Parkinson's treatment now could be done while he slept
March 17, 2013 8:00 AM
Toni Pais cuts into a squash in the kitchen at Cafe Zinho in Shadyside.
Toni Pais talks to UPMC neurosurgeon Mark Richardson during a checkup in February. Dr. Richardson has developed a treatment for Parkinson's in which surgery that once required a localized anesthetic can now be performed using a general anesthetic.
Cafe Zinho owner and chef Toni Pais stands inside the Shadyside restaurant on a recent afternoon.
Toni Pais talks to UPMC neurologist Valerie Suski during a February checkup.
By Sally Kalson Pittsburgh Post-Gazette
Toni Pais doesn't look like a man with Parkinson's disease as he stands in his restaurant kitchen chopping vegetables. The right hand that shook uncontrollably six months ago now wields a steady knife making thin slices on the cutting board. When he lifts the lid on a pan of simmering Portuguese mariscada, or a shellfish stew, a cloud of aromatic steam fills the air. He breathes it in, looks up from under his baseball cap and smiles his mischievous grin.
This is where he is happy, at Cafe Zinho, the colorful, intimate bistro in Shadyside where he's been the chef/owner since 1997. He also had two other highly regarded restaurants, the upscale Baum Vivant on Baum Boulevard and Cafe Zao in the Downtown Cultural District. Both of those have closed, but Zinho and the Pais reputation for scrumptious food endure.
"I have Toni's mariscada every Tuesday for dinner," says Becky Pais, the petite, cheerful blonde who has been his wife and partner for 20 years. "I guess I'm a creature of habit."
How much longer her husband, now 58, would be able to feed that habit was unclear last summer. For eight years he'd been living with Parkinson's disease, a progressive disorder of the nervous system that affects movement. The medication he took for his symptoms helped control the hand tremors and stiffness, but the drugs were growing less effective, as they tend to do.
Mr. Pais could still work during his "on" periods -- he shopped and cooked five days a week for the restaurant, worked out five times a week and played soccer on Sundays. But the "on" times were fewer and further between, and the concentration needed to compensate was exhausting.
"It was mind over matter," Ms. Pais said. "When he'd focus on food and what he knew how to do, he'd be fine." But during "off" times, when the symptoms were more pronounced, his arms and hands shook so much that he cut out most of the other things he enjoyed -- wine tastings, speaking at food events, greeting patrons in his restaurant. His appearance took on the so-called "Parkinson's mask," or frozen facial expression caused by lost muscle control.
"The medicines were good enough for me to cook, but socially you change," Mr. Pais said. "I was very uncomfortable going out."
The Paises had looked into deep brain stimulation, or DBS, in which doctors implant electrodes in the brain and run connecting wires through the neck to a pacemaker-type device implanted in the chest. The device gives off impulses to stimulate production of dopamine, which greatly reduces the symptoms.
The catch, for Mr. Pais, was that DBS was done under local anesthetic, so the patient is awake to help guide the surgeons and avoid unwanted side effects.
"I saw a picture of it and almost fainted," he said. "I knew I couldn't do that for six hours."
Bring on the anesthesic
Then he learned that Mark Richardson, co-director of functional neurosurgery at UPMC, was doing something new in Pittsburgh -- DBS under general anesthetic, with the patient in an MRI machine instead of in an operating room.
In this treatment mode, doctors are guided by a computer screen and software hooked up to the MRI machine. This allows them to see where they are and pinpoint the spot of dopamine production in the brain, so the electrodes can be implanted precisely without collateral damage. The surgery takes the same six hours as the awake procedure, but the patient is knocked out and has no awareness of the operation.
That sounded a lot better to Mr. Pais. He had the surgery in August -- most of the cost was covered by his Highmark insurance -- and the results were immediate.
"As soon as he stood up I could literally see Parkinson's leaving his body," Ms. Pais said. Her husband added, "I felt it releasing me. I was in control again."
The brain operation was on a Friday. A second operation on Monday connected the wires to the pacemaker. On Wednesday he went back to work.
"He never missed a beat," Ms. Pais said. "The resolve was amazing."
Outcomes from awake and asleep DBS are comparable, Dr. Richardson said, but the latter opens up the procedure to people who wouldn't have it otherwise.
