Reference : Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolat...
Scientific journals : Article
Human health sciences : Neurology
Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia
Bruggemann, N. [> >]
Kuhn, A. [> >]
Schneider, S. A. [> >]
Kamm, C. [> >]
Wolters, A. [> >]
Krause, P. [> >]
Moro, E. [> >]
Steigerwald, F. [> >]
Wittstock, M. [> >]
Tronnier, V. [> >]
Lozano, A. M. [> >]
Hamani, C. [> >]
Poon, Y. Y. [> >]
Zittel, S. [> >]
Wachter, T. [> >]
Deuschl, G. [> >]
Krüger, Rejko mailto [University of Luxembourg > Faculty of Science, Technology and Communication (FSTC) > Life Science Research Unit]
Kupsch, A. [> >]
Munchau, A. [> >]
Lohmann, K. [> >]
Volkmann, J. [> >]
Klein, C. []
American Academy of Neurology
Yes (verified by ORBilu)
United States

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.
Luxembourg Centre for Systems Biomedicine (LCSB): Clinical & Experimental Neuroscience (Krüger Group)

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