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Deep Brain Stimulation

Deep brain stimulation (DBS) is another option used to treat dystonia in NBIA individuals, most often in those with PKAN. It involves placing electrodes in the brain. They are attached to wires leading to a battery-operated neurostimulator implanted in the chest. The neurostimulator sends pulses to targeted areas in the brain and takes “off line” the part of the brain that is sending too many signals and causing the muscles to move in painful ways as they do in dystonia.

DBS is not without risks, although extremely rare in well-trained centers. The most severe is bleeding in the brain. Other surgery risks include infections, seizures and an allergic reaction to implanted materials. Side effects of the surgery may include increased dystonia and speech problems like whispering (dysarthria) and trouble forming words (dysphasia), which are usually reversible. However, the total number of severe complications are in the range of 1 percent to 2 percent. Therefore, the risks are considered acceptable in patients expected to have a good or very good operation outcome.

Because a limited number of persons with NBIA have undergone such treatment and care has been provided at a variety of centers, it is not possible to evaluate the efficacy of DBS for this population at this time. Even with these limitations, studies suggest DBS may hold promise. Larger prospective studies may improve understanding of the factors that influence the outcomes of DBS use in NBIA.