Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia
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Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia. / Brüggemann, Norbert; Kühn, Andrea; Schneider, Susanne A; Kamm, Christoph; Wolters, Alexander; Krause, Patricia; Moro, Elena; Steigerwald, Frank; Wittstock, Matthias; Tronnier, Volker; Lozano, Andres M; Hamani, Clement; Poon, Yu-Yan; Zittel-Dirks, Simone; Wächter, Tobias; Deuschl, Günther; Krüger, Rejko; Kupsch, Andreas; Münchau, Alexander; Lohmann, Katja; Volkmann, Jens; Klein, Christine.
in: NEUROLOGY, Jahrgang 84, Nr. 9, 03.03.2015, S. 895-903.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Short- and long-term outcome of chronic pallidal neurostimulation in monogenic isolated dystonia
AU - Brüggemann, Norbert
AU - Kühn, Andrea
AU - Schneider, Susanne A
AU - Kamm, Christoph
AU - Wolters, Alexander
AU - Krause, Patricia
AU - Moro, Elena
AU - Steigerwald, Frank
AU - Wittstock, Matthias
AU - Tronnier, Volker
AU - Lozano, Andres M
AU - Hamani, Clement
AU - Poon, Yu-Yan
AU - Zittel-Dirks, Simone
AU - Wächter, Tobias
AU - Deuschl, Günther
AU - Krüger, Rejko
AU - Kupsch, Andreas
AU - Münchau, Alexander
AU - Lohmann, Katja
AU - Volkmann, Jens
AU - Klein, Christine
N1 - © 2015 American Academy of Neurology.
PY - 2015/3/3
Y1 - 2015/3/3
N2 - 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.
AB - 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.
KW - Adolescent
KW - Adult
KW - Child
KW - Child, Preschool
KW - Deep Brain Stimulation
KW - Dystonia
KW - Electrodes, Implanted
KW - Female
KW - Follow-Up Studies
KW - Globus Pallidus
KW - Humans
KW - Male
KW - Middle Aged
KW - Retrospective Studies
KW - Treatment Outcome
KW - Young Adult
KW - Case Reports
KW - Journal Article
KW - Multicenter Study
KW - Research Support, N.I.H., Extramural
KW - Research Support, Non-U.S. Gov't
U2 - 10.1212/WNL.0000000000001312
DO - 10.1212/WNL.0000000000001312
M3 - SCORING: Journal article
C2 - 25653290
VL - 84
SP - 895
EP - 903
JO - NEUROLOGY
JF - NEUROLOGY
SN - 0028-3878
IS - 9
ER -