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Implants quiet a body at war

A rare neurological disorder had twisted 19-year-old Ed Cwalinski's body into a prison; now, stimulators in his brain may set him free

Wednesday, April 11, 2001

By Anita Srikameswaran, Post-Gazette Staff Writer

Ed Cwalinski loves going to loud rock concerts, but the huge speakers really turn him off. Their magnetic fields shut off battery power to electrodes planted deep within his brain. And without those few volts of stimulation, his body turns back into a prison.

In a hospital video made when he was 18, Ed Cwalinski Jr. is aided by his father, Ed Cwalinski Sr., and his mother, Emily, while powerful muscle convulsions pull him down. (Allegheny General Hospital video)

A year ago, Cwalinski couldn't do anything except lie down while the muscles in his back, arms and legs tied him into knots. The endless spasms not only hurt, but the exercise made him sweat profusely and pushed up his blood pressure. Pounds melted off his tall frame.

Then, in March 2000, Cwalinski became the youngest person in the United States, at age 18, to get deep brain stimulators for the treatment of dystonia, a neurological disease that few people even know exists. More than 20 other dystonia patients in North America have undergone the procedure.

The stimulators are used most often to stop tremors in older patients. Cwalinski's doctors worried about doing brain surgery to put the stimulators in a young man when there was no guarantee of success.

Cwalinski worried more about what would happen if they didn't do the operation.

"I wanted to go for it," he said. "I couldn't take anymore."

He was a sixth-grader when the symptoms began.

He noticed his right hand didn't work properly when he typed on the computer. He'd push the heel of his hand against the desk or keyboard to try to keep it steady.

Cwalinski is all smiles after he got a score of 99% on a math midterm. He has a perfect attendence record in technical school. (Darrell Sapp)

If he didn't, "it would go all over the place," he said.

Writing became increasingly difficult, and by eighth grade, he had taught himself to use his left hand instead.

Later, Cwalinski and his parents realized that he was experiencing writer's cramp, a common form of focal dystonia. The muscles of the hand contract inappropriately -- gripping a pen too tightly or twisting the wrist awkwardly.

As he got older, his shoulder and upper arm hurt whenever he threw a baseball. An orthopedics specialist told the family that Cwalinski had a winged scapula, caused by a problem with a nerve that controls the muscles around the shoulder blade. The doctor couldn't help, so Cwalinski stopped playing ball after ninth grade.

Then, in the middle of 10th grade, a very strange thing happened.

He was standing in line at Hampton High School's cafeteria and his legs seemed to lock up. Feeling off-balance, he sat down.

"When I went to eat, I started shaking," he remembered.

The school nurse was called. She didn't know what to make of his condition and suggested that his parents take him to the emergency room.

"They wanted me to go on this scale, and I could hardly do it. I was, like, ready to fall over," he said.

To the Cwalinskis' irritation, the doctors concluded that the odd symptoms were psychological.

Neither Cwalinski nor his parents believed them.

Couldn't stand up

After that, he gradually got worse. In February 1998, his junior year, it "came on full blown" one day.

"I had spasms throughout my whole body," Cwalinski remembered. "It was extremely difficult to walk. I couldn't really sit in any position. I stayed at home that day."

He didn't return to school for months. Instead, tutors came to his home. Trapped in his body by a mysterious disease, he rarely went anywhere.

As Cwalinski put it, "I'd pretty much lie on the floor and eat. I couldn't sit on the bathroom toilet. It was hard to find a position to even sleep. Mom was so depressed, she was crying."

One minute "you have a child that's running around and playing sports and doing what other kids do, then BOOM! This comes along," said his mother, Emily Cwalinski. "It was devastating. I was a wreck."

By chance, she saw Dr. Susan Baser, a neurologist at Allegheny General Hospital, talking about movement disorders on television. She called Baser's office to make an appointment for her son.

At first, the neurologist thought that Cwalinski had Sydenham's chorea, an uncommon complication of a streptococcal infection that produces involuntary movements and impairs the coordination of voluntary ones. It usually subsides after six months.

The family's initial relief gave way to frustration as time passed and the boy didn't get better. A variety of medications did little to control his spasms. With Baser's encouragement, they decided to go to the National Institutes of Health in Bethesda, Md., to get a second opinion.

After examining him, the NIH experts showed Cwalinski videotapes of children with Sydenham's chorea and of a person with generalized dystonia.

"I knew it was dystonia right away," he said. "It was a little bit of a relief [to know]. But when the doctor told me it doesn't go away ..."

No family history

Most dystonias are what doctors call idiopathic, meaning the causes are not known.

A dystonia can be focal, such as a writer's cramp, or segmental, such as torticollis or wry neck, in which the neck muscles force the head to tilt to one side. A hemidystonia, in which half the body is affected, is usually the result of a traumatic brain injury. Primary dystonia, which is what Cwalinski has, is often genetically based.

Inherited dystonias follow a dominant pattern. That means that if just one parent passes along the abnormal gene, called DYT1, the child can develop the disease even if he inherits a normal gene from the other parent. Patients with dominantly inherited diseases usually have several family members who also have the disease.

Last year, Cwalinski had a blood test that confirmed he had the faulty gene. His parents have not been tested, but neither has any symptoms, neurologist Baser said. "It was always felt that one of the parents had to have it."

Nobody else in his extended family seems to have dystonia, either.

Baser said a recent study of patients being treated for torticollis, the neck problem, revealed that a quarter of them carried the gene, but had no family history. And, according to the Dystonia Medical Research Foundation, fewer than 40 percent of people who carry the DYT1 gene develop any symptoms.

So it appears the abnormal gene doesn't affect everybody the same way.

