Long-Term Clinical Outcome in Meige Syndrome Treated with Internal Pallidum Deep Brain Stimulation Rene ´ Reese, MD, 1 Doreen Gruber, MD, 2 Thomas Schoenecker, MD, 3 Hansjo ¨ rg Ba ¨ zner, MD, 4 Christian Blahak, MD, 4 H. Holger Capelle, MD, 5 Daniela Falk, MD, 6 Jan Herzog, MD, 1 Marcus O. Pinsker, MD, 6,7 Gerd H. Schneider, MD, 8 Christoph Schrader, MD, 9 Gu ¨ nther Deuschl, MD, 1 Hubertus M. Mehdorn, MD, 6 Andreas Kupsch, MD, 2 Jens Volkmann, MD, 1 and Joachim K. Krauss, MD 5 * 1 Department of Neurology, University Hospital Schleswig-Holstein, Kiel, Germany 2 Department of Neurology, University Medicine Berlin, Campus Virchow, Berlin, Germany 3 Department of Neuroradiology, University Medicine Berlin, Campus Virchow, Berlin, Germany 4 Department of Neurology, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany 5 Department of Neurosurgery, Hannover Medical School, Hannover, Germany 6 Department of Neurosurgery, University Hospital Schleswig-Holstein, Kiel, Germany 7 Department of Stereotactic and Functional Neurosurgery, University Medical Center Freiburg, Germany 8 Department of Neurosurgery, University Medicine Berlin, Campus Virchow, Berlin, Germany 9 Department of Neurology, Hannover Medical School, Hannover, Germany ABSTRACT: 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, sev- eral 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 6 4.4 years, and mean disease duration 8.3 6 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 6 1.5 months; P < 0.001) and of 53% at long- term follow-up (38.8 6 21.7 months; P < 0.001). Subscores for eyes were improved by 38% (P 5 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 5 0.058) and 64% (P 5 0.004). Mean improvements were 25% ( P 5 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. V C 2011 Movement Disorder Society Key Words: deep brain stimulation; globus pallidus; dystonia; Meige syndrome Meige syndrome is a manifestation of segmental dystonia comprising blepharospasm and dystonia of lower facial, jaw, and neck muscles. 1 In idiopathic forms, symptoms usually start in the fifth or sixth dec- ade of life with a twofold higher incidence in women. Blepharospasm is the most frequent initial complaint of patients as by time dystonic movement patterns may spread. 2–4 Apart from primary forms, Meige syn- drome has been described as a tardive movement dis- order after neuroleptic treatment. 5 Similar to other idiopathic dystonias, systemic treatment options in Meige syndrome include ------------------------------------------------------------ Reese and Gruber contributed equally to this work. *Correspondence to: Professor Dr. Joachim K. Krauss, Department of Neurosurgery, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625 Hannover, Germany; krauss.joachim@mh-hannover.de 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. Full financial disclosures and author roles may be found in the online version of this article. Received: 4 August 2010; Revised: 19 October 2010; Accepted: 1 November 2010 Published online 10 February 2011 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/mds.23549 RESEARCH ARTICLE Movement Disorders, Vol. 26, No. 4, 2011 691