGS face a higher chance of early death than those with other types of epilepsy and people without epilepsy. Early deaths are mostly due to complications caused by having a lot of seizures, rather than by the syndrome itself.

The risk of death also depends on how early in life LGS starts and whether the person has other brain conditions. For example, those who also have West syndrome or infantile spasms may have a more serious form of the condition and a lower chance of seizure control. These children may also have more severe developmental delays, which can raise safety risks.

Early deaths are mostly due to complications such as seizure-related injuries, brain damage, and sudden unexpected death in epilepsy.

Some potentially life-threatening complications include seizure-related injuries, brain damage, and sudden unexpected death in epilepsy.

Sudden Unexpected Death in Epilepsy

SUDEP is one of the most serious risks for people with LGS. It happens when someone dies suddenly without an obvious cause. It tends to happen more at night.

While rare, SUDEP is more common in people with hard-to-control seizures. In one small study of children with LGS and other severe epilepsies, 3 out of 13 children died from SUDEP. The risk is higher for people with tonic-clonic seizures, status epilepticus, and high seizure frequency.

The exact cause of SUDEP isn’t fully understood. However, researchers think it may involve problems with breathing or the heart during or after a seizure. Using a nighttime seizure monitor may help reduce the risk by alerting families to any sudden changes in movement or breathing throughout the night. Talk to your healthcare team to learn more about your risk level and additional preventive measures.

Falls and Physical Injuries

People with LGS are at a higher risk of falls and injuries because of the types of seizures they tend to have. Atonic seizures can cause someone to suddenly lose muscle tone and collapse without warning. Tonic seizures can also cause the body to stiffen and fall over. These sudden movements can result in head injuries, broken bones, or bruises.

Over time, repeated injuries can have a serious effect on someone’s health and raise the risk of early death. One study showed that between 3 percent and 7 percent of people with LGS died from brain injuries caused by repeated seizures over a period of eight to 10 years.

Preventive measures can help reduce the damage caused by physical accidents. For example, wearing proper footwear and doing home safety checks can help reduce the risk of tripping. In some cases, changes to medication may help reduce the number of atonic or tonic seizures, which lowers the risk of a fall.

Status Epilepticus

Status epilepticus is a medical emergency that happens when a seizure lasts longer than a set amount of time or when multiple seizures happen close together without recovery in between. This can lead to brain damage, coma, or even death if not treated quickly. As many as half of people with LGS go into status epilepticus at some point.

Children and adults with LGS are at greater risk for status epilepticus because their seizures tend to happen more often and be harder to stop. If you notice a seizure lasting longer than usual, call 911 right away. Some people with LGS are prescribed rescue medications. These can be used at home to try to stop long seizures before they become life-threatening.

Quick action is the key to preventing serious outcomes. Learning when and how to respond can be lifesaving.

Breathing Problems and Choking

During a seizure — especially a tonic-clonic seizure — the muscles in the chest and throat can tighten. This can make it harder to breathe or cause food or saliva to go into the lungs by mistake. When this happens, it can lead to aspiration pneumonia, a type of lung infection.

While pneumonia can usually be treated, it can be serious in people who have a lot of seizures, are less mobile, or have weaker immune systems. In some cases, it can contribute to the risk of early death.

Certain medications used to treat LGS, such as clobazam, may lead to an increased risk of aspiration pneumonia. To help lower the risk, people with LGS may need extra support during meals, safer feeding techniques, or help from speech or swallowing therapists.

Risks Change Over Time, and Support Can Help

The risks linked to LGS can change over time. For many children, the early years are the hardest. This is when seizures are often most frequent and when developmental delays, behavioral problems, and learning challenges begin to appear. Children with LGS often need close supervision. They may also need additional services like occupational therapy, speech therapy, or special education programs.

The risks linked to LGS can change over time. For many children, the early years are the hardest.

Adults with LGS may still have multiple seizures per day and often need full-time support. However, many adults can live stable lives with a consistent care plan and a safe environment. This often depends on how well seizures are controlled and how well the person responds to medication. This can become more difficult as time goes on, as many people develop resistance to anti-seizure medications.

Caregivers should be aware that even as children grow into adults, the risk of accidents, SUDEP, and status epilepticus don’t go away. However, with the right medical care, seizure control strategies, and injury prevention measures, long-term survival is possible for many people living with LGS. Whether you’re a parent, caregiver, or an adult living with LGS, know that support and treatment are available.

Talk With Others Who Understand

MyEpilepsyTeam is the social network for people with epilepsy and their loved ones. On MyEpilepsyTeam, members come together to ask questions, give advice, and share their stories with others who understand life with epilepsy.

What measures do you take to protect yourself or your child with LGS? Where do you find support as you face questions about life expectancy? Share your experience in the comments below, or start a conversation by posting on your Activities page.