Epilepsy's complexity requires a comprehensive, team approach. Precise identification of the location of the seizure allows the team to develop the most effective treatment plans, including the use of antiepileptic drugs (AEDs), surgery, and vagal nerve stimulation.

At first, Dianne Williams' seizures came while she was sleeping. As she became an older teenager, the blizzard of electrical activity in her brain started to occur more often and during the daytime. "I never knew I had a seizure until later, when someone would tell me," recalls Ms. Williams, who took two types of antiepileptic drugs (AEDs) to reduce the frequency and intensity of the seizures.

Dianne Williams

Ms. Williams was tested at University Hospital to determine her eligibility for a temporal lobectomy, a procedure in which the section of the brain that produces the seizures is removed. Identifying that area - the seizure focus - and determining its proximity to certain areas of the brain were questions that needed to be resolved. For about five days, Ms. Williams was in a special unit at University Hospital for continuous video EEG monitoring and neuropsychological testing. Based on the results of these tests and other evaluations, Ms. Williams was given the "green light" to have the surgery.

On December 17, 1999, Dr. Peter Carmel, chairman and professor of neurological surgery at New Jersey Medical School, performed the procedure. Since then, says the 42-year-old woman, she has been seizure free. "I'm a whole new person," says Ms. Williams, who is being tapered off her AED. "I go places by myself, which I didn't before. And while I was reluctant to date when I was having seizures, I'm testing those waters again. When people ask about my surgery, I tell them it's given me a brand new life."

Getting Their Lives Back

Scientist Sir Isaac Newton, founding father James Madison, and rock musician Neil Young are among the well-known people who had or have epilepsy. But epilepsy, described in general terms as recurrent seizures, affects the "average Joe" as well; young and old; and people of all races. Today, more than 2 million Americans have the neurological condition, and approximately 125,000 new cases are diagnosed each year. The highest incidences of epilepsy occur at both ends of the age spectrum: people over 65 (perhaps because seizures had previously gone unrecognized or began following a stroke, head injury, or the like, suggests Dr. Marks), and children under age 14. There are about 300,000 American young people with the condition. (See sidebar "Children and Epilepsy.")

Under normal conditions, tiny electrical charges are sent off between nerve cells in the brain; when an unusual amount of electric activity occurs, it produces a seizure. Seizures can involve loss of consciousness, convulsions, involuntary repetitive movements, or staring and blinking; it all depends on the type of epilepsy a person has.

Epileptic seizures fall into two broad categories: partial seizures, which affect one part of the brain, and generalized seizures, which involve many areas of the brain. Specific seizures have their own, identifiable symptoms and can be very different from other types. For example, simple partial seizures can cause jerking movements and affect vision or hearing, while a tonic-clonic seizure ( a generalized seizure previously referred to as grand mal) can result in loss of consciousness with tongue biting and incontinence. And sometimes a seizure is not epilepsy at all, but another condition, such as narcolepsy or an arrhythmia of the heart, which can cause fainting spells.

Epilepsy's complexity requires a comprehensive, team approach. The team member who first sees the patient is an epileptologist, a neurologist who has special training and interest in the field of epilepsy. At major epilepsy centers, such as the one at University Hospital, the staff includes pediatric and adult epileptologists. Other members of the team include neuroradiologists, who take images of the brain; psychologists, who assess the patient's cognitive function, conduct language studies, and identify and address any psycho/social difficulties; and ultimately, neurosurgeons, should the patient's epilepsy not be well controlled by medication. A nurse schedules tests and assists patients from the beginning to the end of treatment. Additionally, academic-based centers, like New Jersey Medical School/University Hospital, actively conduct epilepsy research.

EEG and advances in imaging have had a dramatic effect on the diagnosis and treatment of epilepsy. With the information these tests provide, the epileptologist can distinguish between epilepsy and other conditions and more precisely determine the type of epilepsy a person has.

The EEG, a non-invasive test in which electrodes are placed on the patient's scalp, records brain waves, electrical impulses that come from the brain. A standard EEG is an outpatient test that produces a snapshot in time, says Dr. Marks, but continuous video EEG monitoring enables epileptologists to study a patient as a seizure develops.

An EEG printout shows seizures starting in the left temporal region.

The patient is hospitalized for at least 24 hours, taken off any AEDs to purposely encourage seizures; and connected to a special EEG with a video camera. "Documenting unusual electrical brain activity over time provides very useful information to the epileptologist," says Dr. Marks. "We can classify the seizure type exactly, enabling us to choose the right medication. It's also a safe environment for the patient, who is followed carefully by the medical staff."

Additional information is gathered by MRI, which produces a three-dimensional image of the brain, and functional MRI, which "maps" the brain and indicates where language, motor, and sensory areas are located. Computed tomography (CT scan) enables doctors to see the fine details of the brain's structure, while Single Photon Emission Computed Tomography, or SPECT, can track blood flow in the brain and detect alterations in brain metabolism between and during seizures, information that can be useful in locating the seizure focus.

A traditional MRI Scan of the hippocorpus, a brain region that frequently gives rise to seizures.		A functional MRI Scan showing blood flow changes during motor activity - as seen in red and yellow.

Once a diagnosis of epilepsy is made, the ultimate goal is to help the patient become seizure free. Reaching that goal typically involves either one or more AED's and, if necessary, another type of treatment. For many patients - about 70 percent - seizures can be well controlled with a single AED. "And yet a person who has even one seizure a month still can't drive," says Dr. Marks. "We want these patients to get fully back into society and live a normal life."

