Epilepsy research

Pursing the link between fevers and epilepsy: Multicenter study seeks answers

Six-year-old Bartolina, a patient in Children's Memorial's Nordstrom Epilepsy Observation Unit, talks with Douglas R. Nordli, Jr., MD, director or the hospital's Epilepsy Center.

Despite the latest diagnostic technology such as video electroencephalogram (EEG), positron emission tomography (PET) scans and more than 50 medications at physicians' disposal, the number of children who develop intractable epilepsy - the kind that doesn't respond to treatment - hasn't changed in half a century, says Douglas R. Nordli, Jr., MD, who holds the Lorna S. and James P. Langdon Chair in Pediatric Epilepsy.

"Seizures are very disabling," says Nordli, who is also associate professor of pediatrics at Northwestern University's Feinberg School of Medicine. "They interfere with getting an education, driving a car, maintaining a job and social relationships." But Nordli also believes in the potential of research to some day eliminate the threat of epilepsy to children. "Ideally, we'd prevent the development of epilepsy in the first place. The second best thing would be to cure it once it develops, and the third would be to treat it more effectively."

In a new National Institutes of Health-funded, multi-center clinical study, Nordli and colleagues are pursuing one of epilepsy's many causes. Neurologists have long debated whether prolonged febrile seizures lead to epilepsy. Febrile or "fever" seizures are very common among children under five years old. They occur in approximately 100,000 U.S. children annually when body temperatures rapidly rise above 101 degrees Fahrenheit. Studies on animals have shown an association between a rapid rise in body temperature and seizure activity. In most children these seizures have no lasting consequences. But the study will focus on the three percent of children who have a febrile seizure lasting more than 30 minutes and develop scar tissue in the brain.

"This is a landmark study," says Nordli, the central EEG reader for the study. "It will show if this population develops epilepsy as a result of scarring, and if so, what the risk factors are," says Nordli. "Principal investigator Shlomo Shinnar, MD, at Einstein Medical School in New York, has assembled a first-class team."

Previous studies have attempted to find a link between febrile seizures and epilepsy, but have lacked the funding, methodology and patient population large enough to answer the question. With six medical centers participating in the current study, investigators hope to enroll 200 children and track them for five years. In addition to collecting traditional data such as a medical history, magnetic resonance images (MRIs), and EEG analysis, two new vital pieces of information will be gathered from each of the patients - evidence of previous viral infection and genetic markers. Leon G. Epstein, MD, head of the division of neurology, is leading the viral component of the study.

"With this information," says Nordli, "we'll be able to determine who's at risk by recognizing if it's this particular set of clinical characteristics, infection with this virus, or this genetic pre-disposition. Then we can do something about it." Nordli points to his basic science research colleagues at Children's Memorial and throughout the country who are investigating "neuroprotective strategies" such as diet and medications that might prevent the development of epilepsy in patients identified as high-risk.

Though it is still in the very preliminary stages, Nordli says he is encouraged by the design of the febrile seizure study. "We've learned that it is practical to do collaborative studies in the field of epilepsy," he says. "Even in one very large institution you won't have enough patients to answer a question. It's critical for this kind of research to be conducted multi-institutionally."

[Note: This article appeared in the Spring 2004 issue of Carousel, the magazine of Children's Memorial Hospital.


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