Deep Brain Stimulation and Dystonia
"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.
"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
"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."
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."
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.