Abstract
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.
Highlights
Myoclonus–Dystonia (M–D) is a movement disorder clinically characterized by myoclonic jerks and dystonic postures or movements of the upper body, often combined with psychiatric symptoms such as depressed mood or anxiety (Zimprich et al, 2001)
The first aim of this study is to report the clinical effects of globus pallidus internus (GPi)-Deep brain stimulation (DBS) on M–D patients who had surgery in our center
Dystonia and myoclonus were assessed at the time of the first and second scan using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS) (Burke et al, 1985) and the Unified Myoclonus Rating Scale (Frucht et al, 2002)
Summary
Myoclonus–Dystonia (M–D) is a movement disorder clinically characterized by myoclonic jerks and dystonic postures or movements of the upper body, often combined with psychiatric symptoms such as depressed mood or anxiety (Zimprich et al, 2001). Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is currently the most promising technique for treatment of patients with severe medically refractory dystonia. Additional case reports describe good response of motor symptoms after VIM-DBS or GPi-DBS in M–D patients (Cif et al, 2004; Foncke et al, 2007; Kuncel et al, 2009; Kurtis et al, 2010). GPi-DBS has been reported in only 19 M–D patients so far and positive results were reported, no class I or II trial has been reported
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