Journal Article

DZNE-2020-04130

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia.

Brüggemann, N. (Corresponding author) ; Kühn, 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. ; Wächter, T.DZNE* ; Deuschl, G. ; Krüger, R.DZNE* ; Kupsch, A. ; Münchau, A. ; Lohmann, K. ; Volkmann, J. ; Klein, C.

2015
Ovid [S.l.]

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Please use a persistent id in citations: doi:10.1212/WNL.0000000000001312

Abstract: 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.

Keyword(s): Adolescent (MeSH) ; Adult (MeSH) ; Child (MeSH) ; Child, Preschool (MeSH) ; Deep Brain Stimulation: methods (MeSH) ; Deep Brain Stimulation: trends (MeSH) ; Dystonia: diagnosis (MeSH) ; Dystonia: therapy (MeSH) ; Electrodes, Implanted (MeSH) ; Female (MeSH) ; Follow-Up Studies (MeSH) ; Globus Pallidus: physiopathology (MeSH) ; Humans (MeSH) ; Male (MeSH) ; Middle Aged (MeSH) ; Retrospective Studies (MeSH) ; Treatment Outcome (MeSH) ; Young Adult (MeSH)

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Contributing Institute(s):

1. Ext Universitätsklinikum Tübingen (Ext UKT)
2. Parkinson Genetics (AG Gasser 1)

Research Program(s):

1. 345 - Population Studies and Genetics (POF3-345) (POF3-345)

Appears in the scientific report 2015

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Database coverage:
; Allianz-Lizenz ; BIOSIS Previews ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Current Contents - Life Sciences ; IF >= 5 ; JCR ; NCBI Molecular Biology Database ; PubMed Central ; SCOPUS ; Science Citation Index ; Science Citation Index Expanded ; Web of Science Core Collection

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