A neurosurgical team at University Hospitals of Cleveland (UHC) has, for the first time in North America, applied a new surgical approach to the treatment of Tourette syndrome, resulting in the immediate and nearly complete resolution of symptoms for the patient, who has suffered from this neurologic disorder since he was a child.
"We were genuinely amazed at the patient's response," says Robert J. Maciunas, MD, neurosurgeon at UHC and professor at Case Western Reserve University School of Medicine. He has used the technique called deep brain stimulation (DBS) for the treatment of Parkinson's disease and tremor, and was impressed with this patient's dramatic reaction: the disappearance of the jerking motions, muscle tics and grunting associated with his Tourette's. "This technique holds great promise for patients suffering from this movement disorder, which often is diagnosed in childhood or early adolescence and can be completely debilitating."
Jeff Matovic, a Lyndhurst, Ohio, resident who grew up in Bay Village, was six years old when he was diagnosed with Tourette syndrome, a neurobehavioral disorder characterized by sudden, repetitive muscle movements (motor tics) and vocalizations (vocal tics). Though standard therapy with medication controlled his movements for much of his boyhood, his condition severely worsened with age.
"I had to drink with a sippy cup; otherwise, I would spill liquid all over and even break glass or plastic cups with the severity of the muscle spasms that were so forceful and unpredictable," says Jeff, now age 31. "To grow up being made fun of, always feeling different, and then, as an adult, unable to hold a job and support a family, I was feeling devastated by my Tourette's. I had heard about deep brain stimulation for other movement disorders. I knew that brain surgery was risky, and the doctors could not guarantee any positive outcome, but I wanted them to take a chance on me. And I'm so grateful that they did."
Prior to brain surgery, physicians at University Hospitals Movement Disorders Center mapped out regions of Jeff's brain, through MRI (magnetic resonance imaging) scans and 3-D computer images. Their goal was to locate the safest and most direct route to reach the cells inside the thalamus portion of the brain, involved in controlling Jeff's movements. By placing electrodes around those cells to deliver continuous high-frequency electrical stimulation, control messages are rebalanced throughout the movement centers in the brain. The electrodes are connected from the brain through wires under the skin (beneath the scalp, neck and upper chest) to an implanted battery just beneath the collarbone. In Jeff's case, since both sides of his body were affected by the movement disorder, he has electrodes implanted on both sides of his brain and tiny battery packs implanted on each side of his chest.
The doctors at University Hospitals of Cleveland are careful to point out that not everyone with Tourette syndrome requires treatment. The first line of treatment is medication, which can be very effective. Surgical treatment is considered a last resort, and it is not clear how effective deep brain stimulation will ultimately prove for patients with this particular disorder.
In the United States, the Food and Drug Administration has approved deep brain stimulation for the treatment of Parkinson's disease, essential tremor and dystonia. "We've seen very positive responses in patients with Parkinson's disease. Studies of the DBS technique show that this stimulation can significantly reduce tremor and other symptoms in about three quarters of appropriately selected patients with Parkinson's." says Brian N. Maddux, MD, PhD, Jeff's neurologist at UHC and assistant professor at Case School of Medicine. "Patients with a different movement disorder called dystonia can take three months to respond to the electrical stimulation. We didn't know how Jeff would respond. Within hours after the stimulator was turned on, we observed the ceaseless movements become completely relaxed and he was able to walk normally. We were awestruck."
Physicians at University Hospitals of Cleveland have submitted a report of this case for consideration for publication in a peer-reviewed journal. They hope to further explore the application of DBS to other patients with Tourette syndrome. It is estimated that approximately 200,000 Americans have Tourette syndrome, though experts believe it is a movement disorder that often remains undiagnosed.
For more information about the surgery, go to http://www.uhhs.com. For more information about Tourette syndrome, go to http://www.tsa-usa.org.