It takes courage and discipline to live every day with the haunting uncertainty of epilepsy. A good doctor helps, too.
By Jerry Adler and Eliza Gray | NEWSWEEK
Published Apr 11, 2009
From the magazine issue dated Apr 20, 2009
The worst thing about the epileptic seizure Dan Wheeless suffered on the first day of eighth grade wasn't dropping to the floor in the hallway and awakening with no memory of how he got there. It wasn't even being kicked to get up by his classmates, who thought his collapse and jerking were an act; like, say, piloting a fighter jet, being known as the class clown holds uncommon risk for people with a seizure disorder. The worst thing was how the drugs he took made his brain slow down, so that processing auditory information became painfully difficult. He had to write down what was said to him, break it into clauses and concentrate on the meaning of each one. "Sometimes my mom would have to say something to me five times before I could understand it," he recalls. Switching medications after several months improved his cognitive problems, but the new drugs caused lethargy and weight gain, which disappeared only when a new doctor, Orrin Devinsky of New York University, figured out the right regimen for him. "He saved my life," says Wheeless, who was back to see Devinsky recently at his office in Manhattan. Wheeless, a handsome, strapping 32-year-old, went on to graduate from the University of North Carolina, marry an actress and begin a career in the theater. It may be an overstatement that Devinsky saved his life, because doctors can't predict who will die from epilepsy—although Wheeless, who estimates he has had 50 major seizures in his life and countless smaller ones, was certainly a candidate. Devinsky couldn't even save Wheeless's front teeth, all of which had to be replaced after he broke them falling down with seizures. But it is safe to say that Devinsky saved his mind.
When Devinsky was in medical school in the early 1980s, he was attracted to studying the brain in all its magnificent complexity and subtlety. But he wasn't sure he wanted to be a neurologist, doctors with the reputation of not actually doing much for patients. "In neurology, you'd see strokes, MS, brain tumors and migraine," he recalls. "You pontificated about whether the problem was in the brain, but in those days there wasn't much you could do for them." Then he discovered epilepsy, a disease that provides "a window into the mind." The study of epilepsy drove many of the earliest discoveries about how the brain was organized. But just as important to him, it was a disease doctors could actually treat, and go home at the end of the day having made someone better. Since 1989 he has run one of the largest epilepsy centers in the country, at NYU Langone Medical Center; he is the author of the definitive guide for patients, "Epilepsy," a cofounder of the Web site epilepsy.com and of the Epilepsy Therapy Project annd founder of an organization called FACES (Finding a Cure for Epilepsy and Seizures) that promotes research into new treatments.
Epilepsy is a uniquely human disorder, like psychosis, with which it used to be confused, or demonic possession, another now discredited diagnosis. Depending on the part of the brain affected, seizures can produce hallucinations, anxiety, feelings of religious ecstasy or bizarre psychological tics such as "hyperfamiliarity," a delusional sense that you're already acquainted with everyone you meet. Most often, though, they fall in a well-documented spectrum of mental and somatic anomalies, from the transient episodes of decreased awareness known as absence (formerly called "petit mal") to the tonic-clonic (or "grand mal") attack characterized by loss of consciousness, collapse and spasmodic stiffening and jerking. There is some debate about the long-term risk from repeated seizures; Devinsky maintains they can result in irreversible damage to the brain; some other researchers are less sure. But it's certain that uncontrolled seizures are associated with the risk of lasting memory problems, cognitive deficits, personality changes—and death.
Seizures can be triggered by a baffling array of stimuli, as blatant as a flashing strobe light or as subtle as, literally, thought. There are credible reports of people whose seizures were brought on by doing arithmetic, or by playing mah-jongg, or the sound of the TV personality Mary Hart's voice on television. The neurologist and author Oliver Sacks ("The Man Who Mistook His Wife for a Hat") described a woman, who was also a patient of Devinsky's, who would go into seizures at the sound of Neapolitan music. (Rx: Move to Sicily.) Patients sometimes use this knowledge to their advantage; one woman who never had more than one seizure a day induced one intentionally on the morning of her wedding day, so she could get through the ceremony on her feet. Alcohol, drugs, emotional stress and sleep deprivation are common triggers for seizures. Jet lag, a minor inconvenience for most people, can be catastrophic for someone with epilepsy. In earlier eras, people believed that seizures were influenced by the phases of the moon, something Devinsky doesn't dismiss out of hand. If the tides can feel the moon's gravity, why not the brain?
The brain, of course, is unique among the organs of the body both in its susceptibility to outside stimuli, and the variety of things that can go wrong with it. By contrast, a heart can fail in only so many ways. Conceptually, the job of the cardiologist is straightforward: he needs to restore a damaged heart to its normal rhythm. But epilepsy is the opposite. A normal brain is governed by chaos; neurons fire unpredictably, following laws no computer, let alone neurologist, could hope to understand, even if they can recognize it on an EEG. It is what we call consciousness, perhaps the most mathematically complex phenomenon in the universe. The definition of a seizure is the absence of chaos, supplanted by a simple rhythmic pattern that carries almost no information. It may arise locally (a "partial" seizure), perhaps at the site of an old injury, a tumor or a structural malformation. A network of neurons begin firing in unison, enlisting their fellows in a synchronous wave that ripples across the brain. Or it may begin everywhere at once ("generalized" epilepsy), with an imbalance of ions across the cell membrane, usually the result of an inherited mutation. At a chemical signal, whose origin is still a mystery, billions of neurons drop the mundane business of running the body and join in a primitive drumbeat, drowning out the murmur of consciousness. And so in contrast to the cardiologist, the epilepsy doctor must attempt to restore not order, but chaos.
Seizures can be fatal, especially the rare status epilepticus, a continuous convulsion lasting longer than 10 minutes. One of Devinsky's patients is a teenage boy who showed up at the hospital in December with status epilepticus of unknown origin, although Devinsky suspects a brain lesion from an undiagnosed infection. The only way to stop his convulsions was to induce a coma, and he has been in one ever since. Devinsky keeps looking for the right combination of drugs to save his life. Occasionally people with epilepsy will go to bed at night, apparently healthy, and die in their sleep; the autopsy may be inconclusive and the death is chalked up to SUDEP—Sudden Unexplained Death in Epilepsy. Among patients with refractory seizures—ones that can't be controlled with medication—the risk factor for SUDEP is a little less than 1 percent a year—small, but not negligible. Some of these patients may be candidates for surgery. But in general Devinsky spends more time thinking about the quality of a patient's life than the length of it. "Imagine you come to see me after a single seizure, just one," he says. "I examine you and do an EEG, an MRI and everything looks good, so I say, you're in luck, there's only a 20 percent chance you'll have another one." That is roughly the same risk a middle-aged man or woman runs of having a second heart attack within five years of the first. "But that could affect your life tremendously. Say you're a truckdriver, or a surgeon. Or if you're a commercial pilot—well, that's done." The biggest quality-of-life issue for most people with seizure disorders is driving; every state restricts, to varying degrees, the licenses of people who have had seizures. Most require them to be seizure-free for a certain number of months or years before restoring driving privileges—but only six require doctors to report seizures, so presumably a lot of people are getting away with it. "You say, 20 percent, that's terrible, I want you to tell me I won't have another one. And I can't. And then if you have two, your chance of a third goes up to 70 or 80 percent."
