Massachusetts General Hospital provides deep brain stimulation as a treatment for a variety of neurological disorders, including XDP.
Deep brain stimulation (DBS) is a surgical treatment in which an implanted device delivers electrical stimulation to the brain in order to alleviate neurologic symptoms.
Stimulation is applied through a surgically implanted wire (also called a lead or electrode) that is inserted into the brain. DBS is most commonly used to treat the symptoms of Parkinson’s disease, essential tremor and dystonia.
In dystonia, the most common stimulation target is the globus pallidus internus (GPi). The electrical stimulation is generated by an implantable pulse generator (IPG) that is placed under the skin in the chest, similar to a cardiac pacemaker. The stimulation can be adjusted non-invasively by a neurologist in order to optimize symptom relief.
DBS is most effective for primary dystonias—that is, inherited dystonias like X-linked dystonia parkinsonism (XDP) and dystonias without a known cause—and less effective for secondary or acquired dystonias such as those due to brain injury from cerebral palsy or stroke. With DBS, primary dystonia symptoms improve by 50-60% on average, although individuals may benefit substantially more or less than average. These benefits appear to last at least 10 years.
There is less known about the long-term benefit of DBS in XDP specifically, although initial findings suggest that DBS can be a very effective treatment for the symptoms of XDP.
In one study of eight individuals with XDP, all eight patients experienced an improvement in dystonia symptoms, with six of eight patients improving by more than 65% at three months (Neurology April 8, 2014 vol. 82 no. 10 Supplement P1.013).
With the DBS surgery, there is a 1-2% risk of brain bleeding or stroke that will result in new symptoms such as weakness, numbness or problems with speech. In the majority of cases, these side effects fade with time. There is also a 3-4% risk of surgical infection, which may require removal of all or part of the stimulator.
Lastly, as the IPG battery depletes, it must be replaced via minor surgery. The interval between replacement varies widely between individuals, depending on the device and stimulation settings, but is typically 2-5 years for non-rechargeable batteries and up to 9 years for rechargeable batteries.
(Content on this page provided by Todd Herrington, MD, PhD)
Deep Brain Stimulation at Massachusetts General Hospital
Neurosurgeons at the Mass General Functional Neurosurgery Center use DBS to treat patients with a wide variety of movement disorders. Our center uses the most advanced stereotactic technology—microelectrode recordings—for optimal lead positioning and the best patient outcome.
Mass General was one of the first hospitals to perform DBS, and today we also use it to treat Parkinson’s disease and many different types of dystonia, including:
- Genetic dystonia, such as XDP
- Generalized dystonia
- Segmental dystonia
- Focal dystonia, such as spasmodic torticollis
Recent publications about use of DBS in XDP
- Association of Pallidal Neurostimulation and Outcome Predictors With X-linked Dystonia Parkinsonism
- Long-term Outcomes of Pallidal Deep Brain Stimulation in X-linked Dystonia Parkinsonism: Up to 84 Months Follow-up and Review of Literature
- Long-term outcomes of bilateral pallidal deep brain stimulation for X-Linked Dystonia-Parkinsonism
- Cost-Analysis of the Different Treatment Modalities in X-Linked Dystonia-Parkinsonism