17 Brüggemann, Norbert Kühn, Andrea Schneider, Susanne A Kamm, Christoph Wolters, Alexander Krause, Patricia Moro, Elena Steigerwald, Frank Wittstock, Matthias Tronnier, Volker Lozano, Andres M Hamani, Clement Poon, Yu-Yan Zittel, Simone Wächter, Tobias Deuschl, Günther Krüger, Rejko Kupsch, Andreas Münchau, Alexander Lohmann, Katja Volkmann, Jens Klein, Christine Ext UKT AG Gasser 1 Neurology Neurology Ovid [S.l.] 0028-3878 10.1212/WNL.0000000000001312 English 895-903 9 84 Deep 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.In 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.At 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.Long-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.This study provides Class IV evidence that long-term GPi-DBS improves dystonia in patients with DYT1, DYT6, and non-DYT dystonia. PUB:(DE-HGF)16, ; 0, ; Adolescent Adult Child Child, Preschool Deep Brain Stimulation: methods Deep Brain Stimulation: trends Dystonia: diagnosis Dystonia: therapy Electrodes, Implanted Female Follow-Up Studies Globus Pallidus: physiopathology Humans Male Middle Aged Retrospective Studies Treatment Outcome Young Adult Journal Article 2015 DZNE-2020-04130 2015 pmc:PMC6170184 pmid:25653290 https://pub.dzne.de/record/137808 https://doi.org/10.1212/WNL.0000000000001312