Pallidal and thalamic deep brain stimulation in myoclonus‐dystonia |
| |
Authors: | Doreen Gruber MD Andrea A. Kühn MD PhD Thomas Schoenecker MD Anatol Kivi MD Thomas Trottenberg MD Karl‐Titus Hoffmann MD PhD Alireza Gharabaghi MD Ute A. Kopp MD Gerd‐Helge Schneider MD Christine Klein MD PhD Friedrich Asmus MD Andreas Kupsch MD PhD |
| |
Affiliation: | 1. Departments of Neurology, University Medicine Berlin, Berlin, Germany;2. Department of Radiology, University Medicine Berlin, Berlin, Germany;3. Department of Neuroradiology, University of Leipzig, Leipzig, Germany;4. Department of Neurosurgery, University of Tuebingen, Tuebingen, Germany;5. Department of Neurosurgery, University Medicine Berlin, Berlin, Germany;6. Department of Neurology, University of Lübeck, Lübeck, Germany;7. Department of Neurodegenerative Diseases and General Neurology, Center of Neurology and Hertie‐Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany |
| |
Abstract: | Deep brain stimulation (DBS) of the internal globus pallidus (GPi) and ventral intermediate thalamic nucleus (VIM) are established treatment options in primary dystonia and tremor syndromes and have been reported anecdotally to be efficacious in myoclonus‐dystonia (MD). We investigated short‐ and long‐term effects on motor function, cognition, affective state, and quality of life (QoL) of GPi‐ and VIM‐DBS in MD. Ten MD‐patients (nine ε‐sarcoglycan‐mutation‐positive) were evaluated pre‐ and post‐surgically following continuous bilateral GPi‐ and VIM‐DBS at four time points: presurgical, 6, 12, and as a last follow‐up at a mean of 62.3 months postsurgically, and in OFF‐, GPi‐, VIM‐, and GPi‐VIM‐DBS conditions by validated motor [unified myoclonus rating scale (UMRS), TSUI Score, Burke‐Fahn‐Marsden dystonia rating scale (BFMDRS)], cognitive, affective, and QoL‐scores. MD‐symptoms significantly improved at 6 months post‐surgery (UMRS: 61.5%, TSUI Score: 36.5%, BFMDRS: 47.3%). Beneficial effects were sustained at long‐term evaluation post‐surgery (UMRS: 65.5%, TSUI Score: 35.1%, BFMDRS: 48.2%). QoL was significantly ameliorated; affective status and cognition remained unchanged postsurgically irrespective of the stimulation conditions. No serious long‐lasting stimulation‐related adverse events (AEs) were observed. Both GPi‐ and VIM‐DBS offer equally effective and safe treatment options for MD. With respect to fewer adverse, stimulation‐induced events of GPi‐DBS in comparison with VIM‐DBS, GPi‐DBS seems to be preferable. Combined GPi‐VIM‐DBS can be useful in cases of incapaciting myoclonus, refractory to GPi‐DBS alone. © 2010 Movement Disorder Society |
| |
Keywords: | myoclonus‐dystonia deep brain stimulation globus pallidus internus ventral intermediate nucleus |
|
|