LLUMC Legacy: Daring to Care
PALLIDOTOMY: A MEDICAL MIRACLE FOR PARKINSON'S DISEASE?
The patient's hands and arms shake violently. These tremors stop instantly. A patient with one-inch steps shuffles into Loma Linda from Rochester, New York, and walks normally out of surgery. A young man arrives in Loma Linda from Brazil, bobbing and weaving from the waist up, arms flailing uncontrollably. His wife walks 10 paces ahead, because she cannot walk by his side. The couple walks out of Loma Linda side by side.
Parkinson's disease is a progressive, central nervous system disorder, affecting possibly one million Americans. It was originally called "shaking palsy" when it was identified by James Parkinson in the 1800s. It can eventually lead to complete helplessness. It occurs mostly in people over 50 when the brain stops producing a chemical neurotransmitter called dopamine. Without dopamine some brain cells become overactive, creating classic Parkinson's symptoms. Symptoms include weakness, unstable or twisted posture, freezing (muscle rigidity), on-off phenomenon, tremor, and impaired voluntary movement. In advanced stages it might cause patients to have an expressionless stare. They may be unable to swallow or even blink. The symptoms are caused by an overly active neuronal pathway in the brain, but the basic cause of Parkinson's disease is unknown.
A medicine called L-dopa is the single most effective drug available for Parkinson's disease. It can suppress the symptoms for several years by trying to replace the dopamine in the brain. It is designed to calm the overactive cells. But as the disease progresses it overcomes the drug's ability to control it, producing less and less benefit and sometimes its own negative, painful side-effects.
During a procedure called a stereotactic pallidotomy, a neurosurgeon tries to neutralize the overactive brain cells and relieve some of the Parkinson's symptoms. The procedure--including magnetic resonance imaging (MRI) scans used to map the brain--may take several hours to accomplish. But when the manifestations respond, they respond instantaneously most of the time. The procedure performed at Loma Linda University Medical Center has enjoyed a high percentage of success. Loma Linda neurosurgeons do not call the pallidotomy a cure, but the patients often do, sometimes running, jumping, and showing off to their doctor and their weeping families.
The pallidotomy is controversial and has been criticized by neurologists who consider it to be a bandage instead of a cure. Although they might acknowledge that the procedure sometimes can produce striking results, they point out that negative side effects can include stroke and impairment of vision, and they say it is premature to predict long-term results.
But people who are suffering from Parkinson's disease are desperate to find any relief, despite the possibility of complications. They don't want to wait, as their disease progresses, for the debate to be resolved.
The pallidotomy surgery begins to relieve the rigidity, tremors, and jerky, uncontrollable movements caused by the disease, sometimes instantly, while the patient is still in the operating room. On rare occasions the results are realized the next day. Thanks to recent research and the refinement of neurosurgical techniques, pallidotomy makes hopelessness a thing of the past. Results are often called "a miracle" by benefiting patients.
Discovered by Accident
Back in the late 1930s doctors noticed that Parkinson's patients sometimes experienced some relief from their symptoms, following a stroke in a particular area of the brain. Subsequent research identified the area as the globus pallidus, or pallidum, a tiny part of the brain that in Parkinson's patients is hyperactive and out of control. Neurons in this particular part of the brain are firing faster than a machine gun. The brain is not able to compensate or rebalance this area. The resulting manifestations of Parkinson's disease are crippling, disabling, and can be stressful to relationships.
In 1952 Lars Leksell, M.D., began to perform a neurosurgical procedure in Lund, Sweden, called "postero-lateral pallidotomy." The results were somewhat satisfactory, but the procedure was mostly forgotten when L-Dopa became available. In 1985 Lauri Laitinen, M.D. (Leskell's student) tested Leskell's procedure on 38 Parkinson's patients using stereotactic postero-ventral procedures and CT scans to provide greater precision. Within six months, studies showed that 92 percent of the patients experienced complete or almost-complete relief of rigidity and slowness of movement. Eighty-one percent suffering from tremor had complete or almost complete relief and improved walking and balance. According to Laitinen, a good test of the procedure is to have the patient dance after surgery. In time it was noticed that other benefits included improved mental acuity, rest and sleep, appetite, and feelings of well-being.
Robert P. Iacono, M.D., has made significant refinements in neurosurgical techniques with 500 cases. He is capable of hitting his target with an accuracy rate of one millimeter. Visual disturbance reported in Laitinen's scientific study was 15 percent. Iacono's refinements have reduced visual disturbances to less than one percent.
During the procedure, a small hole is made under local anesthetic in the top of the skull. The patient is sedated and able to converse with the surgeon and to observe what happens. Iacono places a thin Teflon-coated probe with caution and extreme precision into part of the globus pallidus of the brain. He then electronically stimulates the tiny area to test the accuracy of the probe's location. If the target has not been reached, the probe can be repositioned.
