Once or twice he wandered in a daze into traffic on South Orange Avenue in Newark, his home town, oblivious to the danger. The seizures began when he was eight and pummeled him for 17 years like a playground bully who never grew up.
But David Hugee, now 25, is a fighter, the son of a Newark police detective, a martial arts student with a yellow belt who recites the names of karate kicks as if they were his friends. Front Kick. Pivot and Side Kick. Roundhouse Kick. In recent years he's taught himself to try to do battle with the silent electrical storms in his brain by harnessing the mental toughness he's honed in karate school.
"I sit down, close my eyes, and say 'Come on!'" he says. "I'm not gonna have a seizure. I'm gonna fight it." Walking Side Kick. Front Leg Roundhouse Kick. But mind over matter doesn't come easily when you suffer from epilepsy.
In the last year, though, Hugee has joined forces with the medical equivalent of a team of black belts, and the fight's gone much better. The ultimate kick for Hugee and his team at UMDNJ-University Hospital in Newark is that they have apparently beaten the bully. After an operation last October, his doctors said Hugee may never again suffer a seizure.
The long war against an old medical foe is far from over. Recurring seizures, hallmark of the disorder that takes many forms, affect about 2.5 million Americans and 40 million people worldwide. Often many times a day, abnormal spikes of electrical activity disrupt the normal flow of signals among the billions of cells in their brains. Worldwide, some 75 percent of those with epilepsy get inadequate treatment. Worse, in some countries, they suffer cruel and ineffective remedies. They're beaten, or tied to trees, or starved. The US hasn't always been kind to them either. Until 1956, more than a dozen states prohibited them from getting married. Restaurants had the legal right until the 1970s to forbid them to enter.
Today, the surgical breakthroughs that wiped out David Hugee's brain bully are of no use to most patients. The vast majority are treated with epilepsy's first line of defense: anti-seizure drugs. "All the drugs have a downside," says David Marks, MD, assistant professor of neurology at UMDNJ-New Jersey Medical School. "They all cause sedation because they dampen down the nerve cells. And they don't cure the epilepsy. They just stop the seizures."
But Hugee's triumph is a sign of change and victories to come, thanks to equipment as sophisticated as a spy satellite or laser-guided missile. Recently developed or refined technologies have given doctors a way to envision with road-map accuracy the areas where spikes begin, often without even opening the skull. The computerized equipment can help doctors watch the brain at work. Hues on computer-generated pictures change when patients move an arm or read a sentence. The chameleon-color shifts on a screen help doctors find the boundaries of healthy tissue and avoid "friendly fire" damage to essential brain control centers during surgery. New drugs like lamotrigine, gabapentin and topiramate offer hope to patients when other medications fail to quell seizures.
And in the most serious cases, where neither drugs nor surgery avail, doctors can now implant a kind of brain pacemaker. From its place under the skin in the chest, it shoots mild pulses of electricity through wires to a nerve in the neck that runs directly to the brain. The preemptive firing heads off at least some seizures before they can develop.
"Some fevers in infancy may lead to changes and damage within the brain that will result in long-term epilepsy many years later."
Peter W. Carmel, MD
Overall, changes in diagnosis and treatment over the past five years have been "dramatic," according to Peter W. Carmel, MD, professor and chief of the Division of Neurosurgery at UMDNJ-University Hospital. Hugee's problem began when he was a baby, Carmel says. "He actually had his first seizure during infancy when he was running a fever. We now believe that some fevers in infancy may lead to changes and damage within the brain that will result in long-term epilepsy many years later," with no symptoms in the meantime. The discovery adds another reason for parents to call a pediatrician whenever infants run a high temperature.
Medicine has come a long way since Hugee's uncle told his mother one day when he was eight, "That boy's having a seizure." There was little that anyone could do about it or the hundreds that followed, until the last few years. Fast-forward to Hugee in his mid-20s, when Marks and neuropsychologist David Mahalik, PhD, began the complex battery of tests that would culminate in a meeting with Carmel to decide on the feasibility of surgery. The black-belt team began to form around him.
