Deep Brain Stimulation and Pallidotomy in Primary Meige Syndrome: a Prospective Cohort Study

Overview

Journal Neurol Sci

Specialty Neurology

Date 2024 Sep 12

PMID 39266808

Authors

Qing-Pei Hao

Wen-Tao Zheng

Zi-Hao Zhang

Hu Ding

Guang-Biao Qin

Ye-Zu Liu

Yao Tan

Zhi Liu

Ru-En Liu

Affiliations

Soon will be listed here.

Abstract

Background: Primary Meige syndrome (PMS) is a rare form of dystonia, and comparative analysis of globus pallidus internal deep brain stimulation (GPi-DBS), subthalamic nucleus deep brain stimulation (STN-DBS), and pallidotomy has been lacking. This study aims to compare the efficacy, safety, and psychiatric features of GPi-DBS, STN-DBS, and pallidotomy in patients with PMS.

Methods: This prospective cohort study was divided into three groups: GPi-DBS, STN-DBS, and pallidotomy. Clinical assessments, including motor and non-motor domains, were evaluated at baseline and at 1 year and 3 years after neurostimulation/surgery.

Results: Ninety-eight patients were recruited: 46 patients received GPi-DBS, 34 received STN-DBS, and 18 underwent pallidotomy. In the GPi-DBS group, the movement score of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) improved from a mean (SE) of 13.8 (1.0) before surgery to 5.0 (0.7) (95% CI, -10.5 to -7.1; P < 0.001) at 3 years. Similarly, in the STN-DBS group, the mean (SE) score improved from 13.2 (0.8) to 3.5 (0.5) (95% CI, -10.3 to -8.1; P < 0.001) at 3 years, and in the pallidotomy group, it improved from 14.9 (1.3) to 6.0 (1.1) (95% CI, -11.3 to -6.5; P < 0.001) at 3 years. They were comparable therapeutic approaches for PMS that can improve motor function and quality of life without non-motor side effects.

Conclusions: DBS and pallidotomy are safe and effective treatments for PMS, and an in-depth exploration of non-motor symptoms may be a new entry point for gaining a comprehensive understanding of the pathophysiology.

References

Pandey S, Sharma S . Meige's syndrome: History, epidemiology, clinical features, pathogenesis and treatment. J Neurol Sci. 2016; 372:162-170. DOI: 10.1016/j.jns.2016.11.053. View

Albanese A, Bhatia K, Bressman S, DeLong M, Fahn S, Fung V . Phenomenology and classification of dystonia: a consensus update. Mov Disord. 2013; 28(7):863-73. PMC: 3729880. DOI: 10.1002/mds.25475. View

Colosimo C, Suppa A, Fabbrini G, Bologna M, Berardelli A . Craniocervical dystonia: clinical and pathophysiological features. Eur J Neurol. 2010; 17 Suppl 1:15-21. DOI: 10.1111/j.1468-1331.2010.03045.x. View

Defazio G, Livrea P . Epidemiology of primary blepharospasm. Mov Disord. 2002; 17(1):7-12. DOI: 10.1002/mds.1275. View

Wagle Shukla A, Brown R, Heese K, Jones J, Rodriguez R, Malaty I . High rates of fatigue and sleep disturbances in dystonia. Int J Neurosci. 2015; 126(10):928-35. DOI: 10.3109/00207454.2015.1085035. View

Ma H, Qu J, Ye L, Shu Y, Qu Q . Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update. Front Neurol. 2021; 12:630221. PMC: 8039296. DOI: 10.3389/fneur.2021.630221. View

Jankovic J . Treatment of dystonia. Lancet Neurol. 2006; 5(10):864-72. DOI: 10.1016/S1474-4422(06)70574-9. View

Balint B, Mencacci N, Valente E, Pisani A, Rothwell J, Jankovic J . Dystonia. Nat Rev Dis Primers. 2018; 4(1):25. DOI: 10.1038/s41572-018-0023-6. View

Centen L, Oterdoom D, Tijssen M, Lesman-Leegte I, van Egmond M, van Dijk J . Bilateral Pallidotomy for Dystonia: A Systematic Review. Mov Disord. 2020; 36(3):547-557. PMC: 8048649. DOI: 10.1002/mds.28384. View

10.

Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y . Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord. 2010; 17(2):123-5. DOI: 10.1016/j.parkreldis.2010.11.013. View

11.

Vidailhet M, Vercueil L, Houeto J, Krystkowiak P, Lagrange C, Yelnik J . Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol. 2007; 6(3):223-9. DOI: 10.1016/S1474-4422(07)70035-2. View

12.

Volkmann J, Wolters A, Kupsch A, Muller J, Kuhn A, Schneider G . Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol. 2012; 11(12):1029-38. DOI: 10.1016/S1474-4422(12)70257-0. View

13.

Hao Q, Wang D, Ouyang J, Ding H, Wu G, Liu Z . Pallidal deep brain stimulation in primary Meige syndrome: clinical outcomes and psychiatric features. J Neurol Neurosurg Psychiatry. 2020; 91(12):1343-1348. DOI: 10.1136/jnnp-2020-323701. View

14.

Conte A, Berardelli I, Ferrazzano G, Pasquini M, Berardelli A, Fabbrini G . Non-motor symptoms in patients with adult-onset focal dystonia: Sensory and psychiatric disturbances. Parkinsonism Relat Disord. 2015; 22 Suppl 1:S111-4. DOI: 10.1016/j.parkreldis.2015.09.001. View

15.

Burke R, Fahn S, Marsden C, Bressman S, Moskowitz C, Friedman J . Validity and reliability of a rating scale for the primary torsion dystonias. Neurology. 1985; 35(1):73-7. DOI: 10.1212/wnl.35.1.73. View

16.

Ware Jr J, Sherbourne C . The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992; 30(6):473-83. View

17.

Nasreddine Z, Phillips N, Bedirian V, Charbonneau S, Whitehead V, Collin I . The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005; 53(4):695-9. DOI: 10.1111/j.1532-5415.2005.53221.x. View

18.

Zhan S, Sun F, Pan Y, Liu W, Huang P, Cao C . Bilateral deep brain stimulation of the subthalamic nucleus in primary Meige syndrome. J Neurosurg. 2017; 128(3):897-902. DOI: 10.3171/2016.12.JNS16383. View

19.

Lyons M, Birch B, Hillman R, Boucher O, Evidente V . Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus. 2010; 29(2):E5. DOI: 10.3171/2010.4.FOCUS1067. View

20.

Loher T, Capelle H, Kaelin-Lang A, Weber S, Weigel R, Burgunder J . Deep brain stimulation for dystonia: outcome at long-term follow-up. J Neurol. 2008; 255(6):881-4. DOI: 10.1007/s00415-008-0798-6. View