engBrüggemann, NorbertKühn, AndreaLozano, Andres MHamani, ClementPoon, Yu-YanZittel, SimoneWächter, TobiasDeuschl, GüntherKrüger, RejkoKupsch, AndreasMünchau, AlexanderLohmann, KatjaSchneider, Susanne AVolkmann, JensKlein, ChristineKamm, ChristophWolters, AlexanderKrause, PatriciaMoro, ElenaSteigerwald, FrankWittstock, MatthiasTronnier, VolkerShort- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia.info:eu-repo/classification/ddc/610AdolescentAdultChildChild, PreschoolDeep Brain Stimulation: methodsDeep Brain Stimulation: trendsDystonia: diagnosisDystonia: therapyElectrodes, ImplantedFemaleFollow-Up StudiesGlobus Pallidus: physiopathologyHumansMaleMiddle AgedRetrospective StudiesTreatment OutcomeYoung AdultDeep 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.Neurology 84(9), 895-903 (2015). doi:10.1212/WNL.0000000000001312info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionOvid2015info:eu-repo/semantics/closedAccessDEhttps://pub.dzne.de/record/137808https://pub.dzne.de/search?p=id:%22DZNE-2020-04130%22Researchersinfo:eu-repo/semantics/altIdentifier/issn/1526-632Xinfo:eu-repo/semantics/altIdentifier/pmid/pmid:25653290info:eu-repo/semantics/altIdentifier/issn/0028-3878info:eu-repo/semantics/altIdentifier/doi/10.1212/WNL.0000000000001312