Review: Long-term clinical outcome in Meige syndrome treated with internal pallidum DBS

Movement Disorders
doi: 10.1002/mds.23549

Authors: René Reese MD1, Doreen Gruber MD2, Thomas Schoenecker MD3, Hansjörg Bäzner MD4, Christian Blahak MD4, H. Holger Capelle MD5, Daniela Falk MD6, Jan Herzog MD1, Marcus O. Pinsker MD6,7, Gerd H. Schneider MD8, Christoph Schrader MD9, Günther Deuschl MD1, Günther M. Mehdorn MD6, Andreas Kupsch MD2, Jens Volkmann MD1, Joachim K. Krauss MD5

1 Department of Neurosurgery, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625 Hannover, 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

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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

Summary and review by Dr. Leo Verhagen, MD, PhD, Associate Professor of Neurological Sciences, Rush University Medical Center, Chicago

Deep Brain Stimulation (DBS) is recognized as an effective treatment for primary generalized dystonia (PGD), whereas botulinum toxin therapy is the treatment of choice for focal dystonias such as blepharospasm and cervical dystonia. When blepharospasm is part of more widespread cranio-cervical dystonia the ideal treatment becomes less clear. Meige syndrome is characterized by blepharospasm and oromandibular dystonia and can be associated with complex movements of not only the mouth and jaw but also the tongue, lower facial, pharyngeal and cervical muscles. In this population botulinum toxin injections may lead to unsatisfactory results1 or side effects, and oral drugs frequently fail. It thus seems reasonable to investigate whether DBS can play a role in the treatment of medically refractory cases of Meige syndrome. Early experience with DBS for PGD suggested that 'midline' cranio-cervical dystonia responds less favorably than limb, trunk and neck dystonia2, but specific studies on DBS for Meige syndrome were lacking until recently.

The study by Reese et al.3 in this issue of Movement Disorders addresses that knowledge gap and shows that DBS for Meige syndrome is indeed effective. Using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) they found a 53% improvement at long-term follow up, with the BFMDRS sub-items eyes, mouth and speech/swallowing improving by 47%, 56%, and 64%, respectively. The authors also used the Blepharospasm Movement Scale and the Abnormal Involuntary Movement Scale and documented improvements of 38% and 49%, respectively. There was one infection requiring removal of the DBS lead with re-implantation at a later date, but no other adverse events occurred.

While the number of study subjects (12, with 11 patients providing long-term follow-up) remains small, this is the largest study thus far with the longest follow-up data. The ratings of dystonia severity in this study were based on videotape documentation which offered the possibility of blinding the rater by presenting the baseline, short-term and long-term clips in randomized order. It is unclear why that opportunity was not seized in this study. Another concern in this retrospective study involving 3 centers is that the video protocol may not have been identical or ideally suited to document the performance on three different rating scales, but apparently this was not a problem. Methodological strengths of the study include the use of postoperative confirmation of DBS lead location and the use of additional rating scales that are more specific than the BFMDRS for the body area involved.

Taken together, this contribution by Reese and colleagues is important because it provides long-term data (12-78 months) on a relatively large number of patients. It confirms results from several case reports and more recent case series by Sensi4 (9 patients with cranio-cervico-brachial dystonia, 36 months follow-up), Ghang5 (11 patients, 12-35 months follow-up) and Ostrem6 (6 patients, 6 months follow-up) who documented similar or even better outcomes in their patients with segmental cranio-cervical dystonia. While these studies score low on the 'evidence-based medicine scale', the results are encouraging for patients who have failed medical therapy and continue to be significantly disabled by their segmental cranio-cervical dystonia. As the authors recommend, a randomized controlled study in this population would be appropriate and timely.


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About Dr. Leo Verhagen, MD, PhD

Leo Verhagen, MD, PhD, is a board-certified neurologist, an Associate Professor at Rush Medical College, and the Medical Director of the Neurosurgery Program for Movement Disorders, at Rush University Medical Center, Chicago, Illinois. He specializes in the medical and surgical management of patients with Parkinson's disease and other Movement Disorders such as Essential Tremor and Dystonia