• Norbert Brüggemann, Andrea Kühn, Susanne A Schneider, Christoph Kamm, Alexander Wolters, Patricia Krause, Elena Moro, Frank Steigerwald, Matthias Wittstock, Volker Tronnier, Andres M Lozano, Clement Hamani, Yu-Yan Poon, Simone Zittel, Tobias Wächter, Günther Deuschl, Rejko Krüger, Andreas Kupsch, Alexander Münchau, Katja Lohmann, Jens Volkmann, and Christine Klein.
• From the Institute of Neurogenetics (N.B., S.A.S., S.Z., A.M., K.L., C. Klein), University of Lübeck; Department of Neurology (N.B.), University Hospital Schleswig-Holstein, Campus Lübeck; Department of Neurology (A. Kühn, P.K.), Virchow Clinics, University Berlin Charité; Department of Neurology (S.A.S., G.D.), University Hospital Schleswig-Holstein, Campus Kiel; Department of Neurology (C. Kamm, A.W., M.W.), University Hospital Rostock, Germany; Movement Disorders Center (E.M., Y.-Y.P.), Toronto Western Hospital, University of Toronto, UHN, Canada; Movement Disorders Unit (E.M.), Division of Psychiatry and Neurology, CHU Grenoble, Joseph Fourier University, Grenoble, France; Department of Neurology (F.S., J.V.), University Hospital Würzburg; Department of Neurosurgery (V.T.), University Hospital Lübeck, Germany; Division of Neurosurgery (A.M.L., C.H.), Department of Surgery, University of Toronto, Canada; Center for Neurology and Hertie-Institute for Clinical Brain Research (T.W., R.K.), University Hospital Tübingen, Center for Integrative Neurosciences, University of Tübingen and German Center for Neurodegenerative Diseases (DZNE), Tübingen; Clinical and Experimental Neuroscience (R.K.), Luxembourg Centre for Systems Biomedicine, University of Luxembourg; and Department of Neurology and Stereotactic Neurosurgery (A. Kupsch), Basal Ganglia Research Group, Otto von Guericke University Magdeburg, Germany. norbert.brueggemann@neuro.uni-luebeck.de.
• Neurology. 2015 Mar 3;84(9):895-903.

ObjectivesDeep brain stimulation of the internal pallidum (GPi-DBS) is an established therapeutic option in treatment-refractory dystonia, and the identification of factors predicting surgical outcome is needed to optimize patient selection.MethodsIn this retrospective multicenter study, GPi-DBS outcome of 8 patients with DYT6, 9 with DYT1, and 38 with isolated dystonia without known monogenic cause (non-DYT) was assessed at early (1-16 months) and late (22-92 months) follow-up using Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores.ResultsAt early follow-up, mean reduction of dystonia severity was greater in patients with DYT1 (BFMDRS score: -60%) and non-DYT dystonia (-52%) than in patients with DYT6 dystonia (-32%; p = 0.046). Accordingly, the rate of responders was considerably lower in the latter group (57% vs >90%; p = 0.017). At late follow-up, however, GPi-DBS resulted in comparable improvement in all 3 groups (DYT6, -42%; DYT1, -44; non-DYT, -61%). Additional DBS of the same or another brain target was performed in 3 of 8 patients with DYT6 dystonia with varying results. Regardless of the genotype, patients with a shorter duration from onset of dystonia to surgery had better control of dystonia postoperatively.ConclusionsLong-term GPi-DBS is effective in patients with DYT6, DYT1, and non-DYT dystonia. However, the effect of DBS appears to be less predictable in patients with DYT6, suggesting that pre-DBS genetic testing and counseling for known dystonia gene mutations may be indicated. GPi-DBS should probably be considered earlier in the disease course.Classification Of EvidenceThis study provides Class IV evidence that long-term GPi-DBS improves dystonia in patients with DYT1, DYT6, and non-DYT dystonia.© 2015 American Academy of Neurology.

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