Their earliest move was scoping out the location of the problem. Magnetic resonance imaging (MRI) gave the doctors a complete map of Hugee's brain structures. Resting in a tube big enough to accommodate his whole body, Hugee felt nothing. But the MRI machine bombarded his brain cells with a magnetic field. Atoms in the brain cells responded differently to the field, like dancers in a packed room moving differently to the same beat. The MRI equipment instantly picked up and analyzed the atoms' responses, and delivered computer pictures, much clearer than an X-ray, of his entire brain.
The MRI gave them the first major clue about Hugee's problem. The pictures showed that a part of his brain called the hippocampus was smaller on the right side than the left. That suggested that it could be the place where the seizures began. The team knew that removing part or all of the hippocampus might end the spiking.
But now they faced a couple of critical questions. "The hippocampus is like a computer's RAM useful for storing data for a short time until it can be transferred to long-term memory throughout the brain," Carmel says. "So we had to know what would happen to David Hugee's memory if we took away some or all of his right hippocampus." And where exactly did the seizures begin? No one on the team wanted to remove a speck of brain tissue more than necessary.
To check the possible effect of surgery on Hugee's brain, doctors injected a drug into the right side of his brain, essentially putting it to sleep. "Then they asked me a lot of questions," Hugee says, "like 'Who is the President?', and 'What's today's date?' They gave me numbers to remember. And they read out a grocery list to me: bread, eggs, sausage, milk, cakes, butter, hamburgers, cheese, pizza ...and had me repeat it." Then the same test was done on the left side.
Marks said the test came out exactly the way they hoped it would. "When his right side was anesthetized, he could still count numbers and tell you later what they were, and his ability to speak and to remember were intact," he says. "So we know the left side of his brain was working the way it should."
But when the left side of his brain was anesthetized, Hugee's speech and memory deteriorated. "We knew that the left side was dominant for speech and could support memory, so we could operate safely on the hippocampus on his right side," Carmel explains.
During surgery in October, the team opened Hugee's skull and placed electrodes on the glistening white lobe where they thought his epileptic spikes began. They picked up the equivalent of a local thunderstorm. "It was in the hippocampus," Carmel says, "and nowhere else in the lobe." Now confident that this is where the seizures were coming from, Carmel removed the right-side hippocampus.
When he returned from surgery, Hugee's family made a ring around his bed and waited for him to wake up. "When I opened my eyes, I said, 'How you doin', Ma?'" to his mother Lillian. She started crying with joy. "And I looked at my sister and said, 'How're you, Johnelle?' And I saw them all looking so serious. I said, 'Man, what's wrong?'" But as he moved his eyes around the circle, remembering every name and how far he had come from his days as "seizure head," his family broke into smiles and then applause. It was as good as winning a new karate belt, he says, when someone said: "Your brain is so strong."
Staring but not seeing, deaf to dance-club music from a radio only a few feet away, Cesar Fernandes, 28, was unaware of the electrical tumult in his brain. "A little piece of time went blank," he says. His wife Theresa, 27, described the episode: "I was talking to him and all of a sudden he was looking around the room but not answering me. I said, 'Cesar, what's the matter? What's the matter?'" A minute later it seemed like much longer to Theresa full consciousness filled in his mental blank. "Why are you crying?" he asked her. There was a good reason for her tears. Fernandes subsequently suffered several more seizures because of an abnormal collection of blood vessels in the left side of his brain, called a cavernous malformation. The vessels had leaked blood and were irritating surrounding brain tissue, close to the control center for speech. An MRI helped doctors locate the problem. The computerized machinery also enabled them to map out the boundaries of his speech center. During an operation last August, using technology pioneered at UMDNJ-University Hospital, a computer kept track of the location of surgeon Peter Carmel's scalpel and showed him at every second how close he was to healthy, essential brain tissue. As a result, Carmel was able to remove the malformation without risk. Today, Fernandes free of seizures works with computers as a senior information systems analyst with BASF. He's grateful for the computers that made his operation possible. But he has even more respect for the human ingenuity that designed them. "The computer can't run without our brains," he says. |
