Deep Brain Stimulation of the Pallidum is Effective and Might Stabilize Striatal D(2) Receptor Binding in Myoclonus-Dystonia.

PURPOSE: To assess clinical efficacy of deep brain stimulation (DBS) of the pallidum in Myoclonus-Dystonia (M-D) patients, and to compare pre- and post-operative striatal dopamine D2 receptor availability.

METHODS

Clinical parameters were scored using validated rating scales for myoclonus and dystonia. Dopamine D2 receptor binding of three patients was studied before surgery and approximately 2 years post-operatively using 123-I-iodobenzamide Single Photon Emission Computed Tomography. Two patients who did not undergo surgery served as controls.

RESULTS

Clinically, the three M-D patients improved 83, 17, and 100%, respectively on the myoclonus rating scale and 78, 23, and 65% on the dystonia rating scale after DBS. Dopamine D2 receptor binding did not change after surgery. In the two control subjects, binding has lowered further.

CONCLUSION

These findings confirm that DBS of the pallidum has beneficial effects on motor symptoms in M-D and suggest this procedure might stabilize dopamine D2 receptor binding.