Skip to main content

Advertisement

SpringerLink
Log in

Long-term efficacy of GPi DBS for craniofacial dystonia: a retrospective report of 13 cases

  • Original Article
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

This study evaluated the long-term efficacy of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of craniofacial dystonia (Meige syndrome) and investigated the correlation between the volume of tissue activated (VTA) in the GPi and each subregion and movement score improvement. We retrospectively analyzed the clinical data of 13 patients with drug-refractory Meige syndrome who were treated with GPi DBS. The pre- and postoperative Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS) scores were compared. The relationships between the preoperative baseline variables and improvement in the BFMDRS-Movement (BFMDRS-M) score were analyzed. LEAD-DBS software was used for the three-dimensional reconstruction of the GPi and implanted electrodes. The correlations between the GPi-VTA and score improvement were analyzed. The average follow-up period was 36.6 ± 11.0 months (18–55 months). At 3 months after the stimulation and the final follow-up visit, the improvements in the BFMDRS-M score were 58.2 and 54.6%, and the improvements in the BFMDRS-Disability (BFMDRS-D) score were 53.6 and 51.7%, respectively. At the final follow-up visit, the improvements in the BFMDRS-M scores of the eye, mouth, and speech/swallowing were significant (P < 0.001). Age was an independent predictor of improvement in the BFMDRS-M score after DBS (P = 0.005). A decrease in the BFMDRS-M score was significantly positively correlated with the GPi-VTA (r = 0.757, P = 0.003). GPi DBS is an effective method for treating drug-refractory Meige syndrome. LEAD-DBS software can be used as an effective aid for visualization programming after DBS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Data availability

The data used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Code availability

Not applicable.

References

  1. Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J (1985) Validity and reliability of a rating scale for the primary torsion dystonias. Neurology 35(1):73–77. https://doi.org/10.1212/wnl.35.1.73

    Article  CAS  PubMed  Google Scholar 

  2. Bronte-Stewart H, Taira T, Valldeoriola F, Merello M, Marks WJ Jr, Albanese A, Bressman S, Moro E (2011) Inclusion and exclusion criteria for DBS in dystonia. Mov Disord 26(Suppl 1):S5–S16. https://doi.org/10.1002/mds.23482

    Article  PubMed  PubMed Central  Google Scholar 

  3. Berman BD, Starr PhA, Marks WJ, Ostrem JL (2009) Induction of bradykinesia with pallidal deep brain stimulation in patients with cranial-cervical dystonia. Stereotact Funct Neurosurg 87(1):37–44. https://doi.org/10.1159/000195718

    Article  PubMed  PubMed Central  Google Scholar 

  4. Blahak C, Capelle HH, Baezner H, Kinfe TM, Hennerici MG, Krauss JK (2011) Micrographia induced by pallidal DBS for segmental dystonia: a subtle sign of hypokinesia? J Neural Transm (Vienna) 118(4):549–553. https://doi.org/10.1007/s00702-010-0544-y

    Article  Google Scholar 

  5. Deng Z, Pan Y, Zhang C, Zhang J, Qiu X, Zhan S, Li D, Sun B (2018) Subthalamic deep brain stimulation in patients with primary dystonia: a ten-year follow-up study. Parkinsonism Relat Disord 55:103–110. https://doi.org/10.1016/j.parkreldis.2018.05.024

    Article  PubMed  Google Scholar 

  6. Duffley G, Anderson DN, Vorwerk J, Dorval AD, Butson CR (2019) Evaluation of methodologies for computing the deep brain stimulation volume of tissue activated. J Neural Eng 16(6):066024. https://doi.org/10.1088/1741-2552/ab3c95

    Article  PubMed  PubMed Central  Google Scholar 

  7. Faulstich ME, Carnrike CL Jr, Williamson DA (1985) Blepharospasm and Meige syndrome: a review of diagnostic, aetiological and treatment approaches. J Psychosom Res 29(1):89–94. https://doi.org/10.1016/0022-3999(85)90012-1

    Article  CAS  PubMed  Google Scholar 

  8. Foncke EM, Schuurman PR, Speelman JD (2006) Suicide after deep brain stimulation of the internal globus pallidus for dystonia. Neurology 66(1):142–143. https://doi.org/10.1212/01.wnl.0000191328.05752.e2

