San Diego Dr. Kenneth Ott, a neurosurgeon with Scripps, fights to contain a smile while talking. He has good reason to smile: his successes can be measured in human lives transformed. Ott's specialty is performing deep-brain stimulation surgery on those suffering from Parkinson's disease, and the results are dramatic.
Taber's Cyclopedic Medical Dictionary defines Parkinson's disease as "a chronic nervous disease characterized by a fine, slowly spreading tremor, muscular weakness and rigidity, and a peculiar gate." It is named for British physician Dr. James Parkinson (1755-1828) whose "Essay on the Shaking Palsy" clarified and defined the symptoms of the disease for other doctors.
"I always tell people that Parkinson's is a disease of the whole family, because it devastates and disables the whole family. There are about a million people in America who have Parkinson's disease, and the number is going up each year as our population ages. It's about half a percent of our population. And it's a very complicated, difficult disease to treat because there are so many drugs and the side effects are so complicated. It's one of the most difficult things a neurologist can deal with. Most neurosurgeons just do spinal surgery."
The treatment for Parkinson's disease has changed because of the limitations of traditional therapies. "It's a degenerative disease of the central nervous system for which there is no cure. Part of the cause is a depletion of a drug called dopa within the depths of the brain, and in the '60s they began treating patients by giving them dopa. It is highly useful in treating the symptoms, and there are three main symptoms: Tremor, which everyone knows about; bradykinesia, which means slowness of movement; and the third and very disabling symptom is rigidity. Muscles become rigid, and the patient feels like a prisoner in his own body. The tremor is more of a cosmetic problem. Dopamine is still the most important medication given. In the late '40s, there was a treatment developed called stereotactic surgery when a neurosurgeon discovered that certain areas of the brain, when destroyed, alleviated the symptoms of Parkinson's disease. One of the areas of the basal ganglea, when heated up, destroys a little group of cells, and the symptoms will go away. The surgery is done with an external frame affixed to the head, using internal landmarks of the brain to guide the needle to make the little lesion. It was quite effective, but when dopamine came in the '60s, why operate and risk injuring patients?
"Dopamine had widespread use, but after a long period of time it became evident that the effects of dopamine gradually wore off as the disease progressed. So patients would require higher and higher doses with less and less effect. And the higher doses of dopamine created a movement disorder called dystonia, with jerky, almost writhing movements of the arms and legs. After many years of treatment, patients would reach a very disabled state as they waxed and waned from being 'on' on the medications, and when they'd wear off, they'd freeze, and this would happen several times a day. In the past 15 to 20 years, stereotactic surgery has undergone a revival because imaging of the brain is so much better, and we can see the structures better. The operation is much safer because of two new technologies that allow us not to destroy the brain: One, we can put an electrode in the brain and stimulate these areas without destroying them -- making it reversible. It's placed in the sensitive area of the brain and let out under the skin to a little stimulator much like a pacemaker. The other technology has been the gamma knife, which has very focused radiation that can destroy an area noninvasively without heating up an area with an electrode." The gamma knife is a laser device that allows surgeons to use radiation in treating what were once considered inoperable brain tumors without even making an incision.
The use of stimulators or deep-brain stimulation was first tried by French neurologist A.L. Benabid in 1991. "It's like Newton said, 'If I've seen farther than others, it's because I've stood on the shoulders of giants' -- you've heard that aphorism. That's the way it is with surgical innovations. [Benabid] didn't just invent this. The fact is, part of the operation to destroy an area of the brain with a heating current is to stimulate it first to be sure you were in the right spot. Benabid thought, 'Let's just leave the electrode in there and hook it up to a stimulator.' By then, the technology for pacemakers had been invented, so we had the stimulators. The electrode is placed through a small hole in the head and a wire is let underneath the skin so you can't see it, and the stimulator is placed in the chest wall below the collar bone."
The most important thing about the surgery for Dr. Ott is that it works. "For tremor, it's about 80 to 90 percent successful improvement. There are scales of measuring rigidity and movement, a unified Parkinson's disease ratings scale. It rates activities of living, motor function, rigidity, tremor, and other side effects of Parkinson's. It's a semi-qualitative assessment. The FDA did a scientific study with control groups with the stimulator off and on -- people assessed how well it worked who did not have a vested interest in it -- neurologists who were blinded as to whether the stimulators were off or on. They found a significant improvement in the rating scales."
Ott has been performing deep-brain stimulation surgery for about four years. "The battery just wore out on my first patient yesterday, and I had to replace her stimulator, which is easy to do." One other hospital in San Diego, UCSD, also offers the procedure, but Ott believes it is about to become more available -- even to younger patients such as actor Michael J. Fox, who has battled the disease since 1991. "The results of the stimulators are so good now, that they are considering liberalizing the indications to people who are younger and not yet disabled. Why put up with the expense and problems of dopamine when it can be repaired this way? There's also some theoretical advantages to stimulation -- this is controversial -- that there may be some protection against continuing degeneration of these people. But that needs to be proven.
