Cleveland
Emily Cisar had lived for years with her left arm virtually frozen in a clenched position. But when the 17-year-old’s vocal cords began tightening and her singing pitch began to go, she felt ready to try a more drastic response.
Cassie Cisar
Emily Cisar, seen here at age 13, underwent a deep brain stimulation procedure to treat dystonia.
That decision led to the scene here in a Cleveland Clinic operating room one recent morning. Ms. Cisar, who suffers from dystonia, was sedated, while her newly shorn head was fitted with a metal frame to help guide the surgeon in implanting two electrodes into her brain to begin a process called deep brain stimulation.
DBS, first approved by the U.S. Food and Drug Administration in 1997, is used in adults with Parkinson’s disease and a condition called essential tremor, among other conditions. Some 30,000 people with Parkinson’s have had DBS world-wide, according to the Parkinson’s Disease Foundation.
Cleveland Clinic
Andre Machado examines an MRI scan of Emily Cisar’s brain during her July surgery for deep brain stimulation at the Cleveland Clinic. Dr. Machado verifies the target in the globus pallidus on the right side of Ms. Cisar’s brain. The blue line traces the path of the electrode to its destination.
Now researchers are pushing the boundaries of the treatment by investigating it for use against other conditions. DBS may have particular benefit for children suffering from epilepsy, according to Andre Machado, the director of Cleveland Clinic’s Center for Neurological Restoration, who conducted Ms. Cisar’s operation and who is studying the use of DBS to treat pain and stroke.
Stimulation from electrodes powered by a battery, also implanted in the body, appears to reprogram specific parts of the brain. The FDA has issued limited approval for DBS for dystonia, in which involuntary muscle contractions can lead to repetitive movements or abnormal postures, as well as severe forms of obsessive-compulsive disorder.
With children, DBS used to be employed relatively rarely because DBS-treatable disorders are less likely to affect kids. Also, the operation often used to be conducted when the patient was awake, a scary prospect for most children. There are also ethical considerations with using DBS treatment on children, as with all more invasive treatments. Many experts advocate for a dual process where the parent gives permission and the child must agree.
But as anesthetic techniques have improved, more children may be able to benefit because they do not need to be awake during the procedure. The treatment may actually help children more than adults because their brains are more malleable and able to learn.
In some conditions, such as essential tremor, the uncontrolled movement of the body, the effects of DBS are instantaneous when a misfiring part of the brain is reset. With other disorders, like dystonia, improvement takes weeks or months as the brain relearns how to do things correctly.
Ms. Cisar’s surgery illustrates the intricacies involved with major brain surgery. Dystonia is the third-most-common movement disorder in the U.S., behind Parkinson’s and essential tremor and affects about 300,000 Americans, according to the American Dystonia Society. Doctors don’t know the cause in Ms. Cisar’s case, but say it isn’t genetic.
The high schooler from Lorain, Ohio, started having seizures at the age of 18 months and first developed some signs of muscle weakness, spasms and cramping on one side of her body around age 2. By about age 9, the dystonia was so pronounced she couldn’t do simple two-handed tasks like put her hair in a ponytail or tie her shoes, she said.
She has taken medication and tried Botox, which aims to help relax the muscles. But she found Botox painful and the effects only lasted for a few months. She began considering DBS about 18 months ago.
“At first I was nervous about it. It’s surgery,” Ms. Cisar said. But after reading more about it, she wondered if the treatment, though not a miracle cure, might give her a more longer-lasting benefit than treatments she had already tried.
Her parents, both nurses, initially were against the idea. While Ms. Cisar has had a number of physical disabilities, she is cognitively unaffected, according to her father, Robert Cisar. She is bright, has a good memory and a witty, upbeat personality, and her parents were afraid brain surgery could change that, he said while waiting for his daughter to recover from surgery.
They relented when Ms. Cisar told her family that “20 years from now I don’t want to look back and realize I had a chance and I didn’t take it,” her grandmother, Margaret, recalled.
Early one morning in late July, Ms. Cisar was wheeled into an operating room at the Cleveland Clinic.
A portion of the brain called the globus pallidus was the target of dystonia treatment. But exactly where in the region to go is a matter of trial and error, and there will be potentially months of adjustment of the electrical settings to maximize Ms. Cisar’s symptom improvement, Dr. Machado said.
In the operating room, a curved metal frame was affixed to Ms. Cisar’s head for the duration of the surgery to serve as the navigational guide for the placement of the electrodes.
Dr. Machado made an initial incision, 5 centimeters long, on the top right side of Ms. Cisar’s scalp.
He drilled a small, circular hole through the skull and attached to the metal frame a device that would hold straight the lead, a thin, flexible wire with four tiny electrodes on one end. Dr. Machado guided the lead gently into the brain tissue and capped the end of the wire that still protruded from the skull, neatly coiling it as if storing a garden hose. He tucked the coil under Ms. Cisar’s scalp and closed the incision.
Ms. Cisar, still sedated, then underwent a magnetic resonance imaging test so Dr. Machado could verify that he had implanted the electrode correctly. If the placement of the electrode were more than 2 millimeters away from where he intended to place it, Dr. Machado would have to make a clinical decision about whether to reopen the incision to move the electrode. In Ms. Cisar’s case, the placement of the right lead was within the acceptable range, so he moved to implant the left electrode, which he also determined to be acceptable.
About six hours into the operation, Ms. Cisar was handling the surgery so well—her blood pressure was steady and low—that Dr. Machado decided to implant the battery pack, about half the length and thickness of a deck of playing cards, in the chest area on her left side. He connected the leads from her head to the battery pack. This step often requires a separate surgery a week later.
Ms. Cisar now is recovering from surgery. Her battery will be turned on Sept. 10, once she has had time to heal.
“I’m excited, but I’m also anxious about just how it’s going to feel,” said Ms. Cisar, speaking by phone after her operation.
In the days after surgery, the region where the battery pack was implanted felt bruised, but has since healed and feels like normal skin, she said.
She hopes she will be able to do the simple two-handed tasks that she hasn’t been able to in years. “I hope eventually that I can be independent more and move away and live on my own instead of having to depend on other people so much,” Ms. Cisar said.
Write to Shirley S. Wang at Shirley.Wang@wsj.com
A version of this article appeared August 12, 2013, on page D1 in the U.S. edition of The Wall Street Journal, with the headline: Brain Probe’s Potential for Children.