Surgery unlocks patients' prison of immobility

By Alice Keesing
Advertiser Health Writer

For more than five years Norman Mau could do little more than sit in a chair, so gripped was he by Parkinson's disease, which left his muscles frozen and unresponsive.

Norman Mau, who used to be so crippled by Parkinson's that he couldn't tie his shoelaces, takes a swing at the driving range at Mid-Pacific Country Club. Cory Lum • The Honolulu Advertiser

Mau's remarkable turnaround is due to two electrodes implanted deep in his brain in late August. They emit tiny electric shocks that control the muscle rigidity and tremors that can make life unbearable for Parkinson's patients.

The revolutionary surgery — deep brain stimulation — was approved in the United States in 1997 and recently introduced to Hawai'i by brain

surgeons at St. Francis and Queen's medical centers. The treatment offers new hope to hundreds of Hawai'i's estimated 10,000 Parkinson's patients.

"It is truly like being released from prison," said neurosurgeon Dr. Marcus Keep, who performed the first two procedures at St. Francis Medical Center in early September. "The horror of Parkinson's is that the mind is basically intact, but you're a prisoner inside a body that won't move the way it normally would. This is like opening the door to the prison. Granted, you still have the disease, but you're able to get out the door and move and interact with the world in a way that you couldn't have for years."

Parkinson's is a degenerative brain disorder for which there is no cure. It is common in Asians, making it especially prevalent in Hawai'i. The trembling and rigidity can turn eating or drinking into a battle. The symptoms get worse over time, driving many into a reclusive life.

For Mau, who ran his own real estate appraisal business, it meant a frustrating halt to all he did. Business and golf were replaced by sitting at home watching TV. He would often fall down and hurt himself; he had to drag himself around the house because his legs wouldn't work; he was exhausted from the tension in his muscles; his speech became so confused that his family couldn't understand him.

	Operation chronology
•	7 a.m.: The day begins early for the large team of experts. They secure a crown-like metal frame on the patient's skull that serves as a guide for the delicate procedure.
•	8 a.m.: Doctors map out the cerebral map they must negotiate to reach the subthalamic nucleus — no more than 8 millimeters at its longest point. Images of the brain are produced using CT and MRI scans. The smallest error could mean hitting an artery or vein, triggering a hemorrhage, which could potentially be fatal.
•	9 a.m.: The team creates an even more detailed map around the subthalamic nucleus using a highly advanced Gamma Knife computer.
•	10 a.m.: The patient is taken to the operating room, where Keep makes an inch-long cut in the top of the scalp, then drills a hole the size of a nickel in the skull.
•	11 a.m.: A tiny microphone is maneuvered into the brain to pick up noise created by the firing of neurons. Doctors can tell where they are in the brain by what they hear. First they pass through the silent white matter. When they arrive at the subthalamic nucleus, which has a high cell density, the noise changes to that of falling rain. As the microphone passes out of the subthalamic nucleus and deeper into the midbrain, the single large neurons make a sound like popcorn.
•	Noon: With the trajectory carefully mapped, the surgeon feeds in an electrode connected to a long, thin wire. The electrode is made of platinum alloy and shaped like slightly bent spaghetti. Once it is safely implanted in the subthalamic nucleus, the doctors test it, asking the patient — who is sedated but awake — to move a hand to see whether the tremors or rigidity have gone.
•	12:30 p.m.: The procedure is repeated on the other side.
•	2 p.m.: The first phase is over. Time for a quick break for the doctors while the patient is anesthetized.
•	3 p.m.: The second phase places batteries in the patient's chest to power the electrodes. The thin wires are tunneled under the skin of the scalp, behind the ear and down the chest, where two pockets are created to take the silver dollari sized batteries.
•	6 p.m.: The operation is over. The patient goes to the intensive care unit for monitoring. The doctor visits with the family and writes up his notes.

Patients can be treated with drugs, but Mau, like many others, experienced distressing side effects. These can include hallucinations, confusion and uncontrollable jerking.

Some patients have found relief through a pallidotomy, in which a hole is literally burned into the brain. But that procedure does not treat all of the symptoms of Parkinson's, and surgeons say deep brain stimulation is much more effective. It targets the subthalamic nucleus, a small almond-shaped structure in the brain that helps control body movement.

"It really has revolutionized the treatment of Parkinson's," said neurosurgeon Dr. Leon Liem, who operated on Mau at the Queen's Medical Center, the first hospital in Hawai'i to do the procedure.

The operation requires a large team of experts and high-tech equipment. St. Francis estimates the cost at $35,000, largely covered by Medicare.

Word of the operation has spread among Hawai'i's Parkinson's patients. Damien and Marie Gonsalves of Wailuku first heard about it flicking through the TV channels, on a Mainland news report.

Damien was diagnosed with the disease 13 years ago. By 1991 it had become unsafe for him to continue his work driving a truck. He needed constant help, and his wife of 35 years, Marie, quit her job to look after him. Although it was what she wanted to do, the stress was intense, as her husband needed help 24 hours a day.

Keep had cautioned Gonsalves that the procedure was not for everyone. St. Francis works with the Hawai'i Movement Disorder

Center to find those who will most benefit. Keep, who operated on Gonsalves, describes the ideal patient as someone in the intermediate phase of Parkinson's, without complicating conditions such as dementia.

As with any new procedure, the first attempts were wrapped in tense excitement. Keep had played the procedure over and over in his mind, planning for everything that could go wrong.

"It is brain surgery, so there are risks, but the risks are low — there is probably about a 1 percent chance of a hemorrhage, and probably about the same chance for infection," he said.

He likens the day of the operation to the final phase of a long-planned military operation. But the patient's reaction makes everything worth it, he said.

"Mr. Gonsalves said, 'You are my brother. You have given me my life back,' " he recalled.

Both he and Liem herald their first operations as successes. Four patients have undergone the procedure in Hawai'i, and both hospitals are preparing to do more.

Gonsalves said he feels "like a champ" these days. Before, he wanted to hide away at home, but now he jumps at invitations to parties and to go to church. Marie Gonsalves no longer has to be on constant watch.

Mau also is celebrating the return of his independence. His fingers can tie shoelaces again. His body sometimes moves beyond his bidding, but he's back on the golf course, resolved to get his game back.

"I'm determined to beat a few guys around here who took advantage of me," he said, laughing.

There was an extra glow ofcelebration as both men toasted Thanksgiving with their friends and family last month.

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Reach Alice Keesing at 525-8014 or akeesing@honoluluadvertiser.com.