New Surgery Suppresses Symptoms Of Dystonia

BOSTON -- More than a 250,000 Americans live with a severe neurological movement disorder called dystonia.

It's absolutely devastating, causing involuntary movements and great pain. There is no cure, but this week the Food and Drug Administration approved a surgery with potentially life-changing results.

Indra Howard thinks like most 20-year-olds, but she can't act like one. Dystonia makes normal life nearly impossible.

"Dystonia really means involuntary movement of certain muscle groups and it can affect any part of the body from head through upper extremities, lower extremities or the trunk," said Dr. Diana Apetauerova, of the Lahey Clinic in Massachusetts.

The twisting and contorting is uncontrollable, and it hurts like having a charley horse. Howard's hands must be strapped to her wheelchair so she doesn't scratch herself. And because it affects her voice box, she has trouble talking. Her feet, which she uses like hands, are just about her only source of control.

"Her feet are really amazing. Over the years as a matter of fact, what's happened is her big toe has actually become her thumb. And it rotates like our thumb does," Howard's mother, Claire, said.

But Howard wants to write with her fingers and be more independent. And there's a chance she can. On Wednesday at the Lahey Clinic, she was the first Massachusetts woman to undergo a delicate surgery the FDA approved for dystonia just the day before -- called Deep Brain Stimulation, or DBS.

The surgery uses a product called Activa Dystonia Therapy System, made by Medtronic Inc. of Minneapolis. It was already approved for treating Parkinson's disease tremors. This week's approval broadens its use to people with primary dystonia who get little or no relief with medication.

"What the stimulation hopefully will do is allow a loosening of things and allow a normal posturing also of her limbs and hands, an ability to feed herself, hold things appropriately, and ultimately lead a more normal life," said Dr. Jeff Arle, of the Lahey Clinic.

Arle implants a tiny battery-powered electrode that delivers electrical pulses to areas of Howard's brain involved in movement control. The stimulation blocks brain signals that cause the symptoms. During the surgery, Howard is awake, but not in pain.

Doctors hear a small but immediate improvement in her speech -- a glimpse of what a handful of doctors performing the procedure experimentally have already seen.

"Most of the patients that have been done are at least the way they were and often times better. And many of them have quite dramatic benefits from it," Arle said.

Full results often take months, so Howard and her family must wait and hope.

"I'm cautiously and guardedly optimistic that there's going to be big rewards and dividends with this procedure," Howard's father, Wilfred, said.

Claire Howard said that her daughter is only thinking of success.

"When a couple of her care attendants came the next day, she said to them, 'After I have my surgery, I won't be needing you.' And I thought, 'Oh geez, don't let them go yet,'" she said.

Howard will likely be released from the hospital by Friday. She'll come back in about two weeks to turn on the batteries. That's when she'll begin to see how dramatically this surgery will change her life.

Around 2,000 people a year in the United States are expected to be candidates for this surgery.