When Iacono is satisfied that the target has been reached, he heats the tip of the probe, creating a small, pearl-sized lesion in the brain. This two-millimeter lesion blocks the abnormal signals that are responsible for the symptoms of Parkinson's disease. The patient is able to see the improvement immediately--as it happens. The almost always immediate relief appears to be long-lasting. It is hoped that this relief will be permanent. Time will tell.
The procedure takes two to three hours and has been performed on an outpatient basis--the patient going home within four hours of surgery. Depending on the age and other health factors, many patients go home the next morning. Seventy-five percent of Iacono's patients show significant improvement in their symptoms. His success rate probably would be even greater if he did not operate on so many hopeful and appreciative patients over age 75. Studies show that the surgery performed on younger patients is 90 percent effective for 90 percent of the symptoms.
Mass Media Spreads the Word
About half of Iacono's patients are referred by physicians from around the world. The other half are self-referred after hearing about the operation through the Parkinson's support grapevine. Some patients who have benefited from stereotactic pallidotomy couldn't contain their enthusiasm. They spread the good news over the Internet. Their testimonials soon reached the Wall Street Journal, motivating a story that was published on the front page, February 24, 1995. Because Iacono has performed more pallidotomies than anyone else in the world, he was a major part of the story.
As a result, a follow-up story featuring Iacono was broadcast nationwide the following week by ABC's midweek evening news magazine, "Prime Time." Correspondent Jay Schadler followed Edward Hounsell, a former California Highway Patrol officer, into surgery. The procedure was videotaped, sent by satellite uplink to New York, and broadcast nationwide that night. A presurgery home video shows Hounsell, 59, stumbling, with head twisting and arms flailing uncontrollably.
Before the pallidotomy was performed, Hounsell stated with difficulty, his face contorted and twitching with muscle spasms, "I am looking for a better quality of life."
During the preprogram "teaser," anchor Diane Sawyer asked, "Is it for real? Doctors are debating the results, but in just a few minutes you'll see something remarkable." The segment was entitled, "Medical Miracle?"
By 7:30 a.m. Ed Hounsell has been admitted to "Pre-Op," and meets a fellow patient, a woman who has come to Loma Linda all the way from Israel for a pallidotomy. He says to her, "I wish you well."
A few minutes later a metal frame is fitted and attached to Hounsell's head. This large "halo" will provide a reference point during imaging and allow for his head to be stabilized during the procedure. His tremors continue as he goes into a magnetic resonance imaging scanner, a giant electromagnet generating 20,000 times the magnetic field of the earth. It allows Iacono to see inside Hounsell's head without X rays and to provide navigational maps Iacono will need to place the probe with precision inside Hounsell's brain. These scans will assist Iacono in finding and destroying Hounsell's hyperactive brain cells. "This is 21st-century neurosurgery," reports Iacono.
By 9:00 a.m. Jay Schadler and his ABC camera crew gown up and, with three cameras (one previously mounted to the ceiling), accompany the pallidotomy team into the surgery suite. Hounsell's legs and feet are still erupting in spasms.
After drilling two 13-millimeter burr-holes in Hounsell's skull and calibrating his coordinates, Iacono begins the delicate process of directing the probe toward the targeted cells deep inside Hounsell's brain. X rays provide a navigational update and confirm he is right on target. Before heating the end of the probe, Iacono fires electrical impulses into the brain, further refining his positioning technique and confirming the exact location of his target.
The end of the probe, this time penetrating the right side of Hounsell's brain, is heated. The heating procedure is accompanied by a rising, high-pitched tone. Immediately the tremors on Hounsell's left side begin to diminish. Twenty-three million viewers are watching nationwide. One camera shows the left foot with no tremors, the right one still moving uncontrollably.
Before continuing, Iacono conducts a quick verbal test to confirm that Hounsell's speech has not been affected. The patient repeats the doctor's words.
Iacono: "The ragged rascal ran."
Hounsell: "The ragged rascal ran."
Iacono: "Now is the time for all good men to come to the aid of their country."
Hounsell: "Now is the time for all good men to come to the aid of their country."
Iacono: "I pledge allegiance to the flag of the United States of America."
Hounsell: "And to the Republic for which it stands...."
The room erupts in laughter. The patient's sense of humor also is intact.
Iacono repeats the procedure on the left side of Hounsell's brain. This time, with the lights out, a microphone picks up the sounds of Parkinson's disease. While electronic noise is displayed as a graphic signal moving repeatedly from left to right on a scope, hyperactive neurons emit a loud, static, popping noise.
"The whole area is very hyperactive," says Iacono.