Dystonia is treated primarily with medications for the neurological system, including some used in Parkinson's disease. About 50 percent of patients get moderately better with drug therapy, but it typically loses effectiveness over time.

Cwalinski tried a variety of medicines in the months after his diagnosis, but none kept his body still for long. He returned to Hampton High School at the start of his senior year, but after a month, the muscle spasms forced him back to home-based tutoring.

Muscular tug-of-war

"We kept repeatedly admitting him to the hospital because he'd become dehydrated," Baser recalled. "He was profusely sweating and just in horrible pain."

Normally, when one muscle group contracts, an opposing group relaxes. For example, when the biceps contracts, the triceps relax, and the result is the elbow bends smoothly and without causing pain.

Cwalinski's muscles were engaged in a tug-of-war, and the young man was the rope. His biceps would contract to bend the elbow while his triceps fought to straighten it out. The same was true of his back muscles, each group trying to pull his torso over to their side. That made him arch and twist to the right in an unnatural posture.

"I never got used to that," said Cwalinski's friend, Dave Delbusso, who is now a student at the University of Cincinnati's College -- Conservatory of Music. "He always looked like he was uncomfortable. He couldn't even sit up. He would shake uncontrollably. It was bad there for a while."

The Cwalinskis had become regular readers of a newsletter for dystonia sufferers and kept abreast of the latest findings. They heard about deep brain stimulators through that network.

To Cwalinski, the devices sounded ideal.

His doctors were more cautious, however.

Neurosurgeon Donald Whiting of Allegheny General had implanted electrodes deep inside the brains of people with tremors of a limb. More than a year ago, he put them in Cliff Baguley of Leetsdale as an experimental treatment for the spectrum of motion problems seen in Parkinson's disease.

But Whiting and Cwalinski's parents thought seriously about the risks of doing the procedure in a young person.

"Bleeding, stroke, coma, even death are possible. Unlikely, but that's kind of the gamble you take," the neurosurgeon said. "There's a 1 to 2 percent chance of anything really bad happening."

But the youth was ready to take that chance, knowing also that there was no guarantee the implants would help.

The night before the surgery, Cwalinski's friends filled his basement room and wished him well. He wasn't nervous about the surgery, but his mother was.

"They're all down here having a good time. I'm upstairs crying," remembered Emily Cwalinski. "They said he could be paralyzed, and I thought, 'Gee, paralyzed.' But even that would be better than what he was going through.

"That's a terrible thing to say," she quickly added.

Guiding the electrodes

Cwalinski's first surgery began in the early morning of March 31, 2000. Neurosurgeon Whiting worked out a theoretical target for the electrode with computer technology that married CT and MRI images of Cwalinski's brain with an anatomical atlas. He homed in on the site by listening for the rapid crackle of firing neurons.

Cwalinski was awake but mildly sedated during the surgery. A tiny hole was made in the top of his skull to insert the electrode.

The surgeons put one electrode into the left side of Cwalinski's brain, which controls the body's right side. They decided not to immediately bury the stimulators' battery pack, which was to go under his chest skin, in case Cwalinski awoke unchanged and the procedure had to be reversed.

But it was quickly clear after the surgery that the stimulator was having an effect, even at its lowest voltage setting.

"It was pretty amazing," said his friend, Tim Miller, 19, who attends Penn State University. "Ed was lying there normally. Even when he lay on his back before, he'd be kind of stretched out. But he was lying there perfectly. His hands were fine."

The battery pack was placed in his chest a few days after the procedure. The wires from the stimulators to the batteries run under his scalp and through his neck, beneath the skin. In the following months, voltage adjustments were made with a signal from a laptop computer.

It was enough improvement to let Cwalinski attend his graduation and to go on a senior trip to the Outer Banks in North Carolina.

"I was so glad I went to prom," he said. "I had the best time in my life."

Cwalinski's friends, who faithfully kept him company when he was ill by going to his home to watch TV and play video games, now got him out on the town.

"He was making up for lost time," his mother said. "They were going out every weekend."

Pleased with the results of the first operation, Whiting and Baser concluded that a stimulator on the other side of Cwalinski's brain could alleviate the rest of his symptoms, such as the shaking of his left hand.

The second implant procedure was performed Aug. 7. Cwalinski was able to leave the hospital after a three-day stay.

"With the first one he was still shaking and complaining about being sore," said Delbusso. "But after the second one, he was fine. He was completely still, sitting there watching TV, completely coherent and just talking. Normally he really wouldn't be doing that."

"I was just hoping to keep him out of the hospital," neurologist Baser said. "I never dreamed he would get as good as he did."

A brief relapse

Nearly a year after his first operation, Cwalinski's old symptoms flared up. He began shaking, and his right wrist hurt from writer's cramp. Around that time, a crew from the ABC newsmagazine "20/20" was interviewing the Cwalinskis for an episode that might air next month.

"He's getting a little mad because he was doing so good," said his father, Ed. "But that's the way dystonia works. It's just going to take time."

A visit to the neurosurgeon revealed that one of the electrode leads had burned out. Extra leads are placed to compensate for such a problem, so a simple change was made in the stimulator settings.

"It seemed to make a difference," Cwalinski said. "I'm walking straight and the movements have subsided."

His right hand, though, is still cramping, forcing him to type one handed. He is working on his associate's degree in computers and information technology at ITT, a technical school in Monroeville. He holds a perfect attendance record and is on the honor roll.

Cwalinski isn't convinced that his hand will return to normal, but doesn't regret getting the stimulators. He hopes people who see his story focus on the powerful change they made in his life.

"Before, I couldn't do anything," he said. "They'll be amazed."



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