Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia.

Abstract

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.

• Authors: Norbert Brüggemann, Andrea Kühn, Susanne A Schneider, Christoph Kamm, Alexander Wolters, Patricia Krause, Elena Moro, Frank Steigerwald, Matthias Wittstock, Volker Tronnier, Andres M Lozano, Clement Hamani, Yu-Yan Poon, Simone Zittel, Tobias Wächter, Günther Deuschl, Rejko Krüger, Andreas Kupsch, Alexander Münchau, Katja Lohmann, Jens Volkmann, Christine Klein
• Journal: Neurology (Neurology) Vol. 84 Issue 9 Pg. 895-903 (Mar 03 2015) ISSN: 1526-632X [Electronic] United States
• PMID: 25653290 (Publication Type: Case Reports, Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: © 2015 American Academy of Neurology.

Topics

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