Lennox Gastaut Syndrome in Children (LGS)

Lennox Gastaut syndrome is a type of seizure disorder (epilepsy). Children with LGS have several different types of seizures. They may also have learning disabilities (cognitive impairment) and suffer emotional disturbances. Seizures usually start between the ages of 2 and 6 years.

There may be periods of frequent seizures mixed with brief periods of few or no seizures. Children are more likely to have learning disabilities if:

• The seizures start before the age of 2.
• Seizures are frequent.

What are the causes?

In some children, the cause of LGS is never found (idiopathic LGS). In other children, the cause is identified (symptomatic LGS). Causes of symptomatic LGS may include:

• Brain injury.
• Severe brain infections.
• Failure of the brain to form correctly as it develops.
• Lack of oxygen during birth.
• Tuberous sclerosis. This is a genetic disorder in which tumors that are not cancerous grow in many parts of the body.

What are the signs or symptoms?

Seizures are the most visible symptom of LGS. There are different kinds of seizures, including:

• Tonic. With this type of seizure, the body stiffens and the eyes roll upward.
• Atonic. With this type of seizure, there is a brief loss of muscle tone and consciousness, causing abrupt falls.
• Atypical absence. The main characteristic of this type of seizure is staring spells.
• Myoclonic. A myoclonic seizure results in sudden muscle jerks.
• Many other seizure types and combinations.

In addition to seizures, children with LGS may have emotional and psychological symptoms. These can include:

• Irritability.
• Inability to react quickly in his or her surroundings (slowed reaction time).
• Memory problems.
• Aggressiveness.
• Lack of interest in everyday activities (apathy).
• Poor development and abnormal intelligence.

How is this diagnosed?

There are no lab tests to diagnose LGS. However, other tests may be done to help make a diagnosis. These may include:

• A test that measures the electrical activity in your child’s brain (electroencephalogram, or EEG).
• An MRI of your child’s brain.

How is this treated?

The types of seizures in Lennox–Gastaut syndrome are often hard to control. The goal of treatment is to find the medicine, or combination of medicines, that will control your child’s seizures with the fewest side effects.

Other treatments that may be used along with medicines include:

• Brain surgery, which may help reduce seizures in some children.
• An implantable device that sends electric signals to interrupt seizure activity (vagus nerve stimulation).
• A high-fat diet that is low in sugar and protein (ketogenic diet).

Follow these instructions at home:

Support

• Have a good support system at home. Arrange for extra support, such as:
  • Specialized day care.
  • Special education.
  • Placement in a safe group home, if needed.

Safety

• Ask your child’s health care provider about how to best protect your child from injury:
  • Have your child wear a protective helmet with a face guard.
  • Restrict some of your child’s activities, such as bathing or showering alone, or participating in some sports.
  • Do not let your child swim or do water activities without a life jacket or other approved life preserver. This will prevent your child from going below water. Make sure that your child is closely watched.

General instructions

• Give your child over-the-counter and prescription medicines only as told by the child’s health care provider.
• Work closely with your child’s health care providers to learn about your child’s medicines and what side effects to watch for.
• If your child is on a ketogenic diet, work closely with a dietitian to learn what your child can eat and how to manage possible side effects from the diet.
• Keep all follow-up visits as told by your child’s health care provider. This is important.

Contact a health care provider if:

• Your child has seizures more often than usual.
• Your child’s seizures become harder to control.
• Your child is having side effects from seizure medicine.
• You need more support at home.

Get help right away if:

• Your child is injured during a seizure.
• Your child has a seizure that lasts longer than 5 minutes.
• Your child has trouble breathing.
• You cannot wake up your child after a seizure.

These symptoms may represent a serious problem that is an emergency. Do not wait to see if the symptoms will go away. Get medical help right away. Call your local emergency services (911 in the U.S.). Do not drive yourself to the hospital.

Summary

• Lennox–Gastaut syndrome (LGS) is a type of seizure disorder (epilepsy).
• LGS seizures usually start when children are 2–6 years old.
• Children with LGS have several different types of seizures. They may also have learning disabilities (cognitive impairment) and suffer emotional disturbances.
• The types of seizures in Lennox–Gastaut syndrome are often hard to control. The goal of treatment is to find the medicine, or combination of medicines, that will control your child’s seizures with the fewest side effects.