Deep Brain Stimulation of the Pallidum is effective and might stabilize striatal D2 receptor binding in Myoclonus-Dystonia.

• Main Authors: Richard J. Beukers, Maria Fiorella Contarino, Hans D Speelman, P. Rick Schuurman, Jan eBooij, Marina A.J. Tijssen
• Format: Article
• Language: English
• Published: Frontiers Media S.A. 2012-02-01
• Series: Frontiers in Neurology
• Subjects: Dystonia; Myoclonus; DBS; IBZM-SPECT
• Online Access: http://journal.frontiersin.org/Journal/10.3389/fneur.2012.00022/full

Purpose: To assess clinical efficacy of deep brain stimulation of the pallidum in Myoclonus-Dystonia patients, and to compare pre- and postoperative 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 two years post-operatively using 123I-iodobenzamide Single Photon Emission Computed Tomography. Two patients who did not undergo surgery served as controls.Results: Clinically, the 3 Myoclonus-Dystonia patients improved 83%, 17% and 100%, respectively on the myoclonus rating scale and 78%, 23% and 65% on the dystonia rating scale after deep brain stimulation. Dopamine D2 receptor binding did not change after surgery. In the two control subjects, binding has lowered further.Conclusions: These findings confirm that deep brain stimulation of the pallidum has beneficial effects on motor symptoms in Myoclonus-Dystonia and suggest this procedure might stabilize dopamine D2 receptor binding.