Article (Scientific journals)
Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia
Bruggemann, N.; Kuhn, A.; Schneider, S. A. et al.
2015In Neurology, 84 (9), p. 895-903
Peer Reviewed verified by ORBi


Full Text
Brüggemann et al., Neurology, 2015.pdf
Publisher postprint (440.95 kB)
Request a copy

All documents in ORBilu are protected by a user license.

Send to


Abstract :
[en] OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that long-term GPi-DBS improves dystonia in patients with DYT1, DYT6, and non-DYT dystonia.
Research center :
- Luxembourg Centre for Systems Biomedicine (LCSB): Clinical & Experimental Neuroscience (Krüger Group)
Disciplines :
Author, co-author :
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 ;  University of Luxembourg > Faculty of Science, Technology and Communication (FSTC) > Life Science Research Unit
Kupsch, A.
Munchau, A.
Lohmann, K.
Volkmann, J.
Klein, C.
More authors (12 more) Less
External co-authors :
Language :
Title :
Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia
Publication date :
Journal title :
Publisher :
American Academy of Neurology, United States
Volume :
Issue :
Pages :
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBilu :
since 30 November 2015


Number of views
91 (4 by Unilu)
Number of downloads
2 (2 by Unilu)

Scopus citations®
Scopus citations®
without self-citations
WoS citations


Similar publications

Contact ORBilu