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Parkinsons Disease: Sparks put a spring in an unsteady step Experimental surgery that planted electrodes into his brain shows promise for a man in the grip of Parkinson's disease Sunday, March 12, 2000 By Anita Srikameswaran, Post-Gazette Staff Writer
Every day for more than 30 years, Clifford Baguley hit the ground running.
Springing lightly off the balls of his feet, his legs and arms pumping with fluid grace, Baguley ran each day until all of his workday stresses and personal problems were ground into the dust.
Today, those six-mile runs exist only in Baguley's memory.
"Words can't describe how much I miss it," the 61-year-old Leetsdale man said recently. "I'd have given my right arm to be able to run again."
And why not? Three months ago, his right arm was nearly useless. It twitched so recklessly that tea splashed from the mug that he held in tightly curled, trembling fingers. He not only couldn't run, but sometimes he couldn't walk or get up from a chair. Many times, his body was frozen still.
Baguley has Parkinson's disease, a brain disorder that progressively destroys a person's ability to move.
His story might have played out the way it has for thousands of others, with Baguley becoming more and more incapacitated and eventually depending on others to assist him with dressing, eating and moving around. But he wouldn't let that happen.
Shortly before the New Year, Baguley had experimental brain surgery at Allegheny General Hospital. Doctors implanted tiny electrodes deep within his neural tissue to jolt malfunctioning brain areas into more normal activity. In a small number of patients, the procedure, called subthalamic stimulation, has dramatically restored normal physical function.
In the weeks since the remarkable surgery, Baguley has achieved triumphs that surpass anything he accomplished as a runner. He did it with the help of the two tiny electrodes, and the gifts he already had -- physical resilience, willpower and, above all, grace under pressure.
His surgeon, Dr. Donald Whiting, and neurologist, Dr. Susan Baser, also will be on hand to talk about the subthalamic stimulation surgery and answer questions about the treatment of Parkinson's disease.
For now, Allegheny General doctors can offer the operation to very debilitated patients who are no longer benefiting from drug therapy, which comprises about 10 percent of all patients, or nearly 500 people in this area.
They will not place implants in those who have developed dementia, another problem that occurs in some Parkinson's patients. The subthalamic implants still are considered experimental and insurance companies do not have to cover the costs.
The seminar will be held from 11 a.m. to 2 p.m. on Friday, March 24, at the Radisson Hotel Pittsburgh on Route 22 in Monroeville. Call (877) 284-2000 to reserve seating. To obtain information about the stimulator devices for control of tremor and other Parkinson's symptoms, call (800) 664-5111, Ext. 1055.
PG Online Graphic: Parkinson's disease/symptom relief
Falling forward
Two days before his operation, Baguley was feeling off. So was his friend and fellow Parkinson's sufferer Bill Kelley, who was visiting him at his Leetsdale home. Their already stiff muscles were made worse by the cold weather, which they had braved so that Baguley could get a haircut.
Baguley settled into his recliner, motorized to rise and tilt to help him get up, and began to speak in a voice that had been hushed by the cold and Parkinson's.
He talked about his 1981 arrival here from Melton Mowbray, an English market town known for pork pies and Stilton cheese. He also discussed running and living with Parkinson's disease.
As he talked, his body twisted and contorted until he came close to slithering out of his chair onto the floor. His right lower leg and foot twitched relentlessly. His head tilted so far to the right that his ear nearly touched his shoulder.
On a trip to the kitchen for tea, he swayed as though he were walking down an aisle on a bus that kept changing speed. He almost fell from side to side onto the nearest supports, from dining table to the chair rail along the walls. Life, Baguley said, was "stand up and fall over" -- and over and over. Most of the time, he caught himself. Sometimes, he hit the floor.
Kelley, 45, of Mt. Lebanon, wasn't as far along in his symptoms. His face was a mask nearly devoid of expression and he barely moved, both common features of Parkinson's.
"It's different for everybody," Kelley said. "My medication is working and I'm still stonefaced. [But] what you're seeing with me is very mild compared to what you're seeing with him."
How it started
Baguley was the picture of health until 16 years ago. That's when he noticed that his right arm felt leaden and he couldn't carry it properly as he ran.
