Two million people have it. 11 million will have it. It’s slightly more common in males than females, and although 70 percent of children will outgrow it, it can affect people of any age, starting in infancy or in middle age. It can be hereditary — even if no family members have it — or it can come from a head trauma or out of nowhere. And 30 percent of all cases go untreated.
It’s epilepsy, one of the oldest documented and misunderstood brain disorders in history. In 400 B.C., Hippocrates recognized that epilepsy was a brain disorder, and “he refuted the ideas that seizures were a curse from the gods and that people with epilepsy held prophetic powers,” writes Dr. Orrin Devinsky in “Epilepsy: Patient and Family Guide,” the third edition of the definitive handbook for epilepsy. Witches — who were hunted down and killed — were often characterized by seizures, and by the 19th century, epilepsy was considered a contagious and psychiatric disorder. “In the early 20th century, some U.S. states had laws forbidding people with epilepsy to marry or become parents, and some states permitted sterilization,” Devinsky writes. “We have come a long way.”
Parkinson’s disease has celebrity Michael J. Fox representing it (he is also afflicted), AIDS has Elton John, colon cancer has Terrence Howard (whose mother died from the disease), and breast cancer has those little pink ribbons and myriad celebrities touting the need for a cure. But epilepsy has few famous public advocates. “A lot of people have epilepsy and it’s not widely known,” says Dr. Scott Hirsch, primary neuropsychiatrist at the New York University Comprehensive Epilepsy Center. Compared to other diseases, epilepsy is “underfunded and under-researched and not as vocal,” says Hirsch, who is also a clinical assistant professor of neurology, psychiatry, and child and adolescent psychiatry at the NYU School of Medicine. “It’s important to have that for epilepsy.”
Epilepsy does have that advocate in Devinsky, the founder and director of the NYU Comprehensive Epilepsy Center in New York, the largest epilepsy center in the United States, which has more than 2,500 referrals per year. Devinsky may not be a celebrity, but the professor of neurology, neurosurgery, and psychiatry at NYU School of Medicine has published more than 250 articles and chapters in more than 20 books on everything from the origins of brain delusions to “Embarrassment as the Aura of a Complex Partial Seizure.” Devinsky has been named Best Doctor by numerous publications and organizations, and he co-founded epilepsy.com and the Epilepsy Therapy Project, as well as FACES (Finding a Cure for Epilepsy and Seizures), an organization that promotes research into new treatments.
“I thought I’d do more basic science,” Devinsky recalls, sitting behind his desk in a corner office on Manhattan’s East Side. When he attended Harvard Medical School in the early ’80s, he loved studying the brain but felt unsure about neurology because he thought that, with brain tumors, “you just gave them a diagnosis and sent them home.” But his mentor in medical school, Dr. Norman Geschwind, the father of modern behavioral neurology in America (as well as the creator of the term behavioral neurology), attracted Devinsky and countless others to the field. One of Geschwind’s areas of interest was epilepsy and how it worked; Devinksy decided to focus on that. “Epilepsy was a very attractive area,” he says. “It was one of the few areas of neurology [at the time] where you could do something for someone … You could help people.”
And help people he does, since founding the center in 1989, always searching for better and earlier diagnoses, better treatments and a better quality of life for his patients. One of his major accomplishments is bringing the disorder to the forefront of society — explaining the disorder, its causes, treatments like drugs, surgery, and lifestyle changes.
“A seizure is a brief, excessive discharge of brain electrical activity that changes how a person feels, senses, thinks, or behaves,” he writes. “During an epileptic seizure, the regulatory systems that maintain the normal balance between stimulation (excitation) and dampening (inhibition) of nerve cells break down. For example, there may be a loss of inhibitory activity or an overproduction of an excitory neurotransmitter that causes a group of neurotransmitters to fire excessively. These in turn may stimulate neighboring cells or cells with which they have strong connections. Ultimately, large populations are abnormally activated all at once (synchronously). That is, the electrical discharges of many cells become linked, creating a storm of activity.”