Finding the right drug or combination of drugs - one that provides the best seizure control with the fewest side effects - involves careful dosage calculations. If the dose is too low, the patient might have seizures; if it is too high, he or she could experience an increase in the side effects of AEDs, such as drowsiness, irritability, and confusion. Some of the newer AEDs aren't necessarily more effective in preventing seizures, but they do have fewer side effects. Another plus: Older drugs can take a toll on the bones, causing osteoporosis. "It's another reason to consider the newer medications," says Dr. Marks. "A 45-year-old person who has been taking one of these older drugs since she was 15 might have the bones of a 60-year-old."

	The bright yellow area in this SPECT image shows an increase in brain metabolism, representing the seizure focus.

Finding the right medication and being seizure free is wonderful for people who've struggled with epilepsy for years, but being seizure free and medicine free is as good as it gets. "Patients are taken off medication very gradually," says Dr. Marks. "To even be considered, he or she has to be seizure free for at least two years. It's a trial-and-error process."

There's often more to epilepsy than the seizures. "Most patients whose epilepsy is well controlled aren't impaired cognitively, but for adults and children alike, the condition presents many emotional challenges," says Dr. James Hill, assistant professor of clinical psychiatry at New Jersey Medical School and director of neuropsychology at University Hospital. "Our culture holds having absolute control of oneself as a high standard, and losing control - such as during a seizure - has negative social implications." The shame and stigma of the condition, however undeserved, can cause some people with epilepsy to isolate themselves and avoid relationships or challenging careers. Psychologists can help these patients develop practical coping skills and empowerment strategies so that they can live fuller lives, although in some cases, treatment for clinical depression might be warranted.

The CT scan allows physicians to see fine details of the brain's structure and tissue, to see abnormalities, or the absence of abnormalities - as pictured left.

When Surgery is an Option

Medication is the first line of treatment for epilepsy, but some people's seizures are still not well controlled after a number of AEDs. Or, the frequency and severity of seizures might decrease with medication, but the drug's side effects are unacceptable. In another scenario, a patient whose seizures historically originated from one part of the brain rapidly develops new seizure focus areas in other parts of the brain. In all these cases, epilepsy surgery might bring relief - or even a cure - but first the patients must pass a thorough screening process.

"We're looking for patients in whom there's a good definition of where the seizure begins," says Dr. Carmel, who was recently joined by Dr. Jeffrey Catrambone, assistant professor of neurological surgery at New Jersey Medical School, in the surgical treatment of patients with epilepsy. Another criterion is a single origin point, or focus, that's not near the eloquent cortex, areas of the brain that control memory, speech, and other essential functions. Pre-operative neuropsychological testing, particularly the Wada test, helps the team identify those critical areas of the brain.

An ideal candidate for surgery is a person who has partial seizure epilepsy with complex behavior, such as lip smacking or staring. The focus for this type of seizure is often found in the hippocampus, which is located deep in the innermost part of the temporal lobe. Once the source of the seizures is removed, the patient could become seizure free.

The success rates for these types of epilepsy procedures are very encouraging; at least 75 - 85 percent of patients achieve significant benefit. Between 15 percent and 20 percent of surgical patients will have no seizures and won't need medication; 40 percent to 50 percent will be seizure free but need smaller amounts of medication than before; less than 10 percent will still have seizures, but not as frequently, and need medication; and for the remaining patients, surgery will not bring noticeable relief.

"We typically see better results among patients who have a relatively short seizure history than those who've lived many years with epilepsy," says Dr. Carmel. "Years ago, doctors tried patients on AEDs almost in a random fashion. Now, we know more about combinations of drugs and what specific actions they have. The earlier we intervene, the less likely there will be multiple seizure foci." Nationally, most epilepsy surgery is performed on adults, but at University Hospital, thanks to the expertise of Drs. Carmel and Catrambone, most of the surgical cases are children.

There's another category of patients who can benefit from epilepsy surgery. "These people are quite badly off," says Dr. Carmel. "Their bilateral seizures cause drop attacks, which can be very dangerous. Instead of trying to surgically remove the focus, we're attempting to control the spread of seizure across the brain. In one of these procedures, a band of fibers between the brain's hemispheres is cut. The place where the seizures begin is still there, but the pathway is interrupted. These patients continue to have seizures, but less dangerous ones."

A third type of procedure has helped some epileptic adults and children, although it does not involve operating on the brain: implantation of a vagal nerve stimulator. The vagal nerves are found along the left and right sides of the neck and transport messages to the brain. The left vagal nerve is believed to relay messages to areas of the brain that produce inhibition of brain seizure activity. A vagal nerve stimulator, a "pacemaker for the brain," is programmed to deliver low levels of electrical current intermittently every five minutes or so, regularly stimulating the vagal nerve. People who experience an aura before a seizure - an unusual smell, sensation, or even a feeling of impending doom - can also activate the stimulator with a special magnet. During a one- to two-hour procedure, performed at University Hospital by Dr. Allen Maniker, associate professor of neurological surgery at New Jersey Medical School, a small, battery-operated stimulator is implanted in the patient's upper chest wall, and electrodes are threaded to and then around the nerve. While not a cure for epilepsy, vagal nerve stimulation can result in less frequent or less severe seizures.

Whichever modality of treatment is used, eliminating seizures is the common goal. "Epilepsy is a challenging condition to treat," says Dr. Marks. "It is a varied disease that has many manifestations. At the same time, helping to make major improvements in these patients' lives is very satisfying."

To consult with one of our epileptologists, please call (973) 972-2550. And to learn more about epilepsy treatment at University Hospital/New Jersey Medical School, click on the website of the Comprehensive Epilepsy Center at:

http://www.theuniversityhospital.com/epilepsy/