Then the tightness began affecting his work as a draftsman for MS Consultants Inc. in Coraopolis. He had been famous for his speed at detailing blueprints of bridges and other structures. "I began to notice my drawings were getting a little bit ragged around the edges. Stuff was still going down fast enough. But the writing was terrible."
He was diagnosed with Parkinson's in 1986. He tried to keep running, but he was losing his coordination. "I was sort of lurching around and my ankle kept going over," he said. "It seemed I could always find a pothole."
Finally, after falling on the road with what felt like a broken leg, he decided it was enough.
Parkinson's had stopped Baguley, then 47, from running another step.
First described in 1817 by Dr. James Parkinson, the disease is characterized by motion and balance problems. Stiff limbs resist bending, a sign doctors call "lead pipe rigidity." Since smooth motion is impossible, patients try to connect a series of smaller movements. For instance, to start walking, Baguley would get up on his toes and take a few tiny steps that suddenly accelerated into a fast shuffle.
Tremors are also common, such as one called "pill rolling," where a patient's fingers rub together as if over a pill or paper money. In Baguley's case, his right foot endlessly traces small circles.
Another problem is freezing, when muscles lock up and motion is impossible for seconds or hours. Baguley said that one time when his body froze, he was inching his fingers toward the phone to call for help when he accidentally slid out of his chair onto the floor. A police officer came to lift him into the chair and couldn't believe that his body remained bent into an L, an image that still makes Baguley laugh.
"Not that I make light of everything," he said. "I think that love of life and action has been what's kept me going so long."
His optimism hasn't always prevailed, though.
"I thought many a time I might as well be dead," he said quietly.
Although they are at greater risk for dangerous falls, most people who have Parkinson's disease have normal lifespans -- but the quality of their lives gradually degrades.
Baguley feared losing his independence.
Two years before his diagnosis, his father had died of the neurologic disorder amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. Baguley returned to England after his father complained that he suddenly couldn't manage his garden spade. He was present for his father's diagnosis and the news that the progressive disease could kill him within a year, when the nerve impairment afflicted his breathing muscles.
"First time I seen me dad cry," Baguley said. "They couldn't do anything."
When his father was admitted to the hospital for further assessment, he shared a room with someone Baguley knew from the neighborhood. The man, once an avid cricket player, was a motionless wax image sitting in the bed.
Baguley can't forget the statue-like man next to his dying father.
"He had Parkinson's disease," he said. "That is what you get to."
Losing dopamine
An estimated 1.5 million Americans have the disease, which is thought to be triggered by a combination of genetics and toxic exposures.
For reasons not well understood, specialized cells that make the neurotransmitter dopamine gradually vanish from an area deep within the brain called the substantia nigra.
That's why, when Baguley was diagnosed, his doctors prescribed dopamine-replacing drugs. The dosage was increased over time to offset the gradual decline in the brain's production of the chemical messenger. Most patients will get several years of good results from the drugs, but after five to 10 years, the medications become less beneficial and produce more side effects.
That's what happened with Baguley.
By last year, he was taking maximum medication but still struggling through the days. Drug side effects had produced dyskinesias -- the wild involuntary twitches in his right arm and leg.
At work, it became obvious that the disease was no longer something he could shrug off.
"I couldn't get out of my chair to go to the bathroom," Baguley said.
He'd try a combination of pleading and demanding -- "Come on, legs, MOVE!" -- but he couldn't get them to do anything.
He felt like a prisoner in his own body.
More than anything, Baguley wanted to avoid becoming dependent on someone else. He is separated from his wife, Bonnie, and lives alone.
That triggered his interest in subthalamic stimulation, which he had learned about on a news program last year. Low voltage stimulation through electrodes implanted in his brain might improve many of his symptoms.
His insurance company agreed to pay for the $30,000 surgery.
Doctors at Allegheny General had never performed the operation, but they had done similar ones, known as deep brain or thalamic stimulation, that treated just the tremors caused by the disease. For the experimental procedure, electrodes would be placed in a different part of the brain.
Baguley was their first patient.
Two incisions
On the morning of Dec. 29, the operating room was crowded.
