What Is Epilepsy?

It occurs in about 1 percent of children, and comes in many forms. Here are the facts about this neurological disorder.

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Epilepsy is nothing new -- historians have found references to it in Babylonian texts dating back to 1500 B.C., and several of Shakespeare's characters suffered from it, including Julius Caesar and Othello. But for hundreds of years the condition was woefully misunderstood as a kind of violent madness. In fact, epilepsy is a group of neurological disorders in which patients have repeated seizures. "To 'seize' literally means to take hold of something," says Phillip Pearl, M.D., director of epilepsy and clinical neurophysiology at Boston Children's Hospital. "And a seizure literally takes hold of your body."

Seizures occur when too many neurons in your brain misfire together over a large area. A child may have a one-time seizure as a result of a high fever or a head injury, both of which can trigger that kind of neuron misfiring. But if the seizures recur without an obvious trigger such as fever in a young child, some form of epilepsy will be the diagnosis.

Some types of epilepsy are severe and can even be life-threatening, but many are benign and "the majority of children with epilepsy will outgrow it," notes Orrin Devinsky, M.D., a neurologist and director of the NYU Comprehensive Epilepsy Center at NYU Langone Medical Center. "Whatever type of epilepsy your child has, remember that your role is to advocate for him or her to live as full a life as possible. It's a misconception that epilepsy always limits a child's life."

Here is an overview of the types of epilepsy found in children, starting with the most common and ending with the more rare forms.

    Febrile Seizures

    These seizures happen in 2 to 5 percent of children aged 6 months to 6 years old, and are caused by a high fever. Most febrile seizures happen just once and are therefore not considered epilepsy; most likely they don't require anti-epileptic medication or other treatment. But if your child experiences one, a pediatric neurologist should evaluate him to determine whether he's likely to have more. If the febrile seizure lasted longer than 15 minutes or restricted body movements on one side, or if you have a family history of epilepsy, your child's risk of developing epilepsy may be as high as 10 percent. In general, febrile seizures have a recurrence rate of 50 percent in babies under 1 year old, and 25 percent for older children.

      Benign Rolandic Epilepsy

      BRE (also known as benign epilepsy of childhood with centrotemporal spikes, or BECTS) often doesn't require any treatment and is almost always outgrown by age 15. Your child may report some twitching, tingling, or numbness in her face or tongue, or you may notice that her speech becomes garbled for a few minutes. A pediatric neurologist can diagnose BRE using an electroencephalograph (EEG) test, which will show a clear pattern of abnormal spikes over the central and temporal regions of the brain, especially during sleep. She may also have the occasional tonic-clonic seizure, which causes full-body stiffening and a loss of consciousness, and may require medication.

        Juvenile Myoclonic Epilepsy (JME)

        Patients with JME experience little jerks, also known as myoclonic seizures, which tend to happen in the early morning and are usually quick and not painful, though they can be a warning sign that a bigger seizure is about to happen. JME accounts for about 7 percent of all epilepsy cases and tends to begin around puberty. The disorder is lifelong, but can be well controlled with medication.

          Childhood Absence Epilepsy (CAE)

          Children with CAE may not have the kind of jerking and spasms that are associated with epileptic seizures; instead, they look vacant and stare into space for a few seconds, or lose muscle tone and slump a little forward or backward. These "absence seizures" come on between the ages of 5 and 9 and do not usually affect intellectual development. CAE does require medication (usually for a minimum of two years) but with treatment there is a remission rate of 80 percent.

            Benign Occipital Epilepsy (BOE)

            BOE can be especially disorienting for children because they will experience visual hallucinations -- most often, brightly colored shapes -- known as auras. They may also temporarily lose their vision and begin vomiting, then experience an intense headache and occasional jerking on one side of the body. It's easy to confuse BOE with migraines, but an EEG will show spikes in the occipital region of the brain while your child is sleeping, which would not occur with a migraine. The seizures typically start between 5 and 7 years old. Intellectual and neurological function is normal and these kids with these seizures respond well to medication; about 60 percent of patients attain total seizure control.

              Infantile Spasms (West's Syndrome)

              This rare form of epilepsy happens when a baby is between 3 and 12 months old. His body may suddenly jerk and then stiffen. Sixty percent of infantile spasm cases are caused by a brain disorder or injury (such as oxygen deprivation at birth). Infantile spasms require medication or hormonal therapy. About 20 percent of babies with these spasms will develop Lennox-Gastaut Syndrome (see next paragraph).

              Lennox-Gastaut Syndrome

              In this serious and rare condition, children have generalized seizures that can be difficult to control, as well as mild to severe intellectual impairment. Behavioral problems are also common, especially when high doses of multiple medications are needed to control seizures. The seizures tend to come on between the ages of 1 and 6; lifelong medication is usually required.

                Rasmussen Syndrome

                This rare but devastating form of epilepsy comes on between 14 months and 14 years of age. Children experience seizures along with progressive neurological decline and may lose the ability to speak, walk, or use one side of the body. New research suggests that Rasmussen Syndrome is an autoimmune disorder and may be triggered by a viral infection. Steroids and surgery can help; physical therapy may be needed to regain muscle function.

                  Hypothalamic Hamartoma and Epilepsy

                  This is caused by small tumors at the base of the brain. Symptoms include early puberty, seizures (often punctuated by laughing), and increased irritability and aggression. The seizures can be controlled with medication; depending on the location of the tumors, surgery may be an option.

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