Deep Brain Stimulation and Dystonia

Contact Information
(608) 263-7335
UW Health Services



MADISON - The video is dramatic – a young girl, bones outlined beneath her skin, is in constant motion as her dystonia makes her twist and turn. The scene changes, and the same girl appears, still thin but sitting quietly in a chair. On command, she stands up, walks with slight unsteadiness toward the camera and back to her chair again.
A final change, and the girl appears again, a picture of health. She stretches her arms, walks forward without hesitation, turns with a small hop and walks back. She flips her head back toward the camera and gives a triumphant smile.

"While not all children with dystonia will react as dramatically as this girl did to deep brain stimulation, it can turn a child's life around," says UW Health neurosurgeon A. Leland Albright, MD.
Deep brain stimulation (DBS) has offered a broad range of possibilities for neurological and movement disorders including Parkinson's Disease, epilepsy, tremors and even clinical depression. In children, the American Family Children's Hospital in Madison, Wisconsin, and a few other hospitals across the country have begun to use it to treat dystonia, quieting uncontrolled muscle contractions through carefully timed electrical pulses delivered deep into the brain.

"DBS is rarely the first treatment we try for a child with dystonia," says Dr. Albright. "Many children will respond well to intrathecal baclofen, especially those with secondary dystonia from cerebral palsy. If medication therapies are unsuccessful, however, we've found DBS to be of great benefit in restoring quality of life."

Mapping the Brain to Find Precise Area of Concern
Dr. Albright, along with UW Health neurologist Erwin Montgomery, Jr., MD, has performed the six- to eight-hour DBS implantation surgery on 10 children with dystonia in the last 18 months. Over half of the surgery is spent mapping the brain in an effort to find the precise area of concern.

"We're trying to find a target the size of an olive pit," Dr. Montgomery says. "One of the primary areas of dystonia is the globus pallidus, which lies near the visual pathways as well as centers of emotion, thinking and movement. It's critical that we hit the target straight on and avoid anything that surrounds it."
Using temporary electrodes to observe neuron activity in the brain, Dr. Montgomery hones in on the appropriate place to implant the deep brain stimulator. The pulse generator is placed below the collarbone and can be recalibrated wirelessly once the operation is finished. Depending on the amount of stimulation needed, the battery pack in the pulse generator will need to be replaced every two to five years.

After the operation, most children use deep brain stimulation (DBS) for a few weeks to months before seeing improvement. Different types of dystonia respond differently - children with primary or heredodegenerative dystonias often see a marked improvement, while children with secondary dystonia may see less benefit.

"When you're looking at children with neuromuscular disorders that cause a secondary dystonia, it's hard to say which symptoms are caused by the primary disorder and which by the dystonia," Dr. Montgomery says. "Regardless, there is definitely a benefit. Even in severely handicapped children, reducing the amount of dystonia can make their lives easier, as well as the lives of their parents or caretakers."
Researching New Methods of Electrical Stimulation in the Brain

The potential of deep brain stimulation (DBS) has led to research into new methods of electrical stimulation in the brain. For example, Drs. Albright and Montgomery are examining the effect of epidural motor cortex stimulation, during which two strips of electrodes are placed over the cortex outside the brain, rather than inside the brain.

"As remarkable as deep brain stimulation is, we still don't exactly know how it works," Dr. Montgomery says. "Studying DBS is also giving us greater insights on how the brain operates."

For children with dystonia, deep brain stimulation often means a better quality of life.

"It's important to stress that no matter how severe the dystonia, the majority of these children's lives can be improved," Dr. Albright says. "With early intervention, we can decrease the amount of deformity, disability and discomfort. I believe UW is as good or better at treating dystonia in children as any place in the country."

For more information about care for pediatric dystonia, call (608) 263-7335.
Date published: 4/28/2008