His websites, books, and articles all answer common questions such as: “I had one seizure, will I get epilepsy?” (There’s a 50 percent chance.) “Does epilepsy cause mental illness?” (No, but there is a higher rate of anxiety and depression.) “Do seizures change the brain?” (Brief ones don’t, but grand mal ones lasting longer than 20 minutes do; memory problems often occur in either.) “If I have epilepsy will my children?” (Probably not.) And, “Is epilepsy a lifelong problem?” (No, most people need medication for a short time, and if a person is seizure-free for two to four years, they can often go off meds.)
No one knows exactly what causes epilepsy. Devinsky is one of 150 experts involved in the Epilepsy Phenome/Genome Project, a government-funded study to find genetic causes/influencers of epilepsy and genes affecting the response to treatment. The study involves 5,250 people. According to the project’s website, “The goals are to better understand what causes epilepsy, why some families have several people with epilepsy, and how we can better predict which anti-seizure medications (or no medication) will work best for an individual.”
“If people do know that there are two siblings or parents and children with epilepsy, we would be interested in talking to them,” Devinsky says.
Genetics is only a small part of epilepsy. “Anyone can get it from a car accident, a stroke, a tumor, Alzheimer’s, or an infection like meningitis,” the doctor explains. And while the important piece of the puzzle might be the source of the disorder — although many people believe there is causality where none exists, he says — just as important is controlling it and having a good quality of life. Quality of life could mean finding a new job, because seizures prevent a person from working in certain fields, such as operating heavy machinery or where driving is required.
Quality of life also extends far beyond the seizures: “The disorder continues to carry a significant stigma,” he says. Children can be insensitive to others with the disorder. Other children’s parents, teachers, and others don’t understand the disorder, which is why Devinsky suggests people say they have a “seizure disorder” rather than “epilepsy,” although he recommends in his book that his patients get comfortable with their condition because, “People see you through your eyes. If you are afraid of epilepsy, they will be afraid of it. It is a neurological disorder; if you are comfortable talking about it, then almost everyone else will be.” Devinsky founded FACES to challenge the stigma and educate the public to improve people’s quality of lives.
Quality of life doesn’t only mean not having seizures but few or no side effects from anti-epileptic drugs (AED). “Do not accept troublesome side effects without finding out if they are avoidable,” Devinsky recommends. For example, “Sally” was just starting to speak, but when she was put on Topamax, an anti-epileptic drug, her speech was shut down. “On the lower dose it slowed down and became more effortful, and as we pushed it up she stopped speaking,” one of her parents says. Five days after they took her off the medication, she was speaking again. Side effects or not, parents must build their children’s self-esteem, and cultivate it in themselves.
In addition to AED, people with epilepsy can go on special diets, use alternative therapies like biofeedback and, if all that proves ineffective in controlling the disease, can consider surgery. Today, people whose seizures cannot be effectively controlled for one to two years are considered candidates. “In general, the patient should be treated with at least two single drugs, or a combination of two or more drugs before surgery is considered,” Devinsky says. There are two main types of surgery: Lobotomies, which are resective surgeries, in which the part of the brain causing the seizures is removed; and the less common corpus callostomy, which is a disconnection “interrupting nerve pathways along which seizure impulses spread.”
Experimental trials are under way to test other solutions. “There is an explosion of scientific information on how the individual neurons work and how they communicate in normal and diseased conditions,” Devinsky writes. Solutions include early seizure detection like magnetic resonance spectroscopy (MRS) to discern biochemical changes in the brain, as well as using a device to sense a seizure’s onset and release medication directly to the troublesome brain area to prevent a seizure. “Right now, we’re focusing on treating epilepsy, but this device can eventually be of enormous benefit to patients with Parkinson’s disease, Alzheimer’s disease, and stroke victims,” he says. Other trials include using brain stimulation like biofeedback to prevent seizures, and transplantation of stem or fetal cells.
Ultimately, though, there is not one cure for epilepsy, since it’s a spectrum of disorders. But the vision is still the same, as Hirsch succinctly says: “Our goal to completely stop seizures in every patient. Completely.”