The second target is located and again the probe is heated. And again the most profound symptoms, this time on Hounsell's right side, are relieved. Hounsell waves his arms controllably and snaps his fingers.
"Anybody wanna dance?" he quips.
"Three hours after surgery began," reports Jay Schadler, "Ed Hounsell sits up on the operating room table, anxious and seemingly quite capable for the first time in years to begin a new life."
Because the procedure is done under local anesthetic, Hounsell is not taken to the post-operative recovery room. Instead, he walks out of surgery and, to the delight of the operating room staff, suddenly breaks into a run in front of the ABC cameras.
When the segment ends, anchor Diane Sawyer is shown shaking her head in disbelief. "Jay Schadler talked with Ed Hounsell's sister-in-law just a few minutes ago," Sawyer reports. "She's with him tonight at the hospital and says, ÔHe looks like the man I haven't seen in seven years.'"
Hounsell goes home the next day.
A week later "Prime Time" pays what Sawyer calls a "housecall" on Hounsell. First he is shown as he appeared eight days earlier, his head whipping back and forth, legs moving uncontrollably. Then they show the new man.
Schadler narrates: "This was Ed Hounsell yesterday . . . steady, balanced, and driving himself to a doctor's checkup."
"Life is fun. Life is worth living now," reports Hounsell. "It wasn't worth living before."
During his checkup, Hounsell's physical and mental capacities were tested again.
"He's improved 86 percent, at least initially here," reports Iacono afterward.
Schadler summarizes the results. "Though he has not been cured--some symptoms remain--Ed Hounsell says his step is lighter and his future brighter than he could ever have imagined a week ago."
At the close of the update, Sawyer reports, ". . . and tonight for the first time since his operation Ed Hounsell is back in his own home in Northern California . . . feeling just fine, he told us. And one of the things he says that delights him most is that he is able to pick up and read a book for the first time in years."
During the first two weeks following the broadcast, the neurosurgery office is flooded with inquiries from around the United States--4,500 calls. Iacono already had a waiting list in the hundreds.
Other Postoperative Testimonials
Bill Dickinson of Fullerton, California, had Parkinson's disease for almost seven years before surgery. He suffered from freezing episodes, 30-minute periods of mobility, being homebound to a chair, loss of 30-40 pounds, excessive sweating, one to two hours of sleep per night and difficulty controlling movement.
"The surgery has given me a whole new life," he says. "I was able to return to work in the morning, go out to lunch, and play golf in the afternoon. And I went on a two-week trip to Europe with my wife."
Some Parkinson's patients experience many symptoms. Don Berns, a Presbyterian minister from La Ca–ada, California, had Parkinson's disease for almost 13 years. (Before- and after-surgery videos of Berns were included in the "Prime Time" segment.) Burns's condition could only be described as tragic. He was almost completely immobilized, bothered by impaired and sluggish movement, weakness, sweating, tremors, stiffness, stooped posture, swallowing problems, and speaking difficulty. His eyes and mouth remained partially open during sleep. Burns previously had undergone an adrenal graft surgery at another institution, without benefit, and eventually had become completely disabled.
"I'm ecstatic and feel like I'm in a fantasy land," he says. "But with each passing hour the fantasy becomes more a reality, and what was the reality of disease now becomes a fading memory."
In a few minutes after the procedure, Burns wolfs down food it once took him an hour and a half to eat.
The pallidotomy is not a cure for Parkinson's disease, and it is not recommended for all Parkinson's sufferers. (Some patients who are not initally accepted into the program are asked to return later for reevaluation.) But it can remove or reduce some if not all of the most bothersome symptoms for most of those who qualify--those who do not respond well to medication. Patients report little or no pain using local anesthetic. Medication is not discontinued. The procedure makes the patient more responsive to the medication and allows some patients to reduce their medication.
Some of the symptoms require the patient, like Ed Hounsell, to have the surgery on both sides of the brain. After careful evaluation, Iacono may do both sides in one procedure or each side in separate operations. Postero-ventral pallidotomy is funded by most insurance companies and is covered by Medicare.
If a pallidotomy patient is asked to name his most important benefit from the surgery, he will probably mention his release from one of the physical problems. On an absolute scale of values, however, a more important long-term gift each patient may receive is a biochemical re-energizing of the brain's frontal lobe, cerebral cortex functions. Parkinson's disease does not damage the frontal lobes of the brain; however, it apparently gradually cuts off communication between the frontal lobes and the other parts of the brain and body. Some patients have a near-total disconnect. But a reversal is quite possible after the pallidotomy, apparently resulting from a more plentiful blood supply to the brain. The frontal lobes are the seat of personality and conscience, insight, foresight, judgment, attention span, and the ability to concentrate. They probably provide the closest physical connection to spirituality.