"The problem now is that the FDA doesn't pay for it. The most effective target is the subthalamic nucleus, which is where the stimulators will be placed in the future, but the FDA hasn't approved it for use there yet. This will be released within the next six months by the FDA. We've done several now on younger patients who are not on Medicare. That doesn't mean that it's illegal for doctors to do it. The FDA does not regulate doctors' practices, and we can do what is called 'off-label use.' The doctor has a right to use drugs for conditions other than their primary use. For instance, Viagra was used to treat heart conditions, and they found it had off-label use for you know what! So while many of the medications and things we do in surgery aren't officially approved, it's not against the law, but the federal government won't pay for it."
Deep-brain stimulation is expensive. "The transmitter and electrodes cost between $10,000 and $11,000. With the overnight stay in the hospital and operating room costs, it runs about $20,000 to $30,000. I don't think the hospitals make any money doing it."
Although deep-brain stimulation provides extraordinary relief for Parkinson's sufferers, not everyone is a candidate for the surgery. "The indications are that patients have failed medications -- dopamine -- and are disabled because of that. They must also have Parkinson's disease. There are a number of other degenerative conditions of the central nervous system that look like Parkinson's disease but aren't. They may have some of the signs, but they don't respond well and are not good candidates. They have to have a good response to dopamine. The effect of the stimulator is like the best response a patient has ever had to dopamine without ever having the side effects. It also takes hours to program the stimulators -- Dr. Dee Silver is the neurologist here who does that -- but they have to live nearby and be able to cooperate. It takes between 4 to 16 hours of programming to finally get the correct parameters. Patients can't have any dementia either."
Ironically, Ott performed a similar procedure to this surgery before he entered medical school. "I was a teenage kid in Pasadena and got a summer job in a neurophysiology lab at UCLA, where I was putting stimulating electrodes into cats' brains, doing single-fiber recordings from spinal cords, and so forth. That's how I got into neurology; then I was interested in surgery in medical school so I thought neurosurgery was the way to go. I do a lot of spinal surgery, but as my profession turns, we started the Gamma Knife Center here in town, another incredible technology, which was originally invented to treat movement disorders by Swedish physician Lars Leksell in 1968. As computers and imaging came in, it's now used to treat brain tumors on an outpatient basis. We started that in '94, and I do a lot of brain-tumor surgery with the gamma knife. Then I got back to where I was when I was 16 years old, doing functional surgery."
In spite of Ott's acumen and command, he shows a humility when asked how he feels about the results his patients enjoy. "It's pretty dramatic. Especially the tremors. The bradykinesia and rigidity are less dramatic. They are more disabling, but the tremor is very dramatic. The woman whose stimulator had to be changed had a tremor so bad that she would shake herself out of bed. Can you imagine sleeping with someone shaking so badly all the time? When you turn the stimulator on, the tremor goes away in seconds. It's like a miracle. It's amazing. Obviously, it makes me feel good, because it makes a big change in people's lives and you can see it. When someone has spinal surgery, they'll thank me because the pain in their leg is gone, but you can't see that. With Parkinson's, the effect is much more profound."
Ott believes that the cure for Parkinson's disease will ultimately come from the tests being done on animals. Presently, lab animals are given drugs that induce Parkinson's disease, enabling researchers to explore new treatments. "Some of the work on stimulators and the understanding of how Parkinson's affects the brain comes from the animal model, where we can put an electrode in the subthalamic nucleus of an animal, and the Parkinson's disease goes away just like in a human." When it is suggested that animal-rights activists might not appreciate such experiments, Ott is quick to reply, "They would if they knew what it was like to have Parkinson's disease. You know the saying, 'There are no atheists in foxholes'? Well, my saying is, 'There are no PETA [People for the Ethical Treatment of Animals] members in the National Parkinson's Association.' These experiments have allowed us to understand the fundamental physiology of it and allows us a model with which to treat Parkinson's disease."
Al and Corinne Goodman have been married since 1961. Although he works as an engineer with Ericsson Corporation in the Miramar area, Al's primary job is taking care of his wife, who has suffered with Parkinson's disease for ten years. He is also her spokesman, as the disease has limited her ability to speak. "She has some cognitive impairment, which is a result of the disease. For her, the biggest problems were originally muscle-spasm pain, and her shaking was so severe that she broke the bases of toilets where they bolted to the ground." Corinne was Dr. Ott's first deep-brain stimulation patient; it was her stimulator that he recently changed.