Besides the expected neurosurgeons, anesthesiologists and nurses, there were also a neurologist, technicians from the company that makes the stimulator, surgical trainees who wanted a glimpse of an operation they might routinely perform one day and, of course, curious medical staff and students.
The spotlight was on Baguley, who lay sedated with a halo-shaped device screwed into his head to help guide the surgeons in their delicate work. The skull and brain do not feel pain, but Baguley's scalp was numbed with injections of local anesthetic for the small incisions.
Working at the top of Baguley's head behind a barrier of drapes was neurosurgeon Donald Whiting, surgical director of Allegheny General's movement disorder center. The center's medical director, neurologist Susan Baser, stood by to test Baguley's muscle tone once the neurosurgeon had implanted the electrodes.
The surgeons guided a wire probe into the right side of Baguley's brain, seeking out a group of neurons called the subthalamic nucleus. They were trying to place the wire's tip, one-twentieth of an inch long, exactly in the middle of a structure that at its widest measures about one-quarter inch.
As the wire traveled through layers of tissue, everyone in the room listened to the sounds of Baguley's brain. Neurons fired messages to each other, crackling like radio static. A monitor displayed spikes of electrical activity. Whiting reached a consistent rat-a-tat-tat in the subthalamic nucleus and recorded the location.
Baser examined Baguley's left side, which is controlled by the brain's right hemisphere. She bent his arm back and forth at the elbow, noting the rigid muscles that made the motion choppy, like a gear turning in clicks. His hand and wrist were similarly stiff.
Then a small amount of voltage was sent though the wire to test Baguley's response to mild stimulation of the electrode. His wrist became supple, adopting a more natural pose. Baser worked his forearm again and it moved with ease.
"It's very close, if not normal," she said, delighted.
The team then implanted another electrode on the left side of Baguley's brain to treat the symptoms on the right side of his body.
Baguley was given a general anesthetic for the final part of the operation. The surgeons secured the two wires, one from each side of the brain, to his skull, tunneling them under the scalp, down the sides of his neck and into each breast. There, each wire plugged into its own battery-operated device implanted beneath his chest skin. The small devices allow doctors to adjust the voltage stimulation to Baguley's brain.
A lack of dopamine hyperactivates the subthalamic nucleus, which in turn overstimulates a nearby brain structure. When that site, called the globus pallidus, is turned on, it inhibits activity in the thalamus, which leads to many of the classic symptoms of Parkinson's.
Before the electrode surgery came along, the standard operative treatment was to destroy small areas of either the thalamus or globus pallidus to interrupt the abnormal pathways and improve symptoms of tremor and dyskinesia. Actor Michael J. Fox, whose Parkinson's has been highly publicized, underwent the thalamus removal surgery. The surgery isn't reversible, but the electrode implants are.
The electrodes seem to work by altering the signals to the thalamus so that motion is improved.
The day after the operation, Baguley was feeling well except for pain at the incision sites. He had little memory of the seven-hour surgery.
"You wouldn't recognize me today," he said during a phone call from his hospital bed. "I haven't had any rigidity in my right arm. My right leg has been twitching around a bit, but not like [before]. Everything is going better than I thought it might."
Whiting put the electrodes on the lowest possible setting and sent Baguley home. He cautioned that immediate improvements might not signify much, because brain swelling from the surgery can affect electrical circuits just like the stimulator. As the tissue healed, the need for the device would grow. The doctor would have to adjust the medication doses and voltage over the next few months.
New lease on life
On Jan. 6, a week after the surgery, Baguley was out and about, relishing his progress.
At Junior's Restaurant in Coraopolis, the staff was overjoyed to see their regular customer looking so much better. Before, Baguley's tremors and dyskinesia had made handling cutlery a messy if not impossible task. He also had experienced problems swallowing.
"We pureed his food for him one time because he couldn't get it into his mouth," owner Rudy Bolea said.
Now, as Baguley munched on a forkful of food, Bolea looked impressed.
"You're not jumping around as badly," he said.
Baguley's next stop was his workplace, MS Consultants. His engineer bosses made a point of examining the surgical staples in his head and offering to pull them out with the office staple remover.
