Childhood absence epilepsy (CAE) is an epilepsy syndrome. Children with CAE experience absence seizures. These are brief and sudden lapses of consciousness. They may occur many times a day. But they are hard to notice because symptoms are so subtle.1
CAE can be treated with certain anti-seizure medications. However, behavioral and social complications can continue even with treatment. Talk to your doctor about treatments for CAE and any complications that can stem from them.1
What causes childhood absence epilepsy?
CAE has genetic causes, which means it runs in families. People who have close relatives with CAE have a higher risk of having absence seizures. Experts do not yet know which gene changes (mutations) lead to CAE.1-3
However, these mutations seem to alter the brain's electrical activity. Nerve cells (neurons) in the brain send electrical signals to other neurons. Repeating patterns of abnormal electrical signals lead to absence seizures.4
Who gets it?
Symptoms of CAE start in children between 4 and 10 years old. Symptoms most often start around 6 years old.1-3
CAE is one of the most common types of childhood epilepsy. About 12 percent of children with epilepsy have CAE. It is more common among girls than boys.1-3
What are the symptoms of childhood absence epilepsy?
The hallmark symptom of CAE is the absence seizure. Absence seizures often go unnoticed because they last such a short time. For many children, the first signs of CAE are declines in learning ability and attention.1
Symptoms of absence seizures include:1,2,4
- Vacant stare that lasts 10 to 20 seconds
- Sudden stop in motion without falling
- Lip smacking
- Eyelid fluttering
- Chewing motions
- Finger rubbing
- Small movements of both hands
After absence seizures, children do not remember the incident. They are not confused, achy, or drowsy. Children with CAE may have many episodes every day.2,3
Other types of seizures are rare in CAE. Some children with CAE may experience tonic-clonic seizures, also called convulsions. Symptoms of tonic-clonic seizures include stiffening and rhythmic jerking.1
What are some complications of childhood absence epilepsy?
CAE can lead to several complications. Children with CAE have a higher risk of:2,3,5
- Difficulty with fine motor skills
- Learning difficulties
- Social or behavioral problems
- Attention deficit and hyperactivity disorder (ADHD)
- Anxiety
Even after CAE treatment, these complications can continue. Diagnosing these conditions is important to get effective treatment.1
How is it diagnosed?
Your child's doctor will diagnose CAE by taking a health history, doing physical tests, and using electroencephalography (EEG). EEG measures electrical activity in the brain. Rapid breathing during the EEG test can trigger an absence seizure. The pattern on the EEG can help doctors diagnose the type of seizure.2,3
A diagnosis of CAE requires all of the following:1
- Age at onset of 4 to 10 years old
- Normal development and neurological state
- Brief and frequent absence seizures
- 4 to 10 seconds long
- More than 10 seizures per day
- Certain rhythmic pattern on EEG tests
In addition, doctors use certain criteria to rule out CAE. The following indicates a different epilepsy syndrome:1
- Other seizure types happening before or during absence seizures
- Sustained eyelid, head, or limb muscle jerks
- Mild or no loss of consciousness
How are absence seizures treated?
Treating CAE can help children with epilepsy maintain their quality of life. It also can minimize school performance and social acceptance problems.1
Anti-seizure drugs usually completely control CAE. The first-line therapy for children with CAE is ethosuximide (Zarontin®). Most seizures respond to this drug.1-3,6
Side effects of ethosuximide include nausea, vomiting, and sleepiness. Your doctor can help you and your child understand what side effects to expect. They also can adjust the medication dosage to minimize the risk of side effects.1-3,6
If ethosuximide does not work well or causes side effects, your doctor may suggest other treatments. These include:1,3,6
- Other anti-seizure medications, including valproic acid (Depakene®) or lamotrigine (Lamictal®)
- Ketogenic or other diet therapy
- Vagus nerve stimulation
Other things to know
Some other anti-seizure medications actually worsen absence seizures in children with CAE. Talk to your doctor before beginning any treatment for CAE. Tell them about all of your child's health conditions and any other drugs, vitamins, or supplements they are taking. This includes over-the-counter drugs.1
In most cases, CAE responds to treatment and seizures go away before puberty. Children may continue taking medications for at least 2 seizure-free years. Then, these drugs can be tapered off. However, some children with CAE may develop other seizure types later in life.2,3