DBS doesn't cure the disease, but it can lessen the symptoms dramatically. So, while Mr. Pais still has Parkinson's and still takes medication, the dosage is reduced and he has resumed his former activities. This good response could last for years, Dr. Richardson said, depending on how the disease progresses.
Not everyone is helped by DBS, the doctor said, especially if they have a lot of other medical issues. But he said 70 percent of Parkinson's patients get significant results. With periodic adjustments to the pacemaker and regular exercise, the benefits can last for a decade.
Diagnosing Parkinson's disease can be tricky because there's no test for it.
"It's a diagnosis of exclusion," Dr. Richardson said. When doctors can't find any other cause for tremors, stiffness or rigid facial expression, they try a medication that increases levels of dopamine in the brain. If that gets a good response, it means the patient has Parkinson's.
In Mr. Pais' case, he thought he'd had a mini-stroke because he was walking and playing soccer holding his hand to his thigh. He went to UPMC neurologists Peter Sheptak and Richard Kasdan, who looked at his walk and recognized the symptoms.
For several years he took Sinemet, a common drug for Parkinson's. Then Dr. Kasdan told him about deep brain stimulation, which has been in use for 15 years and helped countless patients, but Mr. Pais did not want to go through the procedure while awake.
Testing for asleep DBS began five years ago. In 2010 the FDA approved it at certain sites. One of them was the University of California, San Francisco, where Dr. Richardson received specialized training. He joined UPMC in 2011; it is now one of about 10 places across the country that has FDA approval to perform the asleep procedure.
Dr. Richardson also does awake DBS -- about two or three per month compared with one a month for the asleep version. But he said asleep surgery is a better option for people like Mr. Pais, patients in wheelchairs or those with severe twitching or contortions.
Half a million people in the United States have Parkinson's, according to the National Institute of Neurological Disorders and Stroke, with about 50,000 new cases reported annually. The numbers are expected to increase as the average age of the population increases. The average onset age is 60 -- Mr. Pais was 50 -- and the disorder affects slightly more men than women.
Given the prevalence of the disorder, Dr. Richardson says many more people could benefit from DBS than are seeking it so far. The procedure is covered for qualifying candidates by Medicare and private insurers such as Highmark, Aetna and UPMC Health Plan.
"Often by the time people get to me they've had Parkinson's a long time," Dr. Richardson said. "But it works better if you do it earlier. You don't have to have tried every medication first. This is not a last resort. DBS increases quality of life beyond what can be obtained by medical management. In properly selected patients, it can really give them control back at an earlier stage. The disease continues to progress, but slower progression has been demonstrated."
After the surgery, the chest-implanted pulse generator is set to deliver the lowest effective level of stimulation. Over time the setting is increased as needed, and can be reset externally with a wand device used by the patient at home or by the doctor.
On a recent morning, Mr. Pais went to visit Valerie Suski, a specialist at the UPMC Movement Disorder Clinic in the Kaufmann Building, who showed him how to set the implant.
"When you put the [wand] device up against it, it can change the voltage, how fast it fires, the shape of the electrical field in the brain," Dr. Suski said. "We can tell what he needs by how his exam goes. If he comes in with a tremor on one side, we would work on stimulating the other side since everything in the brain crosses over."
In the clinic, they talk about holistic mind/body connections. Mr. Pais' tremors are minimal now if at all, Dr. Suski said, but if he gets anxious or nervous, they come back, albeit at a reduced level.
"Cardiovascular exercise at least three times a week is very important to help with mood, movement and memory," Dr. Suski said. "Usually this is a fight with patients, but Toni was already working out a lot and playing soccer. He has exceeded our expectations."
"One thing I tell people with Parkinson's is to stay active," Mr. Pais said. "It makes a big difference."
Looking back, he says, "Sometimes I wonder how I did three restaurants at the same time. I did all the shopping myself, made the soups and sauces. I think I was on autopilot."
Today he's content running Cafe Zinho, whipping up his signature dishes, greeting his patrons and pursuing the same activities he enjoyed before the disease intervened.
It's a welcome return to normalcy, Ms. Pais said: "Toni is Toni again."