Joking aside, they, too, were amazed that Baguley's tremors and dyskinesias were so dramatically better. It was as though a large hand had been placed on him to still his constant vibration. And indeed, Baguley said the stimulator felt as if someone was putting mild pressure on the top of his head.
This day's activity and his accidental tardiness in taking medication exacted a toll on Baguley. The drugs wore off and his gait and tremors grew worse. As he left the office building, he moved slowly and unsteadily and began to freeze more frequently. Eventually, he resorted to walking backward, a technique that seems to help him keep moving.
"You have to be patient," Baguley said. "You can't afford to get frustrated at all."
On Jan. 11, he returned to Whiting's clinic to have his stitches removed and get the first adjustments to the brain stimulator.
Glenn Nacey, a consultant from Medtronic, the company that makes it, was on hand to ensure that the doctors were comfortable with a laptop-sized computer that controls the device's output. Neurologist Baser also was there.
Baguley summarized his progress for them: "I don't get the shakes. I can sit still. I can use my knife and fork just like an Englishman should."
Baser agreed with his assessment and noted the good-humored glint in his eye with mock medical jargon: "He has no deficiency of twinkle."
But there was room for improvement, especially in Baguley's gait, so the doctors increased the voltage. They programmed the desired level, two volts on each side, into the computer. One waved a magnetic wand over the devices in his chest and, like magic, Baguley's arms became looser.
"It's like your arm is sleepy and numb, and it wakes up," he said of the sensations.
Baguley attempted a casual stroll, without a cane, up and down the hallway. He grinned and tried again, using the cane as a mock golf club when he came to a stop. The left leg was a bit tight, he said, and the right leg seemed to drag. He took tiny steps to initiate turning around, and occasionally hung on to the nearest wall.
He walked slowly, but he no longer swayed like he was about to lose his balance.
After a little experimentation with the stimulator's settings, and a few more trips down the hall, everyone agreed to try the two-volt stimulation for a week.
Going through therapy
Baguley's follow-up appointments have gone from weekly to monthly as doctors fine-tune the treatment. "It will take several months before we have him perfectly adjusted," Baser said. "But he's been able to dramatically decrease his medications."
Baguley has just completed four weeks of physical therapy that was initiated to help him deal with the awkward and painful tilt of his head. Scar tissue had developed in his neck muscles, which had been tightly contracted for years, and arthritis has damaged his cervical spine. Baser said his head tilt might always be present.
Not too long ago, even for a time after the surgery, Baguley slept in his lift chair because he couldn't turn while lying down or get up easily.
His physical therapist at Health South gave him strategies to overcome this, so now he can roll around from side to side.
"I'm sleeping in my own bed," he exulted. "I can now turn around and get things done. I'm sleeping better, eating better. I'm not choking on my food, not using a straw."
Although he has made great strides in his quality of life, literal strides have been more challenging.
Betty Kargacos, his physical therapist, at first saw two major problems in Baguley's gait -- his right arm doesn't swing and he can't seem to pick up his left leg to take a step.
Kargacos had him use ski pole-like tools to help him position and bend his arms to find a rhythm and momentum that would allow him to move more naturally.
"Almost immediately, he started walking a bit better," she said.
So much so that he walked through a doorway without pausing, a feat for people with Parkinson's. In the past, doorways, drains, curbs and even sidewalk cracks could throw Baguley off his rhythm. He'd stall out, then have to rapidly shuffle around the obstacle and work on lengthening his stride again.
During the past 101/2 weeks, there have been melancholy moments when Baguley grew frustrated at the slow progress in his walking. In the beginning, he pushed himself to walk far rather than properly, a behavior that Kargacos gently discouraged.
Now, he says he doesn't hope to run again.
"I don't want to be tempted," he said, a bit wistfully. "That will be dangerous to me insofar as I'd be trying to get more out of it than I can probably put in."
But patience and dedication have brought him a long way.
On this last warm and sunny Wednesday, Baguley went for a stroll around his block, a distance of about 350 yards, and negotiated drains and manholes without hesitating or breaking stride.
He walked carrying his cane in hand, arms swinging, feet moving in steady rhythm.
He smiled all the way home.
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