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Curing dystonia with deep brain stimulation

October 16, 2018 | UCI Health
carson creer on a hike
After his surgery, Carson Creer is back to doing all of the things he loves most, including hiking.

It was an otherwise uneventful day in May of 2013. Carson Creer, then 29, was standing in the garage with his parents at their Mission Viejo home. Suddenly his head began to move in a jerky motion. His chin dropped to his chest and then his head rolled to the side and back.

“We asked him if he was doing it voluntarily,” says Carson’s mother, Darci. “He said he couldn’t control it.”

His uncontrollable muscle contractions were soon diagnosed as dystonia.

‘Sounds like science fiction’

That was the start of a harrowing, two-year ordeal for Carson and his family until a UCI Health team of movement disorder specialists came up with a plan that, Darci notes, “sounds like science fiction.” They implanted thin wires deep into Carson’s brain and ran the wires under the skin, attaching them to a neuromodulator, a small pacemaker-like device implanted in his chest.

The neuromodulator is programmed to transmit electronic signals to the area of the brain that controls movement. Called deep brain stimulation (DBS), the treatment worked so well to quell Carson’s tremors that the Creers consider it lifesaving.

“Although not technically a cure, it was everything we’d hoped for,” says Jeff Creer, Carson’s father. “The degree of suffering people with dystonia have to endure is heartbreaking! Yet there is hope, and the hope lies in science-based medical research and the efforts of these fabulous physicians and nursing staff.”

Embracing deep brain stimulation

The UCI Health Movement Disorders Program has a long history of using DBS to treat neurodegenerative diseases such as Parkinson’s disease and essential tremors. It’s used less often for dystonia — a somewhat mysterious movement disorder that causes uncontrollable, repetitive muscle movements.

“It has been shown to be effective but never to the degree as it is with Parkinson’s disease,” says Dr. Frank P.K. Hsu, professor and chair of the UCI School of Medicine’s Department of Neurological Surgery.

It was Carson’s UCI Health neurologist, Dr. Nicolás M. Phielipp, who suggested the DBS treatment after almost 18 months of unsuccessful efforts to reduce Carson’s symptoms using medications and Botox injections. The violent head shaking had brought the young man’s life to a halt.

“It was a living hell,” Carson says.

No known cause of Carson’s dystonia

The Creer family isn’t sure why the condition began. Carson was born prematurely and was diagnosed with learning disabilities as a child. In late adolescence he developed occasional severe leg pain and a speech disability that worsened.

But the head-and-neck dystonia came on suddenly, and some of life’s simplest pleasures were abruptly taken away. He had great difficulty watching television because of the violent head movements. When the Creer family attended a theatrical show, Carson struggled. “We had to take turns holding his head just so he could watch what was going on onstage,” Jeff recalls.

When Phielipp suggested DBS, the Creer family gathered around their kitchen table to discuss the risks and benefits.

Jeff and Darci told Carson the decision was up to him. “Let’s go for it,” he responded.

An innovative approach to DBS

The procedure took place on July 30, 2015. The Creers arrived at UC Irvine Medical Center at 5 a.m.

“We were doubting ourselves; wondering if we’d made the right decision,” Darci says.

“But then we got there and saw this giant team of over a dozen people who are experts, and we relaxed. That is what we love about teaching hospitals. There are so many people who are so smart, who have their eyeballs on the patient. It’s not one person with a huge responsibility — it’s this big team.”

Experience matters in a surgery like DBS implantation, and UCI has plenty of it, says Hsu, who earned a PhD in mechanical engineering before becoming a neurosurgeon. Over the years, the team has devised its own unique protocol to improve outcomes and ease patient discomfort.

Unlike other centers, UCI Health patients are asleep for the procedure instead of being kept awake to communicate as the surgeon stimulates parts of the brain — a situation that can cause patients anxiety.

How DBS is done

In the operating room, intra-operative CT scanning — called the BodyTom® — provides brain images. The CT images are merged with MRI images taken the day before surgery to pinpoint the placement of the wire leads in the exact part of the brain that controls movement.

The ROSA (robotic stereotactic assistance) robot system is used with a microelectrode recording device, which involves placing tiny electrodes in the brain to confirm the placement of the leads.

“It’s a very special, unique program,” Hsu says. “We were the first program in the U.S. to do DBS this way.”

Carson’s operation proceeded as planned, and he spent the night in the neurosciences ICU. Darci and Jeff remained at his bedside. Exhausted, they found comfort — and maybe something of an omen — when fireworks from Disneyland lit up the night sky outside Carson’s window.

The following week Carson underwent a second procedure to pull the wires to the chest and attach them to the neuromodulator in his chest. Six weeks later Carson, Jeff and Darci gathered with Phielipp to turn on the device. The neurologist adjusted the settings of the neuromodulator and asked his patient to take a few steps. Carson stood and strolled down a hallway — his posture perfect, his head still.

“It was life-changing,” says Carson, who can now ride his mountain bike, snow and water ski, hike and play tennis.

“It was so fun to watch him do the things he wanted to do again,” Jeff says. “With the success of DBS, the shackles were taken off.”

Exploring DBS as treatment for other conditions

Deep brain stimulation may eventually be used for other types of brain disorders, Hsu says. Researchers around the world are investigating its use for epilepsy, Tourette’s syndrome and some mental illnesses, such as obsessive-compulsive disorder and depression.

“I think people are recognizing this is a good form of therapy, using electrical stimulation of certain parts of the brain to accomplish what you want to do,” he says.

Although Carson’s case was unusual, Phielipp says he expected his patient to benefit. “I’m very happy with the results. Carson was not a typical candidate for DBS, but we have a lot of experience here.”

Top-notch medicine close to home

A long history of treating movement disorders, multidisciplinary teamwork and innovation distinguish the program, Hsu says. “We have the most experienced team in the region, and we use the latest technology. This type of work could not have been done in a community hospital without all the neurologists, specially certified nurses, rehab people, neurosurgery team and the tools we have here.”

The movement disorders team works closely with patients and their families to find solutions to challenging conditions, Phielipp adds. The program emphasizes education, offering an annual Parkinson’s disease symposium for the community. Some UCI Health patients can also opt to enter clinical trials that seek to advance treatments for movement disorders. UCI Health researchers are conducting studies looking for biomarkers to diagnose Parkinson’s disease at early stages and are working to develop treatments that slow or halt the disease process.

“We are patient-oriented here,” Phielipp says. “Patients feel involved and know they are receiving leading-edge developments.”

The Creer family did not expect to find such a depth of experience and resources so close to home, Darci says.

“When we learned there was this procedure available to treat dystonia, we were willing to travel wherever we had to — New York, Chicago, Miami — to make it happen,” she says. “When Dr. Phielipp told us Carson would be a great candidate for this, and that UCI had one of the best neurosurgeons in the world and who also had a PhD in mechanical engineering, we thought, ‘Wow, that is perfect.’”

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