Treatment of Pediatric Movement Disorders

Surgery: Deep Brain Stimulation (DBS)

Deep brain stimulation (DBS) is a modern technique that is based on the results of deep brain lesions. In both lesions and DBS, the operation interrupts the abnormal circuit that is thought to cause dystonia. In DBS, a pacemaker is implanted in the chest wall and connected to a wire that is implanted within the basal ganglia. The procedure to implant the stimulator is lengthy, since the precise location in the basal ganglia must be found, usually using a combination of MRI and electrical measurement of basal ganglia activity. Older children may remain awake during most of the procedure, but younger children will require sedation or anesthesia. When the pacemaker is turned on at a rapid rate, it may block activity in the region of the basal ganglia near the end of the wire. In dystonia, the effect of treatment may not be evident until after several months, but some children have shown remarkable improvement. DBS is not yet approved by the United States FDA to treat dystonia in children; however, it is being used in clinical research in a few centers in the U.S.

The effects of DBS are thus similar to those of a deep brain lesion (see the previous section). The advantages of DBS are that the strength and frequency of the stimulation may be adjusted and the location may be adjusted since there are usually four possible contacts on the wire that may be selected (using an external magnetic programmer). In addition, should the effects of DBS not be as desired, the wire and pacemaker may be removed. The disadvantages of DBS are the need for occasional reprogramming of the pacemaker; the need for a surgical procedure to replace the batteries every few years; and the potential for infection at the pacemaker or the point where the wire enters the skull. DBS must be used with caution in children; it is difficult to predict who will benefit. The long-term side effects of DBS are not yet known. Nevertheless, this is a promising technique and active research programs continue throughout the world.

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