As Al tells the story of Corinne's and his ordeal, she is relaxing in a chair watching television. There is a slight tremor in her right foot. Al quickly points out that it was once far worse.
While tremors are to be expected, Corinne's case was extreme. "Dr. Silver, her neurologist, always considered her to be a rather challenging patient for Parkinson's. As a result of the medications not working after a while, she became a candidate for the deep-brain surgery. To my knowledge, she was the first one done at Scripps Memorial and certainly the first that Dr. Ott had done.
"Doctors do not take surgery lightly," continued Goodman, "including Dr. Ott. They do not rush to surgical solutions, and they will try all kinds of things first to be sure they've exhausted the arsenal of medicines first. Ott had done a lot of localization of brain-related things as a neurosurgeon and head of Gamma Knife, but he had not done that particular procedure. It's very difficult for surgeons, because they wanted her awake during the actual procedure, because you don't feel pain in the brain, and they wanted to make sure that they hit the right spot, so they'll ask her to do certain things during the operation."
The need to keep Corinne awake made the surgery almost impossible. "It's a balancing act -- they wanted a little shaking so they could get a positive determination to hit the right spot, but they didn't want too much shaking because it would interfere with the operation itself. In fact, they had to abort the first operation because her shaking was so severe. They had to stop it for safety's sake. As a result of that, they even convened a safety committee at Scripps Memorial, so there were lessons learned from that.
"Dr. Ott put in her first brain stimulator and it worked flawlessly. Her tremors absolutely stopped cold turkey. It was unbelievable to see the difference from what he had done. If you could see this lady in a before and after state, you would absolutely not believe it!"
While Al gushes with enthusiasm about the results of his wife's surgery, the Goodmans are both realistic and accept that while the symptoms have been reduced, the disease is still present. "Parkinson's is a progressive neurological disease, and the disease itself moved on. She later developed tremors on the other side of her brain, and she reached a point where they decided to put the stimulator on both sides. You see, the stimulator is put on the right side of the brain to stop the tremor on the left side of the body and vice-versa. They did it on the other side and were even talking about putting the second one in the subthalamic area, but it's not yet approved by the FDA, therefore they did both of them in the thalamic part of the brain."
Goodman praises Dr. Ott's skill effusively; he knows the surgery's success depends on the surgeon's accuracy. "You've got to place that probe on exactly the right spot. If it's a millimeter off, all bets are off. It has to be that precise, so there's a lot of dependence on a first-class, first-rate surgeon to get that placement done properly, because without it, the implanted device just doesn't matter. She currently has two implanted devices in her chest area, and there are actually leads that go up behind her ears into the brain. Unlike previous Parkinson's surgery, this is un-doable. The lead is left in the brain, and it sends a little micropulse to the region that's overactive to sort of short-circuit it out. It's an incredible, incredible thing."
Life is different for the Goodmans since Corinne's surgeries. "When she was shaking, she was totally incapacitated. She was in a ball of sweat! Now, with the stimulator, we're able to do things. On weekends, we'll go on little outings. We'll eat out, we'll go to the flower gardens in Carlsbad, we'll go to the beach, we can visit my granddaughter. Before, she would not be able to do any of that. My wife does depend on some medications, but, thanks to the surgery, it's been reduced from ten down to two. We've been able to reduce not only the number of medications, but the dosage as well.
"Before the surgery, there was screaming through the night, the vivid, wild dreams, yelling because of the muscle spasms. It was absolute hell. There were times when I didn't think I could cope anymore myself. At one point, we had siderails on the bed, so she wouldn't shake out. Even now, I don't leave her alone. I always have a caregiver come in. The folks at Ericsson have been unbelievably gracious. They've allowed me to bring some work home. I've purposely upgraded my home computer to make sure that it's compatible with my work situation."
Corinne's latest surgery, the stimulator replacement, was done on short notice. "Dr. Ott is a very busy surgeon, and even on this go-round when she was experiencing all this dramatic shaking, he worked her in within 24 hours after I contacted him. I am very grateful to him for the compassion that both Dr. Silver and Dr. Ott have shown. To have this whole team pull together in 24 hours in this day and age is remarkable. Just when you've got people figured, they surprise the hell out of you in a very positive way."
Al admits that his wife's illness has been a challenge. "When God handed out patience, I think I was absent that day. It's very difficult to deal with a spouse who has Parkinson's who is very slow by nature. My job is in process improvement for software engineering, and my role is to see if we can compete better, faster, and cheaper in a global economy and produce better-quality products, so there's a radical difference between my home life and my work life!
"When she was first diagnosed, both of us went through all the things that happen emotionally -- the denial, the 'why me?' and all of that. We have two grown sons and one of them lives in town here, and he has stepped up to the plate on several occasions. But I try not to bother them, because they have lives of their own."