    Article  CAS  PubMed  Google Scholar 

  9. Ghang JY, Lee MK, Jun SM, Ghang CG (2010) Outcome of pallidal deep brain stimulation in Meige syndrome. J Korean Neurosurg Soc 48(2):134–138. https://doi.org/10.3340/jkns.2010.48.2.134

    Article  PubMed  PubMed Central  Google Scholar 

  10. Horisawa S, Ochiai T, Goto S, Nakajima T, Takeda N, Kawamata T, Taira T (2018) Long-term outcome of pallidal stimulation for Meige syndrome. J Neurosurg 130(1):84–89. https://doi.org/10.3171/2017.7.JNS17323

    Article  PubMed  Google Scholar 

  11. Horn A, Kühn AA (2015) Lead-DBS: a toolbox for deep brain stimulation electrode localizations and visualizations. Neuroimage 107:127–135. https://doi.org/10.1016/j.neuroimage.2014.12.002

    Article  Google Scholar 

  12. Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, Falk D, Herzog J, Pinsker MO, Schneider GH, Schrader C, Deuschl G, Mehdorn HM, Kupsch A, Volkmann J, Krauss JK (2011) Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 26(4):691–698. https://doi.org/10.1002/mds.23549

    Article  PubMed  Google Scholar 

  13. Reich MM, Horn A, Lange F, Roothans J, Paschen S, Runge J, Wodarg F, Pozzi NG, Witt K, Nickl RC, Soussand L, Ewert S, Maltese V, Wittstock M, Schneider GH, Coenen V, Mahlknecht P, Poewe W, Eisner W, Helmers AK, Matthies C, Sturm V, Isaias IU, Krauss JK, Kühn AA, Deuschl G, Volkmann J (2019) Probabilistic mapping of the antidystonic effect of pallidal neurostimulation: a multicentre imaging study. Brain 142(5):1386–1398. https://doi.org/10.1093/brain/awz046

    Article  PubMed  Google Scholar 

  14. Risch V, Staiger A, Ziegler W, Ott K, Schölderle T, Pelykh O, Bötzel K (2015) How does GPi-DBS affect speech in primary dystonia? Brain Stimul 8(5):875–880. https://doi.org/10.1016/j.brs.2015.04.009

    Article  PubMed  Google Scholar 

  15. Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y, Nagahiro S, Kaji R, Goto S (2011) Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord 17(2):123–125. https://doi.org/10.1016/j.parkreldis.2010.11.013

    Article  PubMed  Google Scholar 

  16. Sobstyl M, Brzuszkiewicz-Kuźmicka G, Zaczyński A, Pasterski T, Aleksandrowicz M, Ząbek M (2017) Long-term clinical outcome of bilateral pallidal stimulation for intractable craniocervical dystonia (Meige syndrome). Report of 6 patients. J Neurol Sci 383:153–157. https://doi.org/10.1016/j.jns.2017.10.017

    Article  PubMed  Google Scholar 

  17. Sensi M, Cavallo MA, Quatrale R, Sarubbo S, Biguzzi S, Lettieri C, Capone JG, Tugnoli V, Tola MR, Eleopra R (2009) Pallidal stimulation for segmental dystonia: long term follow up of 11 consecutive patients. Mov Disord 24(12):1829–1835. https://doi.org/10.1002/mds.22686

    Article  PubMed  Google Scholar 

  18. Schrader C, Capelle HH, Kinfe TM, Blahak C, Bäzner H, Lütjens G, Dressler D, Krauss JK (2011) GPi-DBS may induce a hypokinetic gait disorder with freezing of gait in patients with dystonia. Neurology 77(5):483–488. https://doi.org/10.1212/WNL.0b013e318227b19e

    Article  CAS  PubMed  Google Scholar 

  19. Saleh C, Okun MS (2008) Clinical review of deep brain stimulation and its effects on limbic basal ganglia circuitry. Front Biosci 13:5708–5731. https://doi.org/10.2741/3111

    Article  PubMed  Google Scholar 

  20. Saleh C (2010) Knowing the limitations of applied deep brain stimulation technology for a clearer understanding of clinical outcomes. J Neurol Sci 292(1–2):119. https://doi.org/10.1016/j.jns.2010.01.027

