movement disorders – Inside Pediatrics

Deep brain stimulation for progressive dystonia

_{In 2023, Children’s of Alabama performed deep brain stimulation on progressive dystonia patients for the first time.}

Progressive dystonia disorders, characterized by changes in movement patterns, can profoundly impact a child’s quality of life. In adults, such disorders are routinely treated with a procedure called deep brain stimulation (DBS). However, this intervention is less commonly used in pediatric populations.

In 2023, Curtis Rozzelle, M.D., and Emily Gantz, M.D., performed Children’s of Alabama’s first DBS procedures for progressive dystonia patients. This innovative therapy has shown promising results in several pediatric patients with limited treatment options.

_{Curtis Rozzelle, M.D.}

“We’re excited that this innovative procedure is now transferring over to our pediatric patients,” Rozzelle, a pediatric neurosurgeon at Children’s, said. “Kids with progressive dystonia seem to do particularly well, while those with other types of movement disorders may have mixed results.”

Studies suggest that pediatric patients with progressive dystonia respond well to deep brain stimulation, especially after failing conventional medications. The decision to apply DBS in pediatric cases stems from the specific needs of young patients and advancements in the field.

“Until Dr. Gantz arrived at the University of Alabama at Birmingham, we didn’t have a movement disorder neurologist here who also had training and experience with deep brain stimulation,” Rozzelle said. “When she arrived, Dr. Gantz opened the door for us to be able to perform the technical aspects of the surgical procedure. Now, we’ve done several.”

Not every child is an ideal candidate for DBS. The decision to initiate DBS is patient-specific, based on the severity of symptoms and the inadequacy of other treatments. A careful evaluation of each patient’s unique situation, including factors such as genetic mutations and the progression of the disease, must be completed before offering DBS surgery.

Each deep brain stimulation procedure at Children’s uses stereotactic surgical techniques and the ClearPoint targeting system. This system, employed in an MRI scanner, ensures safe, precise electrode placement in the globus pallidus interna (GPI), a key target for treating progressive dystonia.

_{Emily Gantz, M.D.}

“When we implant the electrode into a specific region of the brain, we can either edit the input throughout the stimulation, or we can take it completely away,” Gantz said. “Think of it as a series of relay circuits in the brain. If someone has dystonia, one of those relay circuits isn’t working properly. By putting in the stimulator and applying an electrical current intermittently, we can suppress the abnormal brain activity.

“The stimulator stays in for life, so the procedure doesn’t need to be repeated,” she continued. “Occasionally, we’ll have to change the device’s battery, but they’re rechargeable and designed to last for up to 20 years.”

After the initial procedure, patients return to see Gantz to have the device programmed. “I set the programming on their stimulator so they can make slight adjustments at home. It can take a little while for the device to be effective; we usually leave it alone for a few months and then reevaluate,” Gantz said. “We have guidelines for which settings will most likely help, and we start there. We’re looking to ensure we don’t get side effects, such as visual disturbances or muscle pulling, more than anything.”

The pediatric patients who have begun DBS for progressive dystonia at Children’s are responding well to the new treatment. “I’m really excited about DBS and its future as a treatment in pediatric neurology, specifically movement disorders,” Gantz said. “It may eventually come into play in other treatment areas, and I’m glad the door is open to us here. I think there will be many more patients who will benefit from it.”

January 30, 2024 by childrensal

Birmingham to Cape Town: Children’s Neurologist Consults Across Continents

^{_{Leon Dure, MD, neurologist trained in pediatric movement disorders at Children’s of Alabama.}}

It’s not easy to find a specialist trained in pediatric movement disorders. At Children’s of Alabama, there are two: Leon Dure, MD, and Emily Gantz, DO. They and their multidisciplinary team provide the bulk of care for children with movement disorders in Alabama and beyond.

Considering the dearth of movement disorder specialists here in the U.S., Dure wondered what the numbers were like in Africa. His curiosity led him to become a consultant to a pediatric neurologist in Cape Town, South Africa.

Dure’s interest in helping physicians in under-developed countries was piqued through his work with the International Child Neurology Association (ICNA), which seeks to foster education and resources for practitioners in low-resource environments, such as South Asia, Africa and South America.

He recalls a meeting where a doctor from sub-Saharan Africa presented. “She said there are 80 million children under the age of 18 within that region and two or three neurologists,” Dure said. “So, I realized that the types of problems and issues they’re facing are very different from what we face here.”

In the U.S., Dure often receives requests from colleagues for his opinion on a child, typically via a video sent by text. Why, he wondered, couldn’t the same be done for clinicians in low- and moderate-income countries?

Turns out it wasn’t quite so simple, given the challenges of moving information from places as disparate as South Africa and Birmingham. Dure persevered and found vCreate, a tech company that works with the U.K.’s National Health System to provide secure video messaging. There is even a separate unit called vCreateNeuro, a cloud-based service that allows registered patients and clinicians to securely share smartphone-recorded videos.

He and vCreate developed a proof-of-concept project with a neurologist who has an interest in movement disorders at the Red Cross Hospital in Cape Town. When he has something he wants Dure to review, the neurologist uploads it to the vCreate platform, and Dure gets an email alert.

“Then we begin a back-and-forth regarding what to call it, what to do about it, how to work it up, et cetera,” he said.

The pilot has been in place for about six months, and Dure has consulted on about eight videos.

“These are relatively unusual conditions that are very difficult to characterize,” he said. “So just having someone else say ‘Yeah, you got it, you’re right,’ is quite helpful.”

Dure would like to grow the program to other countries with other U.S.-based pediatric neurologists providing their expertise, but that will require funding. For now, vCreate has been providing the technology for free. “I don’t know if that’s going to be a long-term possibility,” he said. “But so far, it works. And I’m able to provide my expertise to somebody in Cape Town without a whole lot of effort.”

September 2, 2022 by childrensal