Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation


  • Relevant conflict of interest/financial disclosures: RR, DG, HHC, DF, MOP, HMM, AK, JV and JKK received speaking honoraria from Medtronic. GD, AK and JV received grants from Medtronic. HMM, JKK and JV received consultant fees from Medtronic. René Reese, Doreen Gruber, Thomas Schoenecker, Hansjörg Bäzner, Christian Blahak, H. Holger Capelle, Daniela Falk, Jan Herzog, Marcus O. Pinsker, Gerd H. Schneider, Christoph Schrader, Günther Deuschl, Hubertus M. Mehdorn, Andreas Kupsch, Jens Volkmann, Joachim K. Krauss. Full financial disclosures and author roles can be found in the online version of this article.


Deep brain stimulation of the globus pallidus internus (GPi DBS) is effective in the treatment of primary segmental and generalized dystonia. Although limb, neck, or truncal dystonia are markedly improved, orofacial dystonia is ameliorated to a lesser extent. Nevertheless, several case reports and small cohort studies have described favorable short-term results of GPi DBS in patients with severe Meige syndrome. Here, we extend this preliminary experience by reporting long-term outcome in a multicenter case series, following 12 patients (6 women, 6 men) with Meige syndrome for up to 78 months after bilateral GPi DBS. We retrospectively assessed dystonia severity based on preoperative and postoperative video documentation. Mean age of patients at surgery was 64.5 ± 4.4 years, and mean disease duration 8.3 ± 4.4 years. Dystonia severity as assessed by the Burke–Fahn–Marsden Dystonia Rating Scale showed a mean improvement of 45% at short-term follow-up (4.4 ± 1.5 months; P < 0.001) and of 53% at long-term follow-up (38.8 ± 21.7 months; P < 0.001). Subscores for eyes were improved by 38% (P = 0.004) and 47% (P < 0.001), for mouth by 50% (P < 0.001) and 56% (P < 0.001), and for speech/swallowing by 44% (P = 0.058) and 64% (P = 0.004). Mean improvements were 25% (P = 0.006) and 38% (P < 0.001) on the Blepharospasm Movement Scale and 44% (P < 0.001) and 49% (P < 0.001) on the Abnormal Involuntary Movement Scale. This series, which is the first to demonstrate a long-term follow-up in a large number of patients, shows that GPi DBS is a safe and highly effective therapy for Meige syndrome. The benefit is preserved for up to 6 years. © 2011 Movement Disorder Society