    Article  PubMed  Google Scholar 

  21. Tolosa E, Kulisevsky J, Fahn S (1988) Meige syndrome: primary and secondary forms. Adv Neurol 50:509–515

    CAS  PubMed  Google Scholar 

  22. Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Benabid AL, Cornu P, Lagrange C, Tézenas du Montcel S, Dormont D, Grand S, Blond S, Detante O, Pillon B, Ardouin C, Agid Y, Destée A, Pollak P (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352(5):459–467. https://doi.org/10.1056/NEJMoa042187

    Article  CAS  PubMed  Google Scholar 

  23. Volkmann J, Wolters A, Kupsch A, Müller J, Kühn AA, Schneider GH, Poewe W, Hering S, Eisner W, Müller JU, Deuschl G, Pinsker MO, Skogseid IM, Roeste GK, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Classen J, Naumann M, Benecke R (2012) Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 11(12):1029–1038. https://doi.org/10.1016/S1474-4422(12)70257-0

    Article  PubMed  Google Scholar 

  24. Wang X, Zhang C, Wang Y, Liu C, Zhao B, Zhang JG, Hu W, Shao X, Zhang K (2016) Deep brain stimulation for craniocervical dystonia (Meige syndrome): a report of four patients and a literature-based analysis of its treatment effects. Neuromodulation 19(8):818–823. https://doi.org/10.1111/ner.12345

    Article  PubMed  Google Scholar 

  25. Wang X, Mao Z, Cui Z, Xu X, Pan L, Liang S, Ling Z, Yu X (2019) Predictive factors for long-term clinical outcomes of deep brain stimulation in the treatment of primary Meige syndrome. J Neurosurg 5:1–9. https://doi.org/10.3171/2019.1.JNS182555

    Article  Google Scholar 

  26. Yao C, Horn A, Li N, Lu Y, Fu Z, Wang N, Aziz TZ, Wang L, Zhang S (2019) Post-operative electrode location and clinical efficacy of subthalamic nucleus deep brain stimulation in Meige syndrome. Parkinsonism Relat Disord 58:40–45. https://doi.org/10.1016/j.parkreldis.2018.05.014

    Article  PubMed  Google Scholar 

  27. Zhan S, Sun F, Pan Y, Liu W, Huang P, Cao C, Zhang J, Li D, Sun B (2018) Bilateral deep brain stimulation of the subthalamic nucleus in primary Meige syndrome. J Neurosurg 128(3):897–902. https://doi.org/10.3171/2016.12.JNS16383

    Article  PubMed  Google Scholar 

Download references

Funding

This study was funded by the 135 Project of Outstanding Development of West China Hospital, Sichuan University (No. ZY2017307).

Author information

Author notes

  1. Haibo Ren and Rong Wen contributed equally to this work.

Authors and Affiliations

  1. Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guoxue Alley, Chengdu, 610041, Sichuan Province, China

    Haibo Ren, Wei Wang, Denghui Li, Mengqi Wang, Yuan Gao, Yang Xu & Yang Wu

  2. Department of Neurosurgery, Nanchong Central Hospital, Nanchong, China

    Haibo Ren

  3. Department of Neurosurgery, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China

    Rong Wen

Authors
  1. Haibo Ren
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rong Wen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Wei Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Denghui Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mengqi Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yuan Gao
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Yang Xu
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yang Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. The material preparation, data collection, and analysis were performed by H.R., R.W., and D.L. The first draft of the manuscript was written by H.R. and R.W. W.W., D.L., M.W., Y.G., Y.X., and Y.W. read and approved the final manuscript.

Corresponding author

Correspondence to Wei Wang.

Ethics declarations

Ethical approval

The research protocol was approved by the Ethics Committee on Biomedical Research, West China Hospital of Sichuan University (No. 20202).

Consent to participate

Informed consent was obtained from all individual participants included in the study.

Consent for publication

Informed consent for publication was obtained.

Open access

This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Conflicts of interest

The authors declare no competing interests.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ren, H., Wen, R., Wang, W. et al. Long-term efficacy of GPi DBS for craniofacial dystonia: a retrospective report of 13 cases. Neurosurg Rev 45, 673–682 (2022). https://doi.org/10.1007/s10143-021-01584-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-021-01584-4

Keywords

Search

Navigation