AUTHOR=Beukers Richard J., Contarino Maria F., Speelman Hans D., Schuurman P. R., Booij Jan , Tijssen Marina A.

TITLE=Deep Brain Stimulation of the Pallidum is Effective and Might Stabilize Striatal D2 Receptor Binding in Myoclonus–Dystonia

JOURNAL=Frontiers in Neurology

VOLUME=Volume 3 - 2012

YEAR=2012

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2012.00022

DOI=10.3389/fneur.2012.00022

ISSN=1664-2295

ABSTRACT=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.