What is it like?

Here's a typical story: Over a period of a few months when Anna was 15, her family and teachers noticed that sometimes she would be inattentive and "stare into space" for several seconds. Then one day Anna had a tonic-clonic seizure at school while she was taking a math exam, and juvenile absence epilepsy was diagnosed. Once Anna was treated with Depakote (valproate), the staring spells and inattentiveness seemed to disappear.

All children with juvenile absence epilepsy (JAE) have absence seizures but usually they are limited to a few episodes per day. Tonic-clonic (grand mal) seizures are usually the symptom that brings the patient to the doctor. These seizures often occur shortly after awakening but they can take place randomly throughout the day. Myoclonic seizures may also occur, but they are infrequent and inconspicuous.

Who gets it?

Juvenile absence epilepsy (JAE) is relatively common. The seizures begin near or after puberty, between 10 and 17 years of age, in young people with normal intelligence and neurologic function.

Tell me more

The EEG shows generalized spike and wave discharges with normal background activity. CT and MRI scans of the brain are normal.

Little is known about the causes of JAE, but it is felt to be similar to childhood absence epilepsy . Genetic factors predominate; about one-third of families report a family history of the disorder, and the brothers and sisters of affected individuals have about a 10% chance of developing epilepsy.

How is it treated?

Depakote (valproate) is the medication of choice, satisfactorily controlling both absences and tonic-clonic seizures. Lamictal (lamotrigine), either in combination with Depakote or alone, can be effective. If these strategies fail, Zarontin (ethosuximide) or Diamox (acetazolamide) can be tried in combination with Depakote.

What's the outlook?

The seizures are satisfactorily controlled with medication in 80% of cases. Unlike many other childhood epilepsy syndromes, however, many individuals with JAE need to take medication over their entire lifetime. Some patients with JAE will evolve into juvenile myoclonic epilepsy and develop generalized tonic-clonic and myoclonic seizures. Sleep deprivation and limiting alcohol consumption will reduce the likelihood of seizures.

Topic Editor: Gregory L. Holmes, M.D.
Last Reviewed: 10/24/06